NEUROLOGY Flashcards

Question

What is locked in syndrome? A stroke effecting which artery would lead to this, and what part of the brain is affected in this?

Answer 1

- A condition whereby the patient is fully aware but cannot move or communicate verbally due to almost complete paralysis - All voluntary muscles are generally affected, except for vertical eye movements and blinking It may be caused by a stroke affecting the **basilar artery,** thus denying blood to the pons

Answer 2

a visual field defect involving either the two right or the two left halves of the visual fields of both eyes.

Answer 3

**Contralateral homonymous hemianopia with macular sparing** 1. Visual field defects. 2. Cortical blindness. Contralateral loss of pain and temperature due to **spinothalamic damage** Visual agnosia - An inability to recognise or interpret visual information Prosopagnosia - An inability to recognise a familiar face.

Answer 4

- **Cerebellar** signs - **Reduced consciousness** - **Quadriplegia** or **hemiplegia** quadriplegia, disturbances of gaze and vision, **lockedin syndrome** (aware, but unable to respond

Answer 5

(midbrain infarct) Oculomotor palsy and contralateral hemiplegia

Answer 6

Ipsilateral facial loss of pain and temperature Ipsilateral Horner’s syndrome *(decreased pupil size, a drooping eyelid and decreased sweating on the affected side of the face)* Ipsilateral cerebellar signs sudden vomiting and vertigo Contralateral loss of pain and temperature

Answer 7

**Recognition of Stroke in the Emergency Room (ROSIER) scale** is a variation of **FAST (Face, Arm, Speech, Time)** and is used to differentiate acute stroke from stroke-mimics A stroke is possible if the score is > 0 and requires an urgent non-contrast CT head. Once hypoglycaemia has been excluded, assess the following:

Answer 8

CT scan ASAP Distinguishes ischaemic from haemorr **hypoattenuation (darkness)** of the brain parenchyma loss of grey matter-white matter differentiation, and sulcal effacement **hyperattenuation (brightness) in an artery indicates clot** within the vessel lumen

Answer 9

- **ECG:** assess for AF, MI - **Bloods:** - Screen for risk factors including Hba1c, lipids, clotting screen and rule out stroke mimics such as hypoglycemia and hyponatraemia - In younger patients, consider ESR, autoantibody and thrombophilia screen *(ESR raised in vasculitis)* - **CT angiogram (CTA):** identifies arterial occlusion and should be performed in all patients who are appropriate for thrombectomy - **MRI head:** MRI is an alternative to non-contrast CT head; MRI is more sensitive but CT is safer and easier to obtain

Answer 10

*Maintain stable blood glucose levels, hydration status and temperature* *Blood pressure should not be lowered too much during a stroke because this risks reducing the perfusion to the brain.* - **Thrombolysis: alteplase** given if < 4.5 hours of symptom onset and haemorrhage excluded on imaging **Aspirin 300mg** for 2 weeks Prophylaxis - Lifelong **clopidogrel** (75mg, an Antiplatelet)

Answer 11

Tissue plasminogen activator that rapidly breaks down clots and can reverse the effects of a stroke if given in time. Plasminogen is inactive form of enzyme plasmin

Answer 12

**Warfarin/DOAC**, it's superior to aspirin in Atrial fibrillation / mural thrombus Warfarin blocks one of the enzymes (proteins) that uses vitamin K to produce clotting factors. This disrupts the clotting process, making it take longer for the blood to clot DOAC - eg rivaroxaban inhibit Thrombin

Answer 13

- Stroke mimics e.g. - **Hypoglycaemia** - **Hyponatraemia** - **Hypercalcaemia** - **Uraemia** - **Hepatic encephalopathy** - **Brain tumours** - **Seizures** - **Complicated migraine**

Answer 14

- Stroke is the third leading cause of mortality in the US and the UK - The average age for a stroke is 68 to 75 years old - Stroke rates are higher in Asian and black African populations than in Caucasians - M>F

Answer 15

Sudden onset focal neurological deficit. Older definition: symptoms of a stroke that resolve within 24 hours. New definition: transient neurological dysfunction secondary to ischaemia without infarction. Either time based or tissue based **DOES NOT CAUSES ACUTE INFARCTION**

Answer 16

- **Increasing age** - **Hypertension** - **Smoking** - **Obesity** - **Diabetes** - **Hypercholesterolaemia** - **Atrial fibrillation** - **Carotid stenosis** - **Thrombophilic disorders e.g. antiphospholipid syndrome** - **Sickle cell disease**

Answer 17

**Atherothromboembolism** From Carotid artery – main cause - *listen for a carotid bruit* **Cardioembolism** In Atrial Fibrillation After an MI Valve disease/prosthetic valve **Hyperviscosity**: eg polycythaemia, sickle-cell anaemia, myeloma. **Vasculitis** eg cranial arteritis, PAN, SLE, syphilis, etc. **Dissection**: a rare cause of cerebral ischaemia from tearing of the intimal layer of an artery (typically carotid). This leads to an intramural haematoma that compromises cerebral blood flow.

Answer 18

90% = ICA 10% = Vertebral

Answer 19

Depends on the site of the TIA: ACA - weak/numb contralateral leg MCA - weak/numb contralateral side of body, face drooping w/forehead spared, dysphasia (temporal) PCA -Homonymous hemianopia: visual field loss on the same side of both eyes Hemisensory loss Amaurosis fugax

Answer 20

Diplopia – double vision Vertigo Vomiting Choking and dysarthria Ataxia Hemisensory loss

Answer 21

ABCD2 score – risk score of strokes (max score is 7) A – Age – > 60 (1 point) B – Blood pressure (at presentation), 140/90 or more (1 point) C – Clinical features: Unilateral weakness (2 points), Speech disturbance without weakness (1 point) D – Duration, 60 minutes or longer (2 points), 10-59 minutes (1 point) D – Presence of diabetes (1 point) High risk: ABCD2 score of 4 or more, AF is present, More than TIA in one week or a TIA whilst on anti-coagulation Low risk: None of the above Present more than a week after their last symptoms have resolved

Answer 22

The Face Arm Speech Time Test (FAST test): check for/ ask about facial weakness, arm weakness, speech difficulty - ECG: rule out AF as an underlying cause - Auscultation: listen for carotid bruit - Bloods: - PT time/INR - in case thrombolytic therapy is needed - To exclude hypoglycaemia/hyponatraemia - FBC – looking for polycythaemia CT scan - Request an urgent CT scan of the head Carotid doppler – look for stenosis CT angiography – look for stenosis

Answer 23

Immediate management Immediate loading dose: Aspirin 300mg Refer to specialist – to be seen within 24h of symptom onset Antiplatelet therapy Standard treatment is Aspirin 75mg daily With modified-release Dipyridamole OR Clopidogrel daily

Answer 24

- **Antiplatelet:** initially aspirin 300mg - the first-line, immediate management, if aspirin not appropriate, give an Clopidergol **REFER TO STROKE SPECIALIST*** **Carotid endarterectomy:** surgical procedure to remove the blockage, Done within 2 weeks stenosis of > 70% on Doppler is an indication for urgent endarterectomy

Answer 25

A High intensity Statin - atorvastatin : 20-80 mg orally once daily An anticoauglant for AF - eg A low molecular weight heparin eg dalteparin, or A direct thrombin inhibitor or factor Xa inhibitor - rivaroxaban Give aspirin 300mg OD for 2wks, then switch to clopidogrel 75mg OD.

Answer 26

Increased risk of stroke Increased risk of underlying CVD You cant distinguish until after recovery TIA Sx resolve usually within/<24 hours Stroke Sx last more than 24 hours

Answer 27

Ruptured blood vessel leading to reduced blood flow to the brain

Answer 28

history of fluent, yet confused speech. Wernicke's aphasia can be caused by a blockage in the **inferior division of the left Middle Cerebral Artery** Therefore, a patient with Wernicke's aphasia will talk fluently. However, the content will not make sense.

Answer 29

causes **non-fluent speech.** Patients often have word-finding difficulties. However, comprehension remains intact. Broca's area is within the frontal lobe = often affected by infarction of the **left superior division of middle cerebral** artery.

Answer 30

Intracerebral: bleeding within the brain parenchyma Subarachnoid: bleeding into the subarachnoid space, between the pia mater and arachnoid mater of the meninges Intraventricular: bleeding within the ventricles; prematurity is a very strong risk factor in infants *An intracerebral haemorrhage that involves just the brain tissue is called an intraparenchymal haemorrhage, whereas if the blood extends into the ventricles of the brain which store cerebrospinal fluid, it’s called an intraventricular haemorrhage.*

Answer 31

Hypertension - **Arteriovenous malformations:** blood vessels that directly connect an artery to a vein. Over time these abnormal vessels dilate and can rupture - **Vasculitis** - **Vascular tumours** - aka Haemangioma Cerebral amyloid angiopathy: a degenerative disease where abnormal protein deposits in the walls of arterioles making them less compliant Head trauma

Answer 32

- **Arteriosclerosis:** stiffening of vessels prone to rupture - **Microaneurysms**: called Charcot-Bouchard aneurysms, most likely to be found on small arteries

Answer 33

Ischaemia causes brain tissue death. If there is reperfusion, there’s an increased chance that the **damaged blood vessel might rupture**. Bleeding into dead tissue is called haemorrhagic conversion.

Answer 34

pool of blood which increases pressure in the skull and puts direct pressure on nearby tissue cells and blood vessels downstream tissue from bleed are also deprived of oxygen-rich blood. Healthy tissue can die from both the direct pressure and the lack of oxygen Increased ICP can also lead to CSF obstruction, - Hydrocephalus Midline shift Tentorial herniation

Answer 35

Midline shift is a shift of the brain **past its centre line** it is commonly associated with a distortion of the brain stem Tentorial herniation is the movement of brain tissue from one intracranial compartment to another *Midline shift is often associated with high intracranial pressure (ICP), which can be deadly. In fact, midline shift is a measure of ICP; presence of the former is an indication of the latter.*

Answer 36

Similar to an ischaemic stroke - brain region specific - **Headache** - **Weakness** - **Seizures** - **Vomiting** - **Reduced consciousness** - Anterior or middle cerebral artery stroke: **numbness and sudden muscle weakness.** - Broca’s area or Wernicke’s area stroke: **slurred speech or difficulty understanding speech, respectively.** - Posterior cerebral artery stroke: **vision disturbances.**

Answer 37

Pointers to haemorrhage: Sudden loss of consciousness Severe headache Meningism- the clinical syndrome of headache, neck stiffness and photophobia, often with nausea and vomiting - *can be caused by raised ICP* Coma ***These are unreliable, a CT scan is needed for differentiation***

Answer 38

Request an immediate non-contrast CT scan of the head - will see **hyperattenuation (brightness),** suggesting acute blood, often with surrounding **hypoattenuation (darkness)** due to oedema - **Angiography:** visualise the exact location of haemorrhage - **Check FBC and clotting**

Answer 39

- **Consider intubation, ventilation and ICU care if they have reduced consciousness** - **Correct any clotting abnormality** - STOP ANTICOAGULANTS IF PT IS ON THEM Correct severe hypertension but avoid hypotension Craniotomy, or stereotactic aspiration Drugs to relieve ICP - ***IV MANITOL***

Answer 40

elevates blood plasma osmolality, resulting in enhanced flow of water from tissues, including the brain and cerebrospinal fluid, into interstitial fluid and plasma Mannitol hinders tubular reabsorption of water and enhances excretion of sodium and chloride by elevating the osmolarity of the glomerular filtrate.

Answer 41

- **Craniotomy:** part of the skull bone is removed to drain any accumulated blood and relieve pressure. Good for if the bleed is close to the surface of the skull - **Stereotactic aspiration:** aspirate off blood and relieve intracranial pressure, guided by CT scanner. Good for bleeding that is located deeper in brain tissue

Answer 42

Cerebral Spinal fluid The Circle of Willis

Answer 43

Middle meningeal artery is in the extradural space above the Dura mata Bridging veins are found within the subdural space

Answer 44

The dura mater is composed of two layers: the periosteal/endosteal layer and the meningeal layer. The dural venous sinuses are between these two layers

Answer 45

Subarachnoid haemorrhage (SAH) is a type of intracranial haemorrhage characterised by blood within the subarachnoid space. Space between arachnoid mater & pia mater

Answer 46

Typical age 35-65 Make up 5% of strokes ~50% of people die straight away or soon after 10-20% more die from rebleeding within weeks Half the survivors are left with significant disability

Answer 47

Hypertension Known aneurysm Previous aneurysmal SAH Smoking Alcohol increasing age Family history Polycystic Kidney Disease Ehlers-Danlos syndrome & Marfan syndrome Associated with Berry aneurysms

Answer 48

- **Trauma** - **Atraumatic** cases are referred to as **spontaneous** SAH - often due to a saccular cerebral ***(Berry)*** Aneurysm - 70-80% of SAH cases **Arteriovenous malformation (AVM)** - abnormal connections between artery and vein can dilate and cause rupture - 15% of SAH cases Can be Idiopathic

Answer 49

**Polycystin help regulates cell growth** and division (proliferation), by allowing Ca2+ into cells PKD means there is a reduction in polycystin, which means that the wall of the blood vessels get bigger and bigger, getting stretched, **leading to aneurysm formation** Also PKD can also cause hypertension, which can further increase risk of subarachnoid haemorrhages

Answer 50

Arise at points of arterial bifurcation within the Circle of Willis; the junction between the anterior communicating and anterior cerebral arteries is the most common location Most common in anterior half of brain

Answer 51

Rupture of blood vessel - leads to a rise in ICP, Pressure on healthy tissues can make them die, as well as brain tissue not getting blood it needs due to bleed - Ischaemia Vessels being **bathed in a pool of blood can cause vasospasm** - will further reduce the supply of blood flow to the brain Also can **irritate the meninges, which can lead to scarring which can obstruct CSF outflow** ==> Hydrocephalus

Answer 52

- **Headache** - Severe, sudden onset - Occipital - ‘Thunderclap’ headache - **Meningism: photophobia and neck stiffness** - due to Meningies irritation - **Vision changes** - **Nausea and vomiting** - **Speech changes** - **Seizures** - **Weakness** - **Confusion** - **Coma**

Answer 53

- Neck stiffness - Budzinski's Sign - Flexion of neck = Flexion of Knees - Kernig sign - Knee cannot be fully extended with hip flexed at 90 degrees - Focal neurological deficit - eg if affecting Posterior cerebral artery - Oculomotor palsy - Increased BP Papilledema

Answer 54

Migraine Meningitis Corticol vein thrombosis Carotid/vertebral artery dissection

Answer 55

urgent **Non Contrast CT head** ASAP Detects >95% of SAH in first 24 hours - Subarachnoid and/or intraventricular blood, hyperdense areas in the subarachnoid space “Star” shaped sign **ECG** - Arrhythmias and ischaemic changes, Prolonged QTc, ST segment/T-wave abnormalities. **Lumbar puncture** – if CT normal but SAH still suspected Findings - RBCs or **xanthochromia (yellow pigmentation** due to degradation of haemoglobin to bilirubin) Electrolytes - Moderate hyponatraemia ABG – to rule out hypoxia

Answer 56

Immediately refer to neurosurgeon **Nimodipine** - Ca2+ antagonist, (CCB) Reduces vasospasm and therefore cerebral ischaemia Re-examine CNS often IV fluids -Maintain cerebral perfusion Ventricular drainage for hydrocephalus If features of raised intracranial pressure: consider intubation with hyperventilation, **head elevation (30°) and IV mannitol**

Answer 57

first-line **endovascular coiling of the aneurysm** -reduces blood circulation to the aneurysm inserting one or more microsurgical detachable platinum wires, into the aneurysm until there is no more blood flow occurring. Usually enter through leg. Second-line is **surgical clipping via craniotomy** - opening in the skull is created to reach the aneurysm. Then small metal clip on the neck (opening) of the aneurysm to obstruct the flow of blood.

Answer 58

At 6 months, 25% of patients are dead and 50% are moderately to severely disabled. Important predictors of 30-day mortality include age, level of consciousness on admission and the amount of blood visible on CT.

Answer 59

A hematoma is when some blood that has seeped out of blood vessels collects under the skin. A hemorrhage is active bleeding from a broken blood vessel. Therefore a haemorrhage can cause a haematoma.

Answer 60

A bleed **ABOVE** the dura mater, between the outer endosteal of the dura and the skull.

Answer 61

Mostly in young people Rare in small children, Due to plasticity of skull Rare in >60s, as Dura is more tightly adherent to skull More common in males

Answer 62

- **Head injury** - **Hypertension** - **Aneurysms** - **Ischaemic stroke can progress to haemorrhage** - **Brain tumours** - **Anticoagulants such as warfarin** HEAD INJURY IS BY FAR THE MOST COMMON CAUSE

Answer 63

The most common site where the frontal, parietal, temporal and sphenoid bones join together, ***CALLED THE PTERION*** ===> This section of the skull is **relatively thin** and it’s located right **above the middle meningeal artery.**

Answer 64

Once a meningeal artery is torn, blood will pool between the skull and the external layer of the dura mater, separating it from the inner surface of the skull. This blood **cannot cross the suture lines where the dura mater adheres more tightly** A large epidural haematoma can cause a midline shift = displacement of the whole brain towards the opposite side of the skull. *When there’s active bleeding, it’s called a haemorrhage, and the collection of blood that results is called a haematoma.*

Answer 65

lucid interval which is when several hours pass before the onset of symptoms, if blood is accumulating slowly. Here patient will have mild/little symptoms just after trauma and then decline

Answer 66

Can cause brain to herniate = Either a Supratentorial herniaton, as cerebrum is **Pushed against skull or tentorium,** can lead to an ischaemic stroke or Infratentorial herniation, as **cerebellum is pushed up against brainstem** - can affect consciousness, resp and heart rate.

Answer 67

Characteristic history, Head injury - **Reduced GCS:** loss of consciousness after the trauma due to concussion - There might be a **lucid interval** after initial trauma if there is a slower bleed. This is followed by rapid decline. - **Headaches** - **Vomiting** - **Confusion** - **Seizures** - **Pupil dilation** if bleeding continues - May be focal neurological symptoms e.g. **muscle weakness, hemiparesis, abnormal plantar reflex (upgoing plantar) or sensory problems**

Answer 68

CT scan – **shows biconvex hyperdense haematoma** that is adjacent to the skull: Blood forms **rounded/biconvex shape** as the tough dural attachments to the skull keep it more localised - **Don’t cross suture lines** Skull X-ray – may be normal or show fracture lines crossing the course of the middle meningeal artery Skull fracture increases the extradural haemorrhage risk so do an urgent CT on anyone with suspected skull fracture

Answer 69

Refer to neurosurgeon - - **Clot evacuation** - **Craniotomy**: part of the skull bone is removed in order to remove accumulated blood below. - Followed by **ligation of the vessel.** - **Consider intubation, ventilation and ICU care if they have reduced consciousness** - **Correct any clotting abnormality** - **Correct severe hypertension but avoid hypotension** Stabilise patient – ABCDE management **IV mannitol** – to reduce ICP

Answer 70

A subdural haematoma (SDH) is a collection of blood that forms in the subdural space, the space between the dura mater and the arachnoid mater, due to **Rupture of the bridging veins**

Answer 71

Deoxygenated blood drains into **superficial cerebral veins, or bridging veins**, that sit in the subarachnoid space (along with cerebral arteries) - These veins travel through the arachnoid mater, pass through the subdural space and **penetrate the inner layer of the dura mater to drain into the Dural venous sinuses** located between the two layers of the dura mater. *(the inner meningeal layer and the outer endosteal layer)* - Eventually the blood in the venous sinuses drain into the internal jugular vein and returns to the heart.

Answer 72

- The pia and arachnoid maters, are also called leptomeninges. - Between the leptomeninges, there’s the subarachnoid space, which houses cerebrospinal fluid. CSF is a clear, watery liquid which is pumped around the spinal cord and brain, cushioning them from impact and bathing them in nutrients.

Answer 73

Rupture of bridging veins, usually caused by: - **Brain atrophy:** in the elderly the brain shrinks in size which means that the bridging veins are stretched across a wider space where they are largely unsupported - **Alcohol abuse:** caused the wall of the veins to thin out, and make them more likely to break. - **Trauma/ injury** e.g. - Falls - **Shaken baby syndrome** - Acceleration-deceleration injury: speeding on the road and then suddenly slamming the brakes

Answer 74

- **Head injury** - **Brain atrophy** - **Alcohol abuse** - **Hypertension** - **Aneurysms** - **Ischaemic stroke can progress to haemorrhage** - **Brain tumours** - **Anticoagulants such as warfarin** Epileptics, as they can hit their head when having a siezure

Answer 75

bridging veins are under low pressure = bleeding can be slow = delayed onset of symptoms. Weeks/months later, haematoma starts to **autolyse,** ***(broken down by own enzymes)*** = increase in oncotic pressure so Water sucked in, haematoma enlarges. Gradual rise in ICP over weeks, as haematoma grows A large subdural haematoma on one side of the skull can cause a **midline shift, or can cause brain to herniate**

Answer 76

An acute subdural haematoma causes symptoms within 2 days, a subacute subdural haematoma causes symptoms between 3 and 14 days, and a chronic subdural haematoma causes symptoms after 15 days.

Answer 77

- **Reduced GCS**: loss of consciousness right after the injury or in the ensuing days to weeks as the haematoma increases in size. - **Headaches** - **Vomiting** - **Seizures** - Sometimes there can be focal neurological symptoms e.g. **muscle weakness, unequal pupils, hemiparesis or sensory problems** Confusion May fluctuate Insidious physical & intellectual slowing Personality change Unsteadiness ***They often cannot remember the traumatic injury as it was long ago***

Answer 78

- **Immediate NON CONTRAST CT head** to establish the diagnosis. Shows clot and midline shift. Bleeding is between the dura and arachnoid so subdural haematomas follow the contour of the brain and form a **crescent-shape and cross suture lines.** This is different to an epidural haemorrhage! Check FBC and clotting

Answer 79

- Acute subdural haematoma: **hyperdense mass = looks “more white”** than the surrounding healthy brain tissue - Chronic subdural haematoma: **hypodense masses = “less white”** than the surrounding brain tissue. - Acute on chronic bleeding: combination of hyperdense and hypodense, seen in individuals who have a rebleed in the bridging veins after a chronic haematoma has already formed.

Answer 80

IV mannitol - reduce ICP Burr hole / Craniotomy to relieve pressure Craniotomy a large opening in the skull is created to evacuate the haematoma and relieve the associated mass effect.

Answer 81

A classical syndrome of painless short-lived monocular blindness. It is a term usually reserved for transient visual loss of ischaemic origin. Management will depend on cause.

Answer 82

temporary reduction in the retinal, ophthalmic or ciliary blood flow leading to temporary retinal hypoxia. The most common cause of amaurosis fugax is atherosclerotic embolism, leading to a narrowing of either the carotid or retinal artery Can also be caused by **inflammation of the optic nerve** (optic neuritis), **nervous system (e.g. in multiple sclerosis)** or in head injury. Individuals older than 60 years of age that have had multiple episodes of transient monocular blindness could also be investigated for **giant cell arteritis,** which is characterized by inflammation of the large vessels of the scalp, neck, and arms.

Answer 83

Meningitis describes inflammation of the leptomeninges (the arachnoid and pia mater) and usually occurs due to a bacterial, viral, or fungal infection.

Answer 84

Viral infection is most common Then bacterial infection Can also be caused by fungal/parasite infection, as well as Autoimmune (aka Lupus) Medication injected into CSF (Intrathecal therapy) ***Fungal infection is the most common form of chronic meningitis***

Answer 85

Escherichia coli Group B Streptococcus (Streptococcus agalactiae) Listeria monocytogenes *only group where both Streptococcus Pneumoniae or Neisseria Meningitidis aren't in the most common top 3*

Answer 86

Neisseria meningitidis Haemophilus influenzae *(but less common now due to vaccination)* Streptococcus pneumoniae *if in doubt, just say Streptococcus Pneumoniae or Neisseria Meningitidis)*

Answer 87

Neisseria meningitidis Streptococcus pneumoniae *if in doubt, just say Streptococcus Pneumoniae or Neisseria Meningitidis)*

Answer 88

Streptococcus pneumoniae Neisseria meningitidis Listeria monocytogenes *if in doubt, just say Streptococcus Pneumoniae or Neisseria Meningitidis)*

Answer 89

The two most common causes of meningitis in adults are **both diplococci:** 1. Neisseria meningitidis (gram Negative) 2. Strep. Pneumoniae (gram Positive) Alpha Haemolysis on Blood agar, and Optochin sensitive

Answer 90

-Enteroviruses (echoviruses, Coxsackieviruses, polioviruses)- most common -Mumps in countries without routine childhood immunisation -Herpes simplex virus

Answer 91

- **Direct spread:** Pathogen gets inside the skull or spinal column, and then penetrates the meninges, eventually ending up in the CSF. - either through overlying skin or up nose. Normally due to congenital defect like spina bifida, or acquired defect like a skull fracture - **Haematogenous spread:** Pathogen enters the bloodstream and moves through the endothelial cells in the blood vessels making up the blood-brain barrier and gets into the CSF

Answer 92

- **Kernig’s sign:** extension of the knee when hip is flexed at 90 degrees causes neck pain - **Brudzinski sign:** severe neck stiffness causes the hips and knees to flex when the neck is flexed - **Petechial or purpuric non-blanching rash:** associated with meningococcal disease **(N. meningitidis)** - **Pyrexia** - **Reduced GCS**

Answer 93

FEVER HEADACHE NECK STIFFNESS – ‘MENINGISM’ Might not be able to touch chin to neck Purpuric rash – only in BACTERIAL meningitis Non-blanching plupurent rash = meningococcal septicaemia **(meningitis caused by N. Menigitidis)** Photophobia and/or phonophobia Papilloedema – swelling of optic disc on fundoscopy Usually bilateral

Answer 94

**INVESTIGATIONS AND TREATMENT SHOULD BE DONE IN PARALLEL** Treat first, investigate later – give IM benzylpenicillin Assess GCS - if <8 then can't maintain their own airway, 🡪 intubate Blood cultures – BEFORE ANTIBIOTICS!! Lumbar puncture - to obtain CSF - **Diagnostic** Head CT – to exclude lesions e.g. tumour Blood – blood cultures and PCR for S. pneumoniae and N. meningitidis. Nose and throat swabs – are plated out onto blood and chocolate agar. Stool – stool PCR can be used to detect enterovirus. Serology – blood (to detect a convalescent rise in antibody).

Answer 95

CSF: Appearance - Cloudy/Turbid WCC - High neutrophils Protein - High Glucose - Low Culture - bacterial organism bacteria swimming in the CSF (cloudy) will release proteins (high) and use up the glucose (low). Immune response to bacteria is neutrophils

Answer 96

CSF: Appearance - Clear WCC - High Lymphocytes Protein - Normal/Mildly raised Glucose - Normal (2.8–4.2mmol/L., two thirds of blood glucose) Culture - Negative Viruses cant be seen (clear) don’t use glucose (normal) but may release a small amount of protein (normal/mild inc). Immune response to viruses are lymphocytes

Answer 97

Between L3/L4 **Raised ICP** GCS <9 Focal Neurological signs **coagulopathy** Cardiovascular compromise (bradycardia and HTN), **Infection at the site of LP**

Answer 98

Usually milder and so Supportive Tx If HSV/VZV infection then Acyclovir

Answer 99

IV dexamethasone, ideally administered before or with the first dose of antibiotics once in hospital. Reduces mortality and likelihood of neurological sequelae. ceftriaxone : 2 g intravenously every 12 hours OR cefotaxime : 2 g intravenously every 6 hours

Answer 100

intravenous dexamethasone, ideally administered before or with the first dose of antibiotics once in hospital. Reduces mortality and likelihood of neurological sequelae. ceftriaxone : 2 g intravenously every 12 hours OR cefotaxime : 2 g intravenously every 6 hours AND (for Listeria monocytogenes cover) amoxicillin : 2 g intravenously every 4 hours

Answer 101

Urgent/immediate IM Benzylpenicillin Prior to immediate transfer to a hospital

Answer 102

Cryptococcus Neoformans Candida Very rarely affects immune competent people

Answer 103

Meningococcal meningitis - IV benzylpenicillin, or Cefotaxime Pneumococcal meningitis - IV cefotaxime Listeria Monocytogenes Meningitis - IV Amoxicillin and Gentamicin

Answer 104

Give IM benzylpenicillin and do an immediate hospital referral Can offer close **contacts Ciprofloxacin**

Answer 105

infection of the brain leading to inflammation of the brain parenchyma Most commonly affects frontal and temporal lobes of brain

Answer 106

Encephalitis is generally a viral infection and the viral causes are: almost always **Herpes simplex virus** but occasionally Varicella zoster virus (chickenpox), Parvoviruses, primary HIV, Mumps virus and Flaviviruses such as Japanese encephalitis virus and West Nile virus

Answer 107

TB Malaria Neisseria meningitidis - **Fungal:** - Cryptococcus - **Parasitic:** - Toxoplasmosis - from cats

Answer 108

Fever Headache Confusion Seizures Encephalopathy: Behavioural changes psychotic behaviour mood changes

Answer 109

- **Pyrexia** - **Reduced GCS** - Focal neurological deficit, such as: - **Aphasia** - **Hemiparesis** - **Cerebellar signs** - **May also have signs of meningitis:** meningo-encephalitis

Answer 110

The Purkinje cell layer - loss of the purkinje cells well due to cerebellar degradation will lead to ataxia

Answer 111

- **Blood tests:** FBC, CRP, U&Es and blood culture - **Throat swab:** culture for viral organisms - **HIV serology**: now routinely tested in the emergency department CT/MRI Head: Shows evidence of unilateral encephalitis **Diagnostic - LP and CSF analysis, and PCR** CSF analysis: Viraemia - increased lymphocytes, raised protein if viral aetiology Consider PCR for confirmation Cultures from CSF

Answer 112

Aim to start **acyclovir** within 30min of the patient arriving (10mg/kg/8h IV over 1h) if HSV or VZV Symptomatic treatment: eg phenytoin for seizures

Answer 113

Chronic progressive autoimmune, T-cell mediated inflammatory disorder of the CNS, against the **myelin basic protein of oligodendrocytes** causes demyelination of CNS neurons (brain and spinal chord)

Answer 114

- **Age**: most commonly diagnosed in 20-40 year olds - **Female gender**: MS is 3 times more common in females - **Smoking** - **Vitamin D deficiency** - due to lack of sunlight? so - **Northern latitudes** is also a risk factor - **Family history**: ***HLA-DR2*** is implicated - **Autoimmunity**: patients often have a family history of other autoimmune disorders - **EBV infection**: the virus with the greatest link to MS

Answer 115

Myelin is the protective sheath that surrounds the axons of neurons, allowing them to quickly send electrical impulses. This myelin is produced by oligodendrocytes in the CNS and by schwann cells in the peripheral nervous system

Answer 116

T Cells get through blood brain barrier, and get **activated by Myelin** = T cells can now change BBB *(so has more receptors)* to allow more immune cells to get through it T cells **release cytokines, damaging Oligodendrocytes,** and B cells make antibodies and which allow macrophages to attack the oligodendrocytes, Leaving behind areas of **plaque/sclera** on neurone

Answer 117

regulatory T-cells can inhibit other immune cells, meaning at first there may be remyelination of damaged neurons - but over time this remyelination doesn't keep up and permanent damage occurs. A characteristic features of MS is that lesions vary in their location over time, meaning that different nerves are affected and symptoms change over time.

Answer 118

- **Relapsing-remitting**: - The most common pattern (85% of cases) - Episodic flare-ups (may last days, weeks or months), separated by periods of remission. There isn't full recovery after the flare-ups, so disability increases over time - 60% of patients develop secondary progressive MS within 15 years

Answer 119

- **Secondary progressive:** - Initially, the disease starts with a relapsing-remitting course, but then symptoms get progressively worse with no periods of remission - **Primary progressive:** - Symptoms get progressively worse from disease onset with no periods of remission - Accounts for 10% of cases and is more common in older patients - **Progressive relapsing:** - One constant attack but there are bouts superimposed during which the disability increases even faster

Answer 120

EYES: **Optic neuritis** - most common presentation of MS - It involves demyelination of the optic nerve and loss of vision in one eye - PAIN WHEN LOOKING SIDEWAYS Double Vision - due to lesions with the sixth cranial nerve (abducens nerve). **Plaques in sensory pathways from skin** - Numbness, tingling, sensory loss, paraesthesia *(tingling, itching burning sensation)* **Plaques in autonomic nervous system**- Bladder incontinence, sexual dysfunction - **Trigeminal neuralgia:** stimulation to face causes pain **Cognitive decline** - Poor concentration, critical thinking, depression Charcot's triad due to Cerebellar white matter demyelination

Answer 121

**Dysarthria** – due to plaques in brainstem Difficult or unclear speech – can affect eating, talking, swallowing **Intention tremor** – due to plaques along motor pathways Muscle weakness and spasms Tremors Ataxia Paralysis **Nystagmus** – due to plaques in nerves of eyes Loss of vision Optic neuritis Painful eye movements Double vision

Answer 122

SEEN IN MS Uhtoff’s phenomenon: worsening of symptoms following a rise in temperature, such as a hot bath/sauna/exercise Lhermitte’s phenomenon: electric shock sensation on neck flexion (bending neck forward), caused by stretching the demyelinated dorsal column. *Uhtoff explained - Although the myelin sheath does regenerate, the new myelin is less efficient and temperature dependent - When exposed to high heat – conduction through new myelin drastically decreases*

Answer 123

Diagnosis - neurologist based on the **clinical picture and symptoms suggesting lesions that change location** over time. Often using the McDonald Criteria. - looking for symptoms/signs which demonstrate **dissemination in space** (i.e. different parts of the CNS affected) **and time.** Other causes for the symptoms need to be excluded

Answer 124

MRI with contrast - **Active Lesions will take up contrasts, Old ones will not** ==> Can also see Demyelinated Plaques, known as Dawson's Fingers Lumbar puncture with CSF electrophoresis = inflammatory proteins found in the CSF not serum **eg Oligoclonal IgG bands** = CNS inflammation Evoked potentials – tests how long it takes impulses to travel delayed, visual, brainstem, auditory, somatosensory potentials *E.g. stimulate optic nerve and measure time for impulse to go from eye to occipital cortex* - measures visual evoked potential As there is demyelination, conduction will be slower

Answer 125

Criteria used to diagnose MS: Uses symptoms/signs which demonstrate dissemination in space (i.e. different parts of the CNS affected) and time. **Diagnosis is based on:** - 2 or more relapses (disseminated in space and time **AND EITHER** - Objective clinical evidence of 2 or more lesions **OR** - Objective clinical evidence of one lesion **WITH** a reasonable history of a previous relapse - ‘Objective evidence’ is defined as an **abnormality on neurological exam, MRI or visual evoked potentials**

Answer 126

No cure **Acute attacks:** ***(relapsing-remitting MS)*** – IV methylprednisolone 1000 mg intravenously once daily for 3 days Plasma exchange: to remove disease-causing antibodies Chronic 1st line (frequent relapse) - Subcutaneous **Interferon Beta** For spasticity - **Baclofen**, and **Botulinum toxin injections**

Answer 127

Beta Interferon and Monoclonal antibodes eg atemuzmab (anti-CD52), natalizumab

Answer 128

Tremor – beta blocker Mild to moderate muscle spasticity – **oral medications e.g. diazepam, baclofen** (GABA analogue that reduces Ca2+ influx) Focal disabling muscle spasticity – peripheral nerve blocks, **botulinum toxin - botox injections** Removal of trigger factors e.g. UTI, bed sores Physical treatments e.g. **physio, exercise to maintain strength**. Neuropathic pain – gabapentin Depression - SSRIs

Answer 129

For Peripheral nerves, - cell body is in the spine (spinal nerve) Or brain (cranial nerve)

Answer 130

Guillain-Barré syndrome (GBS) is an autoimmune, rapidly progressive **demyelinating condition of the peripheral nervous system**, often triggered by infection “Equivalent to MS for the PNS”

Answer 131

- **Infections:** typically gastrointestinal or respiratory: - **Bacterial:** e.g. Campylobacter jejuni (30%) and mycoplasma pneumoniae - **Viral:** e.g. Zika virus, influenza, Epstein-Barr virus and cytomegalovirus association with the influenza vaccination (uncommon)

Answer 132

GBS is believed to be caused by ‘molecular mimicry’ - Thought that **infectious organisms have the antigens that resemble myelin gangliosides** on Schwann cells (PNS) 🡪 autoantibody mediated damage to myelin sheath - Process involves the production of **anti-ganglioside antibodies** (anti-GMI is positive in 25% of patients) - The demyelination **occurs in patches** along the length of the axon, so it’s called **segmental demyelination** - Early on, there is remyelination but over time, there's irreversible damage

Answer 133

- **Reduced sensation in affected limbs:** sensory findings on examination are usually mild - **Symmetrical weakness** in lower extremities first, progressing to the upper limbs: proximal muscles often affected earlier than distal muscles **Autonomic dysfunction**: e.g. tachycardia, hypertension, postural hypotension, urinary retention (in severe disease) **Respiratory distress**: shortness of breath, **Ataxia with hyporeflexia** (or areflexia) in affected limbs - ***(Absence of neurologic reflexes such as the knee-jerk reaction)***

Answer 134

- **Tingling and numbness in hands and feet:** often precedes muscle weakness - **Symmetrical, progressive, ascending weakness** - **Unsteady when walking** - **Back and leg pain:** common at some point in disease course - **Facial weakness and speech problems,** - **Double vision** (due to Affected Cranial nerves)

Answer 135

Clinical Dx of Hx of recent infection with progressive weakness and areflexia, in >1 limb, near symmetry of symptoms. - **Bloods:** exclude other causes - **U&Es**: electrolyte abnormalities resulting in neuropathic symptoms - **B12 and folate**: deficiency associated with neurological features - **TFTs**: to exclude hypothyroidism as a cause of weakness - **Anti-ganglioside antibodies**: can be used to differentiate GBS variants, e.g. anti-GQ1b antibody in Miller-Fisher syndrome, or Anti GMI

Answer 136

Nerve conduction studies: findings will typically be suggestive of demyelination, e.g. **reduced conduction velocity** Lumbar puncture for CSF: **raised protein with normal WBC count** is typical, although an initial normal protein level does not exclude GBS Spirometry: to monitor respiratory function as 20% of patients require mechanical ventilation at some stage

Answer 137

- **IV immunoglobulins (IVIg):** 5 day treatment course commenced within the first 2 weeks of symptom onset, *Contraindicated in patients with IgA deficiency as can cause severe allergic reactions* **OR** - **Plasma exchange**: 5 treatments of 2-3L over 2 weeks commenced within the first 4 weeks of symptom onset Don't do Both. *No role for steroids*

Answer 138

- **Thromboprophylaxis:** to prevent venous thromboembolism - eg a direct oral anticoagulant, unfractionated heparin, or a low molecular weight heparin - **Physiotherapy:** for those with impaired mobility or motor disturbance - **Intensive care support:** for those who develop ventilatory failure (20%), intensive care and mechanical ventilation may be required

Answer 139

This is the extrapyramidal triad of: 1 Tremor. Worse at rest; often ‘pill-rolling’ of thumb over fingers 2 Hypertonia/rigidity. - *(is too much muscle tone. For instance, arms or legs are stiff and hard to move)* Rigidity +tremor gives ‘cogwheel rigidity’, felt by the examiner during rapid pronation/supination. 3 Bradykinesia. **Cardinal sign** *(slowness of movement and speed (or progressive hesitations/halts)* Involves **Postural instability or shuffling gait** (1) Expressionless face. *Marche à petit pas = Walk slowly*

Answer 140

Some drugs Drugs - Antipsychotics eg haloperidol - *blocks dopamine receptors* metoclopramide, - antisickness, *a dopamine anatagonist* Wilson’s disease Trauma - (dementia pugilistica) - *seen in boxing* Encephalitis Carbon monoxide ***studies show smoking is protective for parkinson's!!***

Answer 141

SN is made of two parts - Pars Reticulata and **Pars Compacta** The pars compacta sends messages to the **striatum via neurons rich in the neurotransmitter dopamine,** forming the nigrostriatal pathway, which helps to **stimulate the cerebral cortex and initiate movement.** Forms part of the basal ganglia In addition to simply initiating movements, the substantia nigra helps to calibrate and fine tune the way that movements happen.

Answer 142

Parkinson’s Disease (PD) is a neurodegenerative disorder characterised by loss of dopaminergic neurones within the substantia nigra pars compacta (SNPC) of the basal ganglia **(nigrostriatal pathway)**

Answer 143

The second most common neurodegenerative disorder after alzheimers dementia - It’s progressive, adult-onset disease, and it gets more common with age - Prevalence of 1% in those aged 60-70 and up to 1-3% in those ≥80 years old - M>F **Peak onset - 55-65 years**

Answer 144

Idiopathic condition but potentially related to genetics Mutation in Parkin Gene, PINK1 Mutation in alpha-Synuclein gene Secondary causes of Parkinsonism; Vascular parkinsonism Infections – Encephalitis Toxin induced – Carbon monoxide, drugs, Pesticides ***studies show smoking is protective for parkinson's!!***

Answer 145

Neurodegenerative loss of dopamine secreting cells from the pars compacta of the substantia nigra that project to the striatum 🡪 reduced striatal dopamine levels, This means that Subthalamic nucleus will be less inhibited, so it will inhibited the Thalamus more. Less dopamine means the **thalamus will be inhibited** resulting in PROBLEMS INITAIATING MOVEMENT

Answer 146

**eosinophilic inclusion bodies** - consisting of **misfolded α-synuclein** in the dopaminergic neurones of the Pars Compacta, **called Lewy bodies.**

Answer 147

**Bradykinesia** (hypokinesia/akinesia) – slowness or absence of movement **Resting tremor** **Rigidity** – stiffness and Pain (20% initially present with pain), Increased tone in limbs and trunk **Postural instability** - Impaired balance – especially when trying to turn - **Other features** - Micrographia (abnormally small, cramped handwriting) - Hypomimia (reduced degree of facial expression) **Tremor is typically asymmetrical**!

Answer 148

- **Anosmia** (smell blindness) - **Sleep disturbance**: REM sleep is impaired - Psychiatric symptoms - **Depression** - **Anxiety** - **Dementia:** usually develops after motor symptoms, unlike in Lewy-body dementia - **Constipation** **Urinary incontinence not typical**

Answer 149

- PD is a **clinical diagnosis:** it should be suspected in a patient who has **bradykinesia** and at least one of the following: - Tremor - Rigidity - Postural instability **Tremor is typically asymmetrical!**

Answer 150

- **MRI brain:** may help exclude other causes of neurological disease but should not be used to diagnose PD - show substantia nigra atrophy - **SPECT (DaT scan):** single-photon emission computed tomography (SPECT) will show reduced dopamine uptake in the basal ganglia

Answer 151

Incontinence Dementia Symmetry Early falls Tremor in action if these are present, think of differentails......

Answer 152

**Normal pressure hydrocephalus** – increase in CSF 🡪 enlarged ventricles Will See **Magnetic gait - inability to lift the feet off the floor** - also incontinence, and dementia Treatment = **Surgical correction** Shunt from the ventricles to the peritoneum **Benign Essential Tremor** *see next card for manifestations* Very common, can run in families Treat with Beta-blockers

Answer 153

Parkinson’s Tremor **Asymmetrical** Worse at rest Improves with intentional movement May have other Parkinson's features No Change with alcohol Lower frequency (4-6 hertz) Benign Essential Tremor Symmetrical Improves at rest Worsens with intentional movement No other Parkinson's features Improves Change with alcohol Higher frequency (5-8 hertz)

Answer 154

1st line carbidopa/levodopa (Co-careldopa): 100 mg orally Most powerful drug The higher the dose, the greater the risk of SE

Answer 155

**Increase amount of dopamine in CNS** Can’t give dopamine as it can’t cross the BBB 2 DRUGS **LEVODOPA AND CARBIDOPA** Dopamine biosynthetic pathway: Tyrosine 🡪 L-dopa 🡪 dopamine 🡪 dopamine receptor L-dopa converted to dopamine under action of **Dopa Decarboxylase in CNS** As Dopa Decarboxylase also exists outside the CNS, it is commonly combined with **Carbidopa (Dopa Decarboxylase inhibitor) as it can’t cross into the CNS** so it doesn’t affect dopamine production in CNS This way the L-Dopa will only get converted into Dopamine in the CNS, not elsewhere in the body.

Answer 156

- **Dyskinesia:** excessive involuntary movements related to levodopa use (excess dopamine) - **Dystonia**: This is where excessive muscle contraction leads to abnormal postures or exaggerated movements. - **Chorea**: These are abnormal involuntary movements that can be jerking and random. - **Athetosis**: These are involuntary twisting or writhing movements usually in the fingers, hands or feet. Excessive daytime sleepiness and sudden onset of sleep

Answer 157

Initially works well but soon the Px becomes resistant to it and the effects wear off. Therefore want to only use it when Sx are bad enough to prevent early resistance.

Answer 158

Parkinson Sx THEN dementia - PARKINSON'S DEMENTIA Dementia Sx THEN Parkinson's - LEWY BODY DEMENTIA W PARKINSONISM

Answer 159

a movement disorder that causes sudden, unintended, and uncontrollable jerky movements of the arms, legs, and facial muscles. Chorea is seen in many diseases and conditions and is caused by an overactivity of the chemical dopamine in the areas of the brain that control movement.

Answer 160

an autosomal dominant genetic neurodegenerative condition that causes a progressive deterioration in the nervous system

Answer 161

autosomal dominant - ***SOMEONE WITH HD HAS 50% OF PASSING IT ONTO CHILDREN*** It's a **“trinucleotide repeat disorder”** that involves a genetic mutation in the **HTT gene on chromosome 4.**, that codes for the huntingtin protein.

Answer 162

**ON CHROMOSONE 4** On the HTT gene - the base sequence of CAG ***(that codes for Glutamine)*** is repeated more than 35 times. This means that patients have 36 or more glutamines in a row in the huntingtin protein.

Answer 163

Mutated proteins aggregate within the neuronal cells of the caudate and putamen (dorsal striatum) of the basal ganglia causing neuronal cell death. Specific neurons that die are the **GABAergic and cholinergic** neurones in the corpus striatum = **decreased ACh and GABA synthesis.** Without this, levels of dopamine increase leading to excessive movement - **Chorea**

Answer 164

A phenomenon in which the signs and symptoms of some genetic conditions tend to become more severe and/or appear at an earlier age as the disorder is passed from one generation to the next. Anticipation is where successive generations have more repeats in the gene, resulting in: - Earlier age of onset - Increased severity of disease

Answer 165

Full penetrance: All genotypes of Huntington’s will express the phenotype

Answer 166

The expanded CAG not only causes the HTT protein to be mutated, but also **interferes with DNA replication itself** When copying the HTT gene, **DNA polymerase can lose track of which CAG** it’s on and so add extra CAGs. This is called ***repeat expansion*** and happens more frequently in the production of sperm than egg. Therefore symptoms tend to present progressively sooner when Huntington's disease runs in men **REPEAT EXPANSION**

Answer 167

Patients are usually asymptomatic until symptoms begin around aged 30 to 50. It presents with an insidious, progressive worsening of symptoms. Sx present between 30-50 yrs People with HD will have more severe symptoms typically around 60+ yrs

Answer 168

- **Chorea** (involuntary, abnormal movements) - **Eye movement disorders**- Problems with initiating saccades Broken pursuit - **Dysarthria:** speech difficulties - **Dysphagia:** swallowing difficulties Ataxia - Problems with heel to toe walking ^^Due to caudate nucelus and putamen atrophy Often additional touch of parkinsonism Rigidity Slowness of fine finger movements

Answer 169

Dementia Memory problems Psychiatric problems Personality change Depression Psychosis HD usually begins with these psychiatrics symptoms before advancing to physical symptoms.

Answer 170

- Diagnosis is made in a specialist genetic centre using a **genetic test for the faulty gene and identification of the number of CAG repeats**. - This involves pre-test and post-test counselling regarding the implications of the results.

Answer 171

- **Post-test counselling:** to help patients cope with diagnosis - **Genetic counselling** regarding relatives, pregnancy and children - Involvement of **MDT** in supporting and maintaining their quality of life (e.g. occupational therapy, physiotherapy and psychology) - **Speech and language therapy** where there are speech and swallowing difficulties - **Advanced directives** to document the patients wishes as the disease progresses

Answer 172

No cure, or treatment to stop progression. Can provide Sx treatment for chorea: - **Antipsychotics (e.g. olanzapine)** - **Benzodiazepines (e.g. diazepam)** - **Dopamine-depleting agents (e.g. tetrabenazine)** - For depression - **Antidepressants**

Answer 173

Progressive uncurable condition Most common cause of death is due to Aspiration Pneumonia, due to swallowing difficulties Life expectancy of 15-20 years post diagnosis High rate of suicide The MRI typically shows **caudate nucelus and putamen atrophy**

Answer 174

The Purkinje cell layer - degradation of it will lead to ataxia

Answer 175

A syndrome caused by neurodegeneration of various causes resulting in progressive reduction of cognition and difficulty with ADLs

Answer 176

Most common Dementia Rarely affects under 65yrs (otherwise EOAD which accounts for around 5% of cases) Affects more females than males Prevalence significantly rises with age

Answer 177

- **Age**: older age is a major risk for AD - **Genetics**: most cases of AD are sporadic (95%). Small number of inherited causes exist (<5%, autosomal dominant inheritance). Inherited causes suggested by early-onset disease. - **Cardiovascular disease**: smoking and diabetes increase risk. Exercise decreases risk. - **Depression** - **Low educational attainment** - **Low social engagement and support** - **Others**: head trauma, learning difficulties

Answer 178

Sporadic (95%) - Certain alleles of apolipoprotein E (APOE) have also been identified as a risk factor. ---> **APOE E4 - this allele of the APOE gene is not as good at clearing beta plaques** *(APOE E2 is protective, as better at clearing beta plaques)* Familial - (Cause of Early onset Alzheimer's disease) - Mutations in the amyloid precursor protein (APP) and presenilin genes (PSEN1, PSEN2) have been identified as risk factor - APP gene is found on chromosome 21, so people with Down Syndrome **(Trisomy 21) have 3 APP genes** - *Alzheimer's is almost inevitable often <40 years*

Answer 179

Amyloid Beta Plaques: • Amyloid Precursor Protein (APP) - A channel protein in neurons. • If it is broken down by the **Beta secretase enzyme**, the fragment is not soluble and becomes sticky, and will clump together • ==> get in the way of neurones and their signalling ==> Plaques can trigger immune response, leading to inflammation

Answer 180

Beta amyloid plaque build-up outside the neurone, **lead to kinase enzyme activation** in Neuron This leads to **phosphorylation of the tau protein,** that helps hold microtubules in cell together. When Tau is **phosphorylated it stops supporting microtubules and clumps with other Tau proteins,** forming forming neurofibrillary tangles Neurones with tangles and non-functioning microtubules can’t signal as well, and sometimes end up undergoing apoptosis

Answer 181

Agnosia - cant recognise things Apraxia - Cant do basic motor skills Aphasia - Speech difficulties - Cognitive impairment - **Poor memory** - **Agitation and emotional lability** - **Depression and anxiety** - **Sleep cycle disturbance** Motor disturbance: wandering is a typical feature of dementia Poor memory

Answer 182

- **Loss of independence**: increasing reliance on others for assistance with personal and domestic activities - **Early stages**: problems with higher level function (e.g. managing finances, difficulties at work) - **Later stages**: problems with basic personal care (e.g. washing, eating, toileting) and motor function (e.g. walking, transferring) *‘Enduring’ doesn’t mean unfluctuating: cognition comes and goes, allowing poetic insights, as in Iris Murdoch’s poignant self-diagnosis: ‘I am sailing into the dark’.*

Answer 183

Cognitive assessment: e.g. mini mental state examination (MMSE), looks at: - **Attention and concentration** - **Recent and remote memory** - **Language** - **Praxis**: planned motor movement (e.g. perform a task) - **Executive function** - **Visuospatial function** - **Bloods and other investigations e.g. ECG, virology, chest x-ray:** exclude other pathologies - Imaging - **CT/MRI:** exclude other diagnosis and can help determine type of dementia; will show medial temporal lobe atrophy

Answer 184

- **Non-pharmacological:** programmes to improve/maintain cognitive function (e.g. structured group cognitive stimulation programmes). Also exercise, aromatherapy, therapeutic use of music/dancing, massage. - **Pharmacological:** - **Mild-to-moderate AD**: acetylcholinesterase inhibitors (e.g. **donepezil**, rivastigmine). - **Moderate-to-severe AD**: N-methyl-D-aspartic acid receptor antagonist (e.g. memantine). - **Advanced care planning** - **End of life care**

Answer 185

Estimated median survival after diagnosis is 3-9 years (variable). Development of delirium on a background of dementia is associated with more rapid progression. - **Mild**: first 2 years - **Moderate**: next 2-4 years - **Severe**: 4-5 years onwards

Answer 186

Frontotemporal dementia is a neurodegenerative disorder characterised by focal degeneration of the frontal & temporal lobes. - Typically affects patients at a younger age (< 65 years) - The mean age of onset is 58 years old. The onset before 40 years old and after 75 years old is uncommon. **Rapid onset - Overall survival is 8-10 years from symptoms starting**

Answer 187

**3R isoforms of the tau protein get hyperphosphorylated.** =====>As with AD, these can't hold the tubulins together, and **start clumping.** Affected Neurons won't function well, and so neurons get damaged/undergo apoptosis Large scale changes in brain - - Brain atrophy: the gyri get narrower whereas the sulci get wider. - The ventricles expand as the rest of the brain shrinks. ***- The parietal and occipital lobes are spared.***

Answer 188

- Frontal lobe effects: personality and behavior changes - **Disinhibition** (e.g. socially inappropriate behaviour) - **Loss of empathy** - **Apathy** (losing interest and/or motivation) - **Compulsive behaviour** - Temporal lobe effects: language problems - **Effortful speech** - **Halting speech** - **Speech-sound errors** - **Speech apraxia** (i.e. difficulty in articulation) - **Word-finding difficulty** - As atrophy progresses - **Memory loss** - **Lack of concentration** - **Inability to learn new things**

Answer 189

- Diagnosis based on **cognitive assessment** - Imaging: - **MRI:** exclude other pathology; indicates changes in the frontal and temporal lobes - Definitive diagnosis: **brain biopsy** after a person has died

Answer 190

neurones contain a protein called alpha synuclein. In LBD, this gets **misfolded in neurons** misfolded alpha-synuclein aggregates to **form Lewy bodies that deposit** inside neurones, particularly in the **cortex and the substantia nigra.** As the disease progresses, more and more neurones accumulate Lewy bodies and die.

Answer 191

Lewy-Body dementia Parkinson's Multiple System Atrophy

Answer 192

- Early symptoms are typically cognitive ones (Alzheimer's-like) - **Difficulty focusing** - **Poor memory** - **Visual hallucinations** - **Disorganized speech** - **Depression** - Later symptoms are typically motor ones (Parkinson's-like) - **Resting tremors** - **Stiff and slow movements** - **Reduced facial expressions** - Some patients may have **sleep disorders** e.g. sleep walking or talking in their sleep

Answer 193

- **Dopamine analogue e.g. levodopa:** for Parkinson's like motor symptoms - **Cholinesterase inhibitors e.g. donepezil:** increases acetylcholine availability, used for Alzheimer's-like cognitive symptom

Answer 194

Vascular dementia is a chronic progressive disease of the brain caused by cerebrovascular disease, and a lack of oxygen being supplied to neurons.

Answer 195

Brain takes around 20-25% of CO Neurons can only function in aerobic conditions. - Neurons also don’t have long term energy stores, so they need a constant supply of glucose to keep working.

Answer 196

Small vessel diseases eg Atherosclerosis, Ischaemic strokes, haemorrhage, amyloidosis = leads to lack of o2, and a gradual decrease in blood supply = chronic ischaemia Tissue gets damaged = Liquefactive Necrosis Brain necrosis leads to a loss of mental functions that are governed by that area.

Answer 197

No cure - **Management of risk factors** e.g. high BP, high cholesterol, diabetes, smoking. - **Acetylcholinesterase inhibitors e.g. donepezil:** only used if mixed dementia as may have some benefit Non-pharmacological: programmes to improve/maintain cognitive function (e.g. structured group cognitive stimulation programmes), exercise, aromatherapy, therapeutic use of music/dancing, massage

Answer 198

Alzheimer's - 60% Vascular - 20% Frontaltemporal dementia 10% Dementia with Lewy bodies 5% Young Onset Alzheimer's 1-5% Others 1%

Answer 199

Absolutely not

Answer 200

No underlying cause relevant to headache: - syndromes based on symptoms Migraine Cluster Tension (Trigeminal Neuralgia)

Answer 201

Secondary – associated with something else Meningitis Subarachnoid Haemorrhage Giant Cell Arteritis Idiopathic Intracranial Hypertension Medication Overuse Headache

Answer 202

> 50 – Hx of HIV or cancer or trauma or risk factors cerebral ven sinus thrombosis – Changing **personality or cognitive dysfunction** – Vomiting without other obvious cause

Answer 203

- Jaw claudication - Visual disturbance/eye pain - Postural - Sudden onset/thunderclap - Focal neurological symptoms present - Brought on by exercise/Valsalva (eg coughing, laughing, straining)

Answer 204

fever – altered conciousness – neck stiffness – Other abnormal neurological examination

Answer 205

* Thunderclap headache ?SAH * Seizure and new headache * Suspected meningitis * Suspected encephalitis * Red eye ? acute glaucoma * Headache + new focal neurology – including papilloedema – Other abnormal neuro exam or symptom (evidence = orange)

Answer 206

Fever Altered consciousness Neck stiffness/ Kernigs sign Focal neurology signs Fundoscopy Check BP too

Answer 207

**recurrent and throbbing headache**, often **preceded by an aura** & associated with nausea, vomiting and visual changes

Answer 208

- **Family history** - **Female gender**: migraines are three times more common in women - **Obesity** - **Triggers**: CHOCOLATE - **Ch**ocolate - **O**ral **C**ontraceptive - Alcoh**ol** - **A**nxiety - **T**umult - Loud noise - **E**xercise - **Other important triggers** include tiredness, lack of food, dehydration, menstruation, red wine and bright lights, under of over sleeping

Answer 209

Prodrome: Precedes headache by hours/days: yawning, cravings, mood/sleep change Aura: last for 60mins Vision changes minutes before attack (attack follows soon after) ZigZag lines Throbbing Headache: 4-72hrs (Hemiplegic in some cases) Resolution: Headache fades away slowly Recovery

Answer 210

neuronal hyperexcitability. This leads to **trigeminal nerves initiating an inflammatory response with subsequent dilation** of meningeal blood vessels, and sensitisation of surrounding nerve fibres leading to pain

Answer 211

An aura is a perceptual disturbance that can occur before or during a migraine. It is usually experienced as a visual phenomenon, such as flashing lights, zigzag lines, or blind spots. Other types of auras may include sensory disturbances, such as tingling in the arms and legs, or speech and language problems.

Answer 212

- **Migraine without aura** - **Migraine with aura** - **Silent migraine** (migraine with aura but without a headache)

Answer 213

4 diagnostic criteria A – 5 attacks fulfilling B-D B - attacks last 4-72 hours C – Character, two of the following: Unilateral, Pulsing, Moderate/severe, Aggravated by routine physical activity D – during headache at least one of Nausea and/or vomiting Photophobia (light sensitive) and phonophobia (sound sensitive)

Answer 214

3 diagnostic criteria A – at least 2 attacks fulfilling B (character) and C (time) B - > 1 reversible aura symptom - eg - Visual – zigzags, spots - Unilateral sensory – tingling, numbness - Speech – aphasia - Motor weakness – known as “hemiplegic migraine” so rule out stroke and TIA C > 2 of the following 4: -Aura symptoms spread gradually over 5 minutes and/or > 2 aura symptoms occurring in successions - Each aura symptom lasts 5-60 minutes -followed within 60 minutes by headache

Answer 215

Clinical Dx unless pathology is suspected where you do tests to exclude DDx, investigating red flags: **CT/MRI red flags** ie. Indications - Worst/severe headache ie. Thunderclap - Change in pattern of migraine - Abnormal neurological exam - Onset >50yrs - Epilepsy - Posteriorly located headache **Lumbar puncture** indications - Thunderclap headache - Severe, rapid onset headache/ progressive headache/ unresponsive headache

Answer 216

Conservative - Avoid Triggers **ACUTE MANAGEMENT** Mild - Paracetamol/NSAIDs - ibuprofen Severe - Triptans - **sumatriptan** Anti-emetics - metoclopramide Avoid use of Opiates

Answer 217

5-HT receptor agonists (serotonin) that are used to abort migraines when they start to develop So prevents peptide release that would lead to vasodilation, pain, neurogenic inflammation.

Answer 218

Required if >2 attacks per month OR require acute meds >2x per week Beta blockers eg. **propranolol** TCAs eg. **amitriptyline** Anti-convulsant eg. **Topiramate** - CAS patient!

Answer 219

Cluster headaches are intensely painful, **unilateral, periorbital headaches** with associated autonomic dysfunction.

Answer 220

Rarest primary headache subtype - Much rarer than migraines, and more common in males (~4x) Typically affects adults with onset usually 20-40yrs Risk factors: Smoker Alcohol Male Genetics - autosomal dominant gene has a link ***A typical patient with cluster headaches in your exams is a 30 – 50 year-old male smoker. Attacks can be triggered by things like alcohol, strong smells and exercise.***

Answer 221

Clusters of attacks may last for weeks/months before a pain free period follows which may last for years

Answer 222

Crescendo pain, that is **unilateral and periorbital** may also affect temples Have **ipsilateral, autonomic features** - Conjunctival infection and lacrimation Ptosis Miosis Rhinorrhoea (runny nose) lid swelling, lacrimation, 5 similar attacks confirms diagnosis

Answer 223

- **Unilateral, periorbital or temporal headaches** lasting 15 minutes to 3 hours - Ipsilateral **autonomic** symptoms: - **Lacrimation** (teary eye) - **Conjunctival injection** (red eye due to enlargement of conjunctival vessels) - **Nasal congestion** - **Rhinorrhoea** (nasal discharge) - **Nausea and vomiting** - **Photophobia, with agitation and restlessness** Horner's Syndrome - **Ptosis** (eyelid drooping) - **Miosis** (excessive constriction of the pupil of the eye) - **Anhidrosis** (Decreased sweating on on half of face)

Answer 224

Damage to the cervical sympathetic chain causes Horner's syndrome by removing sympathetic innervation to the head and neck = Lack of sympathetic innervation to the head and neck

Answer 225

Acute attacks Analgesics are unhelpful 15L 100% O2 for 15 mins via non-rebreather mask Triptans eg. Sumatriptan

Answer 226

Prophylaxis – Verapamil (CCB), Lithium, Prednisolone, Corticosteroids

Answer 227

The most common form of a primary headache which has a characteristic rubber band feeling around the head.

Answer 228

May be due to muscle ache in the frontalis, temporalis and occipitalis muscles. - Increased tenderness of muscles of head and face leading to activation of vasculature-surrounding nociceptors.

Answer 229

**STRESS** - MAIN RISK FACTOR/TRIGGER Sleep deprivation Bad posture Hunger Eyestrain Anxiety Conflict Overexertion Depression Clenched jaw Noise

Answer 230

Bilateral, mild to moderate intensity pain Not aggravated by exercise No nausea or vomiting (anorexia may occur) No more than one of photophobia and phonophobia

Answer 231

Stress relief Avoid triggers Simple analgesia: Aspirin/Paracetamol

Answer 232

Trigeminal nerve (CN5) has both motor and sensory functions and enters the brainstem at the level of the Pons. Three divisions: Ophthalmic *(exits Superior Orbital fissure)* Maxillary *(Foramen Rotundum)* Mandibular *(exits Foramen Ovale)* Function = General SENSATION of the FACE, SCALP, NOSE MOUTH AND FRONT OF TONGUE, MOTOR = OPEN and CLOSE OF MOUTH (MASTICATION) GENERAL SENSATION OF front 2/3 of tongue. *also inervates tensor tympani for hearing*

Answer 233

A chronic, debilitating condition resulting in intense and extreme episodes of pain, due Compression of the trigeminal Nerve

Answer 234

**Vein or artery compressing** the trigeminal nerve Local pathology pressing on trigeminal nerve (more common in younger people): - Aneurysms - Meningeal inflammation - Tumour - Infarction Petrous bone – **spreading middle ear infection** **Multiple sclerosis**

Answer 235

3 attacks, with unilateral pain that is **Severe intensity, Electric shock like,** shooting, stabbing or sharp, Precipitated by **innocuous stimuli to the affected side of the face** Occurs in **one or more distributions of the trigeminal nerve**, with no radiation beyond the trigeminal distribution No neurological deficit

Answer 236

- Trigeminal neuralgia is a **clinical diagnosis** - **MRI brain:** imaging may be used if a sinister cause is suspected, such as a space-occupying lesion or demyelination, or if the patient is refractory to medical treatment and surgical intervention is being considered

Answer 237

First Line: Carbamazepine (anticonvulsant) Second Line: Phenytoin/Gabapentin (analgesic targeted for neuropathic pain) Surgery possible to decompress/intentionally damage nerve *similar to seizure meds?*

Answer 238

Duration: MH - 4-72 hrs TH - Minutes to dates CH - 15-180 mins TGN - Few seconds Site: MH - Unilateral TH - Bilateral CH - Retro-orbital/unilateral TGN - unilateral V1/2/3 distribution

Answer 239

Character: MH - Throbbing TH - Pressing/tight band CH - Hot poker/boring TGN - Electric shock/stabbing Severity: MH - Moderate-severe TH - Mild to moderate CH - Severe to very severe TGN - Severe to very severe

Answer 240

Acute Tx: MH - Triptan TH - Paracetamol CH - Triptan/100% O2 TGN - Carbamazepine

Answer 241

**Glial cells are the non neuronal cells of the CNS and PNS that do not produce electrical impulses** - Astrocytes - provide: physical support, repair, K+ metabolism neurotransmitter removal and maintenance of the BBB. - Microglia - immune cells of the central nervous - Oligodendrocytes - produce the myelin sheath insulating neuronal axons in CNS - Ependymal Cells produce CSF and help with CSF homeostasis, brain metabolism, and the clearance of waste from the brain.

Answer 242

Astrocytoma - specifically - **Glioblastoma multiforme** is the most common, an aggressive type of astrocytoma - Histologically has **pseudo-palisading pattern** - peripheral tumour cells lined up around necrotic centre **- Grade IV (most malignant)** (90% of Primary brain tumours) 2nd Most common Paediatric cancer

Answer 243

**Meningiomas** - - Graded I through III, relatively slow growing. - Histologically, they form nests of cells or a **multinuclear syncytium** of fused cells. - Can cause calcifications called **psammoma** bodies. **Haemangioblastomas** - Derived from cells with blood vessel origins - Can be anywhere, often found in cerebellum - Typically grade I => Slow growing - Histologically, there are often thin-walled capillaries that are arranged close to one another.

Answer 244

Graded 1-4 1 = Benign Pilocytic astrocytoma - Good prognosis 2 = Diffuse Astrocytoma - >5 yrs (10 years average) 3 = Anaplastic Astrocytoma - 2-5 yrs 4 = Glioblastoma - <1 yr

Answer 245

Brain tumours Intracranial haemorrhage Idiopathic intracranial hypertension Abscesses or infection

Answer 246

Characterised histologically Cellularity Mitotic activity Vascular proliferation Necrosis

Answer 247

Low grade – typically present with seizures (can be incidental finding). High grade – rapidly progressive neurological deficit. Symptoms of raised intracranial pressure.

Answer 248

- Signs and symptoms of **raised intracranial pressure** - **Headache:** worse on waking, coughing and bending - **Vomiting** - **Altered mental state** - **Visual field defects** - **Seizures** (particularly focal) - **Unilateral ptosis** - **Third and sixth nerve palsies** - **Papilloedema** (on fundoscopy) Cushing Triad (Increased PP, Bradycardia, Irregular Breathing) Epileptic Seizures Cancer Sx: Lethargy Malaise Weight Loss **Initially asymptomatic with small tumours**

Answer 249

To maintain cerebral perfusion pressure!! The body prioritises **CPP (cerebral perfusion pressure)** over pretty much everything else . Increase in ICP = will lead to decrease in CPP The body compensates by raising MAP to counteract the rise in ICP – hence hypertension This may then lead to a reflex bradycardia based on the HTN, and breathing can become irregular due to brainstem compression

Answer 250

- **Temporal lobe:** dysphasia, amnesia - **Frontal lobe:** hemiparesis, personality change, Broca’s dysphasia, lack of initiative, unable to plan tasks - **Parietal lobe**: hemisensory loss, reduction in 2-point discrimination, dysphasia, astereognosis (unable to recognise object from touch alone) - **Occipital lobe:** contralateral visual defects - **Cerebellum:** DASHING - Dysdiadochokinesis (impaired rapidly alternating movement), Ataxia, Slurred speech (dysarthria), Hypotonia, Intention tremor, Nystagmus, Gait abnormality -

Answer 251

Constant Nocturnal Worse on waking Worse on coughing, straining or bending forward Vomiting

Answer 252

Papilloedema is a swelling of the optic disc secondary to raised intracranial pressure. Papill- refers to a small rounded raised area (the optic disc) and -oedema refers to the swelling Sheath around the optic nerve is connected with the subarachnoid space. Therefore it is possible for CSF under high pressure to flow into the optic nerve sheath.

Answer 253

- Blurring of optic disc margin - Elevated optic disc (look for the way the retinal vessels flow across the disc to see the elevation) - Venous engorgement - Peripapillary haemorrhage *Vessels are able to flow straight across a flat surface, whereas they will curve over a raised disc.*

Answer 254

Acoustic Neuroma (AKA Vestibular Schwannoma) = *tumours of the Schwann cells surrounding the auditory nerve that innervates the inner ear.*

Answer 255

CT (with contrast) MRI (better especially for pituitary lesions) Biopsy - determine grade Fundoscopy - Papilloedema due to raised ICP NO LP as this is CI in raised ICP - *mass lesion since withdrawing CSF may provoke immediate coning (herniation of brain through foramen magnum 🡪 brainstem compression*

Answer 256

Treatment is non-curative (except for grade I) High grade tumour: Dexamethasone (steroid) – reduce oedema Surgery – biopsy or resection - for diagnosis, and to reduce ICP Radiotherapy – mainstay of treatment Chemotherapy – temozolamide, PCV Prognosis – 6 months no treatment/18 months with

Answer 257

Surgery – early resection or biopsy Radiotherapy alone – delays disease transformation not overall survival Radiotherapy and Chemotherapy – evidence improves long-term survival Functional MRI – used during an awake craniotomy Test speech, sensation, listening ability Better with mapping

Answer 258

Metastases from: Non-Small Cell Lung Cancer (NSCLC) Breast Small Cell Lung Cancer (SCLC) Melanoma Renal Cell Carcinoma Gastric Cancer

Answer 259

Secondary brain tumour from a NSCLC

Answer 260

Aneurysm Abscess Cyst Haemorrhage Idiopathic intracranial hypertension

Answer 261

**glutamate,** NMDA is the primary receptor that responds to glutamate by opening ion channels that let **Ca2+ ions in**.

Answer 262

**GABA**, ***Gamma-aminobutyric acid*** binds to **GABA receptors** that tell the cell to inhibit the signal by opening channels that let in **chloride ions** Cl-.

Answer 263

Seizures are transient episodes of abnormal, excessive, hypersynchronous electrical activity in the brain. There are many different types

Answer 264

A neurological disorder characterised by an increased tendency to have recurrent seizures that are idiopathic and unprovoked. (>2 episodes more than 24hrs apart)

Answer 265

highest at the extremes of life with most cases starting before 20yrs or after the age of 60yrs One of the most common neurological disorders 2% of UK population will have 2 or more seizures

Answer 266

In a seizure, clusters of neurones are temporarily impaired and start sending out **lots of excitatory signals - sometimes said to be paroxysmal.** This is due to an imbalance between inhibition and excitation of neurons, as **Balance of GABA and Glutamate shifts towards glutamate** ===> more excitatory stimulation

Answer 267

Paroxysmal event in which **changes of behaviour, sensation or cognitive processes** are caused by **excessive, (too much voltage), hypersynchronous neuronal discharges** in the brain. *(from lecture)*

Answer 268

Generalised seizures (affect both hemispheres) Focal (affect one hemisphere/lobe)

Answer 269

Tonic Clonic Absence Tonic Myoclonic Atonic

Answer 270

Tonic seizure: the muscles become **stiff and flexed,** which can cause the patient to fall, usually backwards Clonic seizures: **violent muscle contractions** (convulsions). Tonic-clonic seizures: there is **loss of consciousness and tonic (muscle tensing) and clonic (muscle jerking) episodes.** Typically the tonic phase comes before the clonic phase. There may be associated tongue biting, incontinence, groaning and irregular breathing.

Answer 271

Myoclonic seizures: short muscle twitches. The patient usually remains awake during the episode. Absence seizures:** impaired awareness or responsiveness, Patient becomes blank and stares into space before returning to normal. Motor abnormalities are either absent or very minor e.g. eyelid flutters or repetitive lip smacking Atonic seizures:** aka drop attacks. The muscles suddenly relax and become floppy, which can cause the patient to fall, usually forward. These don’t usually last more than 3 minutes **= *common in children*

Answer 272

**Simple focal** - The Focal region of cortex **+ NO basal ganglia/thalamus involvement** **Complex focal** The focal region of cortex **+ Basal ganglia and thalamus are involved.**

Answer 273

No Loss of consciousness The patient is awake and aware Will have uncontrollable muscle jerking and may be unable to speak

Answer 274

There is loss of Consciousness Patient is unaware

Answer 275

a focal seizure may **spread to affect a wider network of neurons involving both hemispheres.** Traditionally termed a secondary generalised seizure.

Answer 276

Temporal – memory, emotion and receptive speech Aura – déjà vu, auditory hallucinations, funny smells, Doing strange things on autopilot Out of body experience, automatisms e.g. lip smacking, chewing, fiddling

Answer 277

motor and thought processing Jacksonian march – seizure “marches” up or down the motor homunculus starting in face or thumb *(primary motor cortex is in frontal lobe)* Post-ictal Todd’s palsy – paralysis of limbs involved in seizure for several hours

Answer 278

sensory disturbances Tingling/numbness Parasthesias *More subjective and difficult to diagnose than other areas*

Answer 279

Visual hallucinations Visual phenomena – spots, lines or flashes Transient blindness Rapid and forced blinking Movement of head or eyes to the opposite side

Answer 280

- **Prodromal phase:** - Confusion, irritability or mood disturbances - **Early-ictal phase:** - Aura: warning felt before a seizure. These can include sensory, cognitive, emotional or behaviour changes. - **Ictal phase:** - Will vary depending on seizure type - **Post-ictal phase:** - Confused, drowsy and irritable during recovery

Answer 281

Must have had 2 or more seizures MORE THAN 24 hrs apart to be considered

Answer 282

Electroencephalogram (EEG)– detects electrical signals in the brain - would be abnormal in epilepsy. Gold standard Need to rule out any other causes of the seizures!!: CT head, or MRI- may see a structural lesion U&Es, - particularly hyponatraemia/hypernatraemia, or uraemia can cause seizures FBC - elevated WBC can indicate a systemic or CNS infection, which can cause seizures Blood glucose - again, extreme hypoglycaemia or hyperglycaemia can cause generalised tonic-clonic seizures Toxicology screen - a variety of illicit substances may cause a provoked GTCS

Answer 283

Status epilepticus (SE) is a single, continuous seizure lasting more than five minutes or two or more seizures within a five-minute period without regaining consciousness in between. It is a **medical emergency**.

Answer 284

ABCDE approach - secure airway and give oxygen. **IV bolus—to stop seizures: eg lorazepam 4mg** (in hostpial) Give 2nd dose of lorazepam if no response after 10–20min **Or Buccal Midazolam, or rectal diazepam** *(try cannulating a fitting person)* Then Phenytoin if second dose doesn't work. Get ITU/Anaesthetist help!!

Answer 285

VITAMIN DE: Vascular - haemorrhage/infarcts Infection - Encephalitis Trauma Alcohol -*(if patient has seizures due to alcohol/alcohol withdrawal, give pabrinex)* Metabolic - Hypocalcaemia, Hypo/hypernatraemia, hypoglycaemia Idiopathic - Epilepsy Neoplasms - causing a lesion in brain Dementia + Drugs (cocaine) Eclampsia + everything else

Answer 286

Paroxysmal event in which changes in behaviour, sensation and cognitive function caused by mental processes associated with psychosocial distress

Answer 287

Tongue biting Eyes open Post ictal fatigue Head turning Muscle pain Loss of consciousness >5min Cyanosis Incontinence Postictal confusion

Answer 288

- No seizures from sleep - No incontinence - No tongue biting - No cyanosis - Longer duraiton, 1-20 mins - Eyes open - pre-ictal anxiety symptoms, crying/weeping in ictal phase History of psychiatric illness

Answer 289

Paroxysmal event in which changes in behaviour, sensation and cognitive processes are caused by an insufficient blood or oxygen supply to the brain

Answer 290

Prolonged upright position Eyes closed Sweating prior to LOC Nausea Presyncopal symptoms Pallor

Answer 291

- **Cerebrovascular disease** - **Head trauma** - **Family history**: epilepsy or neurological illness - Dementia Drug use - **Congenital malformations of the brain** - eg cerebral palsy ***STROKE CAN CAUSE EPILEPSY***

Answer 292

1st line: sodium valproate or lamotrigine.

Answer 293

Carbamazepine for focal seizures

Answer 294

Lamotrigine and Carbamazepine both inhibit voltage gated pre synaptic Na+ channels - therefore reduces pre synaptic excitability

Answer 295

inhibits an enzyme that leads to an increase in an inhibitor of GABA transaminase ==> Therefore GABA is broken down less aka leads to an increase in GABA/inhibitory neurotransmission

Answer 296

All females of childbearing age (15-45) Sodium Valporate is Teratogenic Can also damage liver, and cause hepatitis and pancreatitis, can also lead to hair thinning and weight gain Instead use Lamotrigine

Answer 297

Lamotrigine is associated with Stevens-Johnson syndrome, Toxic Epidermal Necrolysis or Hypersensitivity syndrome. **its a dermatological medical emergency, type IV hypersensitivity reaction** High levels of carbamazepine are associated with **Blurred vision, nystagmus, unsteadiness and incoordination**

Answer 298

**Adults who present with an isolated seizure:** - Should stop driving for 6 months, providing no cause is found on brain imaging and there is no epileptiform activity on EEG Known epileptics Patients must be seizure-free for 12 months before they may qualify for a driving license **If the patient drives a heavy goods vehicle (HGV):** - They must be seizure-free for 5 years before they can start driving

Answer 299

ends at the level of the L1/L2 spinal vertebrae. the terminal end of the spinal chord is known as conus medullaris.

Answer 300

Paralysis to one side of the body (usually due to a brain lesion)

Answer 301

Paralysis of both legs/lower body (usually due to a spinal cord lesion)

Answer 302

Ascending tract for fine touch, 2pt discrimination and proprioception.

Answer 303

Travels in dorsal route (Fasciculus Gracilis/Cuneatus) Decussates in the medulla

Answer 304

Ascending: Enters at spinal level of nerve ascends 1-2 spinal levels and then decussates

Answer 305

Upper motor neurons for movement. Decussates at the medulla

Answer 306

a neurone whose cell body originates in the cerebral cortex or brainstem and terminates within the brainstem or spinal cord. basically used to describe **descending motor neurons in corticospinal and corticobulbar tracts.** Both arise form pre-central gyrus, Corticospinal terminate in the ventral horn of the spinal cord Corticobulbar terminate in and motor nuclei of cranial nerves

Answer 307

multipolar neuron which connects the upper motor neurone (UMN) to the skeletal muscle it innervates. The cell body of a LMN lies within the ventral horn of the spinal cord or the brainstem motor nuclei of the cranial nerves. The axon of a LMN exits the CNS terminates on the muscle fibres which it innervates. The combination of the LMN and these fibres is known as a motor unit. Also known as a motor neuron

Answer 308

Compression of the spinal cord resulting in upper neurone signs and specific symptoms dependent on where compression is This is a medical emergency as it can lead to paralysis.

Answer 309

Vertebral body neoplasms (most common cause) - **Spinal pathology** - **Disc herniation** - When centre of disc (nucleus pulposus) has moved out through the annulus (outer part of disc) resulting in pressure on nerve root and pain - **Disc prolapse** - When nucleus pulposus moves and presses against the annulus - can cause a bulge in the disc - **Primary spinal cord tumour** e.g. glioma, neurofibroma - **Infection** e.g. epidural abscess - **Haematoma**

Answer 310

lung, breast, prostate, thyroid, kidney, myeloma, lymphoma

Answer 311

- **Sensory loss 1-2 cord segments below level of lesion** - **UMN signs below the level of lesion** – everything goes up Progressive weakness of legs (typically symmetrical) with UMN signs e.g. contralateral spasticity and hyperreflexia - **LMN signs at level of lesion** Bladder sphincter involvement – hesitancy, frequency, painless retention

Answer 312

Hypertonia - an abnormally high level of muscle tone or tension Hyperreflexia - overactive or overresponsive bodily reflexes, twitching Spasticity Positive Babinski sign - extension of large toe when plantar surface of foot is stroked

Answer 313

Hyporeflexia/ areflexia - decreased or absent reflexes Hypotonia/ atonia - loss of muscle tone Flaccid muscle weakness or paralysis Fasciculations – small involuntary muscle twitches, Muscle atrophy

Answer 314

All motor and sensory function lost below the SCI level

Answer 315

This is a late and bad sign signalling a poorer prognosis

Answer 316

**Do not delay investigations** - **MRI:** gold standard; identifies cause and site of compression - **Biopsy/surgical exploration** may be required to identify the nature of any mass - **Screening blood tests**: FBC, ESR, B12, U&E’s, syphilis serology, LFT, PSA - **Chest x-ray:** to check for TB or lung malignancy

Answer 317

Neurosurgery to decompress cord Dexamethasone to reduce inflammation

Answer 318

Both anterior and posterior divisions of nerve root L4,L5,S1 and S2

Answer 319

The anterior division of L4,L5,S1 and S2 make up the **Tibial nerve** The posterior division of L4,L5,S1 and S2 make up the **common fibular nerve** Together these join to make the sciatic nerve.

Answer 320

It passes closely behind the **Piriformis muscle** , and becomes the tibial and common fibular/peroneal nerve at the popliteal fossa/lower thigh

Answer 321

Sciatica refers to the symptoms associated with irritation of the sciatic nerve.

Answer 322

**Spinal** Intervertebral disc herniation Disc prolapses Spinal stenosis - *due to degenerative bone disease, or RA* Spondylolisthesis – vertebra becomes displaces and slips on top of one below **Non-spinal** Piriformis syndrome *(spasming of piriformis)* Pregnancy Trauma

Answer 323

Symptoms Pain in buttock, back of thigh, leg, lateral aspect of little toe (sciatic nerve distribution) Signs Unilateral Weak plantar flexion Absent right ankle jerk Decreased sensation over lateral edge and sole of right foot

Answer 324

Cauda Equina syndrome

Answer 325

Clinical Dx generally: Can’t Do straight leg raise test without pain May have: XR CT MRI - if cauda equina suspected

Answer 326

Physiotherapy + Painkillers A SNRI, eg Duloxetine surgery – an operation called decompression surgery can sometimes help relieve sciatica

Answer 327

It's a nerve bundle formed by the lumbar, sacral and coccygeal nerves, as they travel down the spinal canal together to reach their corresponding openings. Distal to level of termination of spinal cord at L1/L2. Cauda equina syndrome caused by damage to the peripheral nerves at the cauda equina

Answer 328

nerves in the cauda equina carry motor innervation for the genitals, both internal and external anal sphincter, detrusor vesicae, and muscles of the leg. They are also responsible for skin sensations in these regions.

Answer 329

Cauda equina syndrome (CES) is a neurosurgical emergency which occurs when the bundle of nerves below the end of the spinal cord are compressed.

Answer 330

Herniation of lumbar disc – most commonly at L4/L5 and L5/S1 Spondylolisthesis – most commonly anterolisthesis (vertebra moves forward) Trauma – car crash, gun shot, bleeding via haematomas Tumours Infection Post-op haematoma

Answer 331

Spondylolisthesis is where one of the bones in your spine, called a vertebra, slips forward most commonly anterolisthesis (vertebra moves forward) Slippage of one vertebra over the one below Nerve root comes out ABOVE the disc therefore root affected will be the one BELOW the disc herniation E.g. L4/L5 herniation 🡪 L5 nerve root compression

Answer 332

***Saddle anaesthesia*** Less bladder and bowel control – increased tone of anal sphincter and muscle wall of bladder Erectile dysfunction (or other sexual dysfunction) Lumbosacral pain Leg weakness – flaccid and areflexic Paraplegia Signs Areflexia Fasciculations Loss of bowel/bladder control Urinary retention **WILL SEE LOWER MOTOR NEURON SIGNS ONLY***

Answer 333

MRI spinal cord (diagnostic) PR exam - feel for any abnormalities, and check anal tone Testing nerve roots/reflexes Knee flexion – test L5-S1 Ankle plantar flexion – test S1-S2 Straight leg raising – L5, S1 Femoral stretch test – L4

Answer 334

Management Refer to neurosurgeon ASAP to relive pressure Surgical decompression High dose dexamethasone Corticosteroids

Answer 335

Spinal cord compression is more likely to be thoracic with neurology in the upper limbs with bladder & bowel changes being a very late sign. Whereas in cauda equina, the lower limbs are classically affected with earlier bladder & bowel dysfunction and saddle anaesthesia.

Answer 336

Tumour MS Trauma Aneurysm Vertebral artery dissection 🡪 infarction Infection – cerebellar abscess from ear

Answer 337

CN 1 Sensory Innervates OLFACTORY EPITHILIUM, -- Function = OLFACTION CN 2 Sensory Innervates RETINA, -NUCLIE LOCATED IN THE LATERAL GENICULATE BODY OF THE THALAMUS Function = **Vision, Pupillary light reflex** CN 3 Motor Innervates RECTUS MUSLCES (not lateral rectus), INFERIOR OBLIQUE, LEVATOR PALPEBRAE Muscles of the EYE, Function = Movement of the EYEBALL, **parasympathetic constriction** and accommodation CN 4 Motor Innervates **SUPERIOR OBLIQUE MUSCLES** of the EYE, Function = the trochlear nerve controls the **abduction and intorsion** of the eye

Answer 338

V1 Ophthalmic, Sensory to forehead, middle nose, frontal and ethmoidal sinuses, and sensory to the cornea - **hence responsible for the corneal reflex** V2 Maxillary, Sensory to Lower eyelid, Gums/teeth of upper jaw, Bony part of cheek V3, Mandibular, Both **Sensory innervation to** Mucosa of oral cavity/palate, General sensation to Ant. 2/3 of tongue, Soft part of cheek/mandible **Motor innervation to** Muscles of mastication, Tensor Tympani

Answer 339

CN6 Motor Innervates the LATERAL RECTUS EYE MUSCLE, Function = eye movement, abduction CN7 Both Innervates SPECAIL TASTE SENSATION TO ANTERIOR 2/3 OF TONGUE (TASTE!) and MOTOR OF FACIAL EXPRESSION MUSLES - Parasympathetic innervation to Submandibular and Sublingual and Lacrimal (tear) glands Also innervation of STAPEDIUS

Answer 340

VIII, Vestibulocochlear, Sensory Innervates Vestibular and cochlear, for hearing and balance IX, Glossopharyngeal, Both Parasympathetic Secretion from parotid gland Taste to post. 1/3 of tongue, sensation from pharynx, salivation, parasympathetic from carotid vessels AND **motor to stylopharyngeus muscle**

Answer 341

Vagus, Both, Parasympathetic to thoracic and abdominal muscle, **sensory to larynx mucosa and epiglottis** AND motor to **pharynx, larynx, soft palate and upper 2/3 of oesophagus** Accessory, Motor, innervates Trapezius and sternocleidomastoid Hypoglossal, Motor, Tongue movement

Answer 342

* superior: lesion of the inferior optic radiations in the temporal lobe (Meyer's loop) * inferior: lesion of the superior optic radiations in the parietal lobe * mnemonic = PITS (Parietal-Inferior, Temporal-Superior so field defect is caused by lesion in opposite optic radiations - (aka **Inferior** homonymous quadrantanopia's are caused by lesions of the **superior** optic radiations

Answer 343

Homonymous hemianopia lesion of optic tract (4) Lesions of both optic radiations (7) macula sparing: Homonymous hemianopia lesion of occipital cortex (8)

Answer 344

Loss of vision in the eye of optic nerve lesion

Answer 345

Ptosis Down and out eye Fixed and dilated pupil Unopposed action of the Trochlear (CN4) and Abducens (CN6) cranial nerves, and Loss of parasympathetic outflow from the Edinger-Westphal nucleus supplying the pupillary sphincter and ciliary bodies.

Answer 346

Raised ICP Diabetes Hypertension Giant cell arteritis

Answer 347

Diplopia (double vision) **when looking down** “walking down stairs”

Answer 348

Loss of function of muscles of mastication - **Jaw deviates to side of lesion** **Loss of corneal reflex** Reduced sensation or dysasthesia over the affected area

Answer 349

Adducted eye Raised ICP MS Wernicke’s Encephalopathy Pontine Stroke

Answer 350

Adducted eye Raised ICP MS Wernicke’s Encephalopathy Pontine Stroke

Answer 351

unilateral facial weakness (motor component), altered taste (sensory component), and a dry mouth (parasympathetic component). Can be caused by Bells Palsy

Answer 352

Neurological condition that presents with a rapid onset of unilateral facial paralysis - Normally post viral infection

Answer 353

UMN injured, lower half on contralateral side is weak but forehead is not as it has contralateral and ipsilateral innervation (bilateral) LMN - weakness of all the muscles on the ipsilateral side of the face

Answer 354

Hearing impairment Vertigo Loss of balance

Answer 355

Gag reflex issues Swallowing issues Vocal issues - hoarse voce

Answer 356

Jugular foramen lesion

Answer 357

11 - Can’t shrug shoulders/turn head against resistance 12 - Tongue deviation towards the side of the lesion

Answer 358

Progressive neurodegenerative disease where both upper and lower motor neurons stop functioning but there is no effect on the sensory neurons.

Answer 359

Male Family history of it Increasing age Genetic mutations - SOD1

Answer 360

Amyotrophic Lateral Sclerosis (ALS) - **most common, 50% of cases** Progressive Muscular Atrophy (PMA) Primary Lateral Sclerosis (PLS) Progressive Bulbar Palsy (PBP)

Answer 361

Anterior horn cell lesion, so LMN signs only. Affects distal muscle groups before proximal. Better prognosis than ALS

Answer 362

UMN + LMN + Cranial Nerve IX, X, XI, XII signs Lower cranial nerve nuclei affected causing dysarthria, dysphagia, nasal regurgitation of fluids, choking - Worst prognosis

Answer 363

UMN signs only. - Rare. Loss of Betz cells in motor cortex - marked spastic leg weakness and pseudobulbar palsy. No cognitive decline

Answer 364

Loss of motor neurons in **motor cortex and the anterior horn of the cord**, so combined UMN + LMN

Answer 365

Degenerative condition affecting motor neurons – **mainly the anterior horn cells** There is relentless and UNEXPLAINED destruction of UMN and anterior horn cells in the brain and spinal cord Causes both UMN and LMN dysfunction UMN and LMN affected but no sensory or sphincter loss – distinguishes from MS Never affects eye movements – distinguishable from myasthenia gravis

Answer 366

UMN – everything goes UP Hypertonia Rigidity + spasticity Hyperreflexia Extensor plantar responses (positive Babinski sign) stroke sole of foot, big toe goes up Power: Arms - Flexors > Extensors Legs - Flexors < Extensors ***If you see a patient with mixed upper and lower motor neurone signs, think Motor neurone disease!!***

Answer 367

Hypotonia Flaccidity + muscle wasting Hyporeflexia Fasciculations Babinski Reflex Negative - big toe goes down when stroking foot Generally loss of power ***If you see a patient with mixed upper and lower motor neurone signs, think Motor neurone disease!!***

Answer 368

- **Fasciculations (twitches in the muscles)** especially on the tongue - **Dysarthria and dysphagia:** particularly in progressive bulbar palsy - **Progressive weakness** often first noticed in the upper limbs - **Clumsiness** - **Fatigue** - **Falls** - **Speech and swallow issues:** particularly in progressive bulbar palsy ***If you see a patient with mixed upper and lower motor neurone signs, think Motor neurone disease!!***

Answer 369

Anywhere on a motor nerve between the pre-central gyrus to the anterior spinal cord

Answer 370

Anywhere on a motor nerve between the anterior spinal cord and the innervated muscle

Answer 371

Eye muscles: Affected in Multiple sclerosis and Myasthenia Gravis Sensory Function and Sphincters: Affected in MS and Polyneuropathies

Answer 372

- Definite: LMN + UMN signs in 3 regions - Probable: LMN + UMN signs in 2 regions - Probable with lab support: LMN + UMN signs in 1 region, or UMN sign in more than 1 region + electromyography (EMG) shows acute denervation in more than 2 limbs

Answer 373

- **Electromyography:** in MND there will be evidence of fibrillation potentials - due to denervation of muscles due to LMN dysfunction - *( EMG is a technique for evaluating and recording the electrical activity produced by skeletal muscles.)* - **Nerve conduction studies:** may show modest reductions in amplitude - **MRI spine:** imaging can help exclude spinal pathology which may mimic MND, such as cervical cord compression and myelopathy - **Lumbar puncture:** to exclude inflammatory causes - **Pulmonary function tests:** patients with MND are at risk of respiratory failure Blood tests e.g. raised Creatinine Kinase (due to muscle destruction),

Answer 374

- **Multiple sclerosis** - **Polyneuropathies** - **Myasthenia gravis** - **Diabetic amyotrophy** - **Guillain-Barre syndrome** - **Spinal cord tumours**

Answer 375

**Riluzole, anti-glutaminergic: an inhibitor of glutamate release and NMDA receptor antagonist** = is thought to protect motor neurons from glutamate-induced damage, *(only drug that can increase survival, 2-4 months)* Antispasmodics: such as baclofen Respiratory support: patients with reduced FVC can use non-invasive ventilation at home, usually BiPAP; prolongs survival by 7 months

Answer 376

- **Analgesia** - **Feeding support:** many patients require nutritional support, often with PEG tube - **Speech and language therapy** - **Physiotherapy** - **Advanced directives** to document the patients wishes as the disease progresses - **End of life care** planning

Answer 377

- **Aspiration pneumonia** and **bronchopneumonia** - **Respiratory failure**: often occurs in advanced disease when respiratory muscles have been affected Most patients die within 3 years from the onset of their symptoms.

Answer 378

(Latin for “Grave Muscle Weakness”), its a Type 2 hypersensitivity reaction/autoimmune disease against nicotinic acetylcholine receptors (AChR) in the NMJ

Answer 379

More common in females than males – although >50 more common in males Peak incidence is 30 for females (Autoimmune related) Peak incidence is 60 for males (Thymoma related)

Answer 380

Action potential arrives at NMJ, causing opening of voltage-gated Ca2+ channels. increase in intracellular Ca2+ causes vesicles containing acetylcholine (ACh) to release their contents into the synaptic cleft. ACh activates **nicotinic ACh receptors** in the muscle fibres’ plasma membrane, resulting in an **influx of sodium ions and depolarisation of the muscle fibre membrane potential**. Leads to generation of action potential in skeletal muscle fibre

Answer 381

type II hypersensitivity reaction B cells produce **anti-AChR autoantibodies** which interfere with the NMJ by **binding to nicotinic AChR** on muscle cells When AChR are bound by the autoantibodies, they **can’t be bound by ACh and don’t respond to “contract” signal from CNS** Results in muscle weakness

Answer 382

- anti-AChR autoantibodies (85%) - Some people produce Muscle specific receptor tyrosine kinase (MuSK) antibodies which target proteins in muscle cells - (15%) - **Thymoma or thymic hyperplasia**: 10-15% of patients have a thymoma, whilst up to 70% have thymic hyperplasia - thought that perhaps T cells made in thymus can lead to B cells creating autoantibodies

Answer 383

Worse later in the day/on exertion Lethargy Muscle weakness that starts at head/neck and moves downwards Weak eye muscles - diplopia, ptosis Ptosis Jaw fatigability - slurred speech/ chewing difficulties - **Dysphagia:** difficulty swallowing

Answer 384

Thyroid ophthalmology MS Myotonic dystrophy Brainstem cranial nerve lesions Lambert-Eaton myasthenic syndrome

Answer 385

Serology - Ab testing: serum acetylcholine receptor (AChR) antibody analysis (85%) muscle-specific tyrosine kinase (MuSK) antibodies (15%) Bedside tests - Count to 50 and as they reach higher numbers their voice becomes less audible arm outstretched and ask patient to keep looking at it without lifting head up – after a few seconds they will unable to keep their eyes raises Nerve Stimulation Tests - decrement in evoked potential after motor nerve stimulation Serial measurements of forced vital capacity (FVC) - looking for pulmonary function, can be reduced in Myasthenic crisis CT Thorax/MRI - look for Thymoma

Answer 386

- **First-line: pyridostigmine**; an acetylcholinesterase inhibitor - **Second-line: prednisolone** (to dampen down immune system) Other: if symptoms remain uncontrolled, consider alternatives such as methotrexate or rituximab Thymectomy: indicated if a thymoma is present on imaging, or in patients aged < 45 years old with positive serology;

Answer 387

- **Myasthenic crisis:** - **Respiratory failure** - **Aspiration pneumonia:** dysphagia increases the risk of aspiration

Answer 388

weakening of the respiratory muscles and is often provoked by infections or medications. Patients present with increasing shortness of breath, which can deteriorate into respiratory failure Monitor forced vital capacity. **Ventilatory support (ie BiPAP)** may be needed. Treat with **plasmapheresis (removes AChR antibodies from the circulation)** or **IV immunoglobulin** and identify and treat the trigger for the relapse (eg infection, medications).

Answer 389

A NMJ syndrome which has similar Symptom to MG. Autoimmunity **against the voltage gated calcium channels** = less ACh release at the NMJ causing muscle weakness.

Answer 390

- Can be **paraneoplastic:** associated with malignancies, **especially small cell lung cancer** - OR **autoimmune** - Can be associated with other autoimmune diseases e.g. Hashimoto’s thyroiditis or diabetes mellitus type 1

Answer 391

Proximal muscle weakness that develops more slowly Symptoms **start at extremities and progress towards the head** - *opposite to MG* Causes defective ACh release at the NMJ resulting in proximal limb weakness with absent reflexes

Answer 392

Weakness tends to improve after exercise – unlike in MG

Answer 393

*Similar to MG* - **Manage underlying malignancy** - **Pyridostigmine** allows more acetylcholine to be released in the neuromuscular junction synapses - **Immunosuppressants** (e.g. prednisolone or azathioprine) - **IV immunoglobulins:** bind and neutralise the autoantibodies. - **Plasmapheresis:** to remove damaging antibodies Do regular CXR/high-resolution CT as symptoms may precede the cancer by >4yrs.

Answer 394

A term used to describe the event of temporarily losing consciousness due to disruption in blood flow. Known as vasovagal episodes *Presyncope: near loss of consciousness with lightheadedness, muscular weakness, blurred vision, and feeling faint without actually fainting.*

Answer 395

**Primary syncope (simple fainting):** - Dehydration - Missed meals - Extended standing in a warm environment, such as a school assembly - A vasovagal response to a stimuli, such as sudden surprise, pain or the sight of blood

Answer 396

- Hypoglycaemia - Anaemia - Anaphylaxis - Arrhythmias - Hypovolaemia, due to a bleed - Pulmonary embolism, causing hypoxia

Answer 397

- **Certain medications that** - Block vasoconstriction e.g. calcium channel blockers, beta blockers, alpha blockers, and nitrates - Affect the volume status e.g. diuretics - That prolong the QT interval e.g. antipsychotics and antiemetics - **Elderly** - **Pregnant women** Are also more prone to syncope

Answer 398

Probem with autonomic NS regulating blood flow to brain Vagus nerve receives strong stimulus, eg emotional event, pain or temperature change = **stimulate the parasympathetic nervous system.** Parasympathetic system counteracts sympathetic NS, ==> **causes blood vessels delivering blood to the brain vasodilate** the blood pressure in the cerebral circulation drops, leading to hypoperfusion of brain tissue. Leads to pt loose consciousness and faint

Answer 399

- variant of neurocardiogenic syncope. This occurs when **mild external pressure on the carotid bodies in the neck is enough to induce Parasympathetic response** *(seen in vasovagal)* Mild pressure could be due to shaving, neck turning, tight collar etc

Answer 400

Syncope due to postural change (standing from sitting) It occurs when there’s a **delay in constriction of the lower body veins,** which is needed to maintain an adequate blood pressure when changing position to standing. As a result, blood pools in the veins of the legs for longer and less is returned to the heart, leading to a reduced cardiac output. defined by either a **drop in blood pressure of more than 20 mmHg** or a reflex tachycardia of more than 20 beats per minute,

Answer 401

Situational Typically, from sitting or standing Rarely from sleep Presyncopal symptoms Occipital – see stars, dizzy and light headed, vision goes black Temporal – noises may sound distorted Back of brain most sensitive to oxygen Duration – 5-30 seconds Recovery within 30 seconds Cardiogenic syncope – less warning, history of heart disease

Answer 402

Clinical Hx and examination Ix to rule out pathological causes: Bloods - infection FBC - anaemia ECG- arrythmia Glucose - Hypoglycaemia B-hCG - ectopic pregnancy

Answer 403

Confirm Dx to exclude underlying pathology Avoid Dehydration Avoid missing meals Avoid standing still

Answer 404

A-alpha – proprioception A-beta – light touch, pressure, vibration A – delta – dull pain and cold C – fibres – sharp pain and warmth

Answer 405

Nerve pathology outside of the CNS that affects the peripheral nerves Mononeuropathy: a process affecting a single nerve Polyneuropathy: many nerves involved. Usually describes a symmetrical disease, and it usually begins distally. Can be sensory, motor or mixed.

Answer 406

DAVIDE: Diabetes Alcohol Vitamin B12 Deficiency Infective - Guillain Barre/Charcot Marie Tooth Drugs - isoniazid Every vasculitis

Answer 407

Demyelination *(Schwann cell damage, seen in MS/GBS)* Axonal Damage - Nerve cut *(eg seen in toxic neuropathies)* Nerve Compression *(Focal demyelination at point of compression, disurpts conduction, eg Carpal syndome)*

Answer 408

Vasa Nervorum Infarction *(eg diabetes and arteritis)* Wallerian Degeneration (Nerve cut/lesion, which causes distal end of nerve to die)

Answer 409

* Carpal tunnel syndrome * ‘Wrist drop’ * ‘Claw hand’ * ‘Foot drop’ Carpal tunnel syndrome is most common

Answer 410

Carpal tunnel syndrome (CTS) is a collection of symptoms and signs caused by compression of the median nerve in the carpal tunnel.

Answer 411

Mostly Idiopathic Also: (HODPARAR) Hypothyroidism Obesity Diabetes **Pregnancy** - *can resolve postpartum* Acromegaly Rheumatoid Arthritis Amyloidosis Repetitive Strain Injury Females due to narrower wrists so more likely to have compression Over 30s *exam tip - if you see a patient with bilateral carpal tunnel syndrome, look for features that might suggest underlying rheumatoid arthritis, diabetes, acromegaly or hypothyroidism.*

Answer 412

Numbness Paraesthesia (pins and needles or tingling) Burning sensation Pain Palmer aspects and full fingertips of: Thumb Index and middle finger The lateral half of ring finger

Answer 413

Numbness Paraesthesia (pins and needles or tingling) Burning sensation Pain Palmer aspects and full fingertips of: Thumb Index and middle finger The lateral half of ring finger Weakness of thumb movements Weakness of grip strength Difficulty with fine movements involving the thumb Wasting of the thenar muscles (muscle atrophy) Thenar Muscles Flexor Pollicis Brevis Abductor Pollicis Opponens Pollicis

Answer 414

Phalen Test Tinel Test Nerve conduction studies: small electrical current is applied by an electrode (nerve stimulator) to the median nerve on one side of the carpal tunnel - Recording electrodes over the median nerve on the other side of the carpal tunnel record the electrical current that reaches them

Answer 415

Phalen’s test - flex the wrists are far as possible and hold that position for a minute, this results in numbness in the areas of the hand innervated by the median nerve in people with carpal tunnel syndrome

Answer 416

tap the **transverse carpal ligament**, this reproduces the symptoms of tingling or feelings of pins and needles in areas of the hand served by the median nerve *TOM TIP: I think of tapping a tin can (Tinel’s) to remember the difference between Phalen’s and Tinel’s test.*

Answer 417

Management options for carpal tunnel syndrome are: Rest and altered activities Wrist splints that maintain a neutral position of the wrist can be worn at night (for a minimum of 4 weeks) Steroid injections Surgery

Answer 418

Wrist drop (with elbow flexed and arm pronated?

Answer 419

BEST: Brachioradialis Extensors of forearm Supinator Triceps

Answer 420

Damage to the radial nerve Compression of the radial nerve at the humerus

Answer 421

Claw hand (4th/5th fingers claw up)

Answer 422

Splint Analgesia

Answer 423

Common peroneal nerve palsy

Answer 424

L4-S1 (Branch off the Sciatic nerve)

Answer 425

A type of peripheral neuropathy where there is damage to several individual nerves due to systemic causes.

Answer 426

WARDS PLC: - Wegener’s granulomatosis - Aids/Amyloid - Rheumatoid arthritis - Diabetes mellitus - Sarcoidosis - Polyarteritis nodosa - Leprosy - Carcinoma

Answer 427

Hemi-section of the spinal cord and therefore loss of sensations of pain temperature and touch and motor movement

Answer 428

Space occupying lesions Intervertebral disc prolapses Vertebral bone fractures Trauma – gunshot wounds, knife wounds Infectious – HIV MS

Answer 429

(Ipsilateral Corticospinal) Ipsilateral *(Same side)* weakness and loss of motor function below the lesion. (Ipsilateral DCML) Ipsilateral *(Same side)* loss of proprioception, 2-point discrimination and fine touch. (Contralateral Spinothalamic) *(opposite side)* Contralateral loss of pain and temperature sensation 1-2 spinal segments below the lesion.

Answer 430

Inherited sensory and motor peripheral neuropathy disease caused by an autosomal dominant mutation in PUP22 gene on chromosome17

Answer 431

Mutation in PUP22 gene Chromosome 17 + multiple others

Answer 432

Generally mutations cause dysfunction of the myelin or axons leading to neuropathy Different mutations in different genes have different pathophysiology’s.

Answer 433

High foot arches (pes cavus) Distal muscle wasting causing “inverted champagne bottle legs” Weakness in the lower legs, particularly loss of ankle dorsiflexion Weakness in the hands Reduced tendon reflexes Reduced muscle tone Peripheral sensory loss

Answer 434

A – Alcohol B – B12 deficiency C – Cancer and Chronic Kidney Disease D – Diabetes and Drugs (e.g. isoniazid, amiodarone and cisplatin) E – Every vasculitis

Answer 435

X-Linked recessive condition caused by a mutation in the Dystrophin Gene Boys Exclusively: Age of onset is around 3-5 years old

Answer 436

**X linked Recessive** condition. Therefore mother with 1 faulty gene: Daughters - 50% chance of being carrier Sons - 50% chance of being affected

Answer 437

Lack of Dystrophin gene (vital part of muscle fibre) means that the muscles are not protected from being broken down by enzymes Therefore in DMD you get progressive wasting and weakness of muscle as they are broken down. The muscle tissue is then replaced by fibrofatty tissue Most Px in wheelchair by teenage years

Answer 438

Dilated cardiomyopathy Dystrophin gene in heart muscle not present which is normally involved in membrane stability. Therefore in DMD there is damage to the cellular mechanisms causing dilation of ventricles due to wasting of the cardiac muscle causing cardiomyopathy

Answer 439

Child struggles to get up from lying down (GOWER’S sign) Skeletal deformities - scoliosis

Answer 440

an inability to lift the trunk without using the hands and arms to brace and push – From the lying position, the patient rolls to the kneeling position, pushes on the ground with **extended forearms to lift the hips and straighten the legs,** so forming a triangle with hips at the apex and hands and feet on the floor forming the base. The **hands are then used to push on the knees** and so lift up the trunk (climbing upon yourself).

Answer 441

Blocks the reuptake of serotonin back into the cells, so more stays outside, to try and lift the mood. Dose can be increased if necesscary

Answer 442

Tricyclicv Antidepressanets, eg anatripoline, not used often now: block the reuptake of serotonin and norepinephrine in presynaptic terminals, which leads to increased concentration of these neurotransmitters in the synaptic cleft. The increased concentrations of norepinephrine and serotonin in the synapse likely contribute to its anti-depressive effect

Answer 443

Selective Norepinephrine reuptake inhibitor, newer eg vendofaxcine, SNRIs block the reabsorption (or reuptake) of serotonin and norepinephrine back into the nerve cells that released them. Both of these neurotransmitters are associated with distinct brain structures and functions and impact various processes such as mood and energy in somewhat different ways.

Answer 444

Continuous low mood or sadness feeling hopeless and helpless having low self-esteem feeling tearful feeling guilt-ridden feeling irritable and intolerant of others having no motivation or interest in things finding it difficult to make decisions not getting any enjoyment out of life feeling anxious or worried having suicidal thoughts or thoughts of harming yourself

Answer 445

moving or speaking more slowly than usual changes in appetite or weight (usually decreased, but sometimes increased) constipation unexplained aches and pains lack of energy low sex drive (loss of libido) changes to your menstrual cycle disturbed sleep – for example, finding it difficult to fall asleep at night or waking up very early in the morning

Answer 446

avoiding contact with friends and taking part in fewer social activities neglecting your hobbies and intere

Answer 447

St John's wort is a herbal medicine used to treat mental health problems. The botanical name for St John's wort is Hypericum perforatum, and it is sometimes marketed and sold as 'Hypericum'. It contains many active substances, including hypericin and hyperforin, which are thought to affect mood. Today St John's wort is mainly used as an over-the-counter remedy to treat mild or moderate depression.

Answer 448

The Purkinje cell layer - degradation of it will lead to ataxia

Answer 449

Give IM benzylpenicillin and do an immediate hospital referral