Neurology Flashcards

Question

What is Weber's syndrome and what are the symptoms of it?

Answer 1

- It is a midbrain infarct that leads to **oculomotor palsy and contralateral hemiplegia**

Answer 2

- **Ipsilateral** facial loss of pain and temperature - **Ipsilateral** Horner’s syndrome: miosis (constriction of the pupil), ptosis (drooping of the upper eyelid), and anhidrosis (absence of sweating of the face) - **Ipsilateral** cerebellar signs - **Contralateral** loss of pain and temperature

Answer 3

The Bamford classification and it categorises strokes based on the area of circulation affected

Answer 4

- Total anterior circulation stroke - Partial anterior stroke - Lacunar stroke - Posterior circulation stroke

Answer 5

total anterior circulation stroke Blood vessel= anterior or middle cerebral artery Criteria: all of - Hemiplegia - Homonymous hemianopia - Higher cortical dysfunction

Answer 6

partial anterior circulation stroke Blood vessel= anterior or middle cerebral artery Criteria is any two of: - Hemiplegia - Homonymous hemianopia - Higher cortical dysfunction

Answer 7

Blood vessel= perforating arteries Criteria: there is no higher cortical dysfunction or visual field abnormality and there is one of: - Pure hemimotor or hemisensory loss - Ataxic hemiparesis - Pure sensomotor loss

Answer 8

posterior circulation stroke Blood vessel= Posterior cerebral or vertebrobasilar artery Criteria: - Cerebellar syndrome - Isolated homonymous hemianopia - Loss of consciousness

Answer 9

**F**- Face **A**- Arm **S**- Speech **T**- Time (this is a stupid one because it is not a symptom just there to make the word fast)

Answer 10

Recognition of Stroke in the Emergency Room (ROSIER) scale.

Answer 11

- Loss of consciousness - Seizure activity New, acute onset of: - Asymmetric facial/arm/leg weakness - Speech disturbance - Visual filed defect

Answer 12

A stroke is possible if they have any of the criteria and hypoglycaemia has been excluded **WOULD REQUIRE URGENT NON-CONTRAST CT** - Aspirin 300mg stat (after the CT)

Answer 13

**First line: non-contrast CT of head** ECG- to asses for AF Bloods to look for hyponatremia/hypoglycaemia Carotid doppler

Answer 14

**Diffusion weighted MRI** is more sensitive but harder to obtain

Answer 15

- Hypoglycaemia - Hyponatremia - Hypercalcaemia - Uraemia - Hepatic encephalopathy

Answer 16

- Antiplatelets: **aspirin** given as soon as possible once haemorrhagic stroke is excluded - Thrombolysis: **alteplase** (tissue plasminogen activator)- given if to re-establish blood flow is <4.5 hours of symptom onset - Thrombectomy must score > 5 on NIH Stroke Scale/Score (NIHSS) and pre-stroke functional status < 3 on the modified Rankin scale

Answer 17

CT angiogram (CTA): identifies arterial occlusion

Answer 18

- **Clopidogrel** an antiplatelet - High dose staitn - Carotid stenting - Manage underlying risks

Answer 19

- Related to the subtype of haemorrhage which will cover later But for now: - **Admit to neurocritical care:** patients will need intensive monitoring - **If features of raised intracranial pressure**: consider intubation with hyperventilation, head elevation (30°) and IV mannitol - **Surgical intervention:** decompression may be needed

Answer 20

For ischaemic stroke, the prognosis depends on the severity. A total anterior circulation stroke confers the poorest prognosis. Regarding thrombolysis, if administered within 3 hours, patients are 30% more likely to have minimal or no disability. In general, mortality for haemorrhagic stroke is significantly higher than for ischaemic stroke and can be as high as 40%.

Answer 21

- Must not drive for 1 month after a stroke and can't drive a HGV for 1 year after a stroke

Answer 22

- A transient episode of neurological dysfunction caused by focal brain, spinal cord or retinal ischaemia without acute infarction. - It usually resolves within 24 hours

Answer 23

ACA: weak numb contralateral leg MCA: 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 24

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

Answer 25

ABCD2 score: A: age greater than 60 B: Blood pressure greater than 140/90 C: clinical feature: Unilateral weakness (2 points), speech disturbance (1 point) D- Diabetes D- Duration 60 minuets or longer (2 points) 10 to 59 (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 26

- Auscultation: listen for carotid bruit - CT scan - Request an urgent CT scan of the head - Carotid doppler – look for stenosis - CT angiography – look for stenosis

Answer 27

- First line is **antiplatelet** initially with aspirin 300mg - Carotid endarterectomy: surgery to remove blockage of >70% on doppler - Manage cardiovascular risk with atorvastatin etc

Answer 28

Where there are two or more TIAs within a week. It carries a high risk of a stroke

Answer 29

10% within 3 months

Answer 30

- Intracerebral where the bleeding occurs within the cerebrum - Subarachnoid when bleeding occurs between the pia and arachnoid matter

Answer 31

- **Hypertension** causing arteriosclerosis and microaneurysms called **bouchard aneurysms** - **Arteriovenous malformations** blood vessels that directly connect an artery to a vein - **Vasculitis/Vascular tumours** - **Secondary to an ischaemic stroke**- ischaemia causes brain tissue death. If there is reperfusion there's an increased chance that the damaged vessel might rupture

Answer 32

- Head injury - Hypertension - Aneurysm - Brain tumour - Anticoagulant

Answer 33

- Once blood starts to spew from damaged vessel it creates a pool of blood that increases pressure in the skill and puts pressure on nearby cells and vessels. This can also lead to **brain herniation** - Haemorrhage also results in less blood flowing downstream to cells. The pressure or lack of blood can lead to tissue death within hours

Answer 34

- **Sudden headache** is a key feature - Weakness - Seizure - Vomiting - Reduced consciousness

Answer 35

- CT/MRI to confirm size and location of the haemorrhage - Check FBC and clotting - Angiography to visualise the exact location of the haemorrhage

Answer 36

- Consider ICU and intubation and ventilation if there is reduced consciousness - Correct any clotting abnormalities - Correct severe hypertension but avoid hypotension - Drugs to relieve intercranial pressure **mannitol**

Answer 37

- Craniotomy part of the skull bone is removed to drain any blood and relieve pressure - Stereotactic aspiration: aspirate off blood and relieve intracranial pressure guided by a CT scanner. Good for bleeding that is located deeper in the brain

Answer 38

- Most common in people 45-70 - More common in women - More common in black patients

Answer 39

- Trauma is a key factor - Atraumatic cases are referred to as **spontaneous** SAH

Answer 40

- **Berry aneurysm**- they account for 80% of cases. - Arise at points of bifurcation within the circle of Willis; the junction between the anterior communicating and anterior cerebral artery - They are associated with PKD, coarctation of the aorta, and connective tissue disorders (Marfan)

Answer 41

- Cocaine use - Sickle cell anaemia - Connective tissue disorders - Neurofibromatosis: tumours form on your nerve tissues - PKD - Alcohol excess

Answer 42

- Blood vessels that are bathing in a pool of blood can start to intermittently vasoconstrict (vasospasm) called. If this occurs in the circle of Willis it will reduce the supply of blood flow to the brain causing further injury . - Over time blood in the subarachnoid space can irritate the meninges and cause inflammation which leads to scarring of the surrounding tissue. The scar tissue can obstruct the normal outflow of CSF causing fluid to build up leading to **hydrocephalous**

Answer 43

- 3rd nerve palsy- if the aneurysm occurs in posterior communicating artery - 6th nerve palsy a non-specific sign which indicates raised intercranial pressure - Reduced GCS

Answer 44

- **Thunderclap headache** during strenuous activity or sex. It's like being hit really hard on the back of the head - Neck stiffness - photophobia - Vison changes

Answer 45

- FBC - Serum glucose - Clotting screening - Urgent non-contrast CT of the head. Blood will cause **hyperattenuation (this means becoming more dense on CT will show as white)** in the **subarachnoid space**

Answer 46

- Lumbar puncture: will show RBCs or xanthochromia (yellow pigmentation due to degradation of haemoglobin to bilirubin)

Answer 47

**Nimodipine** is a CCB and prevents vasospasms

Answer 48

- first-line is **endovascular coiling** of the aneurysm; - second-line is **surgical clipping** via craniotomy - **If features of raised intracranial pressure**: consider intubation with hyperventilation, head elevation (30°) and IV mannitol

Answer 49

- Rebleeding 22% risk at one month - - **Vasospasm**: accounts for 23% of deaths; at highest risk for the first 2-3 weeks after SAH; treated with (induced) hypertension, hypervolemia and haemodilution (triple-H therapy). - **Hydrocephalus**: acutely managed with external ventricular drain (CSF drainage into an external bag) or a long-term ventriculoperitoneal shunt, if required - **Seizures**: seizure-prophylaxis is often administered (e.g. Keppra) - **Hyponatraemia**: commonly due to syndrome of inappropriate antidiuretic hormone secretion (SIADH)

Answer 50

At 6 months, 25% of patients are dead and 50% are moderately to severely disabled. Causes of mortality include medical complications (23%), vasospasm (23%), rebleeding (22%) and initial haemorrhage (19%)

Answer 51

Bleeding below the dura matter

Answer 52

- Elderly - Alcoholics

Answer 53

- **Brain atrophy**: in the elderly the brain shrinks in size meaning the bridging veins are stretched across a wider space where they are largely unsupported - **Alcohol abuse**: causes the wall of veins to thin out making them more likely to break - **Trauma/injury**: falls, shaken baby syndrome, acceleration-deceleration injury

Answer 54

The collection of blood that forms as a result of a haemorrhage. As damaged bridging veins are under low pressure, the bleeding can be slow causing a delayed onset of symptoms as the haematoma gradually increases in size. Over time this will compress that brain and increase intercranial pressure

Answer 55

One that causes symptoms within 2 days

Answer 56

One that causes symptoms between 3-14 dyas

Answer 57

One that causes symptoms after 15 days

Answer 58

- Reduced GCS: which can occur straight away or in the ensuing days and weeks as the haematoma increases in size - Headaches - Vomiting - Seizures

Answer 59

Immediate CT head

Answer 60

A hyperdense mass **more white** than the surrounding healthy brain tissue

Answer 61

A hyperdense mass **more white** than the surrounding healthy brain tissue

Answer 62

A hypodense mass **less white** than the surrounding healthy brain tissue

Answer 63

Bleeding is between the dura and the arachnoid so it follow the contour of the brain and dorms a **crescent shape** and **cross suture lines**.

Answer 64

- **Stroke** - **Dementia** - **CNS masses e.g. tumours or abscesses** - **Subarachnoid haemorrhage** - **Epidural haemorrhage**

Answer 65

**Drainage**: - small SDH are drained via a **burr hole washout** a small tube called - large SDH requires a **craniotomy** which is when part of the skull bone is removed - **IV Mannitol** to reduce ICP

Answer 66

- **Supratentorial herniation:** cerebrum is pushed against the skull or the tentorium, can compress the arteries that nourish the brain leading to an ischaemic stroke - **Infratentorial herniation:** cerebellum is pushed against the brainstem, can compress the vital area in the brainstem that control consciousness, respiration, and heart rate

Answer 67

Bleeding above the dura matter

Answer 68

They usually occur in young adults

Answer 69

It is most commonly caused by head trauma. The meningeal arteries are protected by the skull but can be damaged by serious head trauma

Answer 70

The **Pterion** which is the spot where the frontal, parietal and temporal and sphenoid bone join together. It is a thin area of the skull and located just above the **middle meningeal artery**

Answer 71

Blood will pool between the skull and the external layer of the dura mater, separating it from the inner surface of the skull. The blood builds up between the skull and the outer layer of the dura mater but cannot cross the suture lines where the dura mater adheres more tightly. If blood accumulates slowly, there may be a **lucid interval** which is when several hours pass before the onset of symptoms.

Answer 72

- **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

Answer 73

Hyperdense mass = looks “more white” than the surrounding healthy brain tissue.

Answer 74

Epidural haemorrhages cause blood to build up between the outer layer of the dura mater and the skull. Epidural haematomas don’t cross suture lines and they push on the brain forming a biconvex shape.

Answer 75

- **Epilepsy** - **Carotid dissection** - **Carbon monoxide poisoning** - **Subdural haematoma** - **Subarachnoid haemorrhage**

Answer 76

- **Clot evacuation** - **Craniotomy**: part of the skull bone is removed in order to remove accumulated blood below. - Followed by **ligation of the vessel.** - **IV mannitol** to reduce ICP

Answer 77

physiological nervous system response to increased intracranial pressure (ICP) that results in Cushing's triad of: - increased blood pressure - irregular breathing, - bradycardia.

Answer 78

Inflammation of the leptomeninges (the arachnoid and pia) and usually occurs due to a bacterial or viral cause

Answer 79

- S.pneumoniae - N. meningitidis

Answer 80

- Enteroviruses such as coxsackievirus - Herpes simplex virus (HSV) - Varicella zoster virus (VZV)

Answer 81

- **Cryptococcus neoformans** - **Candida**

Answer 82

Group B streptococcus (GBS) S. agalactiae usually contracted during birth from the GBS bacteria that can often live harmlessly in the mothers vaginas.

Answer 83

- Immunocompromised: such as being in the extremes of age, infection (HIV), and medication (Chemotherapy) Listeria monocytogenes M. Tuberculosis - Non-immunised: at risk of H. influenza, pneumococcal and meningococcal meningitis - Crowded environments: students living in halls of residence are a commonly affected demographic

Answer 84

- Direct spread - Hematogenous spread

Answer 85

- Pathogens get inside the skull or spinal column and penetrate the meninges - Sometimes the pathogen will have come through the overlying skin or up through the nose but more likely through anatomical defect or acquired like a skull fracture

Answer 86

- Pathogens enter the blood stream and move through the endothelial cells in the blood vessels making up the blood brain barrier

Answer 87

- Once in CSF pathogen will start multiplying, This will cause WBC in CSF to release cytokines and recruit additional immune cells. This will massively increase the number of WBC in CSF - Additional immune cells will attract more fluid to the area and start causing local destruction this will cause CSF pressure to rise - Immune reaction will cause the glucose concentration of the CSF to fall and protein level increase

Answer 88

- Bacterial and viral meningitis are usually acute - Fungal is more chronic

Answer 89

Kernigs Test Brudzinski’s Test

Answer 90

- Involves patient lying on their back and flexing one hip and knee to 90 degrees and then slowly straightening the knee whilst keeping the hip flexed. This causes the meninges to stretch - A positive test will be where there is spinal pain or resistance to the movement

Answer 91

- Involves lying a patient on their back and gently using your hands to lift their head and neck off the bed and flexing their chin to their chest - A positive test is when a patient involuntarily flexes their hips and knees

Answer 92

. **non-blanching rash”** that everybody worries about as it indicates the infection has caused disseminated intravascular coagulopathy (DIC) and subcutaneous haemorrhages. Other causes of bacterial meningitis do not cause this rash

Answer 93

- Headache - Photophobia - Neck stiffness - Fever - Nausea vomiting - Seizures

Answer 94

- **FBC:** leukocytosis - **CRP:** raised inflammatory markers - **Coagulation screen**: required prior to lumbar puncture (LP) - **Blood glucose**: required in all patients and for comparison with CSF glucose - **Blood culture:** positive in the case of bacterial infection - **Whole-blood PCR for *N meningitidis***

Answer 95

Lumbar puncture

Answer 96

Raised ICP GCS <9 Focal Neurological signs

Answer 97

Between L3/L4 Since spinal cord ends L1/2

Answer 98

- Bacteria will release proteins and use up glucose - Virus don't use glucose but may release small amounts of proteins The immune system releases neutrophils for bacterial and lymphocytes for viral

Answer 99

(IM or IV) of benzylpenicillin prior to transfer to hospital as time is so important: < 1 year – 300mg 1-9 years – 600mg > 10 years and adults – 1200mg This shouldn’t delay transfer. Where there is a true penicillin allergy transfer should be the priority rather than other antibiotics.

Answer 100

Ideally a blood culture and a lumbar puncture for cerebrospinal fluid (CSF) if the patient is acutely unwell antibiotics should not be delayed. There should be a low threshold for treating suspected bacterial meningitis, particularly in babies and younger children. Always follow the local guidelines however typical antibiotics are: < 3 months – cefotaxime plus amoxicillin (the amoxicillin is to cover listeria contracted during pregnancy from the mother) > 3 months – ceftriaxone **Vancomycin** should be added to these if there is a risk of penicillin resistant pneumococcal infection such as from recent foreign travel or prolonged antibiotic exposure. **Dexamethasone** is given 4 times daily for 4 days to children over 3 months if the lumbar puncture is suggestive of bacterial meningitis. to reduce chances of neurological symptoms afterwards

Answer 101

- Conservative management is appropriate most of the time - Give **Aciclovir** if HSV or VZV

Answer 102

**Yes** obviously the only reason this question is in here is so I can say that it is

Answer 103

- **Hearing loss** - seizures and epilepsy - Cognitive impairment - Memory loss - Limb weakness or spasticity

Answer 104

Inflammation of the brain parenchyma. It mostly affects the frontal and temporal lobes

Answer 105

The herpes simplex virus HSV-1 accounts for 95% of cases from cold sores. In neonates HSV-2 from genital herpes is the most common

Answer 106

- **Immunocompromised** - **Blood/fluid exposure**: HIV and west Nile virus - **Mosquito bite**: west Nile virus - **Transfusion and transplantation**: CMV, EBV, HIV - **Close contact with cats**: toxoplasmosis

Answer 107

- An immune response to an invading pathogen - HSV gets into the sensory ganglia by travelling retrograde from skin and recurrent infection happens when it travels anterograde back to the skin. I - If it travels to the CNS, it leads to encephalitis. This is usually along olfactory or trigeminal nerves.

Answer 108

- Pyrexia - Reduced GCS - Aphasia - Hemiparesis - Cerebellar signs

Answer 109

- Fever - Headache - Fatigue - confusion

Answer 110

- Memory disturbance - Psychotic behaviour - Withdrawal or change in personality

Answer 111

- Throat swab - HIV serology - MRI of head will show evidence of inflammation will be normal in 1/3 of cases - Lumbar puncture and CSF investigation including a PCR for HSV

Answer 112

- **Meningitis** - **Encephalopathy** - **Status epilepticus** - **CNS vasculitis**

Answer 113

- **Aciclovir** should be given in all cases where it is suspected - **Ganciclovir** may be preferred in other herpesvirus infections, such as HHV-6

Answer 114

Untreated HSV encephalitis is associated with a 70% mortality. This is significantly reduced with early antiviral therapy. Survivors often have neurological sequelae such as short-term memory impairment and behavioural changes

Answer 115

An autoimmune cell-mediated demyelinating disease of the central nervous system

Answer 116

- More common in women - 20-40 most common age for diagnosis - More common in white - More common in northern latitudes Symptoms will improve in pregnancy and post-partum period

Answer 117

- **Vitamin D deficiency** - **Family history**: HLA-DR2 is implicated; 30% monozygotic twin concordance - **EBV infection**: the virus with the greatest link to MS - Smoking - Obesity

Answer 118

- T-cells get through the blood brain barrier and are activated by myelin. The T-cell then changes the BBB to allow more immune cells to get in the brain - MS is a **type IV hypersensitivity reaction**: T-cells release cytokines and these recruit more immune cells whilst also damaging the oligodendrocytes. - B-cells will make antibodies that will destroy the myelin of the e oligodendrocytes. leaving behind areas of **plaque/sclera**

Answer 119

In early disease, re-myelination can occur and symptoms can resolve. In the later stages of the disease, re-myelination is incomplete and symptoms gradually become more permanent. A characteristic feature of MS is that lesions vary in their location over time, meaning that different nerves are affected and symptoms change over time **MS lesions change location over time is that they are “disseminated in time and space”.**

Answer 120

- Relapsing-remitting: - Secondary progressive - Primary progressive - Progressive relapsing - Clinically isolated syndrome (kind of counts)

Answer 121

- The most common pattern 85% of cases - Episodic flare-ups separated by periods of remission. There isn't full recovery after flare ups so disability increases over time. 60% will develop secondary within 15 years

Answer 122

Initially, the disease starts with a relapsing-remitting course, but then symptoms get progressively worse with no periods of remission

Answer 123

- Symptoms get progressively worse from disease onset with no periods of remission - Accounts for 10% of cases and is more common in older patients

Answer 124

- One constant attack but there are bouts superimposed during which the disability increases even faster

Answer 125

- This describes the first episode of demyelination and neurological signs and symptoms. MS cannot be diagnosed on one episode as the lesions have not been “disseminated in time and space”. - Patients with clinically isolated syndrome may never have another episode or develop MS. If lesions are seen on MRI scan then they are more likely to progress to M

Answer 126

- Optic neuritis - Eye movement abnormalities- double vision VI nerve - Focal weakness (incontinence, limb paralysis, Bells palsy) - Focal sensory symptoms ( numbness, pins and needles, Lhermitte’s sign is an electric shock sensation that travels down the spine and into the limbs when flexing the neck.) - Ataxia - **Uhthoff's phenomenon**: worsening of symptoms following a rise in temperature, such as a hot bath

Answer 127

Demyelination of the optic nerve which present with: - Unilateral reduced vision - Central scotoma (enlarged blind spot) - Pain on eye movement - Impaired colour vision (red)

Answer 128

MRI of brain and spine: - Will show demyelinating plaques called **Dawson's fingers** - High signal L2 lesions - Old lesions will not enhance with contrast, whereas newer lesions will. This provides evidence of dissemination of lesions in time and space which is required for a diagnosis of MS

Answer 129

McDonald criteria

Answer 130

2 or more relapses and either: - Objective evidence of two or more lesions - Objective evidence of one and a reasonable history of a previous relapse ‘Objective evidence’ is defined as an abnormality on neurological exam, MRI or visual evoked potentials

Answer 131

- Oral or IV methylprednisolone - Plasma exchange: to remove disease-causing antibodies

Answer 132

- **Disease-modifying drugs**- don't really need to know them but just in case your feeling keen -- thanks but im not xx - **Beta-interferon**: decreases the level of inflammatory cytokines - **Monoclonal antibodies** e.g. alemtuzumab (anti-CD52) and natalizumab (anti-α4𝛃1-integrin) - **Glatiramer acetate**: immunomodulator drug which acts as a ‘decoy’ - **Fingolimod**: a sphingosine-1-phosphate receptor modulator that keeps lymphocytes in lymph nodes so they can’t cause inflammation

Answer 133

- **Genitourinary:** urinary tract infections, urinary retention and incontinence - **Constipation** - **Depression**: offer mental health support if required - **Visual impairment** - **Mobility impairment**: offer physiotherapy, orthotics and other mobility aids - **Erectile dysfunction**

Answer 134

An acute paralytic polyneuropathy. It is an autoimmune, rapidly progressive demyelinating condition of the peripheral nervous system

Answer 135

- Male - Age 15-35 and 50-75 - Malignancies - Vaccines (flu) - Infections

Answer 136

- Campylobacter jejuni (most common) - Cytomegalovirus - EBV

Answer 137

- A pathogenic antigen resembles myelin gangliosides in the peripheral nervous system. - The immune system targets the antigen and attacks the myelin sheath of sensory and motor neurones - It occurs in patches along the length of the axon so is called segmental demyelination

Answer 138

- Anti-ganglioside antibodies (anti-GMI)

Answer 139

Symptoms usually start within 4 weeks of the preceding infection. The symptoms typically start in the feet and progresses upward. Symptoms peak within 2-4 weeks, then there is a recovery period that can last months to years.

Answer 140

- Symmetrical ascending weakness (starting at feet and moving up the body) - Reduced reflexes - Loss of sensation and pain - Cranial nerve involvement such as facial nerve weakness - Autonomic features (sweating, raised pulse) - Struggling to breathe

Answer 141

A clinical diagnosis that is evidenced by progressive weakness and hyporeflexia in the weaker limbs. The **Brighton criteria** is used for diagnosis. (This does not include letting your manager go to Chelsea or selling all of your best players) (it may include being - at time of writing - 3 positions higher, 4 points better off and having 2 games in hand over Chelsea)

Answer 142

- Myasthenia gravis - Transverse myelitis - Polymyositis

Answer 143

- IV immunoglobulins IV IG - Plasma exchange (alternative to IV IG) - Venous thromboembolism prophylaxis (PE is the leading cause of death)

Answer 144

80% will fully recover 15% will be left with some neurological disability 5% will die

Answer 145

A neurodegenerative disorder characterised by the loss of dopaminergic neurons within the substantia nigra pars compacta (SNPC) of the basal ganglia.

Answer 146

Age: prevalence is 1% in 60-70 and 3% in those above 80 Gender: men are 1.5 times more likely than females to develop PD Family history

Answer 147

- In PD there is progressive loss of dopamine-producing neurons meaning there is a reduction in the amount of dopamine produced at the substantia nigra - Loss of these neurons results in reduction in action of the direct pathway and a resultant increase in the **antagonistic indirect pathway** which has a **restrictive action on movement**. Therefore **bradykinesia and rigidity are key symptoms** - There is also formation of protein clumps **Lewy bodies**

Answer 148

- Bradykinesia - Tremor - Rigidity PD symptoms usually start unilaterally and then become bilateral later in the disease course.

Answer 149

- Incontinence - Dementia - Early falls - Symmetry Can be a sign of normal pressure hydrocephalus

Answer 150

- Handwriting gets smaller - Only take small steps (**shuffling gait**) - Difficulty initiating movement - Difficulty turning around when standing - Reduced facial movements and expressions

Answer 151

- A unilateral **resting** tremor. Described as pill rolling tremor looks like they are rolling pill between thumb and finger - The tremor is worse at resting and when they are distracted like using the other hand Frequency of 4-6 times a second

Answer 152

- If you take their hand and passively flex and extend their arm at the elbow you will feel tension in their arm that gives way to movement in small increments (little jerks) - Described as **cogwheel**

Answer 153

- Depression - Sleep disturbance and insomnia no REM - Loss of sense of smell - Postural instability - Cognitive impairment and memory problems

Answer 154

- PD= asymmetrical BET= symmetrical - PD= frequency= 4-6 BET= 5-8 - PD= worse at rest BET= better at rest - PD= improves with intentional movement BET= worse - PD= no change with alcohol BET= better with alcohol

Answer 155

- Beta blocker (propranolol) - Primidone

Answer 156

PD is a clinical diagnosis and should be suspected in a patient with **bradykinesia** and at least one of the following: - Tremor - Rigidity - Postural instability

Answer 157

Motor symptoms not affecting quality of life: A choice of one of the following: **Dopamine agonist** (non-ergot derived) - Pramipexole, ropinirole **Monoamine oxidase B inhibitor** (MOA-B) - Selegiline, rasagiline - Stop breakdown of circulating dopamine Motor symptoms affecting the quality of life: - Synthetic dopamine **levodopa** given with a drug that stops it being broken down. These are **peripheral decarboxylase inhibitors**. Carbidopa and benserazide. Co-benyldopa (levodopa and benserazide) Co-careldopa (levodopa and carbidopa)

Answer 158

PD is a chronic and progressive condition with no cure. Overall, life expectancy is reduced with the mortality being 2-5 times higher for those aged 70-89 years old. Also, the risk of dementia is up to 6 times higher in PD patients.

Answer 159

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

Answer 160

- It is a **trinucleotide repeat disorder** that involves a genetic mutation in the HTT gene on **chromosome 4** - There is a repeat of CAG which codes for glutamine **36 times in a row** so patients have 36 glutamine in a row on the Huntington protein - These mutated proteins aggregate within neuronal cells of the caudate and putamen causing neuronal cell death. This leads to decreased ACh and GABA synthesis. This leads to **dopamine increase** leading to excessive movement

Answer 161

- It is a feature of trinucleotide repeat disorders. 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. This leads to successive generations having more repeats in the gene resulting in: - Early age of onset - Increased severity of the disease

Answer 162

Patients will be asymptomatic until 30-50. Symptoms: - Begin with Cognitive, psychiatric or mood problems - Chorea (involuntary, abnormal movements) - Eye movement disorders - Dysarthria: speech difficulties - Dysphagia: swallowing difficulties - Dementia

Answer 163

Made at a specialist genetic centre that looks for the number of CAG repeats. This will involve pre and post test counselling

Answer 164

There are currently no treatment options for slowing or stopping the progression of the disease. The key to management of the condition is supporting the person and their family. Effectively breaking bad news 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 Genetic counselling regarding relatives, pregnancy and children Advanced directives to document the patients wishes as the disease progresses End of life care planning

Answer 165

Medications that can suppress the disordered movement: Antipsychotics (e.g. olanzapine) Benzodiazepines (e.g. diazepam) Dopamine-depleting agents (e.g. tetrabenazine)

Answer 166

Life expectancy is around 15-20 years after the onset of symptoms. As the disease progresses patients become more susceptible and less able to fight off illnesses. Death is often due to respiratory disease (e.g. pneumonia). Suicide is a more common cause of death than in the general population.

Answer 167

- Most common form of dementia - More than 500,000 people with it in the UK - More common in women

Answer 168

Age CVD Depression Low educational attainment - Low social engagement and support - head trauma, learning difficulties

Answer 169

- <5% are inherited so the familial but genes such as **APP and presenilin genes (PSEN1, PSEN2)** have been identified as causes. - APP is on chromosome 21 so people with down syndrome have an increased risk - 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)

Answer 170

- **Senile plaques** deposits of **beta amyloid** aggregate. Amyloid precursor protein is found on neurons (helps it to grow and repair after injury). - If it is broken down normally by **alpha secretase and gamma secretase** it is soluble and easy to remove - However if it is broken by **beta secretase** the leftover fragment isn't soluble and creates a monomer called beat amyloid. These are sticky and can in between neurones and their signalling, They also increase inflammation which damages surrounding neurones

Answer 171

- Neurons are held together by their cytoskeleton which is made up of microtubules a protein called **tau** makes sure they stay together. - Beta amyloid build-up outside the neurones initiates pathways which leads to the activation of kinase. This leads to the **phosphorylation of tau** - The tau protein then changes shape, stops supporting the microtubules, and clumps up with other tau proteins, forming neurofibrillary tangles - Neurones with tangles and non-functioning microtubules can’t signal as well, and sometimes end up undergoing apoptosis. As neurones die, the brain starts to atrophy.

Answer 172

- **Agnosia**- can't recognise things - **Apraxia** can't do basic motor skills - **Aphasia** speech difficulties Cognitive impairment Agitation and emotional lability Depression and anxiety Sleep cycle disturbance: Motor disturbance: wandering is a typical feature of dementia

Answer 173

**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)

Answer 174

- Functional ability: inability to carry out normal functions - Impairment in **2 or more cognitive domains** - **Differentials excluded**

Answer 175

CT/MRI: exclude other diagnosis and can help determine type of dementia; will show **medial temporal lobe atrophy**

Answer 176

- Brain biopsy after autopsy

Answer 177

- Mini mental state examination (MMSE) - Montreal cognitive assessment scale (MoCA)

Answer 178

- Attention and concentration - Recent and remote memory - Language - Praxis (planned motor movements) - Executive function - Visuospatial function

Answer 179

- **Mild**: MMSE 21-26, MoCA 18-25, CDR 1 - **Moderate**: MMSE 10-20, MoCA 10-17, CDR 2 - **Severe**: MMSE <10, MoCA <10, CDR 3

Answer 180

- Non-pharmacological: programmes to improve/maintain cognitive function - Mild to moderate AD: acetylcholinesterase inhibitors e.g., **Donepezil and rivastigmine** - Moderate to severe AD: N-methyl-d-aspartic acid receptor antagonist memantine

Answer 181

A neurodegenerative disorder characterised by focal degeneration of the frontal and temporal lobes. It is a heterogenous condition with various subtypes e.g., Pick's disease

Answer 182

- It is an uncommon cause of dementia (2% of dementias) - Typically affects patients at a younger age (< 65 years)

Answer 183

**C9ORF72 gene**: found on chromosome 9. Most common genetic cause of inherited FTD. Also implicated in hereditary motor neuron disease. - **MAPT (microtubule associated protein)**- found on chromosome 17 - **Granulin precursor (GRN)**: found on chromosome 17.

Answer 184

- In Pick disease 3R isoforms of the tau protein become hyperphosphorylated. This means they change shape and stop being able to tie together the tubulins in the neurones cytoskeleton. - These proteins start to clump together forming tangles of tau proteins known as **pick bodies**

Answer 185

Personality and behaviour change: - Disinhibition - Loss of empathy - Apathy - Hyperorality (e.g. dietary changes, attempt to consume non-edible products, eat beyond satiety) - Compulsive behaviour (e.g. cleaning, checking, hoarding)

Answer 186

Language problems: - Effortful speech - Halting speech - Speech sound errors - Speech apraxia - Word-finding difficulty - Surface dyslexia or dysgraphia: mispronouncing difficult words (e.g. yacht)

Answer 187

- 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 188

- **Serotonin reuptake inhibitors (SSRI)**: used for difficult behavioural symptoms. Have been shown to decrease disinhibition, anxiety, impulsivity and repetitive behaviours. - **Atypical anti-psychotics**: can help with agitation and behavioural symptoms.

Answer 189

Overall survival is 8-10 years from symptoms onset.

Answer 190

- Makes up 20% of dementias - It is the second most common form

Answer 191

- Ischaemic stroke - Small vessel disease - Haemorrhage - Other: cerebral amyloid, which is a cause of small vessel disease. Deposition of amyloid in small arteries. CADASIL, which is due to mutation in the NOTCH3 gene and leads to arterial thickening and occlusion.

Answer 192

- The brain uses around 20-25% of oxygen - Neurons can only function in aerobic conditions and don't have long term energy stores so need a constant supply

Answer 193

- Once blood supply to the brain falls below the demands to the tissue, it' considered an ischaemic stroke. The tissue damage is **permanent** because the dead tissue liquifies in a process called **liquefactive necrosis** - Brain tissue necrosis leads to a loss of mental function in the area where the loss has occurred

Answer 194

- They appear suddenly and the brain function decline is step-wise e.g. it decreases with each stroke - Symptoms will vary depending on which vessels are affected in stroke/atherosclerosis

Answer 195

A type of dementia characterised by fluctuating cognitive impairment, visual hallucinations and parkinsonism

Answer 196

- Neurons contain a protein called **alpha synuclein** and in LBD this proteins is misfolded within the neurons - This misfolded protein aggregates to form Lewy bodies that deposit inside the neurones particularly in the **cortex and the substantia nigra**.

Answer 197

- Parkinson's - Multiple system atrophy

Answer 198

- Alzheimer's like cognitive ones: - **Difficulty focusing** - **Poor memory** - **Visual hallucinations** - **Disorganized speech** - **Depression**

Answer 199

- 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 200

- **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 symptoms

Answer 201

Antipsychotics as they can have harmful side effects

Answer 202

No underlying cause relevant to the headache: - Migraine - Cluster - Tension - (Trigeminal Neuralgia)

Answer 203

- Sudden-onset reaching worse severity within 5 minuets (subarachnoid haemorrhage) - New-onset over 50 (GCA/ space occupying lesion) - Progressive/persistent headache or one that has changed dramatically (space-occupying lesion/ subdural haematoma)

Answer 204

- Recent head trauma within 3 months (subdural haematoma) - Headache worse lying down (raised ICP) - Headache worse on standing (CSF leak) - Household contacts with similar symptoms (CO poisoning)

Answer 205

- Fever, photophobia or neck stiffness (meningitis or encephalitis) - New neurological defect (raised ICP/stroke) - Visual disturbance (GCA/ acute closure glaucoma) - Vomiting (raised ICP, brain abscess and CO poisoning)

Answer 206

- Fever, photophobia or neck stiffness (meningitis or encephalitis) - New neurological symptoms (haemorrhage, malignancy or stroke) - Dizziness (stroke) - Visual disturbance (temporal arteritis or glaucoma) - Sudden onset occipital headache (subarachnoid haemorrhage) - Worse on coughing or straining (raised intracranial pressure) - Postural, worse on standing, lying or bending over (raised intracranial pressure) - Severe enough to wake the patient from sleep - Vomiting (raised intracranial pressure or carbon monoxide poisoning) - History of trauma (intracranial haemorrhage) - Pregnancy (pre-eclampsia)

Answer 207

**fundoscopy** which will look for **papilledema** which indicates a raised intercranial pressure

Answer 208

- Family history - Female gender 3 times more common - Obesity - Triggers

Answer 209

**CH**- Chocolate **OC**- Oral contraceptive **OL**- alcohOL **A**- anxiety **T**- travel **E**- exercise CHOCOLATE Other triggers can be red wine, bright lights and menstruation

Answer 210

- Migraine without aura - Migraine with aura - Silent migraine (just the aura without the headache) - Hemiplegic migraine

Answer 211

- Headache is thought to be due to neuronal hyperexcitability. This leads to trigeminal nerves initiating an inflammatory response with dilation of meningeal blood vessels and sensitisation of surrounding nerve fibres leading to pain - Aura is thought to occur due to cortical spreading depression which is a propagating wave of depolarisation across the cerebral cortex causing the brain to become hypersensitive to certain stimuli

Answer 212

Last between 4-72 hours: - Pounding or throbbing in nature - Usually unilateral (can be bilateral more common in children) - Photophobia - Phonophobia (loud noises) - Aura - Nausea and vomiting

Answer 213

Aura is the term used to describe the visual changes associated with migraines symptoms can be: - Sparks in vision - Blurring vision - Line across vision - Loss of different visual fields

Answer 214

They can mimic a stroke. Need to rule out if patient has symptoms: - typical migraine - Sudden onset - Hemiplegia - Ataxia - Change in consciousness

Answer 215

- **Prodromal stage** subtle symptoms such as yawning, fatigue or mood changes - **Aura** - **headache** - **Resolution** the headache can fade away and be relieved by vomiting or sleeping - **Postdromal stage**

Answer 216

At least two headaches filling criteria shown in picture

Answer 217

At least five headaches filling criteria shown in picture

Answer 218

- Analgesia (avoid opioids) - Oral triptan ( 500mg sumatriptan) as the headache starts - Antiemetics metoclopramide

Answer 219

They **5HT (serotonin) receptor agonists** and they cause: - smooth muscle contraction in arteries - Peripheral pain receptors to inhibit activation of pain receptors - Reduce neuronal activity in the central nervous system

Answer 220

- Propranolol - Topiramate (don't use in pregnancy as it is teratogenic and can cause cleft lip) patient must be fitted with coil if age where they can get pregnant - Amitriptyline

Answer 221

The combined pill it increases the risk of a stroke

Answer 222

- **Acupuncture**: if both propranolol and topiramate are ineffective or unsuitable - **Riboflavin (vitamin B2)**: **may be effective in some people, but avoid in pregnancy

Answer 223

- A classical syndrome of painless short-lived monocular blindness. Is mainly caused by transient obstruction e.g. an emboli but can be caused by GCA - Often described as a black curtain coming across the vision.

Answer 224

The most common form of a primary headahce

Answer 225

Between L3/L4 Since spinal cord ends L1/2

Answer 226

Most common primary headache Can be episodic (<15 days/month) or chronic (>15 days a month for at least 3 months)

Answer 227

Missed meals Stress Overexertion Lack of sleep Depression

Answer 228

- Bilateral with a pressing/tight sensation of mild-moderate intensity - Nausea or vomiting - Photophobia - Phonophobia

Answer 229

Severe unilateral headaches often periorbital that come in clusters of attacks

Answer 230

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 231

Patient may suffer 3 – 4 attacks a day for weeks or months followed by a pain-free period lasting 1-2 years. Attacks last between 15 minutes and 3 hours.

Answer 232

- Severe and intolerable pain - unilateral - Red swollen watering eye - Pupil constriction - Eyelid dropping - Nasal discharge - Facial sweating

Answer 233

- Triptans (6mg sumatriptan subcut) - High flow oxygen 100%

Answer 234

Verapamil (CCB) Lithium Prednisolone (a short course for 2-3 weeks to break the cycle during clusters)

Answer 235

- females - 50-60 - increases with age - unilateral - MS

Answer 236

Normally due to compression of the trigeminal nerve by a vascular loop often the superior cerebellar artery

Answer 237

- Light touch - Washing - Shaving - Talking - Cold weather

Answer 238

Electric Shock Pain that lasts for seconds to minutes across the face 90% unilateral 10% bilateral

Answer 239

Clinical Dx 3 or more attacks with characteristic unilateral facial pain and Symptoms MRI - exclude secondary causes/other pathology

Answer 240

-First line: Carbamazepine - Second line: microvascular decompression or ablative surgery may be considered in refractory patients

Answer 241

A paroxysmal alteration of neurological function as a result of excessive hypersynchronous discharge of neurons within the brain

Answer 242

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

Answer 243

VITAMIN DE - Vascular - Infection - Trauma - Autoimmune- SLE - Metabolic - Idiopathic - Neoplasms - Dementia and drugs (cocaine) - Eclampsia

Answer 244

- Genetic - Structural - Metabolic- visible neurological abnormalities that predispose to seizures (e.g. chronic cerebrovascular disease, congenital malformation) - Immune - Infectious- a chronic one e.g HIV - Unknown

Answer 245

- Clusters of neurons in the brain become temporarily impaired and start sending put lots of excitatory signals (said to be paroxysmal which means rapid onset). Happen either due to too much excitation glutamate or nor enough inhibition GABA - It is often noticed by obvious outward signs like jerking, moving and losing consciousness but can also be subjective and only noticed by the person experiencing it like fears or strange smells

Answer 246

- **Generalised**:when both hemispheres are affected **always a loss of consciousness** - **Focal** : when the affected area is limited to one half of the brain or sometimes even smaller like a single lobe can progress to bilateral

Answer 247

- Tonic - Atonic - Clonic - Tonic-clonic - Myoclonic - Absence

Answer 248

Simple (without impaired awareness) Complex (with impaired awareness)

Answer 249

- **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 250

- Tonic seizure: the muscles become stiff and flexed which will cause the patients to **fall backwards** - Clonic seizures: violent muscle contractions (convulsions) Tonic-clonic: there is loss of consciousness and tonic (muscle tensing) and clonic (muscle jerking episodes). Typically the tonic phase comes before the clonic phase. (tongue biting, incontinence, groaning and irregular breathing. After the seizure there is a prolonged post-ictal period where the person is confused, drowsy and feels irritable or depressed.

Answer 251

First line: **sodium valproate** Second line: **Lamotrigine** or **carbamazepine**

Answer 252

Known as **drop attacks**. This is where the muscles suddenly relax and become floppy which can cause the patient to fall **usually forward**. They don't usually last longer than 3 minuets. They typically begin in childhood. They may be indicative of **Lennox-Gastaut syndrome**.

Answer 253

First line: **sodium valproate** Second line: **Lamotrigine**

Answer 254

- They present as sudden brief muscle contractions like a sudden jump. The patient usually remains awake during the episode. - They occur in various forms of epilepsy but typically happen in children as part of juvenile myoclonic epilepsy.

Answer 255

First line: sodium valproate Other options: lamotrigine, levetiracetam or topiramate

Answer 256

- 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**. - Common in children. Most patients (> 90%) stop having absence seizures as they get older.

Answer 257

First line: sodium valproate or ethosuximide

Answer 258

Known as **West syndrome**. It is a rare (1 in 4000) disorder starting in infancy at around 6 months of age. It is characterised by clusters of full body spasms. There is a poor prognosis: 1/3 die by age 25, however 1/3 are seizure free.

Answer 259

Prednisolone Vigabatrin

Answer 260

- No loss of consciousness - The patient is aware and awake - Will have uncontrollable muscle jerking

Answer 261

- There is loss of consciousness - Patient is unaware

Answer 262

Temporal lobe

Answer 263

They affect hearing, speech, memory and emotions: - Hallucinations - Memory flashbacks - Déjà vu - Doing strange things on autopilot Can also include audio symptoms such as buzzing, ringing and vertigo

Answer 264

- Motor symptoms: pelvic thrusting, bicycling and tonic posturing - Bizarre behaviour - Vocalisations - Sexual automatisms

Answer 265

- Paraesthesia - Visual hallucinations - Visual illusions More subjective and difficult to diagnose than other areas

Answer 266

- Visual hallucinations - Transient blindness - Rapid and forced blinking - Movement of head or eyes to the opposite side

Answer 267

Focal to bilateral tonic-clonic: a focal seizure may spread to affect a wider network of neurons involving both hemispheres. Traditionally termed a secondary generalised seizure.

Answer 268

Opposite to generalised: First line: **Carbamazepine** Second line: **sodium valproate**

Answer 269

- Must have had 2 or more seizures more than 24 hours apart - MRI/CT: examine the hippocampus look for underlying cause - EEG: 3H2 wave absence - Bloods: rule out metabolic/infection

Answer 270

It works by increasing the activity of GABA which has a relaxing effect on the brain: - **Teratogenic don't give to females of child bearing age** - Liver damage - Hair loss - Tremor

Answer 271

- **Carbamazepine** - Sodium channel blocker; prevents repetitive and sustained firing of action potentials. - Agranulocytosis - Aplastic anaemia - Induces the P450 system so there are many drug interactions

Answer 272

**Status Epilepticus**

Answer 273

ABCDE Give IV **lorazepam 4mg** and repeat 10 minuets after if it doesn't work If seizure persist then give IV **phenobarbital or phenytoin**

Answer 274

**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 **If the above conditions are not met or the patient has known epilepsy:** - Patients must be seizure-free for 12 months before they may qualify for a driving license - If seizure-free for 5 years, a 'til 70 licence is usually reinstated

Answer 275

Results form processes that compress or displace arterial, venous and cerebrospinal fluid spaces as well as the cord itself.

Answer 276

Metastatic cancer lesions

Answer 277

- Breast - Lung - Prostate - Thyroid - Kidney - Myeloma - Lymphoma

Answer 278

- Thoracic 60% - Lumbar 30% - Cervical 10%

Answer 279

- Disc herniation - Disc prolapse - Primary spinal cord tumour - Infection - Haematoma

Answer 280

- Back pain - Progressive weakness of legs with UMN signs - **Sensory loss 1-2 cord segments** below level of lesion - **UMN signs below level of lesion** - **LWN signs at the level of the lesion**

Answer 281

Bladder and anal sphincter involvement: hesitancy, frequency and painless retention

Answer 282

Loss of all motor and sensory function below the level

Answer 283

Disruption of the anterior spinal cord: - Loss of motor function below the level - Loss of pain and temperature sensation - Preservation of fine touch and proprioception

Answer 284

Disruption of posterior spinal cord or posterior spinal artery (rare) - Loss of fine touch and proprioception (posterior column) - Preservation of pain and temperature sensation (anterior column)

Answer 285

Hemi section of spinal cord: - Ipsilateral paralysis - Ipsilateral loss of vibration and proprioception - Contralateral loss of pain and temperature

Answer 286

- MRI of spinal cord - Biopsy/surgical exploration

Answer 287

Surgical decompression - Laminectomy: removal of the lamina/spongy tissue between the discs to relieve pressure - Microdiscectomy: removal of the herniated tissue from the disc

Answer 288

Surgical decompression - Laminectomy: removal of the lamina/spongy tissue between the discs to relieve pressure - Microdiscectomy: removal of the herniated tissue from the disc

Answer 289

L4/S3 lesion Sensory loss and pain in the **back of the thigh** and **lateral aspect of little toe**

Answer 290

Common peroneal Tibial

Answer 291

- Unilateral pain from the buttock radiating down the back of the thigh to below the knee or feet - Paraesthesia numbness and motor weakness

Answer 292

- Herniated/prolapsed disc - Tumours - Spinal stenosis - Piriformis syndrome

Answer 293

Cauda equina syndrome

Answer 294

Physiotherapy + Analgesia: Amitriptyline Duloxetine

Answer 295

It is a neurosurgical emergency which occurs when the bundle of nerves below the end of the spinal cord are compressed

Answer 296

- **Lumbar disc herniation**: the most common cause of CES - **Trauma** - **Spinal tumour** - **Lumbar spinal stenosis**: narrowing of the spinal cord may result in CES. This can be congenital or acquired e.g. spinal osteoarthritis (spondylosis), rheumatoid arthritis, and a slipped vertebra (spondylolisthesis) - **Epidural abscess or haematoma**

Answer 297

- Saddle anaesthesia (loss of sensation in the perineum) can you feel wiping after poo - Loss of sensation in bladder and rectum (not knowing when they're full) - Urinary retention or incontinence - Faecal incontinence

Answer 298

- Examinations: PR exam, knee and ankle reflexes - MRI spine is gold standard - Bladder ultrasound: to determine whether there us urinary retention

Answer 299

Emergency decompressive laminectomy: surgery should be performed within 24-48 hours of symptom onset. The incidence of thromboemboli in patients with CES is remarkably high, therefore patients require adequate thromboprophylaxis

Answer 300

Conus medullaris

Answer 301

- Ptosis - **Down and out eye** - Fixed a dilated pupil

Answer 302

- Raised ICP - Diabetes - Hypertension - GCA

Answer 303

- Diplopia when looking **down**. Compensation by tilting head away from the affected side

Answer 304

- Trauma to the orbit - Diabetes - Infarction secondary to hypertension

Answer 305

- Weakness of jaw (jaw deviates to weak side when mouth is open - Loss of corneal reflex

Answer 306

- Trigeminal neuralgia - HSV - Nasopharyngeal carcinoma

Answer 307

- Bulbar palsy - Acoustic neuroma

Answer 308

- Bulbar palsy - Acoustic neuroma

Answer 309

Adducted eye the inability to look laterally

Answer 310

- Raised ICP - MS - Pontine stroke

Answer 311

- Unilateral facial weakness (motor) - Altered taste (sensory) - Dry mouth (parasympathetic)

Answer 312

- Bell's palsy - Fractures to pteroid bone - Parotid inflammation - Otitis media

Answer 313

Neurological condition that presents with rapid onset of unilateral facial paralysis

Answer 314

The lower half of the faces only has contralateral innervation Top half has bilateral. forehead sparing

Answer 315

UMN injured: means the contralateral side is weak but the forehead is not LMN - weakness of all the muscles on the ipsilateral side of the face

Answer 316

- Hearing impairment - Vertigo - Loss of balance

Answer 317

- Skull fracture - Otitis media - Tumours - Compression

Answer 318

- Gag reflex issues - Swallowing issues - Vocal issues- hoarse voice

Answer 319

Jugular foramen lesion

Answer 320

Can't shrug shoulders/turn head against resistance

Answer 321

Tongue deviation towards the side of the lesion

Answer 322

Motor neurone disease is an umbrella term that encompasses a variety of specific diagnoses. They are neurodegenerative diseases that affect both upper and lower motor neurones but sensory neurons are spared

Answer 323

Amyotrophic lateral sclerosis (ALS) accounts for 50% of cases

Answer 324

- **Progressive muscular atrophy**: LMN only - **Primary lateral sclerosis**: UMN only - **Progressive bulbar palsy**: affects muscles of talking and swallowing (second most common) LMN only

Answer 325

- Increasing age (over 60) - Male - Pesticides - Heavy metals - Rugby (unlucky louis)

Answer 326

- Progressive weakness of the muscles throughout the body affecting the limbs, trunk, face and speech. - The weakness is often first noticed in the upper limbs. There may be increased fatigue when exercising. - They may complain of clumsiness, dropping things more often or tripping over. They can develop slurred speech (dysarthria).

Answer 327

- Muscle wasting - Reduced tone - Reduced reflex - Fasciculations - Loss of power - Babinski reflex negative

Answer 328

- Increased tone - Brisk reflexes - Rigidity + spasticity - Babinski reflex positive

Answer 329

- Eye muscles - Sensory function and sphincters

Answer 330

- Progressive weakness - Fatigue - Falls - Speech and swallow issues

Answer 331

**MND is a clinical diagnosis** - **Electromyography**: in MND there will be evidence of fibrillation potentials - - **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

Answer 332

- MS - Polyneuropathies - Myasthenia gravis - GBS

Answer 333

- **Riluzole** prolongs survival by 2-4 months by protecting motor neuron damage form glutamate - **Respiratory support:** patients with reduced FVC can use non-invasive ventilation at home, usually BiPAP; prolongs survival by 7 months - **Supportive treatment:** - **Antispasmodics:** such as baclofen

Answer 334

A chronic autoimmune disorder of the **postsynaptic membrane** at the neuromuscular junction of skeletal muscle

Answer 335

- Symptoms peak in women in 20/30s - Symptoms peak in men 50/60s

Answer 336

- Female 2x as common - Autoimmune: linked to rheumatoid and SLE -**Thymoma or thymic hyperplasia**: 10-15% have a thymoma and 70% have thymic hyperplasia

Answer 337

- Axons of motor nerves are situated across a synapse from from the post-synaptic membrane on the muscle cell - The axons release a neurotransmitter from the pre-synaptic membrane. This neurotransmitter is **acetylcholine**. - **ACh** travels across the synapse and attaches to **nicotinic** receptors on the post-synaptic membrane stimulating muscle contraction

Answer 338

- In around 85% of patients there are **acetylcholine receptor antibodies present** - These bind to the receptors on the post-synaptic membrane and prevent ACh from binding and causing muscle contraction - The problem is worsened during exercise as more of the receptors become blocked up. So the more the muscles are used the weaker they get. Gets better with rest These antibodies also activate the **complement system** which damages cells further making the problem worse

Answer 339

- **Muscle specific kinase (MuSK)** - **low-density lipoprotein receptor-related protein 4 (LRP4).** They are both proteins that are important for making the acetylcholine receptor. These antibodies lead to inadequate acetylcholine receptors causing MG

Answer 340

- Proximal muscle weakness: often affecting the face and neck - Ptosis (drooping eyelid) - **Complex ophthalmoplegia:** cannot be isolated to one cranial nerve - **Head drop:** a rare sign due to weakness of cervical extensor muscles - **Myasthenic snarl:** a ‘snarling’ expression when attempting to smile

Answer 341

- Muscle weakness that gets worse throughout the day - Diplopia: double vision - Slurred speech - Fatigue in jaw while chewing

Answer 342

Look for presence of autoantibodies: - Acetylcholine receptor (ACh-R) antibodies (85% of patients) - Muscle-specific kinase (MuSK) antibodies (10% of patients) - LRP4 (low-density lipoprotein receptor-related protein 4) antibodies (less than 5%) CT/MRI of thymus gland to look for thymoma

Answer 343

**Edrophonium test**: patients given IV Edrophonium chloride (neostigmine). Will prevent breakdown of ACh by cholinesterase enzymes and improve symptoms temporarily

Answer 344

First-line: **Reversible acetylcholinesterase inhibitors** (neostigmine/pyridostigmine) Second-line: Immunosuppressants: prednisolone/azathioprine Consider monoclonal antibodies (rituximab) Thymectomy can also improve symptoms.

Answer 345

- Myasthenic crisis often triggered by another illness such as respiratory tract infection . Causes an acute worsening of symptoms and can lead to **respiratory failure**

Answer 346

- Patients may require **non-invasive ventilation with BiPAP or full intubation and ventilation.** Medical treatment of myasthenic crisis is with immunomodulatory therapies such as **IV immunoglobulins and plasma exchange**.

Answer 347

Lambert-Eaton myasthenic syndrome

Answer 348

Lambert-Eaton myasthenic syndrome has a similar set of features to myasthenia gravis. It causes progressive muscle weakness with increased use as a result of damage to the neuromuscular junction. The symptoms tend to be more insidious and less pronounced than in myasthenia gravis.

Answer 349

- Typically occurs in patients with **SCLC**. It is a result of antibodies produced by the immune system against **voltage gated calcium channels** in SCLC but this also targets the pre-synaptic terminal - These channels are responsible for assisting the release of ACh at the synapse

Answer 350

- Proximal muscle weakness that develops more slowly - All other same symptoms really e.g., double vision ptosis, jaw tiredness, slurred speech

Answer 351

Lambert Eaton Syndrome symptoms tend to improve following a period of strong muscle contraction. Post Tetanic Potentiation

Answer 352

- **Amifampridine** allows more ACh to be released in the neuromuscular junction synapses by blocking **voltage gated potassium channels**

Answer 353

- Immunosuppressants (e.g. prednisolone or azathioprine) - IV immunoglobulins - Plasmapheresis

Answer 354

A term used to describe the event of temporarily losing consciousness due to **disruption in blood flow**. Known as vasovagal episodes

Answer 355

- Dehydration - Missed meals - Extended standing in warm environment - Vasovagal response to stiumli

Answer 356

- Hypoglycaemia - Anaemia - Hypovolaemia e.g. due to haemorrhage, GI bleeding, ruptured aortic aneurysm - Infection - Anaphylaxis - Arrhythmias - Valvular heart disease - Hypertrophic obstructive cardiomyopathy - Pulmonary embolism: causing hypoxia

Answer 357

- Elderly - Pregnant women - Certain medications such as - 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

Answer 358

- Caused by problems with the autonomic nervous system regulating blood flow to the brain - When the vagus nerve receives strong stimulus it causes an increase in parasympathetic activation which causes vasodilation and reduction in BP and blood flow to brain leading to the loss of consciousness

Answer 359

This occurs when mild external pressure on the carotid bodies in the neck is enough to induce this reflex response (vagal stimulation). Mild pressure could be due to shaving, neck turning, tight collar etc

Answer 360

- A drop of blood pressure of more than 20mmHg or a reflex tachycardia of more than 20 beats. When a person goes from lying down to standing - 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.

Answer 361

- **Hot or clammy** - **Sweaty** - **Heavy** - **Dizzy or lightheaded** - **Vision going blurry or dark** - **Headache**

Answer 362

- Suddenly losing consciousness and falling to the ground - Unconscious on the ground for a few seconds to a minute as blood returns to their brain - Twitching, shaking or convulsion activity, which can be confused with a seizure

Answer 363

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

Answer 364

A process of nerve damage that affects a single nerve

Answer 365

Disorders of peripheral or cranial nerves whose distribution is usually symmetrical and widespread

Answer 366

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

Answer 367

WARDS PLC - Wegner's granulomatosis - AIDS - Rheumatoid arthritis - Diabetes - Sarcoidosis - Polyarteritis nodosa - Leprosy - Carcinoma

Answer 368

Nerve pathology outside of the CNS that affects the peripheral nerves

Answer 369

- Demyelination - Axonal damage - Nerve compression

Answer 370

ABCDE - A- Alcohol - B- B12 deficiency - C- Cancer and CKD - D- Diabetes and drugs - E- Every vasculitis

Answer 371

amiodarone isoniazid vincristine nitrofurantoin metronidazole

Answer 372

numbness and tingling in the feet or hands burning, stabbing or shooting pain in affected areas loss of balance and co-ordination muscle weakness, especially in the fee

Answer 373

Compression of the median nerve as it travels through the carap tunnel in the wrist

Answer 374

- Swelling of the contents e.g. swelling of tendons putting pressure on nerve - Narrowing of the tunnel

Answer 375

Females due to narrower wrists so more likely to have compression Over 30s

Answer 376

The **palmar aspects and full fingertips** of the: - Thumb - Index and middle finger - The lateral half of the ring finger Note that the palmar cutaneous branch of the median nerve provides sensation to the palm. However, this branch originates before the carpal tunnel and does not travel through the carpal tunnel. Therefore, it is not affected by carpal tunnel syndrome.

Answer 377

The three **thenar muscles** which make up the muscular budge at the base of the thumb: - **Abductor pollicis brevis** (thumb abduction) - **Opponens pollicis** - **Flexor pollicis brevis** (thumb flexion) The other muscle that controls thumb movement is the adductor pollicis (thumb adduction). However, this is innervated by the ulnar nerve.

Answer 378

Most cases are **idiopathic** but can be caused by: - Repetitive strain - Obesity - Perimenopause - RA - Diabetes - Acromegaly - Hypothyroidism If it is bilateral then think underlying cause

Answer 379

- Numbness - Paraesthesia (pins and needles or tingling) - Burning sensation - Pain worse at night wakes from sleep This occurs in areas innervated so most of the palmar aspects of everything but little finger. Patients may shake hand to relieve pain

Answer 380

- Weakness of thumb movements - Weakness of grip strength - Difficulty with fine movements involving the thumb - Weakness of the thenar muscles

Answer 381

- Phalen’s test - Tinel’s test - Durkan’s test

Answer 382

- Involves fully flexing the wrist and holding it in this position. Often this is done by asking the patient to put the backs of their hands together in front of them with the wrists bent inwards at 90 degrees. - The test is positive when this position triggers the sensory symptoms of carpal tunnel, with numbness and paraesthesia in the median nerve distribution.

Answer 383

- Involves tapping the wrist at the location where the median nerve travels through the carpal tunnel. This is in the middle, at the point where the wrist meets the hand. - The test is positive when this position triggers the sensory symptoms of carpal tunnel, with numbness and paraesthesia in the median nerve distribution.

Answer 384

- 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 (last resort)

Answer 385

Females due to narrower wrists so more likely to have compression Over 30s

Answer 386

Wrist drop

Answer 387

Damage to the radial nerve e.g. mid shaft fracture of humerus or compression of radial nerve at humerus

Answer 388

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

Answer 389

A group of diseases that affect the peripheral motor and sensory nerves

Answer 390

CMT1 and CMT2

Answer 391

Autosomal dominant

Answer 392

- Caused by mutations in the **PMP22 and MPZ** genes which encode proteins that are part of the myelin sheath made by Schwan cells. - Loss of myelin slows down the transmission of nerve impulses. Over time Schwann cells try and replace myelin. - As a result, under a microscope, there’s often onion bulb formation - the axon is surrounded by layers of new myelin with underlying damaged layers of myelin.

Answer 393

- Caused by mutations in **MFN2** gene which encodes for a protein in neuronal mitochondria. - When the protein is defective mitochondrial function is disrupted leading to neuronal death

Answer 394

- When motor neurones are affected muscles begin to atrophy. - When sensory neurones are affected it first affects the feet and toes as those are the longest axons and most sensitive to damage

Answer 395

- **Pes cavus**: high foot arches - Hammer toes - Distal muscle wasting - Hand and arm muscle wasting - Thickened palpable nerves

Answer 396

- **Weakness in the lower legs, particularly loss of ankle dorsiflexion** - **Foot drop** - **High-stepped gait** - **Weakness in the hands** - **Reduced tendon reflexes** - **Reduced muscle tone** - **Peripheral sensory loss** - **Tingling and burning sensations in the hands and feet** - **May be neuropathic pain**

Answer 397

- Nerve conduction studies: measures ability of nerves to conduct impulses - Neurologists and geneticists make the diagnosis by looking for mutations

Answer 398

A genetic condition that cause gradual weakening and wasting of muscles.

Answer 399

X-linked recessive

Answer 400

- There is a mutation which codes for the **dystrophin** protein which is used to stabilise the muscle fibres. Without it the sarcolemma wilts and becomes unstable. - Overtime cellular proteins e.g. creatinine kinase starts escaping the damaged cell and calcium starts to enter the cell leading to its death - In the short term there is muscle regeneration resulting in muscle fibres of different sizes but in the long term the muscles atrophy and are infiltrated by fat and fibrotic tissue making them really weak

Answer 401

- Biopsy of the tissue show changes in the muscle itself but not in the nerves which is used to distinguish it between neuropathies

Answer 402

Symptoms usually present at around **3-5**: - Weakness is muscles around pelvis - Walking late - Waddling gait - Enlarged calves

Answer 403

Children with proximal muscle weakness use a specific technique to stand up from a lying position. This is called Gower’s sign. To stand up, they get onto their hands and knees, then push their hips up and backwards like the “downward dog” yoga pose. They then shift their weight backwards and transfer their hands to their knees. Whilst keeping their legs mostly straight they walk their hands up their legs to get their upper body erect. This is because the muscles around the pelvis are not strong enough to get their upper body erect without the help of their arms.

Answer 404

- High **creatinine kinase** level - Genetic testing looking for dystrophin mutations (can be diagnostic) - Muscle biopsy - Electromyogram: distinguish between neuropathic and myopathic pathology

Answer 405

- Occupational therapy - Oral steroids have been shown to slow the progression of muscle weakness - Creatine supplementation can give an improvement in muscle strength

Answer 406

- **Respiratory failure** because of a weak diaphragm - **Scoliosis** - **Dilated cardiomyopathy and arrhythmias:** dystrophin protein is also expressed in heart muscle.

Answer 407

Duchenne's muscular dystrophy: patients have a life expectancy of around **25 – 35 years** with good management of the cardiac and respiratory complications. Becker's muscular dystrophy has a better prognosis

Answer 408

- Gliomas - Meningiomas - Pituitary - Hemangioblastoma - Acoustic neuroma Accounts for less than 2% of all malignant tumours but 20% of childhood cases

Answer 409

- They are tumours in the brain or spinal cord there are 3 types

Answer 410

- **Astrocytoma** - **Glioblastoma multiforme** is the most common - Oligodendroglioma - Ependymoma

Answer 411

- **Glioblastoma multiforme** is the most common - Most commonly found in cerebral hemisphere - Histologically has **pseudo-palisading pattern - peripheral tumour cells lined up around necrotic centre** - Grade IV (most malignant)

Answer 412

- Most common in 40s-50s - Adult oligodendrogliomas typically form in the frontal lobes - Categorised as grade II or III, slow-growing tumours, - Histologically, prominent features can vary from fairly small, round nuclei, surrounded by well-defined “halos” or thick white borders of cytoplasm giving them a “fried egg" appearabce

Answer 413

Arise from ependymal cells (form the epithelial lining of the ventricles in the brain and the central canal of the spinal cord)

Answer 414

- They grow from cells found in the arachnoid mater of the meninges - They are usually benign and this can lead to raised intercranial pressure and lead to the neurological symptoms These tumours may also cause the formation of calcifications called psammoma bodies.

Answer 415

They are initially asymptomatic . - As they develop they present with **focal neurological symptoms**. - Will show signs of raised ICP

Answer 416

- Hemiparesis - personality change, - Broca’s dysphasia, - lack of initiative, - unable to plan tasks

Answer 417

- Dysphagia - Amnesia

Answer 418

- Hemisensory loss - Dysphasia - reduction in 2-point discrimination - astereognosis (unable to recognise object from touch alone

Answer 419

DASHING - - Dysdiadochokinesis (impaired rapidly alternating movement), - Ataxia, - Slurred speech (dysarthria), - Hypotonia, - Intention tremor, - Nystagmus, - Gait abnormality

Answer 420

- Headache - Vomiting - Visual field defect - Seizures - Papilloedema (on fundoscopy) - cushings reflex

Answer 421

- **CT/ MRI** - **Blood tests** e.g. FBC, U&Es, LFT’s, B12 - **Tissue biopsy:** to determine cancer grade and guide management

Answer 422

Severity is classified by the **World Health Organisation’s (WHO) scale** The scale goes from I to IV based on the morphologic and functional features of the tumour cells; a grade IV tumour being the most abnormal looking cells that also tend to be the most aggressive.

Answer 423

- Brain metastasis affects up to 40% of patients with cancer - 10 times more common than primary brain tumours

Answer 424

- Ling - Breast - Melanoma - Renal cell - GI