Neuro (3a Cards)

Question 1

what is a cluster headache?

Answer 1

severely disabling episodic headaches.

more common in men and smokers.

Question 2

how do cluster headaches present?

Answer 2

rapid onset excruciating pain around one eye
eye becomes watery, bloodshot, lids swollen, lacrimation
facial flushing
rhinorrhoea
pain is strictly unilateral, will usually affect one side only
course - headaches last 15-60min, once or twice a day, often nocturnal
clusters last 4-12 weeks, then they might have months/years before next cluster

Question 3

how do you treat cluster headaches?

Answer 3

acute attack - 100% oxygen for 15 mins via non-rebreathe, sumitriptan SC or nasal spray at onset (nasal spray not licensed).
preventative - verapamil (start at 40mg and build up to 960mg max, requires ECG monitoring for AV block once at high doses/whilst raising dose)
prednisolone (second line, but can be preferred as started at top dose - then quickly wean off! may relapse)
lithium - if verapamil not tolerated, requires lots of monitoring
other options:
melatonin
topiramate
sodium valproate

Question 4

give some possible triggers for cluster headaches

Answer 4

• alcohol precipitates attacks when in a cluster, but can be drunk between clusters
• histamine and nitroglycerine
• for some patients - heat, exercise and solvents
• disruption to sleep patterns (e.g. by shift work, jet lag, etc)

Question 5

list some headache red flags that should prompt further investigation

Answer 5

• change in pattern of headache.
• new headache at age > 50.
• onset of seizures.
• headache with systemic illness.
• personality change.
• symps suggestive of raised ICP (morning headache, headache with coughing, sneezing, straining).
• acute onset of the worst headache ever (possible intracranial aneurysm).

Question 6

list some differentials to consider for an acute single episode of headache

Answer 6

with meningism (stiff neck etc):
  - meningitis
  - encephalitis
  - subarachnoid haemorrhage
head injury
sinusitis
glaucoma (acute closed-angle)
tropical illness e.g. malaria, typhus

Question 7

what features of an acute single episode of headache would make you consider meningitis?

Answer 7

meningism - acute, severe headache felt all over with neck stiffness.
fever, photophobia, purpuric rash (although not always!)

admit urgently for CT head/LP if CT negative

Question 8

what features of an acute single episode of headache would make you consider encephalitis?

Answer 8

neck stiffness, fever, odd behaviour, fits, reduced consciousness
admit urgently for CT head/LP if CT negative

Question 9

what features of an acute single episode of headache would make you consider subarachnoid headache?

Answer 9

SUDDEN ONSET, ‘worst ever’ headache, often occipital (been kicked in back of head), stiff neck, focal signs, reduced consciousness
admit urgently for CT head/LP if CT negative

Question 10

what features of an acute single episode of headache would make you consider head injury? what would prompt you to consider further investigations?

Answer 10

hx of trauma!
pain usually around site of trauma, but can be generalised.
CT head to exclude subdural/extradural haemorrhage if drowsiness ± lucid interval or focal signs.

Question 11

what features of an acute single episode of headache would make you consider sinusitis? how do you treat it?

Answer 11

dull, constant ache over frontal or maxillary sinuses, with tenderness ± postnasal drip. pain worse on bending over. preceding coryzal symptoms. lasts 1-2 weeks.
if bacterial (likely Strep pneumoniae, haemophilus influenzae, moraxella catarrhalis) treat with abx - amoxicillin - although some evidence says this doesn't achieve anything.

Question 12

give some home treatment measurers you would advise for a patient with acute sinusitis

Answer 12

• paracetamol/ibuprofen for pain/fever.
• intranasal decongestant (oral is not recommended for sinusitis) for a max of a week.
• nasal irrigation with warm saline solution.
• warm face packs, which may provide localised pain relief.
• adequate fluids and rest.

Question 13

what features of an acute single episode of headache would make you consider acute angle-closure glaucoma?

Answer 13

elderly, long-sighted people
constant, aching pain develops round one eye, radiating to forehead.
markedly reduced vision, visual haloes, N&V.
red congested eye, cloudy cornea, dilated non-responsive pupil (may be oval), reduced acuity.

seek urgent expert help, if delay in treatment >1hr likely start acetazolmide 500mg IV, plus lay supine and give any topical agents not contra-indicated in the patient
topical agents inlude:
Beta-blockers - eg, timolol, cautioned in asthma.
Steroids - prednisolone 15 every 15 minutes for an hour, then hourly.
Pilocarpine 1-2% (in patients with their natural lens).
Phenylephrine 2.5% (in patients who do not have their own lens).

Question 14

what are tension headaches?

Answer 14

main cause of bilateral, non-pulsatile headache ± scalp muscle tenderness, with no vomiting/sensitivity to movement. often described as band round scalp, pressure, tightness.

divided into

• episodic TTH. This occurs on fewer than 15 days each month. It can evolve into the chronic variety.
• chronic TTH. This occurs on more than 15 days each month and has all the features of the episodic TTH.

chronic is more likely due to medication overuse.

Question 15

how should you manage tension type headache?

Answer 15

reassurance, advise on stress management, hydration, risk of medicines overuse.
drug therapy - avoid codeine, ibuprofen first line (OTC), naproxen second. can try paracetamol but usually not as effective.
if nothing’s working - try amitriptyline.

Question 16

what are the characteristic features of headache due to raised ICP?

Answer 16

worse on waking, lying, bending forward, coughing.
vomiting, papilloedema, odd behaviour, false localising signs.
must image before do LP - contraindicated otherwise!

Question 17

what is a medication overuse headache?

Answer 17

chronic daily headache (> 15 days per month, with opiate/triptan use on >10 or paracetamol/NSAIDs on >15) due to overuse of analgesics - mixed opiates and paracetamol (Cocodamol) common culprit.

Question 18

how do you manage a medication overuse headache?

Answer 18

reassurance/education.
withdrawal of analgesia - warn patient headache will worsen initially.
headache should resolve in 10 days or so.

Question 19

what are the symptoms of trigeminal neuralgia? what are the common triggers?

Answer 19

paroxysms of intense stabbing pain, lasting seconds, in trigeminal nerve distribution (facial).
unilateral, typically maxillary/mandibular.
may have preceding symptoms e.g. tingling, numbness.
triggers - washing affected area, shaving, eating, talking, dental prostheses.
typical pt >50yo, woman (F:M = 2:1)

Question 20

what are some red flags in a trigeminal neuralgia history that should prompt referral/further investigation?

Answer 20

Sensory changes, deafness or other ear problems.
Difficulty achieving pain control, poor response to carbamazepine.
History of any skin lesions or oral lesions that could lead to perineural spread.
Ophthalmic division only or bilateral as suggestive of benign or malignant lesions or multiple sclerosis.
Age of onset under 40 years.
Optic neuritis.
Family history of multiple sclerosis.

if investigation needed it’d normally be a brain MRI.

Question 21

list some possible causes of secondary trigeminal neuralgia

Answer 21

• compression by anomalous or aneurysmal intracranial vessels or a tumour
• chronic meningeal inflammation
• MS
• varicella zoster
• skull base malformation e.g. Chiari malformation

MRI needed to exclude these causes.

Question 22

how do you treat trigeminal neuralgia?

Answer 22

carbamazepine PO at 100mg every 12hrs, max 400mg/6hrs.

then try lamotrigine, phenytoin or gabapentin.
if drugs fail, surgery - rhizotomy, microvascular decompression, stereotactic gamma knife surgery can work.

Question 23

list some differential diagnoses for facial pain

Answer 23

migraine
trigeminal neuralgia
post-herpetic neuralgia - shingles
cervical disk pathology
sinusitis
bone neoplasia
temperomandibular joint dysfunction
dental problems
giant cell arteritis
cluster headaches
MS

Question 24

what is giant cell arteritis?

Answer 24

needs excluding in anyone >50yrs with headache lasting a few weeks.
systemic immune-mediated vasculitis affecting medium-sized and large-sized arteries, particularly the carotid artery and its extracranial branches. associated with PMR in 50%.
can cause sudden bilateral vision loss so considered an emergency.

Question 25

how does giant cell arteritis present?

Answer 25

recent onset temporal headache, myalgia, fever. temporal artery and scalp tenderness (e.g. when combing hair), jaw claudication (pain comes on gradually when chewing), amaurosis fugax or sudden blindness. extracranial symps - dyspnoea, morning stiffness, unequal/weak pulses.

Question 26

how do you manage a patient presenting with possible giant cell arteritis?

Answer 26

do an ESR blood test and give high dose (40mg) prednisolone immediately - if claudication give 60mg, if visual symptoms admit for IV methylpred. other Ix - CRP, platelets, alk phos all raised, might see anaemia. get a *temporal artery biopsy* within 7 days of starting treatment. typically a 2 yr course before it resolves - reduce pred once symps controlled and ESR reduced, but don't be afraid to bump it back up as necessary.

Question 27

how do migraines present?

Answer 27

- visual (or other) aura for 15-30 mins before onset within 1hr of severe, throbbing, unilateral headache - headache without aura - aura without headache - headache is incredible severe with N&V, photo/phonophobia - lasts 4-72h

Question 28

what are the criteria for diagnosing a headache as migraine if there's no aura?

Answer 28

5+ headaches lasting 4-72hrs plus N&V or photo/phonophobia plus any 2 of the following: - unilateral - pulsating - impairs usual activity

Question 29

what are the possible triggers for migraine?

Answer 29

``` CHOCOLATE: Chocolate Hangovers Orgasms Cheese Oral contraceptives Lie-ins Alcohol Tumult Exercise ```

Question 30

what kinds of aura may be experienced in migraine?

Answer 30

visual - chaotic distorting lines/shapes/patterns, hemianopia somatosensory - paraesthesiae spreading from fingers to face motor - dysarthria and ataxia (basilar migraine), ophthalmoplegia or hemiparesis speech - dysphasia or paraphasia

Question 31

how do you manage/treat migraines? (not prophylaxis)

Answer 31

- reassurance, education, encourage headache diary and identification of triggers, lifestyle (stress, diet and exercise, sleep) 1) soluble aspirin or ibuprofen, prochlorperazine buccal for anti-emetic if needed 2) rectal diclofenac and domperidone (anti-emetic) - not often used due to patient preference! 3) triptans or ergotamine - 'abortive' so must be taken immediately after onset of headache/aura MUST stop COCP - stroke risk

Question 32

list some contra-indications for triptans

Answer 32

People with uncontrolled hypertension. People with coronary heart disease or cerebrovascular disease People with risk factors for coronary heart disease or cerebrovascular disease People with coronary vasospasm (Prinzmetal's angina)*.

Question 33

what are the options for migraine prophylaxis?

Answer 33

offer if 2+ per month, impacting daily activity for 3+ days each time 1) beta blockers e.g. atenolol 2) amitriptyline 3) topiramate, sodium valproate (these are started in specialist care) 4) pizotifen 5) 12 weekly botulinum toxin A injections

Question 34

list some differentials for stroke (there's bloody loads)

Answer 34

``` head injury hypo/hyperglycaemia subdural haemorrhage intracranial tumours hemiplegic migraine epilepsy (Todd's palsy) CNS lymphoma pneumocephalus Wernicke's encephalopathy hepatic encephalopathy herpes encephalitis abscesses ```

Question 35

what are the two main types/mechanisms of stroke?

Answer 35

haemorrhagic vs infarction ``` arterial embolism (infarction) – from a distant site; e.g. carotids, vertebral or basilar arteries. embolus occludes an artery of brain resulting in infarction. may also come from heart valves in endocarditis. Haemorrhage – can be in the cerebrum itself, or also a subarachnoid haemorrhage may cause a similar effect ```

Question 36

list some uncommon causes of stroke

Answer 36

``` Venous infarct Carotid/vertebral artery dissection (spontaneous or from neck trauma) Polycythemia Fat / air embolism – e.g. in divers MS – demyelinating plaque may act as an embolus Mass lesions (e.g. tumour) Migraine Thrombocythaemia and thrombophilia Venous sinus thrombosis Vasculitis Amyloidosis Drugs – particularly cocaine and OTC cold remedies that contain vasoconstrictors. Sepsis - sudden BP drop ```

Question 37

list some modifiable risk factors that can reduce the risk of a stroke

Answer 37

``` mostly affect risk of infarctive stroke: HTN smoking DM heart disease - valvular, ischaemic, AF peripheral vascular disease past TIA carotid bruit COCP hypercholesterolaemia alcohol misuse clotting disorders syphilis ```

Question 38

what is CADASIL?

Answer 38

rare, inherited cause of stroke and vascular dementia. Caused by a defective NOTCH3 gene multiple small infarcts in the brain. Often presents as migraine and depression in teenagers, and by the 20’s and 30’s there are often TIA’s and strokes. Dementia may follow after age 40-50

Question 39

list the main causes of infarctive stroke

Answer 39

60% – Atherosclerosis of the carotid arteries and aortic arch 20% – valvular heart disease 20% – disease of the vessels in the brain itself

Question 40

what symptoms would you expect to see for a middle cerebral artery infarction?

Answer 40

most common stroke presentation! Contralateral: Hemiparesis Hemiplegia (initially flaccid, becomes spastic) Limbs usually floppy, and reflexes reduced/absent Facial weakness (not always) Hemianopia – visual field defect in which vision is lost in half of the visual field in one/both eyes. Aphasia – when the dominant hemisphere is affected - ask about left and right handedness! symptoms develop rapidly, over a period of minutes, or less commonly they can develop over a few hours.

Question 41

what symptoms/signs indicate a cerebral infarct rather than brainstem/lacunar?

Answer 41

``` depending on site - contralateral sensory loss contralateral hemiplegia - initially flaccid (floppy limb falls like dead weight when lifted), then spastic (UMN signs) dysphasia homonymous hemianopia visuo-spatial deficit ```

Question 42

what symptoms/signs indicate a brainstem infarct rather than cerebral/lacunar?

Answer 42

wide range of effects, incl (features - location): Hemi/tetraparesis - Corticospinal tracts Sensory Loss - Medical lemniscus / spinothalamic tract Diplopia - Occulomotor nuclei Facial Numbness - 5th nerve nuclei Facial weakness - 7th Nerve nucleus Nystagmus and vertigo - Vestibular connections Dysphagia / dysarthria - 9th/10th nuclei Horner’s syndrome - Sympathetic fibres Altered consciousness - Reticular formation

Question 43

what symptoms/signs indicated lacunar infarct rather than cerebral/brainstem? where are these infarct occurring?

Answer 43

``` occurring in brainstem, internal capsule, thalamus, pons 5 main syndromes: 1) ataxic hemiparesis 2) pure motor 3) pure sensory 4) sensorimotor 5) dysarthria (clumsy hand) ``` may be symptomless, or present with very localised symptoms (as in pure motor/sensory) due to the very localised effect it has. consciousness/cognition stay intact (except in thalamic stroke)

Question 44

give some info on the use of thrombolysis in acute stroke

Answer 44

must be sure it's non-haemorrhagic (imaging) and that you are acting within 4.5 hours of onset of symptoms (so if they woke up with it, you can't do it!) and there's no CIs. IV recombinant tissue plasminogen activator (tPA) e.g. alteplase. must do CT 24hrs post-thrombolysis to check for bleeds.

Question 45

give some absolute contra-indications for thrombolysis

Answer 45

Previous intra-cranial haemorrhage / haemorrhagic stroke Major surgery or trauma <2 weeks Active internal bleed (excluding menses) Prolonged / traumatic CPR Pregnancy, or <18 weeks postnatal Severe liver disease / known oesophageal varices Hypertension >200/120 Cerebral neoplasm Previous allergy Head/face trauma < 3months Previous ischaemic stroke < 3 months (except current episode)

Question 46

give some relative contra-indications for thrombolysis

Answer 46

``` Hx of severe (>200/120 hypertension) Peptic Ulcer Anticoagulant therapy Infective Endocarditis Known coagulation disorder ```

Question 47

what symptoms indicate an anterior cerebral artery infarct?

Answer 47

Weak leg (± shoulder), on the contralateral side

Question 48

what symptoms indicate a MCA infarct?

Answer 48

Weak arm and face on the contralateral side. Hemiplegia Hemianopia Asphasia Visuospatial problems

Question 49

what symptoms indicate a posterior cerebral artery infarct?

Answer 49

Eye Problems

Question 50

what features of a history might indicate carotid/vertebral artery dissection as the cause of a stroke?

Answer 50

Accounts for 20% of strokes in those under 40 often the result of trauma - but may be spontaneous symptoms resembling stroke, TIA, or migraine, with pain at the site of the dissection

Question 51

what is locked in syndrome and what causes it?

Answer 51

patient is aware and awake, but virtually all motor neurons are paralysed, and thus the patient cannot move. Usually the eyes are the only structures not affected. Caused by an upper brainstem infarct.

Question 52

what is pseudo-bulbar palsy and what causes it?

Answer 52

the result of a lower brainstem infarct. Results in bilateral impairment of the 9-12thcranial nerves. There is dysarthria and dysphagia

Question 53

what is Wallenberg’s syndrome / lateral medullary syndrome / posterior inferior cerebellar artery thrombosis (PICA)? - these all mean the same!

Answer 53

Syndrome resulting from brainstem infarction. presents with acute vertigo and other cerebellar signs. Due to the nature of the cerebellar pathways, there are the following signs: ``` Contralateral Spinothalamic sensory loss Hemiparesis (usually mild, quite rare) Ipsilateral Facial numbness (V) Diplopia (VI) Nystagmus Ataxia Horner’s syndrome 9th and 10th nerve lesions ```

Question 54

explain the appearance of stroke on CT and MRI

Answer 54

Infarctions will always show up as a wedge shape on both CT and MRI. Haemorrhage – blood appears bright white (dense) on CT – but the longer it has been present, the darker it becomes. After 1-2 weeks it may look the same as normal brain tissue. can roughly estimate the length of time the haemorrhage has been present by density of blood on a scan. Depending on the site of the bleed, it will have a different shape on the scan The damage is easier to see the longer it has been present For example, a ‘new’ stroke (e.g. <2 hours old) may not show up at all, but after 6-12 hours will be clearly visible. This has clinical consequences – because the sooner you can see the stroke, the sooner you can treat it.

Question 55

how do you investigate a stroke

Answer 55

send for urgent CT/MRI if considering thrombolysis, or high risk for haemorrhage, otherwise aim to do within 24hrs diffusion weighted MRI most sensitive for acute infarct, CT helps rule out primary haemorrhage. further tests: BP / examine for signs of HTN e.g. retinopathy. look for cardiac source of emboli - 24h ECG (for AF), might do an echo. check for carotid artery stenosis - carotid Doppler USS ± CT/MRI angiography - 70+ % stenosis = consider carotid endartectomy. bloods - ESR (for vasculitis), FBC/platelets/clotting, glucose, cholesterol/lipids

Question 56

how do you manage a suspected infarctive stroke if thrombolysis not an option? - also cover secondary prevention after stroke.

Answer 56

give everyone aspirin 300mg/day for 2 weeks THEN clopidogrel - long-term secondary prevention. if not tolerated give: aspirin 75mg day, plus dipyridamole (antiplatelet) if AF - start on NOAC. Carotid endartectomy – carotid stenosis of either >70% or >50% (NICE quotes two criteria??) should be referred for assessment for this within 1 week, and have procedure within 2.

Question 57

explain the oxford stroke classification system

Answer 57

The following criteria should be assessed: 1. unilateral hemiparesis and/or hemisensory loss of the face, arm & leg 2. homonymous hemianopia 3. higher cognitive dysfunction e.g. dysphasia Total anterior circulation infarcts (TACI, c. 15%) - involves middle and anterior cerebral arteries all 3 of the above criteria are present Partial anterior circulation infarcts (PACI, c. 25%) - involves smaller arteries of anterior circulation e.g. upper or lower division of middle cerebral artery 2 of the above criteria are present Lacunar infarcts (LACI, c. 25%) - involves perforating arteries around the internal capsule, thalamus and basal ganglia presents with 1 of the following: 1. unilateral weakness (and/or sensory deficit) of face and arm, arm and leg or all three. 2. pure sensory stroke. 3. ataxic hemiparesis ``` Posterior circulation infarcts (POCI, c. 25%) - involves vertebrobasilar arteries presents with 1 of the following: 1. cerebellar or brainstem syndromes 2. loss of consciousness 3. isolated homonymous hemianopia ```

Question 58

explain the ROSIER criteria for assessing likelihood of stroke

Answer 58

like a grown up version of FAST. Exclude hypoglycaemia first, then assess the following: (assessment --> score) Loss of consciousness or syncope --> - 1 point Seizure activity --> - 1 point New, acute onset of: • asymmetric facial weakness --> + 1 point • asymmetric arm weakness --> + 1 point • asymmetric leg weakness --> + 1 point • speech disturbance --> + 1 point • visual field defect --> + 1 point A stroke is likely if > 0

Question 59

how is haemorrhagic stroke managed?

Answer 59

Treatment is essentially supportive. If anticoagulants and antiplatlets have been given, then the effect can be reversed with vitamin K, fresh frozen plasma (FFP) and platelet transfusions. Hypertension should only be treated if systolic is >185 If the haemorrhage causes a mass of >3cm diameter, then surgery can be lifesaving.

Question 60

what is a transient ischaemic attack (TIA)?

Answer 60

mini stroke! symptoms resolve completely within 24hrs 80% are due to thromboembolus. "sudden onset of focal CNS symptoms/signs due to temporary occlusion of part of the cerebral circulation"

Question 61

what symptoms/signs in a TIA indicate an anterior circulation lesion? where is the thrombus likely to be from?

Answer 61

Likely thrombus from carotid system Most likely affecting cerebral function ``` Symptoms/signs: Aphasia/dysphasia Hemiparesis Amaurosis fugax Sensory loss (hemi) Hemianopic visual loss ```

Question 62

what symptoms/signs in a TIA indicate a posterior circulation lesion? where is the thrombus likely to be from?

Answer 62

Posterior Circulation Likely thrombus from vertebrobasilar system Most likely affecting cerebellar/brainstem function ``` Symptoms/signs: Diplopia, vertigo, vomiting Chocking / dysarthria Hemianopic visual loss Sensory loss (hemi) Transient global amnesia Tetraparesis LOC (rare) ```

Question 63

what is amaurosis fugax?

Answer 63

sudden loss of vision in one or both eye(s), caused by an infarct in the retinal artery(ies). classically - curtain coming down.

Question 64

how do you decide how to manage someone presenting with a possible TIA?

Answer 64

``` assess risk of stroke using ABCD2 - score of 4+ needs urgent referral to TIA clinic (within 24h - everyone with TIA should be seen within 7 days): Age 60+ --> 1 pt BP >140/90 --> 1pt Clinical features - unilateral weakness --> 2 pts - speech disturbance only --> 1 pt Duration of symptoms - 1+ hrs --> 2 pts - 10-59 mins --> 1pt Diabetes --> 1 pt ``` start everyone on 300mg aspirin daily (unless current bleeding disorder, already on it, or otherwise CI'ed). then switch to clopidogrel (or aspirin + dipyridamol as per stroke secondary prevention) investigate/treat for cause, esp carotid artery doppler/carotid endartectomy

Question 65

give info on driving and stroke/TIAs

Answer 65

avoid for 1 month, DVLA doesn't need informing unless multiple attacks (for TIA) or residual deficit (for stroke) after 1 month. if HGV/passenger carrying vehicle - must inform DVLA.

Question 66

explain MRC muscle weakness grading

Answer 66

grade 0 - no muscle contraction grade 1 - flicker of contraction grade 2 - some active movement when gravity removed (e.g. swinging leg sideways across bed) grade 3 - active movement against gravity grade 4 - active movement against resistance (can be split into 4-, 4 and 4+) grade 5 - normal power (allowing for age)

Question 67

what are upper motor neurone lesions? signs/symptoms?

Answer 67

damage to motor pathways (corticospinal tracts) anywhere from motor nerve cells in prefrontal gyrus of frontal cortex --> internal capsule --> brainstem --> cord --> synapse with anterior horn cells. pyramidal weakness loss of skilled fine finger movements to greater extent than you'd expect from weakness spasticity in stronger muscles (arm flexors, leg extensors) hyperreflexia - brisk reflexes upgoing plantars (+ve babinksi sign) ± clonus ± positive Hoffman's reflex

Question 68

what is pyramidal weakness?

Answer 68

seen in UMN lesions distribution of weakness, typically involving arm extensors, small muscles of hand, lower limb flexors. weakness of extension in upper limb, and flexion in lower limb no muscle wasting

Question 69

what is spasticity?

Answer 69

increased tone that is velocity-dependent and non-uniform i.e. faster you move the muscle, the greater the resistance - usually seen with brisk reflexes as well. compare to rigidity, which is constant throughout the movement

Question 70

what is the Babinski reflex?

Answer 70

draw thumbnail from heel to toes - upgoing plantar response = positive Babinski

Question 71

what is Hoffman's sign/reflex?

Answer 71

flick the pulp of middle finger away from palm --> brief flexion of thumb and index finger in pincer movement may be more pronounced if neck extended.

Question 72

what is clonus?

Answer 72

elicited by rapid dorsiflexion of foot - 3 or less downward, rhythmic beats is normal, any more suggests UMN lesion

Question 73

what are lower motor neurone lesions? signs/symptoms?

Answer 73

damage anywhere from anterior horn cells in cord --> nerve roots --> plexi --> peripheral nerves weakness distribution is according to muscles supplied by the involved nerve/cord segment etc. affected muscles show wasting ± fasciculation hypotonia/flaccidity reduced/absent reflexes Babinski negative (flexor plantars)

Question 74

what is fasciculation?

Answer 74

spontaneous involuntary twitching of a muscle, might be seen in a LMN lesion.

Question 75

what is idiopathic intracranial hypertension?

Answer 75

occurs typically in young, obese women. symps/signs of raised ICP but no mass lesion on CT/MRI. thought to be due to impaired CSF absorption.

Question 76

how does idiopathic intracranial hypertension present?

Answer 76

young, obese woman complaining of morning headache, vomiting and sometimes visual disturbance (e.g. diplopia, visual obscurations). tinnitus common. uni/bilateral sixth nerve palsies = 'false localising sign' of raised ICP. scans normal, LP confirms raised pressure but otherwise normal.

Question 77

what are visual obscurations?

Answer 77

sudden, transient bilateral visual loss with changes in posture

Question 78

how do you manage a patient with idiopathic intracranial hypertension? what are you worried about?

Answer 78

in some, it'll self-resolve, or go with weight loss/a few LPs. if progresses to more chronic - threat to vision from secondary optic atrophy. Rx with acetazolamide, diuretics or corticosteroids, or surgery to insert lumboperiotneal shunt draining CSF.

Question 79

what is Kernig's sign?

Answer 79

pain and resistance to passive knee extension with hip flexed - demonstrates meningism (NB - can be negative even in presence of meningism!)

Question 80

give some differentials for facial pain

Answer 80

``` trigeminal neuralgia post-herpetic neuralgia giant cell arteritis - if presenting with jaw claudication sinusitis dental/oral disease ```

Question 81

what is post-herpetic neuralgia? how can you treat it?

Answer 81

patient who've had shingles in a branch of the optic nerve may end up with persistent facial pain after the rash. may be very severe and intractable, lasting 2-3yrs after rash. might respond to TCAs, carbamazepine, topical capsaicin.

Question 82

outline the GCS scoring system

Answer 82

Motor response 6. Obeys commands 5. Localises to pain 4. Withdraws from pain 3. Abnormal flexion to pain (decorticate posture) 2. Extending to pain 1. None Verbal response 5. Orientated 4. Confused 3. Words 2. Sounds 1. None Eye opening 4. Spontaneous 3. To speech 2. To pain 1. None score = 3-15

Question 83

outline motor scoring in the GCS

Answer 83

``` 6 = obeys command 5 = localises to pain 4 = withdraws from pain 3 = abnormal flexion to pain 2 = extending to pain 1 = none ```

Question 84

outline verbal response scoring in GCS

Answer 84

``` 5 = orientated 4 = confused 3 = inappropriate words 2 = incomprehensible sounds 1 = none ```

Question 85

outline eye opening scoring in GCS

Answer 85

``` 4 = spontaneously 3 = to speech 2 = to pain 1 = none ```

Question 86

list some causes of altered level of consciousness

Answer 86

Structural - infratentorial (directly involving brain stem) e.g. trauma, infarction, haemorrhage, tumour, demyelination - supratentorial (compressing brainstem) e.g. as above, esp if affect R hemisphere Diffuse - hypoxia - hypoglycaemia - renal/liver failure - hypothermia - vitamin deficiencies - epilepsy - inflammation - meningitis, encephalitis - drugs and toxins - opiates, antidepressants, hypnotic, alcohol

Question 87

explain criteria for brainstem death

Answer 87

Criteria for brain stem death testing: - Deep coma of known aetiology. - Reversible causes excluded - No sedation - Normal electrolytes Testing for brain death: - Fixed pupils which do not respond to sharp changes in the intensity of incident light - No corneal reflex - Absent oculo-vestibular reflexes - No response to supraorbital pressure - No cough reflex to bronchial stimulation or gagging response to pharyngeal stimulation - No observed respiratory effort in response to disconnection of the ventilator Notes: The test should be undertaken by two appropriately experienced doctors on two separate occasions. Both should be experienced in performing brain stem death testing and have at least 5 years post graduate experience. One of them must be a consultant. Neither can be a member of the transplant team (if organ donation contemplated).

Question 88

give some causes of syncope - also what is it?

Answer 88

syncope = LOC due to transient reduction in blood flow to brain - cardiac arrhythmias - prolonged standing, esp in warm surroundings - psychogenic e.g. around needles - excessive reflex vagal stimulation e.g. micturition syncope, cough syncope

Question 89

what features in a LOC hx suggest syncope?

Answer 89

prodrome - lightheadedness, nausea, blurred/tunnel vision, pallor, sweating rapid recovery - once supine, will recover in seconds to 1-2 mins. situation e.g. standing in hot room, around needles

Question 90

list some differentials for LOC

Answer 90

- epilepsy - syncope - hypoglycaemia (warnings = anxiety, tremor, unsteadiness, sweating, hunger) - drop attacks (in middle aged and elderly woman - random falls)

Question 91

what are the 4 clinical features you might see in narcolepsy?

Answer 91

1) daytime sleep attacks - lasting 10-20 mins, pt awake refreshed. irresistible and occur inappropriately e.g. during meals, driving, mid-convo 2) cataplexy - episodes of loss of postural control + limb weakness, with preserved consciousness - often provoked by emotional events e.g. laughter 3) sleep paralysis - inability to move while falling asleep/waking 4) hypnagogic hallucinations - frightening visual hallucinations on falling asleep

Question 92

what is cataplexy?

Answer 92

episodes of loss of postural control and limb weakness, with preserved consciousness - often provoked by emotional events e.g. laughter Ix - sleep studies, EEG, MRI (for SOLs)

Question 93

how can you treat narcolepsy/cataplexy?

Answer 93

narcolepsy - amphetamines, but be sure you've got the diagnosis right before starting due to addictive properties! also modafinil. cataplexy - clomipramine, other antidepressants.

Question 94

what brain functions is the frontal lobe responsible for?

Answer 94

- higher intellectual function - personality, mood - social conduct, behaviour - posterior frontal region contains motor areas - frontal eye fields (conjugate eye movements) - language (in dominant hemisphere)

Question 95

what brain functions is the temporal lobe responsible for?

Answer 95

- memory - language (in dominant hemisphere) - visual pathway (optic radiation)

Question 96

what higher brain functions is the parietal lobe responsible for?

Answer 96

``` dominant hemisphere: - language, reading, writing - calculation - praxis (learning complex motor skills) non-dominant: - visuo-spatial function both: - higher sensory function - visual pathway (optic radiation) ```

Question 97

what higher brain functions is the occipital lobe responsible for?

Answer 97

- visual cortex and visual association areas

Question 98

what is aphasia/dysphasia?

Answer 98

impairment of language function as a result of brain damage | distinguish from dysarthria - impairment of articulation due to disease of muscles/neves involved with speech production

Question 99

what is dyspraxia/apraxia?

Answer 99

inability to perform complex motor acts despite normal muscle power, sensation and coordination, with good comprehension and cooperation. results from parietal lobe damage

Question 100

what is Broca's aphasia?

Answer 100

aka expressive aphasia due to a lesion of the inferior frontal gyrus speech is non-fluent, laboured, and halting comprehension is normal

Question 101

what is Wernicke's aphasia?

Answer 101

due to a lesion of the superior temporal gyrus this area 'forms' the speech before 'sending it' to Broca's area. Lesions result in sentences that make no sense, word substitution and neologisms but speech remains fluent comprehension is impaired

Question 102

what is conduction aphasia?

Answer 102

classically due to a stroke affecting the arcuate fasiculus - the connection between Wernicke's and Broca's area speech is fluent but repetition is poor. Aware of the errors they are making comprehension is normal

Question 103

what clinical features would you expect to see in a parietal lobe lesion?

Answer 103

``` sensory inattention apraxias astereognosis (tactile agnosia) inferior homonymous quadrantanopia Gerstmann's syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation ```

Question 104

what clinical features would you expect to see in an occipital lobe lesion?

Answer 104

homonymous hemianopia (with macula sparing) cortical blindness visual agnosia

Question 105

what clinical features would you expect to see in a temporal lobe lesion?

Answer 105

Wernicke's aphasia: this area 'forms' the speech before 'sending it' to Brocas area. Lesions result in word substituion, neologisms but speech remains fluent superior homonymous quadrantanopia auditory agnosia prosopagnosia (difficulty recognising faces)

Question 106

what clinical features would you expect to see in a frontal lobe lesion?

Answer 106

``` expressive (Broca's) aphasia: located on the posterior aspect of the frontal lobe, in the inferior frontal gyrus. Speech is non-fluent, laboured, and halting disinhibition perseveration anosmia inability to generate a list ```

Question 107

what clinical features would you expect to see in a cerebellar lesion?

Answer 107

midline lesions: gait and truncal ataxia | hemisphere lesions: intention tremor, past pointing, dysdiadokinesis, nystagmus

Question 108

what's CN I called and what does it do?

Answer 108

olfactory nerve --> sense of smell.

Question 109

what's CN II called and what does it do?

Answer 109

optic nerve --> vision: | visual acuity, visual fields, colour vision, pupillary responses.

Question 110

what kind of vision loss is seen in a lesion of the optic nerve?

Answer 110

monocular visual loss - essentially 'blind' in one eye (ipsilateral to lesion)

Question 111

what kind of vision loss is seen in a lesion of the optic chiasm?

Answer 111

bitemporal hemianopia - loss of temporal halves of vision on both sides. due to damage to decussating fibres from the nasal halves of retinae, as light from the temporal half-field is processed by nasal part of the retina.

Question 112

what kind of vision loss is seen in a lesion of the optic tract?

Answer 112

homonymous hemianopia e.g. left half-fields in each eye missing (lesion would then be in R optic tract i.e. contralateral). damage is to fibres from temporal half of one retina (in this e.g. the L one) and the decussated fibres from nasal half of the other (R one).

Question 113

which lobes are damaged in homonymous inferior vs superior quadrantanopias?

Answer 113

PITS Parietal = inferior Temporal = superior contralateral!

Question 114

what is a central scotoma and what might cause it?

Answer 114

loss of central vision, usually associated with reduction in visual acuity diseases of optic nerve and macular region of retina

Question 115

what causes enlargement of the physiological blind spot?

Answer 115

papilloedema - swelling of the optic disc - due to raised ICP. usually have preserved visual acuity.

Question 116

what is macular sparing and what kind of lesion could cause it?

Answer 116

preservation of macular (central) region in patients with homonymous hemianopia lesion of visual cortex sparing occipital pole

Question 117

what is tunnel vision and what can cause it?

Answer 117

loss of peripheral fields, with preservation of central region - ophthalmological disease - chronic simple glaucoma - retinal disease - retinitis pigmentosa - cortical disease

Question 118

what does an afferent pupillary defect look like?

Answer 118

when torch shines in affected eye, the light isn't perceived so neither pupil constricts (direct or consensual). when torch is shined in the normal eye, they both constrict.

Question 119

what does an efferent pupillary defect look like?

Answer 119

light is perceived by affected eye but pupil can't respond - the other pupil constricts consensually but direct reflect absent. when light shined on unaffected eye, you get direct response but not consensual

Question 120

what is a relative afferent pupillary defect? what condition is it strongly linked with?

Answer 120

affected eye can still respond directly to light but not as well as the other side important sign of optic neuritis - persists after symptoms resolve. aka the Marcus-Gunn pupil

Question 121

what is a Holmes-Adie pupil?

Answer 121

benign cause of dilated pupil, commonly seen in women: unilateral in 80% of cases dilated pupil once the pupil has constricted it remains small for an abnormally long time slowly reactive to accommodation but very poorly (if at all) to light aka myotonic pupil

Question 122

what is a Argyll Robertson pupil?

Answer 122

sometimes seen in neurosyphilis mnemonic = Accommodation Reflex Present (ARP) but Pupillary Reflex Absent (PRA) Features: small, irregular pupils no response to light but there is a response to accommodate Causes: diabetes mellitus syphilis

Question 123

what are the names of CNs III, IV and VI - what do they do?

Answer 123

occulomotor, trochlear, abducens nerves trochlear (IV) supplies superior oblique abducens (VI) supplies lateral rectus the rest are supplied by occulomotor, which also supplies levator palpebrae superioris (opens eye lid)

Question 124

what is ptosis?

Answer 124

drooping of eyelid (partial) or unable to open eye (complete) - weakness of levator palpebrae suprioris

Question 125

what is diplopia? what causes it?

Answer 125

``` double vision - neurologically, arises from malalignment of eyes distinguish binocular (occurs when both eyes open) from monocular (persists when one eye covered) - monocular = ophthalmological rather than neurological ```

Question 126

what would you see in a third nerve palsy?

Answer 126

ptosis due to paralysis of levator palpebrae superioris when examiner opens eyelid, eye is in 'down-and-out' position due to unopposed action of superior oblique and lateral rectus muscles pupil may be 'fixed' (no reflex responses) or dilated ('surgical' 3rd nerve palsy) or spared ('medical' third nerve palsy)

Question 127

list some causes of third nerve palsies

Answer 127

diabetes mellitus vasculitis e.g. temporal arteritis, SLE false localizing sign due to uncal herniation through tentorium if raised ICP posterior communicating artery aneurysm (pupil dilated) cavernous sinus thrombosis Weber's syndrome: ipsilateral third nerve palsy with contralateral hemiplegia - caused by midbrain strokes other possible causes: amyloid, multiple sclerosis

Question 128

what would you see in a fourth nerve palsy?

Answer 128

unilateral paralysis of superior oblique, causing vertical diplopia noticed when descending stairs or looking down at a book - may tilt head towards normal side to correct. causes - mild head trauma

Question 129

what would you seen in a sixth nerve palsy?

Answer 129

unable to abduct affected eye because of unopposed action of medial rectus. diplopia on looking to affected side with horizontal separation of images. if isolated sixth nerve palsy - vascular prob, secondary to diabetes/HTN also can be false localising sign of raised ICP due to long, tortuous intracranial course

Question 130

what is Horner's syndrome?

Answer 130

``` Features - miosis (constricted pupil) ptosis (partial) enophthalmos (sunken eye) anhidrosis (loss of sweating on affected side of face) ``` lesion of sympathetic nerve supply to eye - hypothalamus --> brainstem --> cervical cord --> T1 nerve root --> cervical sympathetic chain --> eye

Question 131

what is nystagmus?

Answer 131

involuntary rhythmic oscillatory movement of eyes, might be present on attempted sustained horizontal/vertical gaze.

Question 132

what is CN V called? what does it do?

Answer 132

trigeminal nerve - facial sensation and masticatory muscles divided into ophthalmic, maxillary, mandibular. corneal reflex lesions - loss of facial sensation and corneal reflex, problems chewing, jaw deviation - all depends which branch is affected.

Question 133

what is CN VII called? what does it do?

Answer 133

facial nerve - muscles of facial expression, taste sensation to anterior 2/3rds of tongue, lacrimation, salivation. ``` lesions: flaccid paralysis of upper + lower face loss of corneal reflex (efferent) loss of taste hyperacusis ```

Question 134

what is Bell's palsy? features?

Answer 134

acute, unilateral, idiopathic, facial nerve paralysis - cause unknown, might be to do with HSV. Features - rapid sudden onset: lower motor neuron facial nerve palsy - forehead affected* patients may also notice post-auricular pain (may precede paralysis), altered taste, dry eyes, hyperacusis

Question 135

how do you manage Bell's palsy?

Answer 135

10 day course of pred if within 72hrs of onset | advise artificial tears, eye lubricants and taping eyelid down to protect the cornea

Question 136

what is CN VIII called? what does it do?

Answer 136

vestibulocochlear nerve - hearing and balance. lesions cause: Hearing loss Vertigo, nystagmus Acoustic neuromas are Schwann cell tumours of the cochlear nerve

Question 137

explain Rinne's and Weber's tests

Answer 137

Performing both Rinne's and Weber's test allows differentiation of conductive and sensorineural deafness. Rinne's test: 512 Hz tuning fork is placed over the mastoid process until the sound is no longer heard, followed by repositioning just over external acoustic meatus air conduction (AC) is normally better than bone conduction (BC) if BC > AC then conductive deafness Weber's test: 512 Hz tuning fork is placed in the middle of the forehead equidistant from the patient's ears the patient is then asked which side is loudest in unilateral sensorineural deafness, sound is localised to the unaffected side in unilateral conductive deafness, sound is localised to the affected side

Question 138

what is CN IX called? functions?

Answer 138

glossopharyngeal nerve taste (posterior 1/3rd of tongue) Salivation Swallowing Mediates input from carotid body & sinus lesions - loss of gag reflex, hypersensitive carotid sinus reflex (afferent)

Question 139

what is CN X called? functions?

Answer 139

vagus functions: Phonation Swallowing Innervates viscera ``` lesions: uvula deviates away from site of lesion loss of gag reflex (efferent) swallowing difficulties, dysarthria vocine cough ```

Question 140

what is CN XI called? functions?

Answer 140

accessory nerve supplies sternomastoid and trapezius muscles sternomastoid - turning head against resistance trapezius - shrugging shoulders

Question 141

what is CN XII called? functions?

Answer 141

hypoglossal tongue muscles! lesion (if LMN) - uni/bilateral wasting and fasciculation. if unilateral - tongue deviates to affected side on protrusion.

Question 142

differentiate between bulbar and pseudobulbar palsies

Answer 142

bulbar palsy = LMN lesion of CN IX, X and XII. | pseudobulbar palsy = UMN of CN IX, X and XII.

Question 143

list some causes of bulbar palsy

Answer 143

``` brainstem vascular disease MND syringobulbia tumour brainstem encephalitis e.g. polio Guillain-Barre syndrome skull base or meningeal infiltration myasthenia gravis some muscular dystrophies polyomyositis ```

Question 144

what are the features of a bulbar palsy?

Answer 144

``` nasal speech absent jaw jerk palatal weakness, nasal regurg of food reduced or absent gag reflex wasted, fasciculating tongue ```

Question 145

list some causes of pseudobulbar palsy

Answer 145

``` bihemispheric vascular disease MND MS tumour extrapyramidal disease ```

Question 146

what are the features of pseudobulbar palsy?

Answer 146

slow, monotonous speech, sometimes explosive brisk jaw jerk dysphagia brisk gag reflex shrunken, immobile tongue emotional lability - spontaneous laughing/crying

Question 147

describe the gait you would see in a spastic paraparesis

Answer 147

spastic paraparesis = UMN lesion affecting both legs described as scissoring, 'wading through mud'

Question 148

describe the gait you would see in a spastic hemiparesis

Answer 148

spastic hemiparesis = UMN lesion affecting one leg leg is rigid and circumducts - described as semicircle rotating at hip

Question 149

describe the gait you would see in a bilateral foot drop

Answer 149

bilateral foot drop = LMN lesion of both legs steppage gait - legs lifted high to avoid scraping toes

Question 150

describe the gait you would see in a cerebellar lesion

Answer 150

wide-based gait, staggering, unable to walk heel-toe

Question 151

describe the gait you would see in a Parkinsons

Answer 151

stooping posture, rigid shuffling gait, no arm swing

Question 152

describe the gait you would see in a proximal myopathy

Answer 152

waddling

Question 153

what is epilepsy?

Answer 153

tendency to recurrent seizures - paroxysmal cerebral cortical neuronal discharges result in intermittent, stereotyped attacks of altered consciousness, motor or sensory function, behaviour or emotion

Question 154

explain how epileptic seizures are classified

Answer 154

focal vs generalised focal aware = consciousness maintained through focal impaired awareness = consciousness impaired at any stage generalised onset involved both sides of / whole brain, LOC occurs immediately note focal seizures may become secondary generalised (aka bilateral tonic-clonic) - pt loses consciousness and there's clinical evidence of more generalised involvement

Question 155

give some info on generalised onset seizures and the subtypes

Answer 155

``` these engage or involve networks on both sides of the brain at the onset consciousness lost immediately. further subdivided into motor (e.g. tonic-clonic) and non-motor (e.g. absence) specific types include: → tonic-clonic (grand mal) → tonic → clonic → typical absence (petit mal) → myoclonic: brief, rapid muscle jerks → atonic ```

Question 156

what are the features of a tonic-clonic / grand mal seizure?

Answer 156

prodrome/aura - dizziness, irritability LOC ± epileptic cry and fall to the ground then initial tonic phase - generalised muscle spasms for a few secs then clonic phase - sharp, repetitive muscle jerks. may see tongue biting, incontinence and salivation. when jerks stop, patients usually unconscious for another half an hour or so, then are drowsy and confused for several hours (recovery) may have headache (from fall) or back pain (from muscle spasms) in recovery period.

Question 157

what are the features of a focal, temporal lobe epilepsy?

Answer 157

aura - psychic symptoms (e.g. deja/jamais vu), hallucinations (olfactory, gustatory, visual images), epigastric rising sensation automatisms - chewing and lip smacking. ``` HEAD: Hallucinations Epigastric rising/emotional lability Automatisms Deja vu/dysphasia post-ictal ```

Question 158

what are the features of a focal, frontal (motor) lobe epilepsy?

Answer 158

Head/leg movements , posturing, post-ictal weakness

Question 159

what are the features of a focal, parietal (sensory) lobe epilepsy?

Answer 159

paraesthesiae

Question 160

what are the features of a focal, occipital (visual) lobe epilepsy?

Answer 160

floaters/flashers

Question 161

what are the features of a Jacksonian march seizure?

Answer 161

focal motor attack beginning in the corner of the mouth, the thumb and index finger, or the big toe movements rapidly spread across face or ascend limb associated with organic brain disease causing the epilepsy e.g. tumour in motor region afterwards, affect limb may remain weak - Todd's paralysis

Question 162

what is Todd's paresis/paralysis?

Answer 162

focal weakness after a partial seizure, usually resolves completely within 48h

Question 163

describe the features of febrile convulsions

Answer 163

``` seizures associated with fever child aged 6 months - 5 years brief (< 15mins) and generalised, although some might have focal and prolonged attacks usually isolated attack doesn't require prophylactic AEDs ```

Question 164

what is West's syndrome (infantile spasms)?

Answer 164

triad of: 1) brief spasms beginning within first few months of life - shock-like flexion of arms, head and neck with drawing up of feet (salaam attack) 2) progressive learning difficulties 3) hypsarrhythmia on EEG usually has an identifiable cause e.g. perinatal asphyxia, encephalitis, metabolic disorders, cerebral malformations Rx - vigabatrin/steroids

Question 165

what are petit mal / absence seizures?

Answer 165

generalised epilepsy, usually starts in childhood (peak onset 4-8yrs) attacks occur without warning - child stares into space and stops talking, eyes may flutter/roll up under lids. recovery within seconds, may have many attacks per day EEG: 3Hz generalized, symmetrical sodium valproate, ethosuximide good prognosis: 90-95% become seizure free in adolescence

Question 166

what is juvenile myoclonic epilepsy / Janz syndrome?

Answer 166

onset: teens; F:M = 2:1 1) Infrequent generalised seizures, often in morning 2) Daytime absences 3) Sudden, shock like myoclonic (involuntary jerking) seizure - usually in morning (patient inexplicably spills/throws breakfast across room) usually good response to sodium valproate - carbamazepine can make it worse!!

Question 167

list some causes of epilepsy in neonates

Answer 167

``` birth trauma intracranial haemorrhage hypoxia hypoglycaemia hypocalcaemia ```

Question 168

list some causes of epilepsy in children

Answer 168

congenital anomalies | tuberous sclerosis

Question 169

list some causes of epilepsy in young adults

Answer 169

head injuries | drugs and alcohol

Question 170

list some causes of epilepsy in middle-aged adults

Answer 170

cerebral tumour

Question 171

list some causes of epilepsy in the elderly

Answer 171

cerebrovascular disease | degenerative disorders e.g. Alzheimer's

Question 172

list some causes of epilepsy across all age groups

Answer 172

infection - meningitis, encephalitis, abscess inflammation - MS (rare), vasculitis metabolic encephalopathy NB - all the ones on cards in specific age groups can probs cross age groups also majority of epilepsy occurs without specific cause!

Question 173

list some differentials for epilepsy

Answer 173

``` syncope cardiac dysrhythmia pseudoseizures hyperventilation/panic attacks TIAs migraine narcolepsy hypoglycaemia vestibular disorders ```

Question 174

how is epilepsy investigated/diagnosed?

Answer 174

aim of investigation is to confirm/support clinical diagnosis, classify epileptic syndrome and establish a cause if there is one. EEG - usually only after second seizure due to frequent false positives and negatives. telemetry = EEG + video recording EEG may be ambulatory/prolonged. brain CT/MRI - especially if later onset epilepsy, partial attacks ± focal neurological signs and EEG abnormalities realistically most adults presenting to A&E with isolated, first seizure would get scanned!

Question 175

explain basic principles of drug therapy for epilepsy

Answer 175

usually only started after a second attack | careful outpatient follow up needed - aiming for lowest effective dose of single drug

Question 176

give some reasons for refractory epilepsy

Answer 176

non-compliance with meds pseudoseizures/non-epileptic attacks (either alone or in combo with genuine seizures) associated structural brain disease alcohol and lifestyle (e.g. sleep deprivation)

Question 177

when would you consider starting AEDs after only a single seizure?

Answer 177

- the patient has a neurological deficit - brain imaging shows a structural abnormality - the EEG shows unequivocal epileptic activity - the patient or their family or carers consider the risk of having a further seizure unacceptable

Question 178

what is the usual drug of choice for generalised tonic-clonic seizures?

Answer 178

sodium valproate may use - phenytoin, carbamazepine, lamotragine

Question 179

what is the usual drug of choice for partial seizures?

Answer 179

carbamazepine also - sodium valproate, phenytoin, lamotragine

Question 180

what is the usual drug of choice for absence seizures?

Answer 180

ethosuximide also - sodium valproate, lamotrigine NB - carbamazepine can make them worse!

Question 181

what is the usual drug of choice for myoclonic seizures?

Answer 181

sodium valproate also - clonazepam, lamotrigine

Question 182

when might surgery be considered for epilepsy?

Answer 182

if a defined, operable site of seizure onset is identified, particularly if intractable to drug treatment. in some, surgery may be performed even if onset site unclear - hemispherectomy, disconnection (section of corpus callosum) - RARE

Question 183

what lifestyle/general advice should be given to someone with epilepsy?

Answer 183

avoid triggers if identified e.g. flickering lights, alcohol driving - can only drive after seizure free interval of 6 months, must inform DVLA (for HGV/passenger carrying must be seizure free, without AED for 10 years) - unless epilepsy has only ever occurred during sleep. employment - not allowed to work in armed or emergency services, aircraft pilot etc leisure - NEVER swim alone, inform lifeguards, wear bike helmets etc

Question 184

what causes subarachnoid haemorrhage?

Answer 184

bleeding into subarachnoid space, commonly due to: - head injury / trauma - rupture of an aneurysm - congenital weakenings at junctions in circle of Willis - arteriovenous malformation (angiomas) - malformed vessels, also congenital - enlarge and present in adult life - pituitary apoplexy - arterial dissection - mycotic (infective) aneurysms - perimesencephalic (an idiopathic venous bleed)

Question 185

how does a subarachnoid haemorrhage classically present?

Answer 185

- headache: typically sudden-onset (‘thunderclap’ or ‘baseball bat’), severe (‘worst of my life’) and occipital - nausea and vomiting - meningism (photophobia, neck stiffness) - coma - seizures - sudden death - raised ICP signs on fundoscopy etc - focal neurology due to false localisation from raised ICP

Question 186

how would you investigate someone with a suspected SAH?

Answer 186

- CT head - shows acute blood (hyperdense/bright on CT) is typically distributed in the basal cisterns, sulci and in severe cases the ventricular system. LP - to confirm SAH if CT is negative - performed at least 12 hours following the onset of symptoms to allow the development of xanthochromia (straw coloured CSF). xanthochromia helps to distinguish true SAH from a ‘traumatic tap’ (blood introduced by the LP procedure) cerebral angiography (if pt stable) to look for source of bleeding and direct treatment to look for cause - CXR/ECG for pulmonary oedema and cardiac arrhytmias, clotting for bleeding disorders

Question 187

how is an SAH managed?

Answer 187

treat according to cause: - most intracranial aneurysms are now treated with a coil by interventional neuroradiologists, minority require a craniotomy and clipping by a neurosurgeon - patient should be kept on strict bed rest, well controlled BP and should avoid straining in order to prevent a re-bleed of the aneurysm - vasospasm is prevented using nimodipine and treated with hypervolaemia, induced-hypertension and haemodilution - hydrocephalus is temporarily treated with an external ventricular drain (CSF diverted into a bag at the bedside) or, if required, a long-term ventriculo-peritoneal shunt§

Question 188

list some possible complications of aneurysmal SAH

Answer 188

Re-bleeding (in around 30%) Vasospasm (also termed delayed cerebral ischaemia), typically 7-14 days after onset Hyponatraemia (most typically due to syndrome inappropriate anti-diuretic hormone (SIADH)) Seizures Hydrocephalus Death

Question 189

what is hydrocephalus?

Answer 189

condition in which there is an excessive volume of cerebrospinal (CSF) fluid within the ventricular system of the brain - caused by an imbalance between CSF production and absorption, can occur in raised ICP if CSF flow obstructed

Question 190

what can cause a spontaneous intracerebral haemorrhage (haemorrhagic stroke -think they're the same??) ?

Answer 190

- HTN, with microaneurysm formation (Charcot-Bouchard aneurysms) - bleeding into tumours - trauma - blood disorders - blood vessel disorder - AV malformations, vasculitis, amyloid

Question 191

what is the classical clinical triad that occurs in parkinsons?

Answer 191

1) akinesia/bradykinesia - poverty of movement 2) rigidity 3) tremor - shaking back and forth, usually of upper limbs

Question 192

describe the pathophysiology of Parkinson's

Answer 192

thought to be to do with atrophy/neuronal loss of dopaminergic neurones (the ones that produce dopamine) in the substantia nigra remaining neurones contain intracellular inclusions called Lewy bodies. increasingly common with age (onset usually around 60+ yo)

Question 193

what are the bradykinesic features of Parkinson's disease?

Answer 193

- difficulty with complex motor tasks (dressing, shaving etc) - handwriting becomes smaller (micrographia) - 'mask-like' face due to poverty of facial expression - quiet and monotonous speech - abnormal gait and stance

Question 194

what are the gait features of Parkinson's disease?

Answer 194

- flexed/stooped posture - unable to maintain normal stance in response to pressure (i.e. you can knock them over really easily if push gently from behind or in front - propulsion/retropulsion) - 'freezing' - difficulty initiating walking - small, shuffling steps ('festinant') - loss of normal arm swing - severe postural instability = risk of falls

Question 195

describe the rigidity seen in Parkinson's disease

Answer 195

increase in muscle tone - relatively constant throughout range of movement of joint - 'lead pipe' rigidity (rather than spasticity!) cogwheel rigidity is more due to tremor superimposed on lead pipe - repeated flexion and extension of arm or rotation at wrist.

Question 196

describe the tremor seen in Parkinson's disease

Answer 196

- mostly affects hands, may involve upper/lower limb or jaw - not the head or neck - hands = pill rolling - present at rest, exacerbated by anxiety or stress - improves/disappears on movement - early on - asymmetrical (true for other physical signs as well!) - then progresses to bilateral

Question 197

define tremor

Answer 197

tremor = involuntary, repetitive, rhythmic sinusoidal movement usually affecting 1+ limbs, occasionally involving the head (titubation), face, jaw or trunk

Question 198

give some more features of Parkinson's (beyond - bradykinesia, gait, tremor, rigidity)

Answer 198

``` mask-like facies flexed posture micrographia drooling of saliva psychiatric features: depression is the most common feature (affects about 40%); dementia, psychosis and sleep disturbances may also occur impaired olfaction REM sleep behaviour disorder fatigue postural hypotension ```

Question 199

how is Parkinson's disease diagnosed?

Answer 199

generally clinical based on the triad of features. CT/MRI head unhelpful. I‑FP‑CIT single photon emission computed tomography (SPECT) can be considered if struggling to differentiate from essential tremor - looks for nigrostriate dopaminergic lesion. OR - see if they respond to drugs

Question 200

what are the features and management of essential tremor?

Answer 200

autosomal dominant condition which usually affects both upper limbs Features - postural tremor: worse if arms outstretched improved by alcohol and rest most common cause of titubation (head tremor) Management - propranolol is first-line primidone is sometimes used

Question 201

what drugs are used first-line in Parkinson's?

Answer 201

if the motor symptoms are affecting the patient's quality of life: levodopa if the motor symptoms are not affecting the patient's quality of life: dopamine agonist (non-ergot derived), levodopa or monoamine oxidase B (MAO‑B) inhibitor

Question 202

give some info on levodopa

Answer 202

usually combined with a decarboxylase inhibitor (e.g. carbidopa or benserazide - becomes co-carelodpa or co-beneldopa) to prevent peripheral metabolism of levodopa to dopamine reduced effectiveness with time (usually by 2 years) unwanted effects, particularly of prolonged use: dyskinesia (involuntary writhing movements), 'on-off' effect, dry mouth, anorexia, palpitations, postural hypotension, psychosis, drowsiness no use in neuroleptic induced parkinsonism

Question 203

explain 'wearing-off' / 'on-off' effects regarding L dopa

Answer 203

start to occur after 2-5yrs wearing off = when individual doses produce only short-lived effects before they wear off on-off effects = where patient can switch from symptomatic benefit from meds (on) to akinetic-rigid state (off), often without predictable relationship to dose timings

Question 204

what are dyskinesias, in relation to L dopa?

Answer 204

involuntary movements occurring in relation with drug treatment e.g. twisting, turning movements when dopamine levels high or painful sustained muscle contractions, usually in feet, when levels low (peak dose vs wearing off dyskinesias)

Question 205

what is selegiline?

Answer 205

monoamine oxidase type B inhibitor - inhibits breakdown of dopamine secreted by what dopaminergic neurons remain potentially useful in early Parkinson's disease - might be neuroprotective and delay needing L-DOPA

Question 206

what are COMT inhibitors?

Answer 206

e.g. Entacapone, tolcapone COMT is an enzyme involved in the breakdown of dopamine, and hence may be used as an adjunct to levodopa therapy used in conjunction with levodopa in patients with established PD tolcapone - highly restricted due to very rare occurrence of fatal liver failure

Question 207

what are dopamine agonists, what is their role in parkinson's management?

Answer 207

e.g. bromocriptine, cabergoline, pergolie, ropinirole, pramipexole, rotigotine, apomorphine useful in early PD for delaying need for L-DOPA the ergot derived ones (first 3) can cause pulmonary/retroperitoneal fibrosis, so the others usually preferred.

Question 208

what is chorea/choreiform movement?

Answer 208

irregular, random, variable - flowing/dancing quality. | can affect any part of body

Question 209

give some causes of chorea

Answer 209

acquired: - chronic L-DOPA therapy - postinfectious (Sydenham's chorea + rheumatic fever) - polycythaemia rubra vera - SLE - hyperthyroidism - pregnancy, oral contraceptives - phenytoin, alcohol, neuroleptics Huntington's chorea - associated with Huntington's disease

Question 210

what are athetoid movements?

Answer 210

slower, more writhing quality than chorea | associated with cerebral palsy

Question 211

list some causes of tremor

Answer 211

rest tremor: - Parkinson's postural tremor (worse with maintained posture): - essential tremor - physiological, exaggerated by anxiety, thyrotoxicosis, alcohol, drugs (salbutamol) kinesis tremor (worse during movement/intention tremor): - cerebellar disease - MS, hereditary ataxias - tumours, infarct, haemorrhage of cerebellum

Question 212

what is brain herniation?

Answer 212

displacement of the brain as raised ICP hits pathological levels - where it's displaced depends on where the lesion causing the raised ICP is. causes compression of important structures - most dangerous is 'coning', the compression of the brainstem downwards - neurosurgical emergency

Question 213

what is coning?

Answer 213

aka tonsillar herniation - displacement of the cerebellar tonsils through the foramen magnum. In raised ICP this causes compression of the cardiorespiratory centre. Can occur in Chiari 1 malformation without raised ICP

Question 214

how is hydrocephalus classified?

Answer 214

obstructive - CSF flow blocked within ventricles communicating - CSF flow blocked 'beyond' ventricular system infantile hydrocephalus 'normal-presssure' hydrocephalus

Question 215

what can cause obstructive hydrocephalus? how is it managed?

Answer 215

congenital abnormalities - cerebral aqueduct stenosis, Arnold-Chiari malformation mass lesions e.g. tumour, haematoma requires urgent surgical management - removal of excess CSF via ventricular drain permanent management = ventriculoperitoneal shunt insertion

Question 216

what can cause communicating hydrocephalus?

Answer 216

damage to meninges/arachnoid villi - meningitis, SAH (both can also cause obstructive hydrocephalus) increased CSF viscosity due to high protein conc.

Question 217

list some common causes of head injury

Answer 217

``` RTAs falls assault - blunt injuries industrial injuries domestic accidents sporting accidents missile injuries - bullets and bomb fragments ```

Question 218

what two mechanisms cause brain damage as a result of head injury?

Answer 218

1) damage at impact - contusion/laceration of cerebral cortex at site of impact; diffuse white matter lesions due to axonal shearing 2) secondary complications - haematoma (extra/subdural, intracerebral), cerebral oedema, cerebral ischaemia, coning, infection

Question 219

what are the clinical features of head injury?

Answer 219

remember - do ABCDE approach and treat major chest injury/abdo bleeds before focussing on head injury consider - any LOC? lucid interval? any confusion after regain of consciousness? - persistence of headache and vomiting - haematoma? examine: - external evidence of trauma - signs of basal skull fracture e.g. bilateral periorbital haematoma, subconjunctival haematoma, CSF discharge from nose/ear, bleeding from ear - GCS

Question 220

what investigations would you do for a patient with a head injury?

Answer 220

- skull XR if transient/persistent impairment of consciousness, signs suggestive of skull fracture, focal neurology - urgent CT head if level of consciousness deteriorating or skull fracture + neuro signs/seizures/confusion

Question 221

outline management of head injury

Answer 221

admit if - reduced consciousness, skull fracture, focal neurology - neurological observation, clean and suture scalp lacerations. if severe: surgical - evacuation of space-occupying haematoma, urgent surgery for compound skull fraction medical - IV mannitol bolus for raised ICP (also used before surgery for haematoma), prophylactic abx for basal skull #, manage seizures appropriately

Question 222

list some possible complications/consequences of head injury

Answer 222

- may have residual disability/impairment - small number = persistent vegetative state - CSF leakage due to dural tear - may present late with meningism, requires surgical repair - post-traumatic epilepsy - post-conscussion syndrome - headache, depression, impaired concentration

Question 223

what is normal-pressure hydrocephalus? how is it treated?

Answer 223

unique form of non-obstructive hydrocephalus characterised by large ventricles on CT but normal intracranial pressure classic triad = dementia, incontinence and disturbed gait. Rx - ventriculopertioneal shunting - variable results

Question 224

outline emergency management of raised ICP

Answer 224

ABCDE correct hypotension (MAP >90mmHg) and Rx seizures elevate head of bed, hyperventilate if intubated, mannitol IV helps lower ICP initially but can cause rebound raised ICP. dexamethasone only helpful if tumour (lowers oedema surrounding them). restrict fluids, monitor closely, try and work out what's causing it and treat it.

Question 225

what syndrome is seen in uncal/transtentorial herniation (also what is this)?

Answer 225

Displacement of the uncus of the temporal lobe under the tentorium cerebelli. syndrome - ipsilateral fixed, dilated pupil (due to parasympathetic compression of the third cranial nerve) and contralateral paralysis (due to compression of the cerebral peduncle) - then coma Rx as for raised ICP emergency

Question 226

what syndrome is seen in tonsillar herniation? also what is this?

Answer 226

Displacement of the cerebellar tonsils through the foramen magnum - ‘coning’. increased pressure in posterior fossa forces cerebellar tonsils through foramen magnum. ataxia, 6th nerve palsies, upgoing plantar reflexes --> LOC, irregular breathing, apnoea -- proceed rapidly!!

Question 227

what syndrome is seen in subfalcian/cingulate herniation? also what is this?

Answer 227

Displacement of the cingulate gyrus under the falx cerebri - caused by frontal mass. may be silent unless anterior cerebral artery compressed causing stroke.

Question 228

outline classification of brain tumours

Answer 228

benign - normally not of the brain (meninges, cranial nerves etc) - histologically benign but still a SOL in cranial cavity so can be life-threatening malignant - often gliomas (glial cell origin - locally invasive but rarely metastasise) or secondary mets from elsewhere in body

Question 229

what are the three main ways malignant brain tumours can present? how would you investigate for them?

Answer 229

``` epilepsy raised ICP (chronic cause, although may acutely detiorate and cause coning) focal neurological deficit - depends on site of lesion e.g. dysphasia, hemiparesis, personality change etc ``` Ix - CT/MRI scans, screening for primary neoplasm (esp CXR).

Question 230

what are meningiomas? what are the specific ways they can present?

Answer 230

benign meningeal tumours - can present similarly to malignant brain tumours i.e. epilepsy, raised ICP, dysphasia, hemiparesis, dementia. if in olfactory groove = uni, then bilateral anosmia, papilloedema, frontal lobe dysfunction. in parasagittal region - spastic paraparesis mimicking spinal cord lesion, UMN signs in both legs as tumour is between cerebral hemispheres in cavernous sinus - unilateral ophthalmoplegia (III, IV and VI palsies) and trigeminal sensory loss optic nerve - contralateral papilloedema

Question 231

what are acoustic neuromas? how do they present?

Answer 231

benign, aka scwannoma - arises from nerve sheath cells in VIII nerve complex in internal auditory meatus. initially - unilateral sensorineural hearing loss due to damage to nerve within meatus. as it expands it can involve V and VII, then eventually get ipsilateral ataxia due to compression of brainstem/cerebellum and bulbar nerve palsies. eventually - raised ICP.

Question 232

what are pituitary adenomas? how do they present?

Answer 232

neurologically - classic is bitemporal hemianopia due to compression of optic chiasm + will have endocrine disturbances

Question 233

outline management options for brain tumours

Answer 233

surgery - benign tumours usually operable and curative, malignant/secondary normally not - although operation often needed to get histological diagnosis. radiotherapy - directed at tumour in gliomas, whole brain radiation if multiple metastases. drugs - AEDs for epilepsy, dexamethasone for raised ICP (only works in tumours!), chemo might be indicated in gliomas.

Question 234

what are the usual causative organisms of meningitis in adults?

Answer 234

``` Neisseria meningitidis (meningococcus) Streptococcus pneumoniae (pneumococcus) ``` rarer - mycoplasma tuberculosis - immunocompromised listeria monocytogenes - immunocomp, neonates, >60yos staphylococci - pts w/ head injuries, neurosurgical shunts

Question 235

list some neurological sequelae of menignitis

Answer 235

deafness (most common) other neurological: epilepsy, paralysis infective: sepsis, intracerebral abscess pressure: brain herniation, hydrocephalus

Question 236

what are the clinical features of meningitis?

Answer 236

severe headache - rapid onset (minutes to hours) pain/stiffness in neck and back vomiting photophobia might present w/ altered consciousness, seizures O/E - fever, tachycardia, shock, petechial/purpuric rash if meningococcal

Question 237

what neurological signs would you see in meningitis?

Answer 237

'meningism' - neck stiffness on attempted flexion, high-pitched 'meningeal' cry in infants Kernig's positive deteriorating level of consciousness raised ICP - papilloedema, bulging fontanelles CN palsies and other focal signs

Question 238

what would you see in CSF of untreated bacterial menigitis?

Answer 238

turbid CSF, raised CSF pressure polymorph leucocytosis raised protein, low glucose (eats sugar, sh*ts out protein)

Question 239

what would you see in CSF of viral meningitis?

Answer 239

clear or turbid CSF glucose slightly low (60-80% of plasma glucose) protein normal/raised lymphocytosis

Question 240

what would you see in CSF of tuberculous meningitis?

Answer 240

slightly cloudy CSF, fibrin web lymphocytosis raised protein, low glucose (eats sugar, sh*ts out protein)

Question 241

give some CI's to LP in meningitis - how else would you investigate?

Answer 241

focal neurological signs papilloedema deteriorating consciousness head CT needed to excluded mass lesion mimicking meningitis before you can do LP

Question 242

what investigations would you do for meningitis, beyond head CT/LP?

Answer 242

``` full blood count (neutrophilia) U&Es (hyponatraemia) CRP coagulation screen (for DIC) blood culture (can be +ve even with sterile CSF) whole-blood PCR blood glucose blood gas ```

Question 243

list some possible complications of meningitis

Answer 243

acute - seizures, abscess formation, hydrocephalus, inappropriate ADH secretion, septic shock DIC (if meningococcal) can be left with LDs, epilepsy, hearing loss - particularly if a child!

Question 244

how do you treat bacterial meningitis in adults? give initial, empirical treatment?

Answer 244

if pre-hosp and suspecting meningococcal (e.g. rash) - IM benpen immediately then urgent transfer. IV cefotaxime (+amoxicillin if >50yrs old)

Question 245

once CSF/blood cultures have come back, how do you treat bacterial meningitis?

Answer 245

*according to local policy/guidelines* BNF says: Meningococcal meningitis = IV benpen or cefotaxime Pneuomococcal meningitis = IV cefotaxime Haemophilus influenzae = IV cefotaxime Listeria = IV amoxicillin + gentamicin

Question 246

outline contact treatment for bacterial meningitis

Answer 246

give household contacts rifampicin or ciprofloxacin - if meningococcal disease. notify local health protection agency and/or PHE.

Question 247

what can cause brain abscesses?

Answer 247

otitis media, paranasal sinuses, head injury/surgery | distant spread e.g. bronchiectasis, pelvis, bacterial endocarditis

Question 248

what are the clinical features of a brain abscess?

Answer 248

collection of pus, effectively a SOL so features depend on location: raised ICP - common focal signs e.g. hemiparesis, ataxia etc seizures can have fever, but not always progression is over days or even a few weeks so a major differential is brain tumour

Question 249

how do you investigate a brain abscess?

Answer 249

CT or MRI LP is CI'ed due to coning risk. bloods - FBC (neutrophil leucocytosis) and blood cultures.

Question 250

how do you manage a brain abscess?

Answer 250

typically surgical - decompress/drain abscess, it may reform after wards. might give broad-spec abx until cultures done e.g. cefotaxime + metronidazole steroids may help with oedema

Question 251

list the different forms of neurosyphilis

Answer 251

- mild, self-limitng meningitis due to secondary syphilis - meningovascular syphilisy - gumma - tabes dorsalis - "general paralysis of the insane" - congenital neurosyphilis

Question 252

what is meningovascular syphilis?

Answer 252

inflammation of meninges and cerebrospinal arteries in tertiary syphillis presents as subacute meningitis w/ focal signs e.g. CN palsies, hemiparesis, wasting of intrinsic hand muscles (syphilitic amyotrophy)

Question 253

what is syphilitic amyotrophy?

Answer 253

wasting of intrinsic hand muscles seen in neurosyphilis

Question 254

what is gumma?

Answer 254

focal meningovascular disease seen in neurosyphilis - presents as intracranial mass lesion e.g. w/ epilepsy, focal deficits, raised ICP etc also present generally in tertiary syphilis as granulomatous lesions of skin/bone

Question 255

what is tabes dorsalis? what are the features?

Answer 255

seen in neuro/ tertiary syphilis - optic atrophy, Argyll Robertson pupils, ptosis - painful abdo crises - truncal analgesia - painless urinary retention - lancinating pains in legs - absent knee and ankle reflexes - ataxic stomping gait - positive Romberg's test (sways/falls when eyes closed) - downgoing plantars - loss of deep pain sense - can get ulcers

Question 256

what are the features of "general paralysis of the insane" as seen in neurosyphilis?

Answer 256

``` dementia, delusions of grandeur, frontal lobe features, epilepsy Argyll Robertson pupils, optic atrophy dysarthria tremulousness spastic paraparesis upgoing plantars ```

Question 257

how is neurosyphilis diagnosed and treated?

Answer 257

syphilis serology in blood/CSF CSF might also show lymphocytes, raised protein, oligoclonal bands Rx - IM procaine pencillin + oral probenecid often steroids given concurrently to prevent Jarisch-Herxheimer reaction (inflammatory response to rapid killing of spirochaetes)

Question 258

aetiology/pathogenesis of viral encephalitis

Answer 258

viral invasion of brain --> lymphocytic inflammatory reaction w/ necrosis of neurones and glia HSV1 most common in developed world (95%) also - herpes zoster, CMV, EBV, adenovirus, mumps

Question 259

what are the clinical features of encephalitis?

Answer 259

headache, fever, deteriorating level of consciousness over hours -> days. focal neuro signs - HSV commonly affects temporal lobe so e.g. aphasia seizures, vomiting, psychiatric symptoms

Question 260

how is encephalitis investigated/diagnosed?

Answer 260

CSF: lymphocytosis, elevated protein, normal glucose, raised pressure PCR for HSV CT: in HSV - medial temporal and inferior frontal changes (e.g. petechial haemorrhages) - normal in one-third of patients MRI is better EEG pattern: HSV = characteristic lateralised *periodic discharges* at 2 Hz

Question 261

how is encephalitis managed?

Answer 261

IV aciclovir (for HSV encephalitis) prognosis depends on how early this is started. ganciclovir is CMV, but prognosis a lot worse for this and other weird causes.

Question 262

give basic info on shingles vaccine

Answer 262

offered to all patients aged 70-79 years is live-attenuated and given sub-cutaneously main contraindications are immunosuppression. Side-effects injection site reactions less than 1 in 10,000 individuals will develop chickenpox

Question 263

what is shingles?

Answer 263

acute, unilateral, vesicular, itchy/painful rash in a single dermatome - due to reactivation of dormant VZV that has sat in the nerve root.

Question 264

how is shingles treated?

Answer 264

oral aciclovir - helps initial symps and reduced risk of post-herpetic neuralgia

Question 265

what is post-herpetic neuralgia?

Answer 265

persisting pain in a dermatome following shingles - can be severely painful, can't really treat it, lasts 1-2 yrs.

Question 266

what is herpes zoster ophthalmicus?

Answer 266

reactivation of the varicella zoster virus in the area supplied by the ophthalmic division of the trigeminal nerve features: vesicular rash around the eye, which may or may not involve the actual eye itself Hutchinson's sign: rash on the tip or side of the nose. Indicates nasociliary involvement and is a strong risk factor for ocular involvement

Question 267

how do you treat herpes zoster ophthalmicus?

Answer 267

oral antiviral treatment for 7-10 days, ideally started within 72 hours Topical antiviral treatment is not given in HZO oral corticosteroids may reduce the duration of pain but do not reduce the incidence of post-herpetic neuralgia ocular involvement requires urgent ophthalmology review complications - ocular: conjunctivitis, keratitis, episcleritis, anterior uveitis ptosis post-herpetic neuralgia

Question 268

what is Ramsay Hunt syndrome? features/management?

Answer 268

(herpes zoster oticus) - reactivation of the varicella zoster virus in the geniculate ganglion of the seventh cranial nerve. Features auricular pain is often the first feature facial nerve palsy vesicular rash around the ear other features include vertigo and tinnitus Management oral aciclovir and corticosteroids are usually given

Question 269

what kind of motor symptoms/signs might a patient with a spinal cord lesion present with?

Answer 269

usually UMN signs affecting both legs - spastic paraparesis if cervical spine lesion, might affect all 4 limbs - spastic tetraparesis - may also have mixed LMN/UMN features in upper limps due to damage to cord and nerve roots in the neck

Question 270

what kind of sensory symptoms/signs might a patient with a spinal cord lesion present with?

Answer 270

they'll have a sensory level - a threshold at which sensation below is impaired and above is normal. in presence of a spastic paraparesis, this indicates a spinal cause - although not that useful for localisation e.g. sensory level at T10 indicates a lesion at, or above, T10 rather than just at T10

Question 271

what kind of autonomic symptoms/signs might a patient with a spinal cord lesion present with?

Answer 271

bladder involvement is an early feature - urgency/frequency and then incontinence. bowel symps less common, poss will see constipation. erectile dysfunction common. general features indicating spinal disease = hx of neck/back pain or injury

Question 272

explain the difference between extrinsic and intrinsic spinal cord lesions, and how they might present?

Answer 272

``` extrinsic = compression by e.g. tumour, prolapsed disc - produces sensory loss including sacral dermatomes (saddle anaesthesia) intrinsic = damage more central parts of spinothalamic tract first, producing sacral sparing - but this is not a strict rule. ```

Question 273

what is Brown-Sequard syndrome?

Answer 273

characteristic pattern of sensory/motor deficits occurring when lesion damages only one side of cord (complete = cord hemisection) sensory pattern is accurately localising. ipsilateral UMN weakness (as descending corticospinal tracts decussate in medulla) proprioception/vibration loss is also ipsilateral (as posterior column ascending fibres don't decussate till medulla) BUT pain/temperature (spinothalamic) loss is contralateral (because these fibres decussate two levels above entry level)

Question 274

what are the tracts involved in Brown-Sequard syndrome and how does each one produce symptoms?

Answer 274

1. Lateral corticospinal tract --> Ipsilateral spastic paresis below lesion 2. Dorsal columns --> Ipsilateral loss of proprioception and vibration sensation 3. Lateral spinothalamic tract --> Contralateral loss of pain and temperature sensation

Question 275

what is syringomyelia?

Answer 275

CSF filled cavity (syrinx) develops centrally in the spinal cord, producing characteristic deficits - usually starts in lower cervical cord but could expand ‘cape-like’ (neck and arms) loss of sensation to temperature but preservation of light touch, proprioception and vibration - classic e.g. is they've burned their hands without noticing. Other sympts/signs - spastic weakness (predominantly of the upper limbs), paraesthesia, neuropathic pain, upgoing plantars and bowel and bladder dysfunction

Question 276

give some causes of syringomyelia

Answer 276

a Chiari malformation: strong association trauma tumours idiopathic

Question 277

how do you investigate/manage syringomyelia?

Answer 277

Ix - full spine MRI with contrast to exclude a tumour or tethered cord. brain MRI - to exclude a Chiari malformation. Treatment will be directed at treating the cause of the syrinx. In patients with a persistent or symptomatic syrinx, a shunt into the syrinx can be placed.

Question 278

what is cervical spondylosis?

Answer 278

most common cause of spinal cord disease >50yrs old degenerative (osteoarthritis) disease of C spine leads to compression due to: - calcification, degeneration, protrusion of intervertebral discs - osteophytes - calcification and thickening of longitudinal ligaments

Question 279

list some causes of spinal cord disease

Answer 279

MS (if <40yrs - most common) cervical spondylosis (most common if >50yrs) trauma - vertebral fractures, disc protrusions (although normally spontaneous rather than traumatic) infection - epidural abscess, Pott's disease of spine, syphilis, HIV inflammation - MS, postviral transverse myelitis, sarcoid, SLE, ankylosing spondylitis vascular - infarct of spinal cord, AV malformation, epidural haematoma congenital - Arnold-Chiari malformation --> syringomyelia inherited - hereditary spastic paraplegia neoplasm - vertebral mets compressing cord, benign extrinsic tumours (neurofibroma, meningioma), intrinsic tumours (ependymoma, glioma, mets) degenerative - in cord = MND, in spine = spondylosis w/ cord compression

Question 280

how do you manage a patient presenting with possible spinal cord compression?

Answer 280

MRI imaging/myelography give high dose oral dexamethasone to reduce oedema. treat cause.

Question 281

what is degenerative cervical myelopathy? give some symptoms

Answer 281

degenerative condition of cervical cord. symptoms can include any of the following - keep an eye out for progressive/deteriorating symptoms: - pain in neck, upper or lower limbs - loss of motor function - loss of digital dexterity, preventing simple tasks such as holding a fork or doing up their shirt buttons, arm or leg weakness/stiffness leading to impaired gait and imbalance - loss of sensory function causing numbness - loss of autonomic function (urinary or faecal incontinence and/or impotence)

Question 282

how should degenerative cervical myelopathy be diagnosed and treated?

Answer 282

C spine MRI is gold standard - shows disc degeneration and ligament hypertrophy, with accompanying cord signal change. early surgical decompression is key.

Question 283

what is cervical radiculopathy?

Answer 283

impingement of cervical nerve roots, often due to prolapse of degenerate cervical intervertebral disc backwards. also caused by spondylosis or tumours (rare)

Question 284

what clinical features might you see in a cervical radiculopathy?

Answer 284

neck pain radiating down arm (often in myotome distribution rather than dermatome) segmental muscle weakness loss of relevant tendon reflexes dermatomal sensory impairment

Question 285

outline the segmental innervation of upper limb muscles (ie by nerve route)

Answer 285

C5 - most shoulder movements, biceps C6 - brachioradialis (elbow flexion), wrist extension and abduction C7 - triceps (elbow extension), wrist extension and adduction, finger extension C8 - wrist flexion and adduction, finger flexion T1 - intrinsic muscles of hand

Question 286

how do you manage cervical radiculopathy?

Answer 286

if disc disease - conservative management with NSAIDs, muscle relaxants, use of collar, physiotherapy some will need cervical MRI with view to surgery (to widen foramen/remove disc material)

Question 287

what is cauda equina syndrome?

Answer 287

damage to / compression of cauda equina (bundle of nerves at bottom of spinal cord) usually due to lumbar disc herniation

Question 288

what are the features of cauda equina syndrome?

Answer 288

severe back pain urinary incontinence/retention reduced sensation in the perianal area (saddle anaesthesia) decreased anal tone

Question 289

how should you manage a patient presenting with cauda equina syndrome?

Answer 289

it can be rapidly progressive so refer for urgent MRI and surgical review - urgent surgical decompression needed (laminectomy) to prevent permanent paralysis/sphincter dysfunction. if malignant cause - dexomethasone to reduce oedema around the tumour and minimise compression.

Question 290

what clinical features might suggest a lumbar disc prolapse?

Answer 290

low back pain and tenderness sciatica (pain radiating down back of leg from buttock to ankle) wasting/weakness of gastrocnemius and soleus S1 sensory loss depressed ankle reflex if L5 root lesion compressed by L4/5 prolapse = sciatic pain + foot drop, + L5 sensory impairment

Question 291

how is sciatica/lumbar disc prolapse managed?

Answer 291

conservative - gentle exercise, NSAIDs, physiotherapy | root injections with local anaesthetic/steroids

Question 292

what is multiple sclerosis?

Answer 292

chronic cell-mediated autoimmune disorder characterised by demyelination in the central nervous system. *lesions disseminated in time and space!* first lesion usually optic neuritis

Question 293

briefly explain pathophysiology of MS

Answer 293

affects white matter of brain/spinal cord and optic nerves. chronic inflammatory damage to myelin - form 'plaques' (areas with lots of demyelination) reduction in conduction velocity leads to loss of signals down these pathways.

Question 294

what causes MS?

Answer 294

main theory is some kind of environmental agent triggers it in a genetically susceptible individual (major histocompatibility complex, MHC, has genetic role). it's 3x more common in women, typically diagnosed in a 20-40yo, and linked to latitude (how far north/south you live).

Question 295

what is optic neuritis? features?

Answer 295

inflammatory demyelination of one (less commonly = both) optic nerves - either part of MS or clinically isolated syndrome (or syphilis/diabetes can cause) features: unilateral decrease in visual acuity over hours or days poor discrimination of colours, 'red desaturation' pain worse on eye movement relative afferent pupillary defect central scotoma

Question 296

what is anterior cord syndrome?

Answer 296

caused by ischemia of the anterior spinal artery, resulting in loss of function of the anterior two-thirds of the spinal cord this incl descending corticospinal tract, ascending spinothalamic tract, and autonomic fibers. characterized by a corresponding loss of motor function, loss of pain and temperature sensation, and hypotension. symptoms come on really fast, Ix = MRI, Rx = treat cause (e.g. aortic aneurysm, vasculitis, sickle cell)

Question 297

how do you treat optic neuritis?

Answer 297

should resolve over weeks-months | sometimes give high dose oral steroids

Question 298

give some visual symptoms you might get in MS, apart from optic neuritis

Answer 298

optic atrophy Uhthoff's phenomenon: worsening of vision following rise in body temperature (can occur with sensory/motor features too) internuclear ophthalmoplegia

Question 299

what sensory features are typically seen in MS?

Answer 299

pins/needles numbness trigeminal neuralgia Lhermitte's syndrome: paraesthesiae in limbs on neck flexion

Question 300

what motor features are typically seen in MS?

Answer 300

spastic weakness: most commonly seen in the legs

Question 301

what cerebrellar/other features are typically seen in MS?

Answer 301

cerebellar - ataxia: more often seen during an acute relapse than as a presenting symptom tremor Others - urinary incontinence sexual dysfunction intellectual deterioration

Question 302

what are the different sub-types of MS?

Answer 302

- relapsing-remitting disease - secondary progressive disease - primary progressive disease - progressive-relapsing disease

Question 303

describe the course of relapsing-remitting MS

Answer 303

``` most common form, accounts for around 85% of patients acute attacks (e.g. last 1-2 months) followed by periods of remission with NO neurological signs ```

Question 304

describe the course of secondary progressive MS

Answer 304

describes relapsing-remitting patients who have deteriorated and have developed neurological signs and symptoms between relapses around 65% of patients with relapsing-remitting disease go on to develop secondary progressive disease within 15 years of diagnosis gait and bladder disorders are generally seen

Question 305

describe the course of primary progressive MS

Answer 305

accounts for 10% of patients progressive deterioration from onset more common in older people

Question 306

describe course of progressive relapsing MS

Answer 306

patient has acute attacks, with steadily worsening/progressing features in between rare

Question 307

how is MS diagnosed?

Answer 307

key is lesions dissemninated in time and space brain/spine MRI can show plaques - not specific to MS so need clinical features as well. LP - CSF shows oligoclonal bands by electrophoresis - indicates local synthesis of immunoglobulins.

Question 308

how do you manage an acute relapsed of MS?

Answer 308

high dose methylpred, IV or PO for 3-5 days - improves speed of recovery but not severity of the exacerbation

Question 309

how is modification of MS course achieved/attempted?

Answer 309

beta interferon can reduce relapse rate (certain criteria must be met before you can use it though) other drugs: - glatiramer acetate: immunomodulating drug - acts as an 'immune decoy' - natalizumab: a recombinant monoclonal antibody - fingolimod: sphingosine 1-phosphate receptor modulator

Question 310

what are the criteria for starting an MS patient on beta interferon?

Answer 310

- relapsing-remitting disease + 2 relapses in past 2 years + able to walk 100m unaided - secondary progressive disease + 2 relapses in past 2 years + able to walk 10m (aided or unaided) reduces number of relapses and MRI changes, however doesn't reduce overall disability

Question 311

describe symptomatic management options used in MS

Answer 311

for spasticity - baclofen, gabapentin, dantrolene, tizanidine, diazepam, botox injections into specific muscles, also physio! for cerebellar tremor - clonazepam, isoniazid, gabapentin for fatigue - excl other causes (e.g. anaemia, depression), trial amantadine - also mindfulness/CBT for bladder problems - oxybutynin or tolterodine, intermittent self-catheterisation for erectile dysfunction - sildenafil

Question 312

define mononeuropahty

Answer 312

damage to a single peripheral nerve e.g. by trauma (esp. pressure) or damage to their loody supple

Question 313

what is polyneuropathy?

Answer 313

multiple peripheral nerves affected by inflammatory/metabolic/toxic processes leading to a diffuse, distal, symmetrical pattern of damage - usually affects lower limbs before upper

Question 314

what is carpal tunnel syndrome?

Answer 314

compression of median nerve at wrist, as it passes through the carpal tunnel occurs e.g. in isolation (manual labour, typing at computers) or with causes of multiple neuropathies (e.g. diabetes) or with abnormal soft tissue 'crowding' the carpal tunnel

Question 315

give some medical conditions associated with carpal tunnel

Answer 315

``` pregnancy diabetes mellitus local deformity e.g. secondary to osteoarthritis, fracture rheumatoid arthritis myxoedema acromegaly amyloidosis ```

Question 316

what are the clinical features of carpal tunnel?

Answer 316

- pain/parasthesiae in hand - thumb, index and middle fingers - wasting and weakness of thenar eminence - sensory loss in hand (thumb + 2.5 fingers) - positive Tinel's / phalen's tests - often bilateral

Question 317

what are Tinel's / Phalen's tests?

Answer 317

``` Tinel's = tapping over carpal tunnel produces tingling paraesthesiae in thumb/2.5 finger distribution Phalen's = prayer sign (maximal wrist flexion) for 60 secs produces same tingling ```

Question 318

how is carpal tunnel diagnosed and treated?

Answer 318

electrophysiology shows prolonged action potential (motor and sensory) Rx - splinting of hand, esp at night; local steroid injection; surgical decompression

Question 319

what are the clinical features of an ulnar neuropathy?

Answer 319

ulnar can be damaged anywhere on course, but commonly at elbow - pain/tingling paraesthesiae from elbow down to forearm, down ulnar border of hand (little finger +0.5) - wasting/weakness of intrinsic muscles - sensory loss to little + 0.5 fingers - characteristic claw hand deformity in chronic lesions

Question 320

how is an ulnar neuropathy diagnosed/treated?

Answer 320

nerve conduction studies might localise site of lesion mild - splinting of arm at night with elbow extended surgical decompression - not as successful as for carpal tunnel

Question 321

what is a radial palsy?

Answer 321

pressure on radial nerve in upper arm can lead to acute wrist drop ± sensory loss in superficial radial nerve (back of hand, not really fingers) due to prolonged abnormal posture of arm - Saturday night palsy (arm draped awkwardly over arm chair)

Question 322

what is meralgia paraesthetica?

Answer 322

compression of lateral cutaneous nerve of the thigh as it passes under inguinal ligament sensory loss in an oval on the front/lateral thigh associated with change in weight

Question 323

what is a lateral popliteal palsy?

Answer 323

damage to common peroneal nerve common as it winds around fibular neck leads to foot drop - weakness of ankle dorisflexion and eversion and of extensor hallucis longus, w/ variable sensory loss foot drop can also be caused by an L5 lumbar root lesion.

Question 324

list some causes of multifocal neuropathy/mononeuritis multiplex

Answer 324

- malignant infiltration - vasculitis/connective tissue disease - RA, SLE, polyarteritis nodosa, Wegener's granulomatosis - sarcoidosis - diabetes mellitus - infection - leprosy, herpes zoster, HIV, Lyme disease - hereditary neuropathy with liability to pressure palsies

Question 325

list some causes of polyneuropathies

Answer 325

infection - leprosy, diphtheria, Lyme disease, HIV inflammatory - GBS, CIDP, sarcoid, Sjogren's, vasculitis (SLE, polyarteritis nodosa) neoplastic - paraneoplastic, paraproteinaemia metabolic - DM, uraemia, myxoedema, amyloid nutrition - vit deficiency esp thiamine, B12 toxic - alcohol, lead, arsenic, gold, mercury etc drugs - isoniazid, vincristine, cisplatinum, metronidazole, nitrofurantoin, phenytoin, amiodarone

Question 326

what is chronic inflammatory demyelinating polyneuropathy?

Answer 326

- cause of peripheral neuropathy - antibody mediated inflammation results in segmental demyelination of peripheral nerves - males more commonly affected than females - features similar to GBS, with motor features predominating - more insidious onset, over weeks to months - often thought of as the chronic version of GBS - high protein content in the CSF - treatment with steroids and immunosuppressants may have a role (unlike in GBS)

Question 327

outline the major causes of peripheral neuropathy according to motor vs sensory loss

Answer 327

Predominately motor loss: Guillain-Barre syndrome porphyria lead poisoning hereditary sensorimotor neuropathies (HSMN) - Charcot-Marie-Tooth chronic inflammatory demyelinating polyneuropathy (CIDP) diphtheria ``` Predominately sensory loss: diabetes uraemia leprosy alcoholism vitamin B12 deficiency amyloidosis ``` Alcoholic neuropathy: secondary to both direct toxic effects and reduced absorption of B vitamins sensory symptoms typically present prior to motor symptoms Vitamin B12 deficiency: subacute combined degeneration of spinal cord dorsal column usually affected first (joint position, vibration) prior to distal paraesthesia

Question 328

how do polyneuropathies present? treatment?

Answer 328

depends on cause, commonly distal numbness/pain (glove and stocking) motor - distal weakness, wasting foot/hand deformity if long standing - pes cavus/claw hand Rx - depends on cause - CIDP/vasculitis = steroids/immunomodulation

Question 329

what is myasthenia gravis?

Answer 329

autoimmune disorder - pts have circulating antibodies to acetylcholine receptors at the neuromuscular junction associated with thymus pathology (hyperplasia, atrophy, tumour/thymoma)

Question 330

what are the clinical features of myasthenia gravis?

Answer 330

key feature is muscle fatigability - muscles become progressively weaker during periods of activity and slowly improve after periods of rest: - extraocular muscle weakness: diplopia - proximal muscle weakness: face (myasthenic snarl), neck, limb girdle - ptosis - dysphagia, dysarthria (nasal speech) - bulbar signs - involvement of respiratory muscles - this makes it an emergency!

Question 331

how do you investigate myasthenia gravis?

Answer 331

- single fibre electromyography: high sensitivity (92-100%) - CT thorax to exclude thymoma - CK normal - serum acetylcholine autoantibodies: around 85-90% have these - in the rest, about about 40% have anti-muscle-specific tyrosine kinase antibodies - Tensilon test: IV edrophonium reduces muscle weakness temporarily - not commonly used anymore due to the risk of cardiac arrhythmia

Question 332

list some exacerbating factors for myasthenia gravis

Answer 332

most common = is exertion resulting in fatigability - symptoms become more marked during the day ``` drugs: penicillamine quinidine, procainamide beta-blockers lithium phenytoin antibiotics: gentamicin, macrolides, quinolones, tetracyclines ```

Question 333

what are the management options for myasthenia gravis?

Answer 333

long-acting anticholinesterase e.g. pyridostigmine - symptomatic relief, can end up needing increasing doses so get muscarinc side effects (salivation, vomiting, abdo pain, diarrhoea) immunosuppression: prednisolone then add azathiprine if still no response thymectomy - if there's a thymoma if really severe - plasma exchange/IVIG

Question 334

how does Duchenne's muscular dystrophy present? how do you differentiate Becker's from this?

Answer 334

progressive proximal muscle weakness from 5 years calf pseudohypertrophy Gower's sign: child uses arms to stand up from a squatted position 30% of patients have intellectual impairment Becker's = develops after the age of 10 years intellectual impairment much less common

Question 335

what are the dystrophinopathies?

Answer 335

- X-linked recessive - due to mutation in the gene encoding dystrophin, dystrophin gene on Xp21 - dystrophin is part of a large membrane associated protein in muscle which connects the muscle membrane to actin, part of the muscle cytoskeleton - in Duchenne muscular dystrophy there is a frameshift mutation resulting in one or both of the binding sites are lost leading to a severe form - in Becker muscular dystrophy there is a non-frameshift insertion in the dystrophin gene resulting in both binding sites being preserved leading to a milder form

Question 336

what are the common features of the myopathies? list some causes as well

Answer 336

Features: symmetrical muscle weakness (proximal > distal) common problems are rising from chair or getting out of bath sensation normal, reflexes normal, no fasciculation ``` Causes inflammatory: polymyositis inherited: Duchenne/Becker muscular dystrophy, myotonic dystrophy endocrine: Cushing's, thyrotoxicosis alcohol post-polio syndrome ```

Question 337

what is myotonic dystrophy?

Answer 337

autosomal dominant disorder - abnormally sustained muscle contraction (myotonia) - manifests as inability to release grip. features start age 20-30, involves cardiac, skeletal and smooth muscle. General features: - myotonic facies (long, 'haggard' appearance) - frontal balding - bilateral ptosis - cataracts - dysarthria Other features: - myotonia (tonic spasm of muscle) - weakness of arms and legs (distal initially) - mild mental impairment - diabetes mellitus - testicular atrophy - cardiac involvement: heart block, cardiomyopathy - dysphagia diagnosis - electromyopathy Rx - phenytoin, mexiletine

Question 338

what are the inflammatory myopathies?

Answer 338

polymyositis - can occur on its own or w/ autoimmune connective tissue disorders e.g. systemic sclerosis, fibrosing alveolitis, Sjogren's dermatomyositis - lilac (heliotrope) rash affecting face ± purple-red rash to knuckles and anterior chest wall, other estensor surgaces features - proximal myopathy ± dysphagia due to pharyngeal muscle involvement, muscle pain, tenderness, arthralgia, Raynaud's Rx - need histological confirmation, then treat with steroids and immunosuppressants e.g. azathioprine.

Question 339

give some info on statin associated myopathy

Answer 339

myopathy: includes myalgia, myositis, rhabdomyolysis and asymptomatic raised creatine kinase. Risks factors for myopathy include advanced age, female sex, low body mass index and presence of multisystem disease such as diabetes mellitus. Myopathy is more common in lipophilic statins (simvastatin, atorvastatin) than relatively hydrophilic statins (rosuvastatin, pravastatin, fluvastatin)

Question 340

what kind of myopathy can glucocorticoid steroids cause?

Answer 340

proximal myopathy

Question 341

what is cerebral palsy?

Answer 341

range of mostly motor neurological deficits arising from pre- or perinatal insults often in comb with LDs, behavioural problems and epilepsy

Question 342

list some risk/causative factors for cerebral palsy

Answer 342

prematurity antenatal - foetal hypoxia or infection, developmental abnormalities, twins, maternal age perinatal - postmaturity, traumatic delivery w/neonatal intracranial haemorrhage postnatal, if preterm - hypoxia, hypoglycaemia, cerebral ischaemia or haemorrhage, hypothermia postnatal, fullterm - infection, trauma, kernicterus

Question 343

explain some different patterns/manifestations of cerebral palsy

Answer 343

``` spastic diplegia (Little's disease) - congenital spastic parapareis, with shortening/deformity of legs --> walking difficulty. upper limbs spared. spastic hemiplegia - w/ hemianopic and hemisensory deficits, epilepsy, LDs athetoid - choreoathetiod movements developing in early childhood, usually normal cognitive function although dysarthria can make communication difficult ```

Question 344

how is cerebral palsy managed?

Answer 344

- multidisciplinary approach is needed - treatments for spasticity include oral diazepam, oral and intrathecal baclofen, botulinum toxin type A, orthopaedic surgery and selective dorsal rhizotomy - anticonvulsants, analgesia as required

Question 345

give some info on spina bifida

Answer 345

- Non fusion of the vertebral arches during embryonic development - Three categories; myelomeningocele, spina bifida occulta and meningocele - Myelomeningocele is the most severe type with associated neurological defects that may persist in spite of anatomical closure of the defect spina bifida occulta - skin and tissues (but not not bones) may develop over the distal cord, site may be identifiable by a birth mark or hair patch management is surgical. prevent with folic acid supplements in pregnancy

Question 346

what is an Arnold-Chiari malformation?

Answer 346

herniation of cerebellar tissue, and sometimes lower part of brainstem through foramen magnum - congenital.

Question 347

what is Huntington's disease? outline the genetics

Answer 347

inherited neurodegenerative condition, progressive and incurable condition resulting in death 20 years after the initial symptoms develop. Genetics: autosomal dominant trinucleotide repeat disorder: repeat expansion of CAG results in degeneration of cholinergic and GABAergic neurons in the striatum of the basal ganglia due to defect in huntingtin gene on chromosome 4 MRI - caudate atrophy, enlarged frontal horns

Question 348

what clinical features are typical of Huntington's disease?

Answer 348

presents at age 35-40ish (although genetic anticipation = might present earlier and earlier down the generations) progressive chorea and dementia/personality changes also - dystonia, saccadic eye moveemnts

Question 349

how do you manage Huntington's disease?

Answer 349

no treatment slows progression. | counselling and genetic testing for relatives.

Question 350

what is Wilson's disease?

Answer 350

autosomal recessive disorder characterised by excessive copper deposition in the tissues - esp liver and basal ganglia Metabolic abnormalities include increased copper absorption from the small intestine and decreased hepatic copper excretion. caused by a defect in the ATP7B gene located on chromosome 13.

Question 351

how does Wilson's disease present? how is it treated?

Answer 351

in childhood - cirrhosis in adolescence - neuro features - akinetic rigid syndrome, dystonia, cerebellar signs, psychiatric signs Kayser-Fleischer rings on slit lamp Ix - serum copper and caeruloplasmin, urinary copper excretion Rx - copper chelation with penicillamine

Question 352

what is Friedrich's ataxia?

Answer 352

an early-onset hereditary ataxias - autosomal recessive, trinucleotide repeat disorder characterised by a GAA repeat in the X25 gene on chromosome 9 (frataxin). The typical age of onset is 10-15 years old. Gait ataxia and kyphoscoliosis are the most common presenting features.

Question 353

what are the features of Friedrich's ataxia?

Answer 353

``` Neurological features: absent ankle jerks/extensor plantars cerebellar ataxia optic atrophy spinocerebellar tract degeneration ``` Other features: hypertrophic obstructive cardiomyopathy (90%, most common cause of death) diabetes mellitus (10-20%) high-arched palate

Question 354

what is Charcot-Marie-Tooth disease?

Answer 354

most common hereditary peripheral neuropathy results in a predominantly motor loss no cure, and management is focused on physical and occupational therapy. Features: - Frequently sprained ankles - Distal muscle weakness - Distal muscle atrophy - Hyporeflexia - Stork leg deformity - Pes cavus - Hammer toes

Question 355

describe the genetics of Alzheimer's disease

Answer 355

- most cases are sporadic - 5% of cases = autosomal dominant trait - mutations in the amyloid precursor protein (chromosome 21), presenilin 1 (chromosome 14) and presenilin 2 (chromosome 1) genes are thought to cause the inherited form - apoprotein E allele E4 - encodes a cholesterol transport protein

Question 356

describe the underlying pathology of alzheimer's diease

Answer 356

- macroscopic: widespread cerebral atrophy, particularly involving the cortex and hippocampus - microscopic: cortical plaques due to deposition of type A-Beta-amyloid protein and intraneuronal neurofibrillary tangles caused by abnormal aggregation of the tau protein - biochemical: there is a deficit of acetylcholine from damage to an ascending forebrain projection Neurofibrillary tangles = paired helical filaments are partly made from a protein called tau in AD tau proteins are excessively phosphorylated

Question 357

describe the non-pharmacological management of Alzheimer's disease

Answer 357

NICE recommend offering: - 'a range of activities to promote wellbeing that are tailored to the person's preference' - group cognitive stimulation therapy for patients with mild and moderate dementia other options to consider include group reminiscence therapy and cognitive rehabilitation

Question 358

describe the pharmacological management of Alzheimer's disease

Answer 358

NICE updated it's dementia guidelines in 2018: - three acetylcholinesterase inhibitors (donepezil, galantamine and rivastigmine) as options for managing mild to moderate Alzheimer's disease - memantine (an NMDA receptor antagonist) is in simple terms the 'second-line' treatment for Alzheimer's, NICE recommend it is used in the following situations: → moderate Alzheimer's in patient intolerant of, or have a contraindication to, acetylcholinesterase inhibitors → as an add-on drug to acetylcholinesterase inhibitors for patients with moderate or severe Alzheimer's → monotherapy in severe Alzheimer's

Question 359

what are the characteristic pathological features of Lewy body dementia?

Answer 359

alpha-synuclein cytoplasmic inclusions (Lewy bodies) in the substantia nigra, paralimbic and neocortical areas

Question 360

what are the features of Lewy body dementia? how is it diagnosed?

Answer 360

Features: - progressive cognitive impairment - parkinsonism - visual hallucinations (other features such as delusions and non-visual hallucinations may also be seen) Diagnosis: - usually clinical - single-photon emission computed tomography (SPECT) can be used (commercially known as a DaTscan)

Question 361

how is Lewy body dementia managed?

Answer 361

- both acetylcholinesterase inhibitors (e.g. donepezil, rivastigmine) and memantine can be used as they are in Alzheimer's. - AVOID antipsychotics/antiparkinsonian drugs - will make them worse!

Question 362

describe the underlying pathology of frontotemporal dementia (in Pick's disease)

Answer 362

Focal gyral atrophy with a knife-blade appearance is characteristic of Pick's disease. Macroscopic - Atrophy of the frontal and temporal lobes ``` Microscopic - Pick bodies - spherical aggregations of tau protein (silver-staining) Gliosis Neurofibrillary tangles Senile plaques ```

Question 363

what clinical features suggest a vascular dementia?

Answer 363

- abrupt onset and stepwise progression rather than smooth or gradual - presence of vascular disease - nocturnal confusion, fluctuating cognition - emotional lability it's due to multiple small infarcts in the brain

Question 364

what is a chronic subdural haemtoma?

Answer 364

mostly occurs in elderly/alcoholic, can follow minor head injury - pay not be able to identify history of trauma! Rupture of the small bridging veins within the subdural space rupture and cause slow bleeding - common in elderly/alcoholic because they have brain atrophy so bridging veins are held taught

Question 365

how does a chronic subdural haematoma present? what would you see on CT? management?

Answer 365

several week to month progressive history of either confusion, reduced consciousness or neurological deficit. On CT - crescentic in shape, not restricted by suture lines and compress the brain (‘mass effect’). chronic subdurals are hypodense (dark) compared to the substance of the brain (acute subdurals are hyperdense and appear bright) If incidental finding / no associated neurological deficit then manage conservatively If pt confused, has an associated neurological deficit or has severe imaging findings = surgical decompression with burr holes is required.

Question 366

what is motor neurone disease?

Answer 366

``` neurological condition of unknown cause which can present with both UMN + LMN signs. rarely presents before 40 years various patterns of disease: - amyotrophic lateral sclerosis - primary lateral sclerosis - progressive muscular atrophy - progressive bulbar palsy. ``` combination of clinical patterns seen in some

Question 367

give some info on amyotrophic lateral sclerosis MND

Answer 367

typically LMN signs in arms and UMN signs in legs in familial cases the gene responsible lies on chromosome 21 and codes for superoxide dismutase

Question 368

what type of signs would you see in primary lateral sclerosis MND?

Answer 368

UMN signs only

Question 369

what type of signs would you see in progressive muscular atrophy MND?

Answer 369

LMN signs only affects distal muscles before proximal carries best prognosis

Question 370

what type of signs would you see in progressive bulbar palsy MND?

Answer 370

palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei signs of mixed bulbar and psuedobulbar palsy e.g. wasted, fasciculating tongue but brisk jaw reflex carries worst prognosis

Question 371

what clinical features suggest MND?

Answer 371

- fasciculation - the absence of sensory signs/symptoms - the mixture of LMN and UMN signs - wasting of the small hand muscles/tibialis anterior is common Other features: - doesn't affect external ocular muscles - no cerebellar signs - abdominal reflexes are usually preserved and sphincter dysfunction if present is a late feature

Question 372

how do you investigate MND?

Answer 372

diagnosis is usually clinical, but: - nerve conduction studies show normal motor conduction and can help exclude a neuropathy - Electromyography (EMG) shows a reduced number of action potentials with an increased amplitude - MRI is usually performed to exclude the differential diagnosis of cervical cord compression and myelopathy

Question 373

MND management/prognosis?

Answer 373

Riluzole: - prevents stimulation of glutamate receptors - used mainly in amyotrophic lateral sclerosis - prolongs life by about 3 months Respiratory care: - non-invasive ventilation (usually BIPAP) is used at night Symptomatic: - anticholinergics for reducing saliva secretion when swallowing difficult - baclofen/dantrolene/diazepam for spasticity - antidepressants - quinine for cramp - laxative for constipation - phyiotherapy - home adaptations Prognosis: poor: 50% of patients die within 3 years

Question 374

list some metabolic causes of an acquired acute ecephalopathy (presenting with confusion/coma/seizures)

Answer 374

hypoxia hypoglycaemia respiratory/renal/hepatic failure electrolytes - sodium (high/low), calcium/magneisum high/low vitamin deficiencies endocrine disorders - hypopituitarism, thyroid up/down, myxoedema, insulinoma toxins - CO, lead, alcohol

Question 375

what deficiency causes Wernicke-Korsakoff syndrome?

Answer 375

``` vitamin B1 (thiamine) deficiency - urgent IV pabrinex (contains vitamins B and C - thiamine, riboflavin, pyridoxine, nicotinamide and ascorbic acid) usually occurs in chronic alcoholism, can also get in hyperemesis gravidarum! ```

Question 376

how does Wernicke's encephalopathy present? how does this link to Korsakoff's?

Answer 376

triad of: ophthalmoplegia - nystagmus, third/sixth nerve palsies ataxia confusion when this goes untreated, pt ends up with antero and retrograde amnesia + confabulation = Korsakoff's - can't really treat this!

Question 377

what is Lambert-Eaton myasthenic syndrome?

Answer 377

seen in association with small cell lung cancer, and to a lesser extent breast and ovarian cancer caused by an antibody directed against pre-synaptic voltage gated calcium channel in the peripheral nervous system. Features: - repeated muscle contractions lead to increased muscle strength - limb girdle weakness (affects lower limbs first) - hyporeflexia - autonomic symptoms: dry mouth, impotence, difficultly micturating - ophthalmoplegia and ptosis not commonly a feature (unlike in myasthenia gravis) EMG - incremental response to repetitive electrical stimulation Management - treatment of underlying cancer immunosuppression, for example with prednisolone and/or azathioprine intravenous immunoglobulin therapy and plasma exchange may be beneficial

Question 378

what is status epilepticus?

Answer 378

recurring seizures, patient doesn't regain consciousness between attacks, for 30 mins or more - though cut off to being treating usually 5 mins. risky because if untreated --> anoxia --> brain damage/death

Question 379

give some possible causes of status epilepticus

Answer 379

``` if existing seizure disorder - non-compliance, alcohol, sleep withdrawal no previous seizures: - trauma - infection - meningitis, encephalitis, abscess - tumour - stroke - metabolic, hypoxia - drug OD, alcohol ```

Question 380

describe management of status epilepticus

Answer 380

ABCDE !!!! in community - buccal midazolam or rectal diazepam, unless they have IV access in which case IV lorazepam - call ambulance in hospital: 1) IV lorazepam, if unavailable give IV diazepam, if can't get IV access - buccal midazolam 2) give two doses of first-line treatment 3) still no luck - IV phenobarbital or phenytoin if still nothing = 'refractory status epilepticus': adults - IV midazolam, propofol or thiopental sodium children/young people - IV midazolam or thiopental sodium

Question 381

what is Guillain-Barre syndrome?

Answer 381

acute inflammatory demyelinating polyneuropathy, often triggered by an infection (classically Campylobacter jejuni). mostly motor involvement, often including respiratory nd bulbar muscles making it an emergency

Question 382

describe the pathogenesis of Guillain-Barre syndrome?

Answer 382

- cross reaction of antibodies with gangliosides in the peripheral nervous system - correlation between anti-ganglioside antibody (e.g. anti-GM1) and clinical features has been demonstrated anti-GM1 antibodies in 25% of patients

Question 383

describe the features of Guillain-Barre

Answer 383

characteristic features = progressive weakness of all four limbs - maximum deficit reached within hours - days. - classically ascending weakness i.e. the lower extremities are affected first, however it tends to affect proximal (within the limb i.e. proximal lower limb, then rest of lower limb, then proximal upper limb etc) muscles earlier than the distal ones - sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs. Some patients experience back pain in the initial stages of the illness Other features: - areflexia - cranial nerve involvement e.g. diplopia - autonomic involvement: e.g. urinary retention Less common findings - papilloedema: thought to be secondary to reduced CSF resorption

Question 384

how do you investigate/diagnose GBS?

Answer 384

- LP = raised CSF protein w/ normal cell count - EMG + nerve conduction may confirm demyelinating neuropathy (all of these can be normal early in disease) - 25% of pts will have circulating ganglioside abs

Question 385

how do you manage a patient with GBS?

Answer 385

- regular vital capacity measurements to monitor for involvement of respiratory muscles - ECG montoring also - admit to ICU if VC deteriorating or swallow problems - can need artificial ventilation and NG feeding etc - LMWH for DVT prophylaxis - mouth and eye care immunological treatment - plasma exchange or high dose IVIG - speeds rate of recovery, reduces complications c. 80% will make complete recovery, but this can take months

Question 386

give some indicators for poor prognosis in Guillain-Barre syndrome

Answer 386

- increasing patient age - rapid onset of weakness - need for ventilation - antiganglioside abs - preceding diarrhoeal illnes - electrophysiology showing signif. axonal degeneration

Question 387

what is a myasthenic crisis?

Answer 387

acute myasthenia gravis symptoms affecting bulbar muscles, diaphragm, intercostal muscles can occur at any point in someone with severe MG. Rx - airway/ventilation support, plasma exchange/IVIG.

Question 388

what are the NICE criteria indicating a head injury patient requires immediate CT?

Answer 388

GCS < 13 on initial assessment GCS < 15 at 2 hours post-injury suspected open or depressed skull fracture. any sign of basal skull fracture (haemotympanum, 'panda' eyes, cerebrospinal fluid leakage from the ear or nose, Battle's sign). post-traumatic seizure. focal neurological deficit. more than 1 episode of vomiting

Question 389

what are the NICE criteria indicating a head injury patient requires CT within 8 hours?

Answer 389

adults with any of the following risk factors who have experienced some LOC or amnesia since the injury: age 65 years or older any history of bleeding or clotting disorders dangerous mechanism of injury (a pedestrian or cyclist struck by a motor vehicle, an occupant ejected from a motor vehicle or a fall from a height of greater than 1 metre or 5 stairs) more than 30 minutes' retrograde amnesia of events immediately before the head injury any patient on warfarin

Question 390

give some basic info on how head injury damages the brain

Answer 390

- primary brain injury may be focal (contusion/haematoma) or diffuse (diffuse axonal injury) - diffuse axonal injury occurs as a result of mechanical shearing following deceleration, causing disruption and tearing of axons - intra-cranial haematomas can be extradural, subdural or intracerebral, while contusions may occur adjacent to (coup) or contralateral (contre-coup) to the side of impact - secondary brain injury occurs when cerebral oedema, ischaemia, infection, tonsillar or tentorial herniation exacerbates the original injury. The normal cerebral auto regulatory processes are disrupted following trauma rendering the brain more susceptible to blood flow changes and hypoxia - the Cushings reflex (hypertension and bradycardia) often occurs late and is usually a pre terminal event

Question 391

what are the features of an extradural (epidural) haematoma?

Answer 391

Bleeding into the space between the dura mater and the skull. Often results from acceleration-deceleration trauma or a blow to the side of the head. majority of epidural haematomas occur in the temporal region where skull fractures cause a rupture of the middle meningeal artery. Features: - raised intracranial pressure - some patients may exhibit a lucid interval

Question 392

what are the features of a subdural haematoma?

Answer 392

Bleeding into the outermost meningeal layer. Most commonly occur around the frontal and parietal lobes. Risk factors include old age, alcoholism and anticoagulation. Slower onset of symptoms than a epidural haematoma.

Question 393

what are the features of a subarachnoid haemorrhage?

Answer 393

Classically causes a sudden occipital headache. Usually occurs spontaneously in the context of a ruptured cerebral aneurysm but may be seen in association with other injuries when a patient has sustained a traumatic brain injury.

Question 394

describe the CT findings of an extradural haematoma

Answer 394

hyperdense (bright), biconvex (or lentiform) collection around the surface of the brain.

Question 395

describe the CT findings of an acute subdural haematoma

Answer 395

hyperdense (bright), crescenteric collection surrounding the brain that is not limited by suture lines

Question 396

describe the CT findings of a chronic subdural haematoma

Answer 396

hypodense (dark), crescenteric collection around the surface of the brain that is not limited by suture lines

Question 397

describe the CT findings of an intracerebral haemorrhage

Answer 397

hyperdensity (bright lesion) within the substance of the brain.

Question 398

describe the CT findings of a SAH

Answer 398

hyperdensity within the cisterns or the brain and the sulci

Question 399

describe the CT findings of an intraventricular haemorrhage

Answer 399

hyperdensity within the dark CSF spaces within the ventricles.

Question 400

what are the clinical features of an extradural haematoma?

Answer 400

loss of consciousness, a lucid interval and then a rapid decline in consciousness. Mass effect on the brain will cause uncal herniation and a fixed, dilated pupil due to third cranial nerve compression.

Question 401

what are the clinical features of an acute subdural haematoma?

Answer 401

caused by high-speed injuries or acceleration-deceleration injuries and is therefore commonly associated with other brain injuries. spectrum of severity of clinical presentation from an asymptomatic patient to those who are severely comatose.

Question 402

what are the clinical features of a chronic subdural haematoma?

Answer 402

typically present several weeks after a mild head injury with progressive confusion, loss of consciousness, weakness or higher cortical function

Question 403

what is the definitive management of an extradural haematoma?

Answer 403

craniotomy and evacuation of the haematoma.

Question 404

what is the definitive management of an acute subdural haematoma?

Answer 404

decompressive craniectomy.

Question 405

what is the definitive management of a chronic subdural haematoma

Answer 405

definitive treatment is burr hole drainage - if symptomatic, if not just leave to resolve

Question 406

what is squint? how can it be divided?

Answer 406

Squint (strabismus) is characterised by misalignment of the visual axes. concomitant = due to imbalance in extraocular muscles, convergent is more common than divergent paralytic = due to paralysis of extra-ocular muscles can be examined using corneal light reflection test prefixes 'eso' = inward deviation 'exo' = outward

Question 407

what causes concomitant squint? management?

Answer 407

usually congenital if tested separately, they have full movement of both eyes extraocular muscles and nerves grossly normal no diplopia Rx - refer - correct refractory error, glasses with prisms, maybe surgery - needs correcting to avoid amblyopia

Question 408

what causes paralytic squint? management?

Answer 408

usually acquired damage to extraocular muscles or nerves third/fourth/sixth nerve palsies etc Rx - refer, treat cause if poss, prisms on glasses if not

Question 409

what is amblyopia?

Answer 409

``` lazy eye usually caused by: - constant strabismus. - asymmetric refractive errors. - any orbital pathology affecting ocular growth or clarity e.g. early onset cataract ``` presents either with visible strabismus, or unilateral deterioration in acuity

Question 410

list some treatment options for amblyopia

Answer 410

- correct refractory errors - treat any treatable cause - patch to occlude the healthy eye

Question 411

list some features of a seizure that might indicate NEAD

Answer 411

- tend to last longer than epileptic - e.g. up to and over 25 mins - might go in and out of episodes - atypical episodes - eyes screwed shut (you can't open them), thrashing about or stiff as a board, move (slowly) away when you approach, back arching, thrusting of hips - might still see tongue biting/incontinence - more aware of surroundings - can remember conversations occurring during episode - won't drop their O2 sats like in epilepsy - if they're getting loads, they tend to only come on in stressful situations/when people will definitely be around - no post-ictal confusion, recovery 'instant' (<1 min) - don't tend to injure themselves like epileptic seizures do, because they don't happen when driving/cooking etc

Question 412

list some vascular causes of unilateral visual loss

Answer 412

- amaurosis fugax/central retinal artery occlusion - central retinal vein occlusion - anterior ischaemic optic neuropathy - GCA

Question 413

list some non-vascular causes of unilateral visual loss

Answer 413

- optic neuritis - retinal detachment - floaters with reduced acuity - vitreous haemorrhage - also floaters - acute angle-closure glaucoma

Question 414

give some features suggestive of optic neuritis

Answer 414

- reduced acuity over a few days - pain on moving eye - exacerbated by heat/exercise - afferent pupillary defect - dyschromatopsia (reduced colour vision, especially loss of red)

Question 415

what is 'pinhole correction'?

Answer 415

used when test visual acuity - corrects refractive error to see if it's a neuro problem or if the patient just needs glasses;

Question 416

what is a Marcus Gunn pupil?

Answer 416

reduced afferent pupillary reflex, sign of past optic neuritis

Question 417

what features suggest acute angle-closure glaucoma?

Answer 417

painful red eye, nausea/vomiting, cloudy elliptical pupil, can be triggered by ipatropium bromide - suspect it if they were fine till they used their inhalers!

Question 418

list the DANISH cerebellar signs

Answer 418

``` Dysdiadochokinesis Ataxia Nystagmus Intention tremor Slurred, staccato speech Hypotonia ```

Question 419

what is syncope?

Answer 419

abrupt and transient LOC with loss of postural tone that follows a sudden fall in cerebral perfusion

Question 420

what is a seizure?

Answer 420

clinical manifestation of disordered electrical activity in the brain