Neurology Flashcards

Question

What are the spaces in between the meninges called?

Answer 1

- Skull and outer dura = narrow extradural space (in some regions) - Inner dura and arachnoid = narrow subdural space - Arachnoid and pia = subarachnoid space - continuous with ventricular system

Answer 2

- Extradural space – meningeal vessels - Subdural space – bridging veins - Subarachnoid space – Circle of Willis and branches

Answer 3

- Lateral surface: most from the middle cerebral artery - Medial surface: mostly from the anterior cerebral artery

Answer 4

- Lower limb - represented on the medial surface of the cerebral hemisphere = territory of the anterior cerebral artery - Upper limb and face represented on the lateral surface = middle cerebral artery territory

Answer 5

- Ischaemic events: - Transient Ischaemic Attack (TIA) (not a stroke) - Cerebral Infarction (ischaemic stroke) (stroke) - Haemorrhagic events: - Intracerebral haemorrhage (stroke) - Subarachnoid haemorrhage (stroke) - Subdural haemorrhage (not a stroke) - Extradural haemorrhage (not a stroke)

Answer 6

- A sudden onset, brief episode of neurological deficit caused by temporary, focal cerebral ISCHAEMIA (lack of O2 + nutrients to the brain) - Symptoms are maximal at ONSET (usually last 5-15 minutes) - Usually last \<24 HOURS - Without intervention, 1/12 will have a stroke WITHIN A WEEK

Answer 7

- 15% of FIRST STROKES are PRECEDED by a TIA - Can also foreshadow an MI - More common in MALES and BLACK PEOPLE - predisposition to hypertension & atherosclerosis

Answer 8

- AGE - HYPERTENSION - SMOKING - DIABETES - Hyperlipidaemia - Heart disease - ATRIAL FIBRILLATION - Combined oral CONTRACEPTIVE PILL - Peripheral arterial disease, clotting disorder, vasculitis

Answer 9

- ATHEROTHROMBOEMBOLISM = from carotid artery - main cause - listen for a CAROTID BRUIT - CARDIOEMBOLISM: in ATRIAL FIBRILLATION, after an MI, valve disease/prosthetic valve - Hyperviscosity - Hypoperfusion

Answer 10

- Hypoglycemia - Migraine aura - Focal epilepsy - Vasculitis - Syncope, e.g. due to an arrhythmia - Retinal bleed

Answer 11

- Carotid artery (90%): - AMAUROSIS FUGAX - caused by temporary occlusion of retinal artery. Unilateral sudden vision loss. Transient – only lasts minutes. “Like a curtain descending” - Aphasia - Hemiparesis - Hemisensory loss - Hemianopic vision loss - Vertebrobasilar artery (10%): - Diplopia, vertigo, vomiting - Choking and dysarthia - Ataxia

Answer 12

- NOT a TIA if these occur on their own: - Syncope - Dizziness - Temporary loss of consciousness - Temporary memory loss - Gradual onset – suggests demyelination, tumour, migraine

Answer 13

ABCD2: - Age (\>60 years) - Blood pressure (\>140/90) - Clinical features (e.g., unilateral weakness) - Duration of TIA - Diabetes mellitus

Answer 14

- Clinical diagnosis based off SYMPTOMS DESCRIPTION - BLOOD TESTS: glucose, FBC (looking for polycythemia), ESR (raised in vasculitis), U&Es, cholesterol, INR (if on Warfarin) - FIRST LINE = BRAIN IMAGING - diffusion weighted MRI or CT - SECOND LINE = CAROTID imaging - DOPPLER ULTRASOUND. MR/CT angiography if stenosis is found - ECG - Echocardiogram

Answer 15

- Immediate management: ASPIRIN 300mg. Refer to specialist – to be seen WITHIN 24h of symptom onset - Control CV risk factors: BP control, smoking cessation, statin, e.g. simvastatin, no driving for 1 month - Antiplatelet therapy: standard treatment is ASPIRIN 75mg daily (with modified-release Dipyridamole) OR Clopidogrel daily - Anticoagulation (e.g. Warfarin) for patients with Atrial Fibrillation - Carotid Endarterectomy: if \>70% carotid stenosis, reduces stroke/TIA risk by 75%

Answer 16

- RAPID ONSET NEUROLOGICAL DEFICIT lasting for OVER 24 HOURS - POOR BLOOD FLOW to the brain causes CELL DEATH

Answer 17

- 3rd MOST COMMON cause of death worldwide - death rate of 20-25% - In the UK, someone has a stroke every 3.5 minutes - 1 in 4 people who suffer a stroke die WITHIN 1 year - 1 in 2 stroke survivors have PERMANENT DISABILITY - Most common in OLDER PEOPLE - rare aged \<40 - Slightly more common in MALES

Answer 18

- ISCHAEMIC (85%) - blood clot in blood vessel to brain - HAEMORRHAGIC (15%) - bleed in small blood vessel in/around brain - An URGENT CT/MRI is needed to determine which type is present - treating the wrong type would be devastating

Answer 19

F.A.S.T: - Face - has it fallen on one side? - Arms - can they raise them? - Speech - is it slurred? - Time - if you notice any of these, do not hesitate to call 999

Answer 20

- Blood vessel to/in brain OCCLUDED by a CLOT - Ischaemia & INFARCTION follow: - Infarcted area DIES, resulting in FOCAL neurological SYMPTOMS - Infarcted area is surrounded by a swollen area (OEDEMA) which can REGAIN FUNCTION with neurological recovery

Answer 21

- AGE, MALE, HYPERTENSION, SMOKING, DIABETES, recent/past TIA - Hyperlipidaemia - Heart disease - IHD, AF, valve disease - Combined oral CONTRACEPTIVE pill - Black, Asian, peripheral vascular disease, clotting disorder, vasculitis, alcohol, infective endocarditis, carotid bruit

Answer 22

- Small vessel occlusion by THROMBUS - ATHEROTHROMBOEMBOLISM, e.g. from carotid artery - CARDIOEMBOLISM - AF, post-MI, valve disease, infective endocarditis - Hyperviscosity - Hypoperfusion - Vasculitis - Fat emboli from a long bone fracture - Venous sinus thrombosis

Answer 23

Depends on site (ACA, MCA, PCA etc.): - ACA: CONTRALATERAL WEAKNESS AND SENSORY LOSS OF THE LOWER LIMB, incontinence, drowsiness - MCA: CONTRALATERAL MOTOR WEAKNESS AND SENSORY LOSS, APHASIA/DYSPHAGIA (due to Wernicke's and Broca's area being affected), FACIAL DROOP - PCA: HOMONYMOUS HEMIANOPIA, visuo-spatial deficit - Vertebrobasilar artery: coordination and balance - Lateral medullary syndrome: ipsilateral HORNER'S syndrome = reduced sweating, facial numbness, dysarthria, limb ataxia, dysphagia

Answer 24

- Varies depending on the site: - QUADRIPLEGIA - LOCKED-IN SYNDROME - Facial numbness & paralysis - Gaze & vision disturbances - Dysarthria & speech impairment - Vertigo, nausea, vomiting - Cerebellar signs - Palatal paralysis & diminished gag reflex - Altered consciousness - Coma

Answer 25

- Small infarcts from occlusion of a single small perforating artery supplying a subcortical area - Can happen in: – Internal capsule – Basal ganglia – Thalamus – Pons

Answer 26

- Depends on the area affected. One of: - Sensory loss - Weakness (unilateral) - Ataxic hemiparesis - Dysarthria - motor speech problems

Answer 27

- NON-CONTRAST CT HEAD SCAN ASAP: DISTINGUISHES ISCHAEMIC from HAEMORRHAGIC. Shows site of infarct. Identifies conditions mimicking a stroke - Diffusion-weighted MRI scan: more sensitive. CT might be negative in first few hours after infarct. Indicated if diagnosis is uncertain - BLOOD TESTS: glucose (to rule out hypoglycaemia), FBC (looking for polycythemia), ESR (raised in vasculitis), U&Es, cholesterol, INR (if on Warfarin) - ECG: AF, MI

Answer 28

- Immediate management: haemorrhagic stroke must be EXCLUDED. Immediate loading dose: ASPIRIN 300MG - Antiplatelet therapy: aspirin 300mg daily for 2 weeks, then Clopidogrel daily long term - Anticoagulation (e.g., Warfarin) for patients with Atrial Fibrillation - THROMBOLYSIS: must happen WITHIN 4.5 HOURS of symptoms onset (the window in which the benefits outweigh the risks). IV ALTEPLASE (loads of CONTRAINDICATIONS as it can cause massive bleeds) - MECHANICAL THROMBOECTOMY: endovascular removal of thrombus - only suitable for some patients and not others - Admit to an acute stroke unit - Swallowing & feeding support - Eventual rehabilitation

Answer 29

- SUDDEN BLEEDING into brain tissue - Due to RUPTURE of a BLOOD VESSEL WITHIN the BRAIN - Like an ischaemic stroke, this leads to INFARCTION, due to O2 DEPRIVATION - Pooling blood INCREASES intracranial pressure (ICP) - ~10% of strokes, higher mortality: up to 50%

Answer 30

- HYPERTENSION - ANTICOAGULATION drugs - Just been given THROMBOLYSIS - Age - Alcohol - Smoking - Diabetes

Answer 31

- HYPERTENSION: can lead to stiff & brittle vessels = prone to rupture and microaneurysms - SECONDARY to ISCHAEMIC stroke: bleeding after reperfusion - Head trauma - Arteriovenous malformations - Vasculitis - Vascular tumours - Brain tumours - Cerebral amyloid angiopathy - Carotid artery dissection

Answer 32

- Puts pressure on skull, brain & blood vessels - healthy tissue can die - CSF outflow obstruction = hydrocephalus - Midline shift = displacement of brain tissue across the centre line of the brain - Tentorial herniation = movement of brain tissue from one intercranial compartment to another - Coning = compression of brainstem = stop breathing because respiratory centre is there = death

Answer 33

- Similar to an ischaemic stroke - Pointers to haemorrhage (these are unreliable, as CT scan is needed for differentiation): - Sudden loss of consciousness - Severe headache - Meningism - Coma

Answer 34

- Same as ischaemic stroke - Brain imaging (CT/MRI) is essential

Answer 35

- Stop anticoagulants immediately: effects reversed with clotting factor replacement, decision to restart after 1-2 weeks on a case by case basis - Control of BP - IV drugs - Reducing ICP: mechanical ventilation, IV Mannitol to reduce ICP - Referred for neurosurgical evaluation if: - Hydrocephalus - Coma - Brainstem compression

Answer 36

- Spontaneous BLEEDING into SUBARACHNOID SPACE (space between arachnoid mater and pia mater) - Can be CATASTROPHIC - About 5% of strokes

Answer 37

Circle of Willis

Answer 38

- 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 39

- HYPERTENSION - KNOWN ANEURYSM - PREVIOUS ANEURYSMAL SAH - Smoking - Alcohol - Family history - Bleeding disorders - Associated with BERRY ANEURYSMS (things that cause Berry aneurysms = PKD, coarctation of aorta, Ehlers-Danlos syndrome + Marfan syndrome

Answer 40

- Traumatic injury - Aneurysmal RUPTURE - BERRY ANEURYSMS. 70-80% of cases, at common points around Circle of Willis (junction of anterior communicating artery with anterior cerebral artery (40%) and bifurcation of the middle cerebral artery (34%)) - ARTERIOVENOUS MALFORMATIONS = abnormal tangle of blood vessels connecting arteries and veins, 15% of SAH cases - Idiopathic = 15-20% of cases

Answer 41

- Tissue ISCHAEMIA: less blood can reach tissue due to bleeding loss, so less O2 & nutrients reach tissue, causing cell death - Raised ICP: fast flowing arterial blood pumped into cranial space - SPACE-OCCUPYING LESION = puts pressure on brain - Blood IRRITATES MENINGES: these inflame – meningism symptoms, can obstruct CSF outflow – hydrocephalus - VASOSPASM - bleeding irritates other vessels, e.g. arteries so they constrict = ischaemic injury

Answer 42

- SUDDEN ONSET EXCRUCIATING HEADACHE: “THUNDERCLAP”, "WORST EVER headache of life”, typically OCCIPITAL. Can get a sentinel headache before main rupture, sign of a ‘warning leak’ - in ~6% - NAUSEA - VOMITING - COLLAPSE - LOSS of/DEPRESSED CONSCIOUSNESS - Seizures - Vision changes - Coma

Answer 43

- Signs of meningeal irritation: - NECK STIFFNESS - KERNIG'S SIGN (knee and hip flexed to 90 degrees + extension of knee is painful or limited in extension) - BRUDZINSKI'S SIGN (passive flexion of neck causes hip and knee flexion) - Retinal, subhyaloid & vitreous bleeds = worse prognosis, with/without also papilloedema - Focal neurological signs, e.g. 3rd nerve palsy - Increased BP

Answer 44

- Headache: - MIGRAINE - Cluster headache - MENINGITIS - Intracerebral haemorrhage - Cortical vein thrombosis - Carotid/vertebral artery dissection

Answer 45

- Brain CT = ASAP!. Detects \>95% of SAH in first 24 hours. Subarachnoid and/or intraventricular blood. “STAR” shaped sign - LUMBAR PUNCTURE but only if normal ICP to prevent coning. Perform if -ve CT but strong suspicion of SAH remains. PERFORMED AFTER 12 HOURS as we are looking for XANTHACROMIA = confirms SAH, CSF is yellowish - filled with breakdown products of RBCs, e.g. bilirubin - MRI/CT angiography = to establish source of bleeding, e.g. aneurysm, in all patients fit for surgery

Answer 46

- Once SAH is proven, IMMEDIATELY REFER TO NEUROSURGEON - NIMODIPINE for 3 weeks - Ca2+ antagonist, reduces vasospasm so reduces cerebral ischaemia - Surgery: - ENDOVASCULAR COILING (preferred) OR surgical clipping if angiography has shown an aneurysm - IV fluids - maintain cerebral perfusion - Ventricular drainage for hydrocephalus

Answer 47

- BLEEDING into SUBDURAL SPACE (space between dura mater & arachnoid mater) - Due to rupture of a BRIDGING VEIN - run from cortex to venous sinuses, vulnerable to deceleration injury - Usually due to HEAD TRAUMA - other causes include dural metastases - Massive LATENT INTERVAL - WEEKS TO MONTHS - Very treatable

Answer 48

- BABIES - traumatic injury, e.g. “shaking baby syndrome” - BRAIN ATROPHY - veins are more susceptible to rupture, e.g. DEMENTIA, ELDERLY, ALCOHOLICS. These people are also more ACCIDENT-PRONE and at risk of FALLS, along with epileptics - People on ANTICOAGULANTS

Answer 49

- Bleeding from bridging veins into subdural space - Forms a haemotoma (SOLID SWELLING of CLOTTED blood) - Then BLEEDING STOPS - WEEKS/MONTHS LATER, haematoma starts to AUTOLYSE. This creates a MASSIVE INCREASE in ONCOTIC AND OSMOTIC PRESSURE. Water sucked in, haematoma ENLARGES - GRADUAL RISE in ICP over weeks - MIDLINE STRUCTURES SHIFTED AWAY from side of clot - TENTORIAL HERNIATION, CONING

Answer 50

- FLUCTUATING level of CONSCIOUSNESS - DROWSINESS - HEADACHE - CONFUSION - may fluctuate - Insidious physical & intellectual slowing - Personality change - Unsteadiness - They can often not remember the traumatic injury as it was long ago!

Answer 51

- RAISED ICP - Seizures - Localising neurological signs: - Unequal pupils - Hemiparesis - Occur late (often \>1 month after injury)

Answer 52

- NON-CONTRAST HEAD CT SCAN: - Shows haematoma - crescent-shaped - BANANA - Shows MIDLINE SHIFT - MRI scan

Answer 53

- SURGERY: - 1st line = BURR-HOLE washout - 2nd line = craniotomy - IV MANNITOL to reduce ICP - Reverse clotting abnormalities - Address cause of trauma, e.g. falls, trauma

Answer 54

- BLEEDING into EXTRADURAL SPACE (space between dura mater & skull bone) - Usually after TRAUMA to TEMPLE: - FRACTURE to TEMPORAL/parietal BONE - RUPTURE of MIDDLE MENINGEAL ARTERY - Can also be due to tear to dural venous sinus - LUCID INTERVAL – can be HOURS. Initial drowsiness/unconsciousness, then recovery, then rapid deterioration later on

Answer 55

- Mostly in YOUNG PEOPLE, but rare in small children due to plasticity of skull - Rare in \>60s as the dura mater is more tightly adherent to skull - More common in MALES

Answer 56

- After lucid interval: - Rapid rise in ICP - Pressure on brain - Midline shift - Tentorial herniation - Coning

Answer 57

- Initial head trauma: injury to temple/pterion, short episode of drowsiness or unconsciousness - Lucid interval: hours to days, period of recovery - Then sudden, rapid deterioration follows…

Answer 58

- RAPIDLY DECLINING GCS (consciousness level) - Increasingly severe HEADACHE - VOMITING - SEIZURES - Hemiparesis - UMN signs - Ipsilateral pupil dilation - Bilateral limb weakness - COMA - Deep and irregular breathing due to coning (brainstem compression) - Late signs include: - Bradycardia - Raised BP - Death from respiratory arrest

Answer 59

- CT scan: shows haematoma, LEMON-SHAPED LESION, doesn’t cross suture lines. Unilateral, shows midline shift - Skull X-ray: fracture lines might be seen in temporal or parietal bone

Answer 60

- Stabilise patient - URGENT SURGERY: clot evacuation, ligation of bleeding vessel - IV MANNITOL to reduce ICP - Airway care: intubation, ventilation - DO NOT GIVE ASPIRIN!!

Answer 61

- Migraine - Cluster headache - Tension headache - Secondary headache, e.g. drug-induced

Answer 62

- Migraine: RECURRENT throbbing headache often preceded by an AURA + ASSOCIATED WITH NAUSEA, VOMITING and VISUAL CHANGES - Most common causes of EPIDSODIC HEADACHE i.e. recurrent - More common in FEMALES (roughly 3x) + 90% have onset before 40yrs - Risk factors: genetics \> FHx, female, age (majority of first migraines are in adolescence)

Answer 63

There is some evidence suggesting that migraines are due to irritation of the trigeminal nuclei within the brainstem due to changes in arterial blood flow; this is why the pattern pictured above is seen (same as trigeminal neuralgia)

Answer 64

- There are no know definite causes, however a number of triggers are known - Mnemonic – CHOCOLATE: - Chocolate - Hangovers - Orgasms - Cheese - Oral contraceptives - Lie-ins - Alcohol - Tumult i.e. loud noises - Exercise

Answer 65

- PRODROME = yawning, cravings, mood/sleep changes - AURA = PRECEDES ATTACK AND CAN BE A VARIETY OF SYMPTOMS: - Visual disturbance, e.g. lines, dots, zig-zags - Somatosensory, e.g. paraesthesia, pins & needles

Answer 66

- Migraines are classified as WITH OR WITHOUT AURA, but to be classified as a migraine a set of general features must also be met: - AT LEAST 2 OF: - Unilateral pain (usually 4-72hrs) - Throbbing-type pain of a moderate\>severe intensity - Motion sensitivity PLUS, AT LEAST ONE OF: - Nausea/vomiting - Photophobia/phonophobia - THERE MUST ALSO BE A NORMAL EXAMINATION AND NO ATTRIBUTABLE CAUSE

Answer 67

- Usually, diagnosis is made clinically with few/no investigations required; however, in certain cases other causes for headaches must be rules out: - Lab tests, e.g. CRP, ESR - CT/MRI if RED FLAGS i.e. indications: - Worst/severe headache i.e. 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 68

- TRIPTANS, e.g SUMATRIPTAN (first line and most important for exams). C/I in IHD, uncontrolled HTN. S/Es = arrhythmias, angina \> MI. Work on 5-HT receptor - NSAIDS, e.g. naproxen - Anti-emetics, e.g. prochlorperazine - AVOID OPIOIDS AND ERGOTAMINE no matter how bad the pain is

Answer 69

5-HT receptor

Answer 70

- Required if \>2 attacks per month OR require acute meds \>2x per week - BETA BLOCKERS, e.g. propanolol - but contra-indicated in asthmatics etc. - TCAs, e.g. amitryptyline - Anti-convulsant, e.g. Topiramate

Answer 71

- EPISODIC HEADACHES lasting from 7 days up to 1 year (although usually 2-3wks) with PAIN-FREE PERIODS in between that last ~4wks - Considered the MOST DISABLING OF PRIMARY HEADACHES - Much rarer than migraines, and MORE COMMON IN MALES (~4x) - Typically affects adults with onset usually 20-40yrs

Answer 72

- SMOKER - ALCOHOL - Male - Genetics - autosomal dominant gene has a link

Answer 73

- RAPID ONSET OF EXCRUCIATING PAIN, CLASSICALLY AROUND THE EYE, however other common areas are the temples or forehead - Pain is strictly UNILATERAL and LOCALISED TO ONE AREA. It rises to a CRESCENDO over a few minutes and last for 15-160 minutes, once or twice a day usually around the same time of day - IPSILATERAL AUTONOMIC FEATURES: - WATERY + BLOODSHOT EYE - MIOSIS (PUPILLARY CONSTRICTION) +/- PTOSIS (SEEN IN 20%) - Facial flushing - Rhinorrhoea (blocked nose)

Answer 74

- Acute attacks: - Analgesics are unhelpful - 15L 100% O2 FOR 15 MINS VIA A NON-REBREATHER MASK (first line for acute) - TRIPTANS, e.g. SUMATRIPTAN

Answer 75

- VERAPAMIL (CCB) = 1st line prophylaxis - Prednisolone - REDUCE ALCOHOL CONSUMPTION AND STOP SMOKING

Answer 76

- MOST COMMON CHRONIC DAILY + RECURRENT HEADACHE - Can be EPISODIC \<15 DAYS/month or CHRONIC \> 15 DAYS/month (for at least 3 months) - There is no known organic cause, however a number of triggers exist

Answer 77

Triggers: - Stress - Sleep deprivation - Bad posture - Hunger - Eyestrain - Anxiety - Noise

Answer 78

- Usually at least one of the following: - BILATERAL - PRESSING/TIGHT + NON-PULSATILE (LIKE AN ELASTIC BAND). Mild/moderate intensity - +/- scalp tenderness - NO AURA, vomiting or sensitivity to head movement - CAN BE SOME 'PRESSURE' BEHIND EYES, but pain isn’t localised to be around the eye

Answer 79

- Avoidance of triggers & stress relief - Symptomatic relief: - Aspirin - Paracetamol - Ibuprofen - AVOID OPIOIDS - LIMIT ANALGESICS TO NO MORE THAN 6 DAYS PER MONTH TO REDUCE THE RISK OF MEDICATION-OVERUSE

Answer 80

- Giant cell/temporal arteritis (most important = vascular disease affecting the temporal artery. One-sided pain in the temple, scalp tenderness when you touch it. Important to rule out in people \>55. Important as can cause BLINDNESS if left untreated) - Sentinel headache - Thunderclap headache - Headache of a morning - Trauma - Medication overuse - Trigeminal neuralgia - Systemic infection - Meningitis/encephalitis

Answer 81

Epilepsy - the recurrent tendency to spontaneous, intermittent, abnormal electrical activity in part of the brain, manifesting in seizures

Answer 82

Criteria, one of: - AT LEAST 2 UNPROVOKED SEIZURES OCCURING MORE THAN 24 HOURS APART - One unprovoked seizure and a probability of future seizures (considered \>60% risk in 10yrs) - Diagnosis of an epileptic syndrome

Answer 83

Can occur at any age, but onset is most common in the extremes of life, e.g. \<20 years and \>60 years

Answer 84

- 2/3RDS IDIOPATHIC, often familial - Cortical scarring, e.g. trauma, cerebrovascular disease, infection - Tumours/space-occupying lesions - Strokes - Alzheimer’s - Alcohol withdrawal (delirium tremens)

Answer 85

- FHx - Premature babies, especially if they are small for their gestational age - Abnormal cerebral blood vessels - Drugs, e.g. cocaine

Answer 86

- PRODROME = precedes the seizures, usually by hours/days. Weird feeling, e.g. mood/behaviour changes - AURA = just before a seizure. Part of the seizure where the patient is aware & often precedes other manifestations, e.g. strange feelings in gut, déjà vu, strange smells, flashing lights. Often implies a PARTIAL SEIZURE - POST-ICTAL = the period after a seizures. HEADACHE, CONFUSION, MYALGIA, SORE TONGUE (OFTEN BITTEN). Temporary weakness after focal seizure in motor cortex = POST-ICTAL TODD'S PALSY (this will go away after a few hours). DYSPHASIA following TEMPORAL LOBE seizure

Answer 87

- Primary generalized (40%): - Simultaneous electrical discharge throughout the whole cortex, with no features that suggest localisation to only one hemisphere/lobe - Bilateral and symmetrical motor manifestations - ALWAYS ASSOCIATED WITH LOSS OF CONSCIOUSNESS & LACK OF AWARENESS (patient will wake up and be told they've had a seziure) - Partial (focal) (60%): - Focal onset with features that can be referrable to a single lobe, e.g. temporal lobe - Often seen with underlying structural disease i.e. there is an underlying cause - Electrical discharge is limited to one lobe, in one hemisphere - These may later PROGRESS to become GENERALIZED SEIZURES (secondary)

Answer 88

- TONIC seizure = high tone (hence the name) = RIGID, STIFF LIMBS. Will fall to floor if standing due to stiff limbs - CLONIC seizure = RHYTHMIC MUSCLE JERKING (i.e. clonus, the UMN sign) - TONIC-CLONIC SEIZURE (“Grand Mal”) = combination of tonic & clonic seizures. STEREOTYPICAL 'SHAKING' SEIZURES due to the mix of on/off rigidity and muscle jerking - MYOCLONIC seizure = isolated jerking of a limb/face/trunk. “DISOBEDIENT LIMB” or “thrown to the floor” - ATONIC seizure = LOSS OF MUSCLE TONE = FLOPPY - ABSENCE seizure (“Petit Mal”) = common in CHILDHOOD but usually goes by adulthood, however there is an INCREASED RISK OF DEVELOPING GENERALIZED TONIC-CLONIC SEIZURES AS AN ADULT. Will go PALE and ”STARE BLANKLY” for a few seconds. Often suddenly stop talking mid-sentence & do not realise they have had an attack

Answer 89

- SIMPLE PARTIAL SEIZURE = NO EFFECT on CONSCIOUSNESS OR MEMORY. Awareness unimpaired but will have focal motor, sensory, autonomic or psychic symptoms depending on the affected lobe. NO POST-ICTAL SYMPTOMS - COMPLEX PARTIAL SEIZURE = memory/awareness is affected before, during or immediately after the seizure. Most commonly arises from the TEMPORAL LOBE \> affects speech, memory & emotion. Post-ictal confusion is COMMON if temporal lobe, whereas RECOVERY is often swift if the FRONTAL LOBE is affected - PARTIAL SEIZURE WITH SECONDARY GENERALISATION. 2/3 patients with partial seizures will develop generalized seizures, usually generalized tonic-clonic. These start focally & spread widely throughout the cortex

Answer 90

a) Temporal lobe i.e. memory, understanding speech, emotion: - AURA (80%): deja-vu, auditory hallucinations, funny smells, fear - Anxiety, out-of-body experiences - AUTOMATISMS (set of brief unconscious behaviours), e.g. lip smacking b) Frontal lobe i.e. motor, thought processing: - Motor features, e.g. posturing, peddling movements of leg - JACKSONIAN MARCH - seizures march up/down the motor homunculus - POST-ICTAL TODD'S PALSY - starts distally in a limb & works its way upwards to the face c) Parietal lobe i.e. sensation: - Sensory disturbances, e.g. TINGLING/NUMBNESS d) Occipital lobe i.e. vision: - VISUAL PHENOMENA, e.g. spots, lines, flashes

Answer 91

- Non-epileptic seizures are entirely situational, e.g. metabolic disturbances (low Na+, hypoxia). Can be related to syncope (afterwards) - Non-epileptic seizures are typically longer with closed eyes & mouth - Non-epileptic do not occur during sleep and do not involve incontinence or tongue-biting - There are pre-ictal anxiety symptoms in non-epileptic, e.g. they know they are about to happen

Answer 92

- Epilepsy-sensitive signs: - Tongue-biting - Head turning - Muscle pain - LOC - Cyanosis - Post-ictal symptoms

Answer 93

- DIAGNOSIS IS MADE CLINICALLY, but there are a number of tests to help: - ELECTROENCEPHALOGRAM (EEG) = not diagnostic, but may help determine the TYPE of epileptic syndrome - MRI/ CT head = used to rule out other potential causes, e.g. space-occupying lesions - Bloods = FBC, Ca2+, electrolytes, U&Es, LFTs, blood glucose. To rule out other potential causes, e.g. metabolic disturbances - Genetic testing = if suspected genetic cause, e.g. juvenile myoclonic epilepsy

Answer 94

- Usually only started after the second epileptic episode, and the drug of choice varies dependent on the type of epileptic syndrome and the individual (esp. age, gender) - You basically give SODIUM VALPROATE unless: - Female of childbearing potential, then LAMOTRIGINE for all generalized except: - Myoclonic = LEVETIRACETAM/TOPIRAMATE - Absence = ETHOSUXIMIDE - Partial (focal) seizure = Lamotrigine

Answer 95

Male = SODIUM VALPROATE Female = LAMOTRIGINE (as most will of childbearing potential)

Answer 96

IT IS HIGHLY TERATOGENIC!

Answer 97

Continuous seizure lasting over 5 minutes or repeated seizures with no recovery of consciousness in-between - Management: - BENZODIAZEPINES (1st line) - IV LORAZEPAM - If ineffective within 10 mins, 2nd line = PHENYTOIN, sodium valproate, levetiracetam, phenobarbital - If no response within 30 mins from onset - induction of GA with Propofol

Answer 98

- Gliomas (tumours of the glial cells, listed from most to least malignant): - Astrocytoma (GLIOBLASTOMA MULTIFORME is the most common) - Oligodendroglioma - Ependymoma - Others: - Meningioma (tumour of the meninges) - Schwannoma - Craniopharyngiomas - Much less common than secondary brain tumours

Answer 99

- Most common origins: - Non-small cell lung cancer = most common - Small cell lung cancer - Breast - Melanoma - Renal cell carcinoma - GI - MUCH MORE COMMON than primary brain tumours

Answer 100

- Often brain tumours do not have any symptoms, particularly when they are small - As they develop they present with FOCAL NEUROLOGICAL SYMPTOMS depending on the location of the lesion, e.g. classic example: patient has an unusual change in personality and behaviour = tumour in the frontal lobe - Also present with signs and symptoms of RAISED INTERCRANIAL PRESSURE - key finding is PAPILLOEDEMA on FUNDOSCOPY

Answer 101

- Depletion of THIAMINE (VITAMIN B1) - Classic triad: - CONFUSION - Ataxia - Ophthalmoplegia - Sign: - ASTERIXIS (‘liver flap’) = general sign of a metabolic encephalopathy

Answer 102

Occurs in patients with poor nutritional absorption, intake, or loss: - CHRONIC ALCOHOLISM - Severe starvation - Prolonged vomiting

Answer 103

- Diagnosis is clinical - Low blood thiamine

Answer 104

- PABRINEX (IV B vitamins including thiamine) - DO NOT give glucose before thiamine

Answer 105

- If not managed appropriately: - Fatal in 20% - Can progress to KORSAKOFF'S SYNDROME

Answer 106

- Irreversible, long-term brain damage (due to B1 deficiency) - Symptoms: - Decreased ability to acquire new memories - Retrograde amnesia - Confabulation (invented memories)

Answer 107

- Dementia is a term used to describe a group of symptoms such as poor memory and difficulty learning new information, which can make it really hard to function independently - Different types: - Alzheimer's disease - Frontotemporal dementia (Pick's) - Vascular dementia - Dementia with Lewy bodies

Answer 108

- Alzheimer's disease is a chronic neurodegenerative disease with an insidious onset and progressive but slow decline - Most common form of dementia - Mainly affects those over 65

Answer 109

- Increased age - Family history - Down's syndrome (Trisomy-21) - Apolipoprotein E-e4 (ApoEe4) allele homozygosity (ApoE normally breaks down beta amyloid, e4 alleles encode less effective ApoE) - Reduced cognitive activity

Answer 110

- Extracellular deposition of BETA-AMYLOID PLAQUES: signalling obstruction -\> deposits around vessels -\> increased haemorrhage risk -\> initiates inflammatory response - In AD, Tau proteins become hyperphosphorylated -\> aggregate, stop supporting microtubules -\> form NEUROFIBRILLARY TANGLES -\> obstruct neuronal signalling -\> neuron apoptosis - Damaged synapses - Atrophy

Answer 111

- Memory - episodic and semantic - Language - difficulty understanding or finding words - Attention and concentration issues - Psychiatric changes, e.g. withdrawal, delusions - Disorientation, e.g. time and surroundings

Answer 112

- MMSE - Bloods - TFTs, B12 (check for other causes) - CT/MRI scan - exclude other dementia causes. AD will show cortical (especially hippocampus) atrophy, gyri narrowing, sulci widening and ventricle enlargement - Memory clinic assessment

Answer 113

- No cure - Supportive therapy, e.g. carers, changes to the home, help with daily activities - Medication to manage symptoms: acetylcholinesterase (AChE) inhibitors, e.g. rivastigmine, galantamine, memantine

Answer 114

- Frontotemporal dementia (Pick's) = atrophy of the frontal and temporal lobes. It is a progressive dementia - 5% of dementias - More common in those under 65 - average onset is between 45-65 years

Answer 115

- Loss of neurons, but no plaque formation - Pick bodies - Tau +ve or TDP-43 +ve inclusions

Answer 116

- Onset tends to be insidious and progressive. Present with 3 main symptoms: - Behavioural issues, e.g. loss of inhibition/empathy, compulsive behaviours, difficulty planning - Progressive aphasia, e.g. slow, difficult speech, grammatical errors - Semantic dementia, e.g. loss of vocabulary, problems understanding

Answer 117

- Bloods - B12, TFTs, U&Es (?other causes) - MRI/CT - frontal/temporal atrophy - FBC and LFTs for suspected encephalopathy - MMSE

Answer 118

- No cure - Supportive therapy, e.g. carers, help setting up a stable routine, home changes for motor difficulties, speech and language therapy - SSRIs - help with behaviour symptoms - Levodopa/carbidopa if Parkinson’s symptoms present - Stopping exacerbating drugs

Answer 119

- Vascular dementia is a chronic progressive disease of the brain bringing about cognitive impairment. The executive functions of the brain such as planning are more prominently affected than memory. It is the result of multiple, small infarcts - Second most common dementia globally - 17% of dementia in the UK - Risk factors: smoking, history of TIAs, AF, hypertension, DMT1, hyperlipidaemia, obesity, coronary heart disease - Detailed pathophysiology is based on the location and size of the original infarct, and number and location of consequent infarcts - A single stroke doubles the risk of developing vascular dementia

Answer 120

Characterised by STEPWISE PROGRESSION - periods of stable symptoms, followed by a sudden increase in severity

Answer 121

- Presentation varies massively but can include: - Visual disturbances - UMN signs (e.g. muscle weakness, overactive reflexes, clonus) - Attention deficit - Depression - Incontinence - Emotional disturbances - If infarct was subcortical, then expect to see dysarthria and parkinsonisms

Answer 122

- Full history important - previous stroke/TIA? - Cognitive impairment screen - orientation, attention, language function, visuospatial functions, motor control - Medication review - ?other cause - MRI - looking for previous infarcts

Answer 123

- Supportive therapy, e.g. carers, home changes, routine help, cognitive simulation programmes - SSRIs or anti-psychotics to control symptoms, e.g. lorazepam - Prognosis is 3-5 years from diagnosis

Answer 124

- Characterised by eosinophilic intracytoplasmic neuronal inclusion bodies (Lewy bodies) in the brainstem and neocortex. Also see substantia nigra depigmentation and amyloid deposits - Lewy body disease includes three overlapping disorders, so this dementia is on a spectrum - DLB is when the dementia is the presenting issue, Parkinson’s dementia is when Parkinson’s is the presenting issue

Answer 125

- Dementia is often presented initially: memory loss, spatial awareness difficulties, loss of cognitive function - Parkinsonisms, e.g. tremor, rigidity, change in gait - Visual hallucinations - Sleep disorders, restless leg syndrome

Answer 126

- International criteria used: - Presence of dementia with 2 of: fluctuating attention and concentration, recurrent well-formed visual hallucinations, spontaneous Parkinsonism - If there is only 1 of the 3 core features, diagnosis can also be made with a SPECT or PET scan showing low dopamine transporter uptake in basal ganglia - MMSE or 6-item cognitive impairment test (6-CIT) - Bloods - B12, TFTs, U&E, MRI (?other cause) - MSU to check for urine infection

Answer 127

- Refer to a specialist - Supportive therapy - cognitive stimulation, exercise programmes, at-home care - Cholinesterase inhibitors, e.g. rivastigmine suggested to treat cognitive decline - Avoid use of neuroleptic drugs, e.g. haloperidol, as they can produce severe sensitivity reactions

Answer 128

- Parkinson’s disease is a condition where there is a progressive REDUCTION of DOPAMINE in the BASAL GANGLIA of the brain, leading to disorders of movement - Ventral tier of the zona compacta in the substantia nigra is affected the most - Second most common neurodegenerative disorder (after Alzheimer’s). Typically develops between 55-65 years of age

Answer 129

- Usually idiopathic - Drug-induced Parkinsonism is caused by dopamine antagonists, e.g. clozepine - Can also be caused by encephalitis or exposure to certain toxins, e.g. manganese dust - Risk factors: age, male sex, pesticide exposure

Answer 130

- The symptoms are characteristically asymmetrical, with one side affected more than the other. There is a classic triad of features in Parkinson’s disease: - RESTING TREMOR - RIGIDITY (COG-WHEEL WALK) - BRADYKINESIA - Other clinical signs include: impaired dexterity, fixed facial expressions, foot drag - Common associated symptoms: dementia, depression, urinary frequency, constipation, sleep disturbances

Answer 131

- Almost entirely clinical diagnosis. 3 step diagnosis: 1) Diagnosis of Parkinsonian syndrome: bradykinesia (required) plus one of rigidity, resting tremor, or postural instability 2) Exclusion criteria (none to be met): history of stroke, repeated head injury, neuroleptic treatment, unilateral features after 3 years, cerebellar signs, Babinski’s sign, early severe dementia, negative response to large L-dopa dose 3) Supportive criteria (3 or more required): unilateral onset, rest tremor present, progressive, excellent response to L-dopa (70-100%), visual hallucinations - DaT SCAN IS USED TO CONFIRM THE DIAGNOSIS OF PARKINSON'S DISEASE

Answer 132

Young onset + biologically fit: 1. Dopamine agonist: ropinirole 2. MOA-B inhibitor: rasagiline 3. L-DOPA: co-careldopa = most effective for symptomatic management. Offered in early disease as well tolerated. Wear-off phenomenon can happen after years of taking levodopa - where dose is no longer effective. Can be combated by taking a COMT inhibitor alongside Biologically frail and comorbidities: 1. L-DOPA 2. MOA-B inhibitor

Answer 133

- Huntington’s disease is an autosomal dominant genetic condition that causes a progressive deterioration in the nervous system. It causes the LOSS of main inhibitory neurotransmitter GABA - Mean age of onset is 30-50 years - It is a 'TRINUCLEOTIDE REPEAT' disorder that affects CAG gene on the Huntington gene, on chromosome 4

Answer 134

- ANTICIPATION. This is where successive generations have more repeats in the gene, resulting in: - Earlier age of onset - Increased severity of disease

Answer 135

- Main sign is hyperkinesia - Characterised by: CHOREA, DYSTONIA, and INCOORDINATION - EYE MOVEMENT PROBLEMS - Psychiatric issues - Depression - Cognitive impairment, behavioural difficulties - Irritability, agitation, anxiety

Answer 136

- Diagnosis is made in a specialist genetic centre using a genetic test (CAG repeat testing) for the faulty gene. It involves pre-test and post-test counselling regarding the implications of the results - MRI/CT - loss of striatal volume

Answer 137

- No treatment options for slowing or stopping the progression of the disease - Benzodiazepines/valproic acid - chorea - SSRIs if depression present - Haloperidol for psychosis - Prognosis is poor. Most common death is from respiratory illness, e.g. pneumonia. Suicide is the 2nd most common cause

Answer 138

SYDENHAM'S CHOREA (rheumatic fever)

Answer 139

- Spinal cord compression (SCC) results from processes that compress or displace arterial, venous, and cerebrospinal fluid spaces, as well as the cord itself - Causes: trauma, tumours, central disc protrusion, prolapsed disc (L4-5 and L5-S1 most common), epidural haematoma, infection, cervical spondylitic myelopathy. Tumours: most common spinal metastases are breast, prostate and lung cancer

Answer 140

- Red flag signs: - Loss of bladder or bowel function - UMN signs in the lower limbs (e.g. clonus, hyperreflexia) - LMN signs in the upper limbs (e.g. atrophy) - Symptoms depend on the injury type and site. Can include paraplegia, pain, paraesthesia, changes to tendon reflexes etc.

Answer 141

- X-ray whole spine - MRI if indicated, e.g. cancer - Renal function - Haemoglobin - monitor blood loss

Answer 142

- Acute spinal cord compression is a neurosurgical emergency - Dexamethasone until treatment plan confirmed - Catheterisation - Analgesia - Surgical decompression if indicated - Chemotherapy if indicated

Answer 143

- Hemisection of the spinal cord (damage limited to one half -\> paralysis on side of lesion (ipsilateral hemiplegia), loss of sensation on the opposite side (contralateral pain and temperature sensation deficits) - this is due to the crossing of fibres in the spinothalamic tract) - Most commonly caused by penetrating trauma - Most cases seen in the cervical region

Answer 144

- Ipsilateral hemiparesis - Contralateral loss of pain and temperature below the lesion

Answer 145

- Plain radiographs for penetrating or blunt trauma - MRI to determine extent of injury - Neurological examination to establish level of injury

Answer 146

- No specific treatment: - Spine immobilisation - Steroids to decrease swelling - Physical therapy - Occupational therapy - Surgery if indicated

Answer 147

- Cauda equina syndrome is a rare and severe type of spinal stenosis where all of the nerves in the lower back suddenly become severely compressed - The cauda equina is formed by the nerve roots caudal to spinal cord termination - Causes: herniation of lumbar at L4/5 or L5/S1 level, tumours, trauma, infection, late-stage ankylosing spondylitis, postoperative haematoma, sarcoidosis

Answer 148

- Sudden onset - hours - SADDLE PARASTHESIA = loss of sensation in the perineum - around the genitals and anus (red flag) - BLADDER/BOWEL DYSFUNCTION, i.e. loss of sensation, urinary incontinence - BILATERAL SCIATICA - Sexual dysfunction (red flag) - Motor problems - Lower back pain (if severe, red flag) - Bilateral LMN weakness, absent ankle reflex

Answer 149

- Medical emergency - immediate referral - Rectal exam - loss of anal tone/sensation - MRI spine (most important)

Answer 150

- CAUDA EQUINA SYNDROME IS A NEUROSURGICAL EMERGENCY: - LUMBAR DECOMPRESSION SURGERY - Immobilise spine - Anti-inflammatory agents - Antibiotics if infection present - Chemotherapy if indicated

Answer 151

Sciatica refers to the symptoms associated with irritation of the SCIATIC NERVE

Answer 152

The spinal nerves L4 – S3 come together to form the SCIATIC NERVE. It exits the posterior part of the pelvis through the GREATER SCIATIC FORAMEN, in the buttock area on either side. It travels down the back of the leg. At the knee, it divides into the TIBIAL NERVE and the COMMON PERONEAL NERVE The sciatic nerve supplies SENSATION to the lateral lower leg and the foot. It supplies MOTOR function to the posterior thigh, lower leg and foot

Answer 153

- The main causes of sciatica are lumbosacral nerve root compression by: - HERNIATED DISC - SPONDYLOLISTHESIS (anterior displacement of a vertebra out of line with the one below) - SPINAL STENOSIS - BILATERAL SCIATICA is a RED FLAG for CAUDA EQUINA syndome

Answer 154

- UNILATERAL PAIN from the buttock radiating down the back of the thigh to below the knee or feet - might be described as an “electric” or “shooting” pain - PARASTHESIA - NUMBNESS - MOTOR WEAKNESS - REFLEXES may be affected depending on the affected nerve root

Answer 155

- X-ray, MRI - confirms disc herniation, stenosis, tumours as its aetiology - SCIATIC STRETCH TEST can be used to help diagnose sciatica. The patient lies on their back with their leg straight. The examiner lifts one leg from the ankle with the knee extended until the limit of hip flexion is reached (usually around 80-90 degrees). Then the examiner dorsiflexes the patient’s ankle. Sciatica-type pain in the buttock/posterior thigh indicates sciatic nerve root irritation. Symptoms improve with flexing the knee

Answer 156

- Initial management = self-management, education, reassurance, analgesia - NICE guidelines state NOT to use medications such as gabapentin, pregabalin, diazepam or oral corticosteroids for sciatica. They suggest considering a NEUROPATHIC MEDICATION if symptoms are persisting or worsening at follow up: AMITRIPTYLINE or DULOXETINE - Chronic sciatica management options: epidural CORTICOSTEROID injections, local anaesthetic injections, radiofrequency denervation, spinal decompression

Answer 157

- Damage to a nerve through compression, trauma, infection etc. - Results in the interruption of axonal continuity - 3 categories of peripheral nerve injuries: stretch related, lacerations, and compressions

Answer 158

- Depends on the nerve affected Can include: numbness, tingling, muscle weakness, dropping objects, sharp pains, buzzing sensations in limbs

Answer 159

- Grades of nerve injury (Seddon 1942): - Neuropraxia (conduction block) - Axonotmesis (axons divided) - Neurotmesis (nerves divided)

Answer 160

- Neurological examination - MRI - Again, depends on the nerve affected

Answer 161

- Splints/braces - Physical therapy - Exercise - Analgesia - Surgery in some severe cases

Answer 162

- Compression of the median nerve in the carpal tunnel - There are 8 carpal bones in the wrist and a ligament that lies across the front of the wrist - between the ligament and the carpal bones is the carpal tunnel - Median nerve gives feeling to thumb, index finger, middle finger, and half of the ring finger - More common in late 50s and late 70s, and pregnant women - IDIOPATHIC. There are a number of key risk factors: ACROMEGALY, HYPOTHYROIDISM, diabetes, rheumatoid arthritis, repetitive strain, obesity, perimenopause

Answer 163

- SENSORY symptoms of the thumb, index finger and middle finger, and the lateral half of the ring finger: NUMBNESS, parasthesia, burning sensation, pain - Symptoms can come and go but are typically WORSE at NIGHT

Answer 164

- Clinical diagnosis - NERVE CONDUCTION STUDIES (first-line) - USS or MRI if other damage suspected - PHALEN'S test (fully flexing the wrist) and TINNEL'S TEST (tapping the wrist at the location of the median nerve passing through the carpal tunnel)

Answer 165

- 1 in 4 cases will be self-limiting (usually 1 year). Pregnancy cases will resolve postpartum - Rest the wrist, and try to avoid gripping/squeezing actions - Splint (1st line) - common for night wear only - Steroid injections - Surgery in severe cases to decompress the carpal tunnel

Answer 166

- Difficulty in lifting the front part of the foot, resulting in dragging of the toes. Can be permanent or temporary - Caused by damage to the COMMON PERONEAL NERVE (also called common fibular nerve) - Causes include: injury, lower back damage, tumour, hip replacement, cauda equina syndrome, and multiple sclerosis

Answer 167

- Unilateral symptoms: - Complaint of one foot dragging across the floor when walking - Tripping - Numbness in the same side - Weakness in the same side

Answer 168

- Foot drop itself can be a clinical diagnosis - Need to find the underlying cause: - X-ray, USS, CT, MRI - Nerve conduction study

Answer 169

- Brace or splint - Physiotherapy - Specialised shoes - prevent foot drop when walking - Nerve stimulation - Surgery if indicated

Answer 170

- Heterogeneous group of inherited peripheral neuropathies. It affects the PERIPHERAL MOTOR and SENSORY nerves and causes dysfunction in the MYELIN or the AXONS - Most common inherited neuromuscular disorder. Autosomal dominant inheritance (can be recessive but rare) - No predilection for race or sex - Disease course is extremely varied

Answer 171

- 4 major categories: CMT1, CMT2, CMT3, CMTX: - CMT1 - production of abnormal, unstable myelin - CMT2 - primarily an axonal disorder - CMT3 - characterised by marked segmental demyelination - CMTX - demyelinating neuropathy

Answer 172

- Onset is insidious and slowly progressive - CMT1: onset by 10 years old. Muscle weakness starting in feet and working up (INVERTED CHAMPAGNE BOTTLE LEGS), sensory loss in the same pattern as weakness, foot drop and spinal deformities can be seen - CMT2: onset between 10-20 years old (can be later). Weakness and wasting pattern same as CMT1 - CMT3: infantile onset, usually by 2 years old. Hypertonia or floppy baby syndrome - CMTX: more severe in males. Similar presentation as CMT1

Answer 173

- Bloods - FBC, TFTs, LFTs, B12, folate - CSF examination - MRI brain and spinal cord - Genetic studies - Nerve conduction studies - Nerve biopsy if genetic studies inconclusive

Answer 174

- No cure or effective treatment - MDT care - Supportive therapy and rehabilitation - Surgical correction of spinal deformity - Analgesia - Avoid neurotoxic drugs, e.g. vincristine, as this can exacerbate the condition

Answer 175

- Muscular dystrophy is an umbrella term for genetic conditions that cause gradual wasting and weakening of the muscles - Duchenne muscular dystrophy - Becker muscular dystrophy

Answer 176

- Inherited disorder characterised by progressive muscle wasting and weakness - X-linked recessive condition - male - Results in damage to the DYSTROPHIN gene, so no dystrophin is produced. Function = strengthen muscle fibres and protect from injury - Prognosis - wheelchair by 12, death by 30

Answer 177

- Present in early childhood, before 3 - Progressive proximal muscular dystrophy with characteristic pseudohypertrophy of the calves - Major milestones delayed - Inability to run, hop or jump - Recurrent falls - Speech delay - Fatigue - really common

Answer 178

- Initial investigation - serum creatinine kinase = very high - Genetic analysis - Muscle biopsy with assay for dystrophin protein - Muscle strength test - Gait assessment

Answer 179

- Joint contractures - Cardiomyopathies and heart failure - Gastric dilation - Learning difficulties - Constipation, osteoporosis, hypertension - Respiratory failure - most common cause of death

Answer 180

- MDT care. Specialist referral - CORTICOSTEROIDS - 1st line - Immunisations - influenza and pneumococcal - Physiotherapy - Vitamin D and bisphosphonates for bone health - Mobility help - wheelchair once cannot walk - Nutritional care - End-of-life directive and palliative set-up

Answer 181

- Beckers muscular dystrophy is very similar to Duchennes, however the DYSTROPHIN gene is LESS SEVERELY AFFECTED and maintains some of its function. The clinical course is less predictable than Duchennes - Symptoms only start to appear around 8-12 years. Some patient require wheelchairs in their late 20s or 30s. Others able to walk with assistance into later adulthood - Management is similar to Duchennes

Answer 182

- Disorder of neuromuscular transmission caused by impaired presynaptic release of acetylcholine. Rare disorder - Caused by an autoimmune attack on P/Q subtype of VOLTAGE-GATED CALCIUM CHANNELS (VGCCs). These VGCSs are responsible for assisting the release of acetylcholine into the synapse of the neuromuscular junction - Typically occurs in patients with SMALL-CELL LUNG CANCER - Presentation can be very similar to MYASTHENIA GRAVIS - be aware of this

Answer 183

- Insidious onset: - PROXIMAL MUSCLE WEAKNESS - DYSPHAGIA - EYELID PTOSIS (drooping) - DIPLOPIA - Depressed tendon reflexes - Gait changes - Dry mouth - Impotence in male patients

Answer 184

- Nerve conduction studies - Nerve stimulation using 50Hz - Serum test for VCGC antibodies - Detection of ACh receptor antibodies indicate Myasthenia gravis - MRI for malignancy. This should be done after 2 years if patient did not present with SCLC

Answer 185

- Initial treatments aimed at any cancers present - AMIFAMPRIDINE - improves muscle strength - Acetylcholinesterase inhibitors - Immunosuppression in severe cases, e.g. prednisolone - Plasmapheresis - Surgical intervention possible

Answer 186

- Myasthenia gravis is an autoimmune condition that causes muscle weakness that gets progressively worse with activity and improves with rest - Affects men and women of different ages - typical patients are either a woman under the age of 40 or a man over the age of 60 - There is a strong link between THYOMA (tumours of the thymus gland) and myasthenia gravis

Answer 187

- Caused by the binding of autoantibodies (85% - ACETYLCHOLINE RECEPTOR ANTIBODIES, 15% = MuSK and LRP4 antibodies) to POSTSYNAPTIC neuromuscular junction receptors - most commonly on the acetylcholine receptor - These antibodies also activate the COMPLEMENT system within the neuromuscular junction, leading to damage to cells at the postsynaptic membrane. This further worsens the symptoms

Answer 188

- Muscle weakness that is worse on exertion and gets better with rest - Almost all patients will have ocular manifestations at some point in the disease (DIPLOPIA, PTOSIS) - Weakness is more marked in proximal muscles. Weakness might be seen in: small muscle of the hands, deltoid and triceps muscles, bulbar muscles, muscles involved in chewing - No muscle wasting, sensation is unimpaired - Seizures can occur

Answer 189

- Diagnosis is mostly clinical - POSITIVE TENSILON/EDROPHONIUM TEST - muscles get stronger after being injected with Tensilon - Detection of ACh receptor antibodies (85%), MuSK (10%), LRP4 (5%) - Pulmonary function tests - TFTs - Crushed ice test - ice is applied to ptosis for 3 minutes, if it improves it is likely myasthenia gravis

Answer 190

- REVERSE ACETYLCHOLINESTERASE INHIBITORS - PYRIDOSTIGMINE = increases the amount of acetylcholine in the neuromuscular junction and improve symptoms - IMMUNOSUPPRESSION (e.g. prednisolone or azathioprine) suppresses the production of antibodies - THYMECTOMY can improve symptoms even in patients without a thymoma - Monoclonal antibodies, e.g. rituximab

Answer 191

- Ciprofloxacin - Azithromycin - Propranolol

Answer 192

- The painless, temporary loss of vision in one or both eyes - 5 causes: embolic, haemodynamic, ocular, neurological, and idiopathic

Answer 193

a) Embolic and haemodynamic causes can include: cardiac emboli, giant cell arteritis, SLE, thrombocytosis, malignancy hypotension b) Ocular causes can include: keratitis, closed-angle glaucoma, myopia, intraocular haemorrhage c) Neurological causes can include: optic neuritis, multiple sclerosis, migraine

Answer 194

- Most common cause is ATHEROSCLEROTIC EMBOLI - This results in the narrowing (stenosis) or occlusion of the INTERNAL CAROTID ARTERY or the CENTRAL RETINAL ARTERY, leading to hypoperfusion (i.e., low blood supply) of the retina

Answer 195

- Described as ‘black curtain coming down vertically into the field of vision’ - Vision loss, blurring, fogging, dimming - Can last seconds to hours (depends on cause)

Answer 196

- Clinical diagnosis due to transient nature. Full history - Ophthalmic exam - look for signs of ocular ischaemia - ESR level - CT head if indicated - More concerned with cause of episode

Answer 197

- Treat the underlying cause, e.g. anticoagulants - If left untreated, it can lead to a stroke

Answer 198

- Damage to one or more peripheral nerves. Results in transmission blockages between the PNS and CNS. Can be acute or chronic - Diabetes is the most common cause of peripheral neuropathy. Can also be caused by dietary deficiencies, medicines, alcohol excess, CKD, injury, infection, connective tissue disorders, inflammatory conditions, or some hereditary diseases - Axonal degeneration results from DM, B12 deficiency, lead poisoning, paraneoplastic syndrome - Segmental demyelination results from GBS, CMT

Answer 199

- Sensory: - Loss of touch, proprioception, temperature/pain sensation, paraesthesia - Numbness, tingling, burning/shooting pains - +ve Romberg test (patient cannot stand with feet together and eyes closed) - sensory ataxia - Motor: - Distal weakness - tripping, difficulty opening jars - Proximal weakness - difficulty climbing stairs - Muscle wasting - Fasciculations - Absent tendon reflexes

Answer 200

- Full history - past medical history, drug history, family history etc. - Bloods - glucose, B12, LFTs, TFTs, FBC, U&Es, ESR, immunoglobulins (?cause) - NERVE CONDUCTION STUDIES useful for classification - Electromyography - Nerve biopsy

Answer 201

- TREAT THE UNDERLYING CAUSE - Pregabalin or gabapentin for pain - Supportive therapy, e.g. walking aids

Answer 202

- Depression can describe both a mood and an illness. Major depressive disorder is a clinical syndrome involving mood, neurovegetative functions, cognition, and behaviour - it is characterised by at least 5 symptoms - Risk factors: prior depression, family history of depression, recent bereavement, stress, or medical illness, postnatal, dementia, corticosteroids - Clinical presentation: depressed mood, anhedonia, weight change, libido changes, sleep disturbance - Investigations: clinical diagnosis, metabolic panel, FBC, thyroid function tests - Treatment: moderate = antidepressant, e.g. citalopram, psychotherapy

Answer 203

- Painful rash caused by REACTIVATION of a nerve infection caused by the varicella-zoster virus. First presents as chickenpox in childhood, remains dormant in the dorsal root ganglia, and travels through peripheral sensory nerves to the skin - Less contagious than primary infection - Risks include: immunocompromised, HIV, malignancy

Answer 204

- Red, painful rash - dermatomal distribution. Most commonly cervical, trigeminal, thoracic and lumbar dermatomes - Fluid-filled blisters - Stabbing or burning pain - Fever, headache, fatigue - Itching

Answer 205

- Often a CLINICAL DIAGNOSIS - PCR test - CSF analysis - Bloods and cultures

Answer 206

- ORAL ANTIVIRAL THERAPY (1st line): acyclovir, valacyclovir, famciclovir - Immunocompromised patients should be treated with IV acyclovir - Analgesia - Antipyretics

Answer 207

- Inflammation of the brain parenchyma due to a viral infection (most commonly) - Can be caused by other conditions: TB, Lyme disease, toxoplasmosis, ticks - HSV-1 is the most common cause, others: CMV, EBV - Infections are most severe in children and the elderly

Answer 208

- TRIAD of FEVER, HEADACHE, and ALTERED MENTAL STATUS - Many present with signs of meningitis (stiff neck, vomiting, headache) - Symptoms of raised ICP - vertigo, nausea, severe headache, photophobia - If not caught, or caught too late, patient’s can rapidly slip into coma

Answer 209

- Bloods - FBC, U&Es, LFTs, TFS, B12, lactate etc. - CSF - viral PCR to detect specific virus - lymphocytosis with normal CSF:plasma glucose ratio - CT/MRI - often bilateral but asymmetrical (MRI preferred) - Blood cultures/gram stain

Answer 210

- Urgent hospital admission - IV ACICLOVIR STAT (1st line) as soon as encephalitis is suspected - Immediate antibiotics - IV Benzylpenicillin - Careful with fluids - cerebral oedema - ICU admission may be required with ventilation - Sedatives may be required

Answer 211

- Guillain-Barré syndrome is an “ACUTE PARALYTIC POLYNEUROPATHY” that affects the peripheral nervous system. It results in demyelination and axonal degeneration. It causes acute, symmetrical, ascending weakness and can also cause sensory symptoms - It is usually triggered by an infection (particularly ~6 weeks post-GI infection) and is particularly associated with to campylobacter jejuni, cytomegalovirus and Epstein-Barr virus - Increased incidence in males. Peak ages are 15-35, and 50-75

Answer 212

- Guillain-Barré is thought to occur due to a process called MOLECULAR MIMICRY - The B cells of the immune system create ANTIBODIES against the antigens on the pathogen that causes the preceding infection. These antibodies also match proteins on the nerve cells. They may target proteins on the MYELIN SHEATH of the motor nerve cell or the nerve axon

Answer 213

- Characterised by weakness, paraesthesia, and hyporeflexia - Sudden onset - TOES TO NOSE WEAKNESS. SYMMETRICAL ASCENDING WEAKNESS - Absent reflexes (LMN sign) - Neuropathic pain - can develop in the legs, back pain is rare - Peripheral loss of sensation - sensory loss in the lower extremities - Involvement of autonomic nervous system: reduced sweating, reduced heat tolerance, paralytic ileus - intestinal obstruction w/o blockage, urinary hesitancy

Answer 214

- Diagnosis is made CLINICALLY - BRIGHTON CRITERIA can be used - Other investigations: - Nerve conduction studies - Lumbar puncture. Raised protein \>0.55g/L. Normal WCC at \<5 WBC per mm^2. These results are called cyto-protein dissociation - Bloods - B12, FBC, U&Es, LFTs, TFTs etc. - Spirometry - to monitor respiratory function - ECG - identify any abnormalities & monitor

Answer 215

- IV IMMUNOGLOBULIN 0.44g/kg/day for 5 days (1st line) - Plasmapheresis (plasma exchange) - DVT prophylaxis - LMWH - AVOID CORTICOSTEROIDS - these can make the condition worse - Good prognosis - most fully recover (80%)

Answer 216

- Chronic traumatic encephalopathy is a progressive brain condition that's thought to be caused by repeated blows to the head and repeated episodes of concussion - It is particularly associated with contact sports, such as boxing or American football

Answer 217

- Similar to other types of degenerative brain disorders, particularly Alzheimer's: - Short-term memory loss - Changes in mood - Increasing confusion and disorientation - Difficulty thinking - As it progresses: dysarthria, significant memory problems, parkinsonism

Answer 218

- There is currently no test to diagnose CTE. A diagnosis is based on a HISTORY of participating in contact sports, plus the symptoms and clinical features - MRI/CT scan - may not show anything

Answer 219

Similar to dementia - supportive treatment

Answer 220

1. Atrial fibrillation – see AF, think stroke 2. Fibrillating atria will result in stasis and pooling of blood, particularly in the pockets of the auricular appendages. AF is irregularly irregular as electrical signals randomly make it across to the Bundle of His in the ventricles, and these contract at disorganised intervals. The stasis of the atrial blood (as per Virchow’s triad) will contribute to the likelihood of clot formation in the heart, which if dislodged may be carried as an embolus through the heart and into the aorta. The path of least resistance tends to be straight into the carotid arteries, though there’s a slight increase in the risk of thromboembolisms going elsewhere in the body.

Answer 221

Cushing's reflex. ## Footnote Raised intracranial pressure: Ischaemic/necrosing tissue creates an inflammatory response. This in turn perpetuates swelling and oedema, which in a large area of the brain is not very helpful and cerebral oedema will cause an increase in the intracranial pressure. If there’s a haemorrhagic stroke, a similar process occurs as frank blood coming in contact with cerebral neurons creates an inflammatory response, as well as bleeding simply taking up space in the brain and raising pressure. When the pressure reaches a point where CSF, venous system, and then arteries have all become squashed, the cerebral perfusion pressure in the arteries is not high enough to properly perfuse the brain, and it starts to become more ischaemic. The arterial smooth muscle will increase the blood pressure by vasoconstricting to try to reperfuse the brain, but the baroreceptors in the aorta will get stressed out with the blood pressure increase, and result in a bradycardia to calm the BP down.

Answer 222

- Multiple sclerosis (MS) is a CHRONIC and PROGRESSIVE condition that involves DEMYELINATION of the MYELINATED neurones in the central nervous system. This is caused by an INFLAMMATORY process involving the activation of IMMUNE CELLS against the MYELIN - Characterised by repeated episodes of inflammation of the nervous tissue in the brain and spinal cord - Female to male ratio; 2:1, \<50. Peak incidence is in young females - Causes not fully understood - genetic and environmental factors

Answer 223

- Results in the loss of the myelin sheath in the CNS due to inflammation. This slows or blocks the transmission of signal to and from the brain - Oligodendrocytes affected (CNS), NOT Schwann cells - 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. The key expression to remember to describe the way MS lesions change location over time is that they are "DISSEMINATED IN TIME AND SPACE"

Answer 224

There are 3 main patterns of MS: 1) Relapsing-remitting MS: symptoms come and go, periods of good health followed by sudden symptoms, most common presentation 2) Secondary progressive MS: follows from relaxing-remitting, gradually worsening symptoms with fewer remissions, ~50% of those with relapsing-remitting MS develop this 3) Primary progressive MS: from the beginning of the disease, symptoms gradually develop and worsen over time, 10-15% of people at onset

Answer 225

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

Answer 226

- Symptoms - TEAM: - Tingling - Eye: OPTIC NEURITIS (loss of vision in one eye, most common presentation). Also double vision (due to lesions in the SIXTH CRANIAL NERVE - two key phrases = INTERNUCLEAR OPTHALMOPLEGIA + CONJUGATE LATERAL GAZE DISORDER) - Ataxia - Motor: paraparesis

Answer 227

- Usually UMN signs - Loss of COLOUR VISION - LHERMITTE SIGN - electric jolt felt down the spine when flexing neck - UHTHOFF PHENOMENON - symptoms worse with heat, e.g. hot bath/exercise

Answer 228

- Symptoms must be disseminated in time (\>1 month apart) and space (clinically or MRI) - Gold standard - MRI BRAIN AND SPINAL CORD = GD-enhancing lesions - Lumbar puncture - Often a clinical diagnosis: McDonald Criteria - Bloods - B12, FBC, U&Es, LFTs, TFTs, HIV serology, calcium, glucose (?other causes)

Answer 229

MIMO: - Acute relapse - METHYLPREDNISOLONE (1st line) - Relapsing-remitting MS - immunomodulators, e.g, INTERFERON BETA 1A (1st line) - Secondary progressive - SIPONIMOD or METHYLPREDNISOLONE (1st line), CLADRIBINE (2nd line) - Primary progressive - OCRELIZUMAB (1st line)

Answer 230

- Trigeminal neuralgia is sudden, severe facial pain. It's often described as a sharp shooting pain or like having an electric shock in the jaw, teeth or gums - Two types: - Classic = caused by artery/vein compressing the trigeminal nerve (CN V) - Secondary = non-vascular lesion compressing the trigeminal nerve - Triggers: touching/moving tongue/lips/face, chewing, shaving, brushing teeth

Answer 231

- Pain paroxysms - last ONE-SEVERAL SECONDS, may REPEAT, usually UNILATERAL - Dull pain between paroxysms - Facial muscle spasms

Answer 232

- MRI/CT head - may identify lesion/vascular compression - Clinical diagnosis

Answer 233

- CARBAMAZEPINE (first line) - Surgery

Answer 234

Syncope is the term used to describe the event of temporarily LOSING CONSCIOUSNESS due to a disruption of blood flow to the brain, often leading to a fall. Syncopal episodes are also known as vasovagal episodes, or simply fainting

Answer 235

Syncope caused by a problem with the AUTONOMIC nervous system regulating blood flow to the brain - When the vagus nerve receives a strong stimulus, e.g change in temperature it can stimulate the parasympathetic nervous system. Parasympathetic activation counteracts the sympathetic nervous system, which keeps the smooth muscles in blood vessels CONSTRICTED - As the blood vessels delivering blood to the brain relax, the blood pressure in the cerebral circulation drops, leading to hypoperfusion of brain tissue. This causes the patient to lose consciousness and “faint”

Answer 236

- Patients often remember the event and can recall how they felt prior to fainting. This is called the PRODROME, and involves feeling: hot or clammy, sweaty, heavy, dizzy or lightheaded, vision going blurry or dark, headache - May be bit groggy but different to POST-ICTAL period after a seizure. May be INCONTINENCE with both seizures and syncopal episodes - A COLLATERAL history from someone that witnessed the event is essential. During a vasovagal episode they may describe the person: 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, there may be some twitching, shaking or convulsion activity, which can be confused with a seizure

Answer 237

- Primary syncope (simple fainting): dehydration, missed meals, extended standing in a warm environment, a vasovagal response to a stimuli, e.g. sudden surprise, pain or the sight of blood - Secondary causes: hypoglycaemia, anaemia, infection, anaphylaxis, arrhythmias, valvular heart disease, hypertrophic obstructive cardiomyopathy

Answer 238

- Syncope: prolonged upright position before the event, lightheaded before the event, sweating before the event, blurring or clouding of vision before the event, reduced tone during the episode, return of consciousness shortly after falling, no prolonged post-ictal period - Seizure: epilepsy aura (smells, tastes or deja vu) before the event, head turning or abnormal limb positions, tonic clonic activity, tongue biting, cyanosis, lasts more than 5 minutes, prolonged post-ictal period

Answer 239

- Full history and examination. Are there any physical injuries as a result of the faint, for example a head injury? Is there a concurrent illness, for example an infection or gastroenteritis? - ECG, particularly assessing for arrhythmia and the QT interval for long QT syndrome - 24 hour ECG if paroxysmal arrhythmias are suspected - Echocardiogram if structural heart disease is suspected - Lying and standing blood pressure - Bloods

Answer 240

- ECG, particularly assessing for arrhythmia and the QT interval for long QT syndrome. 24 hour ECG if paroxysmal arrhythmias are suspected - Echocardiogram if structural heart disease is suspected - Bloods, including a full blood count (anaemia), electrolytes (arrhythmias and seizures) and blood glucose (diabetes)

Answer 241

- SEIZURES or UNDERLYING PATHOLOGY need to be managed by an appropriate specialist - Once a simple vasovagal episode is diagnosed, reassurance and simple advice can be given to: - Avoid dehydration + missing meals - Avoid standing still for long periods - When experiencing prodromal symptoms such as sweating and dizziness, sit or lie down, have some water or something to eat and wait until feeling better

Answer 242

- Inflammation of the meninges. Notifiable disease - Can be caused by a virus, bacteria or fungi - Bacterial meningitis is often more serious than viral. Viral is more common - Infants and teenagers are most at risk - Risk factors: immunosuppression, smoking, CSF shunts, DM, IVDU, adrenal insufficiency, malignancy, sickle cell disease, and crowding (university!)

Answer 243

- Neonates = GROUP B STREP. - Babies, young children and adults = STREPTOCOCCUS PNEUMONIAE - Teens and young adults = NEISSERIA MENINGITIDIS

Answer 244

- HERPES SIMPLEX VIRUS - ENTEROVIRUS - VARICELLA ZOSTER VIRUS

Answer 245

- Non-specific symptoms: fever, headache, vomiting, nausea, lethargy, irritability, muscle pain, chills, shivering, sore throat - Stiff neck - Photophobia - NON-BLANCHING RASH, i.e. do not disappear (meningococcal only - Neisseria meningitidis) - KERNIG'S SIGN (pain on passive knee extension) - BRUDZINSKI'S SIGN (patient lying flat, lift their head and flex their chin to their chest - this causes them to involuntarily flex their hips and knee) - Raised ICP - decreased GCS, seizure, focal deficits - Septic shock

Answer 246

- If suspected do NOT delay treatment - LUMBAR PUNCTURE - CONTRAINDICATED with raised ICP, shock - Bloods - cultures, FBC, U&Es, glucose, lactate - ABG

Answer 247

- Bacterial: appearance = cloudy, opening pressure = elevated, WBC = high \>1000-2000 (neutrophils), protein = high \>200, glucose = low \<40 - Viral: appearance = clear, opening pressure = normal, WBC = low \<300 (lymphocyte), protein = mildly raised or normal \<200, glucose = normal - Fungal: appearance = normal, opening pressure = normal/elevated, WBC = \<500, protein = high \>200, glucose = normal/low - TIP: interpreting lumbar puncture results is a common exam question. It is easier to think about what will happen to the CSF with bacteria or viruses living in it rather than trying to rote learn the results. It makes sense that bacteria swimming in the CSF will release proteins and use up the glucose. Viruses don’t use glucose but may release a small amount of protein. The immune system releases neutrophils in response to bacteria and lymphocytes in response to viruses

Answer 248

- ABCDE + support - Empirical therapy = IV Benzylpenicillin - Assess GCS - FIRST LINE = CEFTRIAXONE/Cefotaxime. Add IV BENZYLPENICILLIN for RASH - CONTACTS = single dose of CIPROFLOXACIN - Penicillin allergy = CHLORAMPHENICOL - Immunocompromised (risk of listeria) = Amoxicillin / Ampicillin

Answer 249

- Viral meningitis = ACYCLOVIR - Prophylaxis = RIFAMPICIN and CIPROFLOXACIN

Answer 250

- An umbrella term for a variety of neurodegenerative disorders selectively affecting motor neurons (mostly the anterior horn cells of the spinal cord and the motor cranial nuclei). Can affect upper or lower motor neurons. NO sensory problems as no effect on sensory neurones - Mostly sporadic. Few familial variants - SOD1 gene, C90RF72 gene. Men slightly more affected; 3:2 - The majority of cases are AMYOTROPHIC LATERAL SCLEROSIS (ALS), however there are 3 other categories of MND (progressive muscular atrophy (PMA), primary lateral sclerosis (PLS), progressive bulbar palsy (PBP))

Answer 251

- Results in a mixed UMN and LMN presentation - LMN signs typically predominate, e.g. weakness, atrophy, muscle fasciculations - Onset in the limb is the typical presentation: - Look for wrist/foot drop - Change in appearance of hands - wasting - Gait disorders/tendencies to trip - Excessive fatigue

Answer 252

- El Escorial Criteria: 1) Presence of: LMN degeneration by clinical, electrophysiological, or neuropathological examination; UMN degeneration by clinical examination; progressive symptoms as determined by the history 2) Absence of: electrophysiological and pathological evidence of other disease processes; neuroimaging evidence of disease processes

Answer 253

- MDT Care - Supportive therapy - Regular physical and occupational therapy - Speech and language therapy - Diet and nutrition support - Non-invasive ventilation - RILUZOLE - neuroprotective glutamate-release inhibitor; life-prolonging treatment - End of life care and advance directives - Prognosis - average survival post-diagnosis is 2-4 years

Answer 254

- Progressive disease characterised by degeneration of the motor neurons with cortical, brainstem, and ventral cord locations - Most common form of MND ~50% - Some familial inheritance with the SOD1 and C90RF72 genes - Common ages of onset 40-50 (familial) and 58-63 (sporadic)

Answer 255

- Presents with signs of degeneration of upper and lower motor neurons (upper extremity weakness, stiffness, with poor-cordination and balance, unsteady gait). Asymmetric onset - Babinski sign +ve - Fasciculations of the tongue - Any corticobulbar signs indicate a worse prognosis: - Brisk jaw reflex - Dysarthria (difficulty speaking) - Dysphagia (difficulty swallowing) - Sialorrhoea (excess salivation)

Answer 256

- Diagnosis via the El Escorial Criteria - There are no specific investigations for MND but tests include: - Nerve conduction studies - EMG - CT or MRI brain and spinal cord (?other cause) - Bloods - B12, folate, HIV serology, Lyme disease serology, creatinine kinase, serum protein electrophoresis (?other causes) - Muscle biopsy

Answer 257

- MDT Care - Supportive therapy, physical therapy - Diet and nutrition care - RILUZOLE - life prolonging medication - Quinine/baclofen - cramps - Hyoscine - sialorrhoea - PEG tube for nutrition - NIV - respiration help

Answer 258

- Around 2 in 10 people with MND have PBP - Bulbar = medulla oblongata (ORIGIN OF CRANIAL NERVES 9-12) - WORST PROGNOSIS

Answer 259

- UMN and LMN - First affected muscles are those used for talking, chewing, and swallowing - Present with palsy of the tongue, difficulty swallowing and palsy of facial muscles

Answer 260

- Diagnosis via the El Escorial Criteria - There are no specific investigations for MND but tests include: - Nerve conduction studies - EMG - CT or MRI brain and spinal cord (?other cause) - Bloods - B12, folate, HIV serology, Lyme disease serology, creatinine kinase, serum protein electrophoresis (?other causes) - Muscle biopsy

Answer 261

- MDT Care - Supportive therapy, physical therapy - Diet and nutrition care - RILUZOLE - life prolonging medication - Quinine/baclofen - cramps - Hyoscine - sialorrhoea - PEG tube for nutrition - NIV - respiration help

Answer 262

- ALS: UMN and LMN, progressive paralysis, eventual respiratory failure, SOD1, C90RF72, ALS2, SETX, VAPB, ANG - PBP: LMN in the brainstem, pharyngeal muscle weakness, progressive loss of speech, tongue atrophy, none - PLS: UMN of the arms, legs and face, movements become slower, pseudobulbar effect, UMN signs, none - PMA: progressive degeneration of only the LMNs, muscle wasting, clumsy hand movements, fasciculations, muscle cramps, LMN only, none

Answer 263

- Progressive muscular atrophy: anterior horn cells affected, LMN ONLY; distal muscle affected first then proximal - Primary lateral sclerosis: rare, progressive tetraparesis (UMN ONLY)

Answer 264

- Olfactory (I): may notice altered taste, rather than a loss of sense of smell. Disorders can manifest as anosmia, hyposmia, distortions (dysosmias), or spontaneous olfactory hallucinations (phantosmias) - Optic (II): typically produce monocular visual loss, which can be sudden or gradual, and may or may not be associated with pain - Occulomotor (III): controls most extra-ocular muscles. Present with paralysis of adduction, elevation, and depression, and when the pupil is involved a large unreactive pupil is noted. This presentation can suggest serious neurological disorders - Trochlear (IV): innervates the superior oblique muscle, which controls depression, intorsion, and adduction of the eye. It is the most common cause of vertical diplopia - Trigeminal (V): biggest cranial nerve (opthalmic (V1), maxillary (V2) and mandibular (V3)). Symptoms depend on the location and aetiology of the lesion and may include loss of sensation in the distribution of one or more trigeminal nerve branches, neuropathic pain, or weakness of the muscles of mastication - Abducens (VI): innervates the lateral rectus muscle and controls abduction. Patients typically present with horizontal double vision - Facial (VII): most common cranial nerve mononeuropathy. There are many aetiologies, and the most important initial step is to rule out central causes of facial weakness, including ischaemic stroke and pontine neoplasms. The most common cause of a peripheral facial palsy is BELL'S PALSY - Vestibulocochlear (VIII): symptoms of dysfunction include hearing loss, tinnitus, and vertigo - Glossopharyngeal (IX): isolated glossopharyngeal neuropathy is rare, as lesions often involve other cranial nerves in close proximity (VIII, X, XI, and XII). Additionally, isolated palsy of the glossopharyngeal nerve can often be asymptomatic, due to redundant innervation of target structures by other cranial nerves - Vagus (X): can result in hoarseness, dysphagia, and dyspnoea, as well as palatal droop and deviation of the uvula to the contralateral side - Accessory (XI): trapezius weakness results in a drooping shoulder at rest and mild scapular winging with attempted shoulder elevation and arm abduction \>90°. When the patient shrugs his or her shoulders against resistance, unilateral weakness may be detected. SCM weakness results in difficulty when turning the head in the opposite direction to the injury. To test, the patient is asked to turn his or her head to the side against resistance - Hypoglossal (XII): unilateral or bilateral tongue weakness with deviation towards the affected side on tongue protrusion, tongue atrophy (with scalloping or accentuation of the midline groove), fasciculation of the tongue at rest, tongue flaccidity, or the inability to move the tongue rapidly from side to side or vertically

Answer 265

- Bell’s palsy is an acute, sudden-onset, unilateral facial palsy of probable viral aetiology - Risk factors: age, diabetes mellitus, pregnancy (third trimester)

Answer 266

- Can be remembered by BELLS Palsy: - Blink reflex abnormal - Ear sensitivity - Lacrimation - deficient or excess - Loss of taste - Sudden onset - Palsy (CN VII muscles)

Answer 267

- UMN: transmits information from brain to spinal cord or brainstem - LMN: transmit signals from spinal cord to skeletal muscle

Answer 268

- Damage to the brain: stroke, infection, tumour - Injury to the white matter of the spinal cord

Answer 269

- Injury to the axons leaving the spinal cord - Injury to the ventral grey matter of the spinal cord

Answer 270

- HYPERTONIA (everything goes up) - Spasticity - Clonus (involuntary muscle contractions) - Overactive reflexes, tight muscles - Babinski sign --\> dorsiflexed toe

Answer 271

- HYPOTONIA (everything goes down) - Muscle ATROPHY - Reduced reflexes - Fasciculations (muscle twitching)

Answer 272

C. Lumbar puncture

Answer 273

d. Nimodipine

Answer 274

To look for cardiac causes of thrombotic emboli * Atrial fibrillation * Myocardial infarction in the past You may also want to do an echocardiogram to look for valvular disease, heart muscle abnormalities, mural thrombi and infective endocarditis

Answer 275

- The stroke has not yet been confirmed as ischaemic - The stroke has been confirmed as haemorrhagic - It has been more than 4.5 hours since the onset of their symptoms - They/their family decline thrombolytic therapy - Contraindications: recent surgery, recent trauma, previous CNS bleed, stroke is mild/non-disabling, AVM or aneurysm is present, severe liver disease, serious head injury or stroke in past 3 months, recent GI or GU haemorrhage, known clotting disorder, on anticoagulants, low platelet count High BP

Answer 276

b. Middle meningeal artery

Answer 277

- IV mannitol - to reduce ICP, if midline shift or hydrocephalus is seen on CT scan - Urgent neurosurgery - to remove the clot and ligate the bleeding vessel - Intubation & ventilation

Answer 278

e. Dizziness and loss of coordination for 10 minutes

Answer 279

e) Cluster headache

Answer 280

d) Lack of sleep

Answer 281

c) Sumatripan

Answer 282

b) Hyperhidrosis

Answer 283

a) Tonic seizure generalised seizure

Answer 284

e) Lamotrigine

Answer 285

D) Stop her clozepine. This is drug induced Parkinsonism. Bradykinesia and a resting tremor are present, however the dopamine antagonist should be stopped before starting her on any other treatments.

Answer 286

B) Vascular dementia. This is classic of vascular dementia. The patient is showing memory problems and behavioural disturbance. He also had a stroke and the history is indicating a stepwise progression

Answer 287

C) X-ray. This is Brown-Sequard syndrome. The patient needs a pain radiograph (X-ray) to assess the damage of the penetrating wound. An MRI might be useful after the initial results, but would not be first-line

Answer 288

E) Advise she rest the wrist and avoid gripping objects. This is a case of Carpal Tunnel Syndrome. As the patient is pregnant, it is very likely that the condition will resolve itself postpartum. For the time being, she should rest the affected wrist

Answer 289

C) Dementia with Lewy Bodies

Answer 290

A) Increased CAG repeats. Huntington’s Disease is an autosomal dominant condition that results in the increase of CAG repeats on the Huntington gene on chromosome 4p16.3

Answer 291

E) Surgical decompression. This is a classic presentation of Cauda Equina syndrome. This is a medical emergency that requires immediate surgical decompression. The other options are all possible treatments if required post-operatively

Answer 292

A) Relapsing-remitting. This patient has symptoms that come and go - this is relapsing-remitting MS

Answer 293

D) Guillain-Barre syndrome. The patient has the classic ‘toe to nose’ weakness, and had a GI infection less than 6 weeks ago. He also has absent reflexes, which is another sign of GBS

Answer 294

C) Riluzole. This patient is suffering from ALS. The symptoms are a mixture of upper and lower motor neuron signs, and the onset was asymmetrical. Riluzole is the life-prolonging drug for MND patients. Natalizumab and interferon beta are multiple sclerosis treatments

Answer 295

D) Lactate

Answer 296

A) Bacterial. There are 2 clues here. First there is a non-blanching rash, which only occurs with bacterial infection. Secondly, the results of the CSF analysis indicate a bacterial cause. It is cloudy, with raised pressure and high WBCs

Answer 297

B) Impaired presynaptic release of acetylcholine. Lambert-Eaton Syndrome is caused by impared presynaptic release of acetylcholine due to damage of voltage-gated calcium channels

Answer 298

E) Over 60 years old. The correct criteria is over 50 years old. All of the others are part of the American College of Rheumatology Classification

Answer 299

B) Reactivation of VZV that was dormant in the dorsal root ganglia. Shingles is caused by the reactivation of the varicella-zoster virus (VZV) that has been dormant in the dorsal root ganglia since the primary infection. CMV = Cytomegalovirus EBV = Epstein-Barr Virus

Answer 300

C) Immediate 70mg prednisolone and urgent referral to rheumatology. ## Footnote D not unreasonable but no indication of eye involvement (yet) If there is any indication of current or previous eye-involvement then they need to go straight to an ophthalmologist but that isn’t the case here. In reality the most important thing is to get them started on high dose pred and get them into hospital (which specialists need to do what and when can then be sorted)

Answer 301

c) IV phenytoin

Answer 302

C. Meningitis

Answer 303

A. Bacterial B. Fungal and TB C. Viral

Answer 304

A. Tension headaches

Answer 305

E. Topiramate

Answer 306

A. Giant cell arteritis B. CRP/ESR for inflammation C. Temporal artery biopsy D. Prednisolone E. Blindness if the inflammation spreads to the optic arteries

Answer 307

A. Generalised seizure B. Another course of IV lorazepam 10 to 20 minutes after the first; if this doesn't work give phenytoin C. No. Patient needs to be investigated for all other causes first. If she is found to have TWO seizures of unknown cause = epilepsy D. Lamotrigine. She is pregnant

Answer 308

D. Focal seizure

Answer 309

A. Order a CT scan

Answer 310

D. Lacunar stroke Aetiological causes of TIA/stroke: large artery atherosclerosis, cardioaortic embolism, small vessel occlusion, undetermined

Answer 311

A. Urgent non-contrast CT head 5 complications: hydrocephalus, vasospasm, seizures, rebleeding, electrolyte abnormalities

Answer 312

D. Rupture of bridging veins 4 risk factors: alcoholism, old age, trauma, anticoagulants use or bleeding disorders

Answer 313

A. Frontotemporal dementia ## Footnote Histopathlogy of AD: amyloid/senile plaque, neurofibrillary tangles, cortical atrophy, loss of Ach production

Answer 314

A. Methylprednisolone 3 types of MS: relapsing-remitting, primary progressive, secondary progressive

Answer 315

1. Symptoms of raised ICP, progressive neurological deficit, epilepsy, lethargy 2. Brain, liver, adrenal, bone 3. Bowel, bladder, breast, renal cell carcinoma 4. Prostate, kidney, breast, thyroid, bone

Answer 316

C. Forced Vital Capacity

Answer 317

B. Antibodies to post-synaptic acetylcholine receptors (Anti-ACh)

Answer 318

a) Upward gaze fatigability b) CT neck scan - thymoma

Answer 319

A. Median neuropathy

Answer 320

e) Common peroneal nerve palsy - most common cause of foot drop

Answer 321

A. DAT scan. You would expect to see degeneration of substantia nigra B. Lewy bodies C. Bradykinesia, rigidity D. Drug-induced, multiple system atrophy E. Levodopa: postural hypotension, dyskinesia (involuntary movements), dry mouth, drowsiness Dopamine agonist (ropinirole, cabergoline, bromocriptine): compulsive gambling (impulsivity), drowsiness, associated with pulmonary fibrosis Monoamine Oxidase B inhibitor (MOA-B inhibitor), e.g. selegiline F. Essential tremor