Neuro MKSAP/Harrison's QBank

Question 1

What is postural orthostatic tachycardia syndrome?

Answer 1

A change from the supine position to an upright position causes an abnormally large increase in heart rate (and causes symptoms, usually presyncopy) It is thought to be related to mutations in the noradrenaline transporter

Question 2

What is Stiff-Person syndrome?

Answer 2

Paraneoplastic syndrome with Anti-amphiphysin antibodies associated with SCLC and breast cancer. These antibodies are pretty much anti-GABA-pathway. Causes progressive rigidity and spasms (particularly trunk in early days)

Question 3

Can you explain anything about EEG lead placement? hint: there are numbers and letters. Do odds = left or right?

Answer 3

The International 10:20 system - usually details to the left of the wave A, earlobe; C, central; F, frontal; Fp, frontal polar; P, parietal; T, temporal; O, occipital. Right-sided placements are indicated by even numbers, left-sided placements by odd numbers, and midline placements by Z.

Question 4

What happens to an EEG during metabolic encephalopathy? Can you comment about what might happen in space occupying lesion?

Answer 4

he EEG generally slows in metabolic encephalopathies, and triphasic waves may be present. Periodic lateralizing epileptiform discharges (PLEDs) are commonly found with acute hemispheric pathology such as a hematoma, abscess, or rapidly expanding tumor

Question 5

What are the major findings seen on EMG? What are the characteristics of myopathic changes vs neuropathic?

Answer 5

TL;DR: 1. myopathic is short, small amp contraction. 2. neuropathic is long, diffuse contraction (think that some nerve fibres are fast, some slow - all over the shop). 3. fibrillations, which are NOT the same as myotonic discharges

Activity recorded during EMG.

A. Spontaneous fibrillation potentials and positive sharp waves.

B. Complex repetitive discharges recorded in partially denervated muscle at rest.

C. Normal triphasic motor unit action potential.

D. Small, short-duration, polyphasic motor unit action potential such as is commonly encountered in myopathic disorders.

E. Long-duration polyphasic motor unit action potential such as may be seen in neuropathic disorders

Question 6

Case from Harrison’s:

37 year old man, first ever seizure. Now post-ictal with nuchal rigidity. Drug screen positive for cocaine.

What’s your investigation sequence?

Answer 6

Harrison’s argues that exclusion of meningitis is most important. CT head may be indicated if there are concerns with elevated ICP, but MRI is not the primary investigation.

Question 7

This is a LOW YIELD QUESTION:

Approximately what percentage of adults can discontinue AEDs? What percent of patients with mesial temporal lobe epilepsy respond to surgery?

Answer 7

60% for discontinuation

70% respond to temp lobe surgery in MTLE

Question 8

Which of the following does NOT have a role in primary or secondary prevention of atherothrombotic stroke ? (from Harrison’s)

1. aspirin
2. BP control
3. clopidogrel
4. statins
5. warfarin

Answer 8

Warfarin.

The WARSS study found no benefit of warfarin over aspirin for 2ndary prevention in atherothrombotic disease. Warfarin’s role is apparently only in AF/cardiocerebral emboli-related stroke.

Question 9

Restless legs syndrome requires four main symptoms for diagnosis. Can you list a few? (all four if you can, champ!)

Are there any investigations we should do for RLS? (any causes of secondary)

Answer 9

1. an urge to move legs accompanied by unpleasant sensation in legs
2. symptoms begin or worsen when at rest
3. feel better after movement
4. worse during evening/night

Harrison’s recommends testing for ferritin (anaemia) and renal failure. Can also be associated with peripheral neuropathy

By the way - Primary RLS has a genetic component.

Question 10

Do you know what complex regional pain syndrome (CRPS) is?

Could you explain a little about the pathogenesis and the symptomatology please.

Answer 10

Used to be known as “Reflex sympathetic dystrophy”.

There are two types. CRPS type I: regional pain syndrome usually after tissue trauma - but not necessarily the same tissue as is affected by the condition (e.g. after MI, stroke, minor shoulder injury). In this version, pain is not confined to a single peripheral nerve area.

CRPS type II: occurs after injury to a specific nerve - and initially symptoms are localised to that nerve, but may eventually spread.

Symptoms of both: spontaneous pain, vasomotor dysfunction, sweat gland abnorm, focal oedema.

Eventually can lead to skin atrophy and contractures

Question 11

Should trigeminal neuralgia have sensory loss associated with it?

Does the absence or presence of sensory loss lead to any other investigation?

Answer 11

Typically trigeminal neuralgia does not have sensory loss. Only pain along the sensory divisions.

Sensory loss would inspire the need for MRI/neuroimaging

Question 12

What diagnosis do you think of with:

progressive lower limb weakness and a less of sensation “below the belly button” and incontinence?

Answer 12

Myelopathy is the thing we were going for in this case.

The next step of the differential is compressive vs non-compressive myelopathy.

Question 13

What are the major causes of compressive myelopathy?

(the answer has 4 things)

Answer 13

tumour

epidural abscess or haematoma

herniated disc

vertebral pathology

Question 14

what are the major causes of non-compressive myelopathy?

5 main types

Answer 14

spinal cord infarction

systemic disorders like vasculitis, SLE, sarcoid

infections (particularly viral)

demyelinating disease

idiopathic

Question 15

What is the typical clinical presentation of syringomyelia?

(also give a little about demographics and predisposing features)

Answer 15

over 50% is associated with a Chiari malformation

usually symptoms start in teen

can be acquired though

classic presentation is central cord syndrome - pain and temperature loss, and upper limb weakness (because the motor nerves cross near the central canal - and the syrinx is usually in the cervical cord)

eventually progresses and patient gets UMN signs in legs

Question 16

what are some of the symptoms of post-concussive syndrome?

Answer 16

this includes a constellation of symptoms including fatigue, headache, dizziness and difficulty concentrating

typically improves over a 6-12 month period

patients who were energetic and high functioning have an excellent prognosis

Question 17

A patient is admitted with seizures. He is immunocompromised 2nd AIDS. Head CT shows ring enhancing lesion in the temporal lobe. Toxo IgG is positive.

CSF is negative Gram stain.

Treatment for toxo and keppra is initiated.

2 weeks down the track, CT looks the same, but no more seizures

What’s the next test? Do we continue the toxo-treatment?

Answer 17

Distinguishing CNS toxo from primary CNS lymphoma can be tricky in the immunocompromised patient.

Standard practice: in neurologically stable patient - treat for toxo for 2-3 weeks, then re-image.

if imaging shows clear improvement, continue toxo treatment.

If there is no improvement -> brain biopsy and toxo therapy does not need to be continued.

When we did the LP on arrival, we should have sent a CSF EBV DNA. If this was no positive (in an immunocompromised patient who didn’t respond to treatment), then we would be able to diagnose CNA lymphoma.

Treatment includes whole brain radiation

Question 18

Which of the pituitary hormones is most sensitive to whole brain radiation?

Which is the least sensitive?

Answer 18

GH is MOST

TSH is least.

therefore, patients with WBRT can become GH-deficient first.

the other pit hormones have intermed sens

Question 19

There are a few types of CJD. What are they associated with?

vCJD, sCJD, fCJD and iCJD (infectious)

Answer 19

variant CJD = the infectious one that people in Europe got from bovine infected with BSE

sporadic = seemingly random in adults in 5th-6th decade. MOST COMMON

familal CJD = autosomal dominant with germ line mutation

infectious = seen in all those poor bastards that got GH in the UK from cadavers

Question 20

What are the treatment options for chronic fatigue syndrome?

Answer 20

CBT and graded exercise therapy are the only that have been shown to be helpful.

at present no evidence for pharmaceuticals

Question 21

Copper deficiency can cause some neurological problems. Any idea what the main thing it causes is?

Are there any metal issues that are associated with copper deficiency?

Answer 21

Copper deficiency most commonly causes a myelopathy (although peripheral neuropathy have been described)

Zinc TOXICITY can lead to acquired copper deficiency.

Question 22

Does pregnancy do anything for MS relapse rates?

Does smoking do anything to MS relapse rate?

Answer 22

Pregnancy decreases the rate of relapse, especially in the 3rd trimester. However, the post-partum period is associated with an increased rate!

smoking increases risk of secondary progression

Question 23

There are a bunch of different proteins associated with neurodegenerative conditions.

Please list the protein for the following:

Parkinson’s, dementia with Lewy body, Frontotemporal dementia, CJD, Alzheimers

Answer 23

Major degenerative dementias associated with abnormal aggregation of a specific protein

1. Alpha synuclein:

multiple systems atrophy

Parkinsons

Dementia with lewy bodies

2. Tauopathies

progressive supranuclear palsy

Frontotemporal dementia

3. Amyloidopathies

Alzheimers – EXTRAcellular plaques of amyloid (Abeta42), also INTRAcellular tangles of Tau and Lewy bodies with alpha synuclein

4. Prion related protein

CJD

Question 24

What condition can cause post-infectious syndrome with sub-acute onset of weakness, sensory changes and bowel or bladder dysfunction? Might be associated with thoracic pain level. How do we treat?

Answer 24

Idiopathic transverse myelitis. Although recurrence might suggest MS. The presence of a sensory spinal cord level and hyper-reflexia localises disease to the spinal cord (ruling out GBS). After exclusion of other potential causes, treat with IV methylpred. If refractory, plasmapheresis or cyclophosphamide.

Question 25

What are some guidelines for sport related concussions? (i.e., exclusion from sport, neuro-imaging etc)

Answer 25

Grade 1 = transient confusion without amnesia or LOC. If exam normal and asymptomatic, can rejoin at 15 minutes. Grade 2 = transient confusion > 15min. No amnesia/LOC. If normal after 1 week, can rejoin. Consider imaging if persistent abn Grade 3 = brief or prolonged LOC. If exam normal = home with supervision. If exam abn (includ confusion) = CT head. No sport for 1 week, unless prolonged (2 weeks!)

Question 26

What is the best choice of AED for reproductive age women?

Answer 26

The best is often considered to be the one that provides best control. Carbamazepine has been shown to have low risk of foetal malformations (risk probably highest in first weeks of gestation). (2-4%) Lamotrigine and levetiracetam are also used and thought safe. Valproate has consistently been shown to be highest risk (6 - 17%). Phenytoin (6-14%) and phenobarbital (3-7%) also higher.

Question 27

What are options for treating post-stroke lysis hypertension? What are our goals?

Answer 27

Ideally <180/105. Labetalol and nicardipine are the options listed in UTD. Nitroprusside is relatively contraindicated because of possibility of increasing intracranial pressue.

Question 28

Patient admitted with stroke and INR of 1.5. What is the most appropriate treatment?

Answer 28

Interestingly, TOAST and IST showed no benefit in preventing stroke. HAEST (Heparin in Acute Embolic Stroke Trial) showed no benefit aspirin V heparin at 14 days post acute ischaemic stroke Acute anticoagulation is only if: 1. mechanical valve; 2. AF with small infarct post-cardiac surgery; 3. cervicocephalic dissection

Question 29

Which causes greater rates of first division pain? Trigeminal neuralgia or herpes zoster affecting CN V? Tell me about management of both.

Answer 29

Only 5% of trigeminal neuralgia affects the ophthalmic branch, whereas zoster has much higher rates. The pain of tic douloureux also lasts only 5 seconds to 2 minutes (by definition). Management of trigeminal neuralgia: first line is carbamazepine, initiated at low dose and slowly uptitrated. Second line agents (limited evidence) - baclofen, gabapentin, clonazepam, lamotrigine. There are surgical options, whereby they cut the nerve fibre

Question 30

What is the definition of episodic versus chronic migraine? What is the definition of a migraine?

Answer 30

episodic means 15 days/month for 3 months. Migraine can be defined as “with aura” or “without aura”. Without aura requires >5 headaches lasting 4-72 hours; 2 of [unilateral, pulsating, aggravation on physical activity, mod-severe pain impacting daily activity]; 1 of [N/v, photo/phonophobia] with aura: 2 attacks fully reversible aura symptoms; one of the aura symptoms develops slowly - over more than 4 minutes duration <60 minutes for aura; headache after aura

Question 31

what are the treatment options for episodic migraine prevention?

Answer 31

MKSAP suggests that only topiramate and onabotulinum toxin A. Older options includes propranolol, timolol, amitriptyline and divalproex sodium.

Question 32

What are the risks with migraine and being child-bearing age female?

Answer 32

GREAT QUESTION! *ahem* 2-4 fold increase in stroke risk if COCP and migraine (any type) Seems to be much higher in women with migraine with aura (recommended to avoid OCP in these women) - stats missing, but just avoid migraine w/ aura + OCP

Question 33

What are the therapeutic options for tension-type headaches?

Answer 33

Aspirin and paracetamol are the best. Addition of caffeine has also been shown to potentiate effects.

Question 34

Treatment options for cluster headaches?

Answer 34

Acute - high flow oxygen is useful in 75%; other options include sumitriptan and zolmitriptan Transitional (short term use to prevent the ongoing attacks) - steroids Preventative - verapamil (often in high doses!)

Question 35

An idea of the electrode numbering system with EEG?

Answer 35

If the electrode is on the right side, even numbers are used. e.g. F8-T8 Left side = odd numbers. e.g. C3-P3

Question 36

Which of the anti-epileptic drugs are most associated with Stevens-Johnson syndrome?

Answer 36

They all are, but actually rapid up-titration of lamotrigine and combination lamotrigine and valproate is higher risk. There is theoretical increased risk of Stevens-Johnson in Asian with HLA-B*1502 allele, so may be a good idea to test before starting lamotrigine, phenytoin or carbamazepine.

Question 37

what is the definition of refractory epilepsy? how would you manage such a patient?

Answer 37

failure to respond to 2 agents. These patients should be referred to subspecialty unit for evaluation, confirmation of diagnosis and consideration for surgery.

Question 38

Dural sinus venous thrombosis has a few characteristic presenting features. Please describe them. Also comment on the different sinuses and any specific presenting features.

Answer 38

Headache (severe), depressed LOC and seizures. Fundoscopic examination can reveal papilloedema in 50% of dural sinus thrombosis. Thrombosis of the cavernous sinus causes lesions of CNIII ,CNIV, CNV1/2 and sometimes CNVI. If the cortical veins are involved, they can get ischaemic and then haemorrhage.

Question 39

Which stroke risk factor has the greatest impact?

Answer 39

According to MKSAP, hypertension has greatest effect.

Question 40

What are the pathologic findings of Alzheimer disease? And the radiographic?

Answer 40

Includes: diffuse brain atrophy with widening of sulci and enlargement of ventricles histologically: neurofibrillary tangles (knotted loops [strands of silver-stained material within cell bodies of neurons] and helices of hyperphosphorylated tau microtubular protein), amyloid-containing neuritic plaques, and granulovacuolar degeneration of neurons.

Question 41

What are the pharmaceutical treatment options for Alzheimer disease?

Answer 41

Focuses on acetylcholinesterase inhibitors, although galantamine also has an agonist effect on nicotine receptor. Donepezil - AChE inhibitor; rivastigmine - AChE inhibitor; Tacrine - AChE inhibitor; Galantamine - AChE inhibitor & nicotinic receptor agonist Memantine - NMDA inhibitor

Question 42

Describe the clinical features and pathological findings in Lewy body dementia please. How to treat? What about hallucinations and psychosis?

Answer 42

May be pattern of cognitive impairment with psychiatric features of personality change, apathy, depression and psychosis. Motor signs of parkinsonism occur and are usually poorly responsive to levodopa. Also get dream-enactment. Pathologically widespread Lewy bodies - eosinophilic intracytoplasmic accumulations of alpha-synuclein. Treat with AChE-inhibitors as per AD. For hallucination Tx = avoid dopamine blockers. Good choice = clozapine.

Question 43

What is the classic triad of normal pressure hydrocephalus?

Answer 43

1. cognitive decline 2. gait impairment - broad base, short shuffling steps, characteristic hesitation/freezing on step initiation and near-normal leg function in supine/sitting position. 3. urinary urgency and incontinence all of these are in the setting of normal CSF pressure (usually <15cm H20)

Question 44

Generally the medications that cause tardive dyskinesia are associated with blockade of a type of receptor. What is that type?

Answer 44

Is it the dopamine - type 2 (D2) receptor.

Question 45

What investigations are appropriate in patients with recurrent thunderclap headache?

Answer 45

If recurrent thunderclap headache, then reasonable to image the intracranial vasculature - as provided by angiography.

Question 46

What is the treatment of Bell’s palsy?

Answer 46

Prednisone, preferably within the first 72 hours of symptoms.

Question 47

Following thrombolysis for acute ischaemic stroke, when do we give the first dose of aspirin?

Answer 47

The trials have all withheld anti-platelet agents for 24 hours post lysis.

Question 48

If a patient is on lamotrigine and starts the OCP, what needs to happen to the medication? Which AEDs should patients not take COCP with?

Answer 48

Oestrogen containing therapies lead to increased “glucuronidation” - reducing the levels of lamotrigine by up to 40%. Caution is also needed during the placebo weeks of the pill, as lamotrigine levels can become supratherapeutic. Carbamazepine, phenytoin, barbiturates, topiramate - all induce enzymes leading to inadequate contraception.

Question 49

What are the differences between trigeminal neuralgia and paroxysmal hemicrania? Recall - paroxysmal hemicrania is a type of primary headache in the same class as cluster headache

Answer 49

1. location - trigeminal neuralgia is typically V2-V3. Hemicrania usually V1. 2. Duration - trigeminal neuralgia is typically 2-3 seconds of pain then seconds - hours of painfree Paroxysmal hemicrania is a cluster headache-like syndrome - 15 min up many times/day 3. Associated - trigeminal neuralgia - typically none Paroxysmal hemicrania is often associated with autonomic symptoms (like cluster headache) 4. Treatment - trigeminal neuralgia - carbamazepine is first line Paroxysmal hemicrania - indomethacin is first line.

Question 50

What are the common complications of subarachnoid haemorrhage - describe as early and late.

Answer 50

Early - aneurysmal re-rupture hydrocephalus Late (from day 5) - arterial vasospasm (which can lead to cerebral infarction) - CT angiography is first line to assess this.

Question 51

What is Capgras syndrome? Where is the lesion?

Answer 51

A delusional disorder in which a person holds a delusion that a friend, spouse, parent, or other close family member has been replaced by an identical-looking impostor. It can also occur with places/locations. It is primarily associated with psychiatric illness, but can occur with dementia. If associated with a focal lesion = non-dominant hemisphere. It can also occur with diffuse disease such as Alzheimer.

Question 52

What are the treatment options for status epilepticus?

Answer 52

First line = benzodiazepines. Lorazepam 0.1mg/kg IV or diazepam IV/PR (shorter acting than lorazepam) Second line agents = phenytoin (or fosphenytoin - its prodrug) or IV valproic acid Third line agents = no specific data exists to guide this.

Question 53

What paraneoplastic syndrome is associated with ovarian neoplasm? What is the typical radiographical finding?

Answer 53

Anti-NMDA receptor encephalitis is characterised by subacute memory disturbance, personality change, psychosis and seizures. There is also an association with oral dyskinesia. Most commonly this syndrome is associated with unilateral or bilateral ovarian teratomas. On brain MRI (FLAIR) - increased signal in temporal lobes (uni or bilateral). Diagnosed by Ab found in serum or CSF. Rx: oophopectomy + plasmapheresis/IVIg/steroids

Question 54

What are the treatment options for MS-related fatigue? (And is there anything that we need to exclude before diagnosing such?)

Answer 54

At minimum: exclusion of anaemia, sleep disorders, depression, hypothyroidism. Treatment options are amantadine (off label use; NMDA-R antagonist) and modafinil (also used for wakefulness in sleep apnoea, MoA = unknown)

Question 55

What is apraxia, and where is the lesion usually?

Answer 55

Apraxia is the inability to perform a previously learned skilled motor task despite intact motor and sensory systems, clear comprehension and full cooperation. The lesion is typically dominant parietal lobe

Question 56

Which migraine prophylaxis should be avoided in patients with a history of kidney stones?

Answer 56

Topiramate has a mild carbonic anhydrase inhib effect, leading to dec. prox tubule reabs of bicarb, leading to increased risk of calcium-containing renal stones.

Question 57

What has strongest benefit in a patient with symptomatic high-grade carotid stenosis - statin, aspirin, stenting or endarterectomy?

Answer 57

Carotid endarterectomy has far and away the greatest benefit. After a stroke, a patient has a 2% risk of stroke recurrence PER DAY for the first 14. NNT = 6 according to stroke lecture (Dr Read) Stenting was shown to be equal to endarterectomy, but has greater risk of peri-op stroke. Statins - trials haven’t investigated safety in immediate post-stroke setting. Aspirin only small benefit compared to surgery

Question 58

What is the “best” clinical test to assess for risk of falls? (particularly backwards fall, apparently)

Answer 58

The pull test has been examined in studies (don’t ask which) and is the best way of predicting the risk of future falls in a patient with a history of BACKWARDS falls. Basically the test is - stand behind patient, warn them, pull backwards. Normal response is small step back to recover. Abnormal is ineffectual short steps, or just plain falling.

Question 59

How do we manage multiple sclerosis related spasticity?

Answer 59

The most commonly used in Australia is baclofen. Also consider diazepam. In the US, tizanidine (centrally acting, alpha2-R agonist) is also used. Refractory symptoms can trial botulinum toxin injections or even intrathecal baclofen pump.

Question 60

What is most appropriate AED for partial seizures in older patients?

Answer 60

There are multiple options for partial seizures, but the side effect profile of lamotrigine makes a compelling argument for it being best in older population. Carbamazepine and phenytoin, for example, are commonly complicated by fizziness, lethargy and gait instability. They are also hepatic enzyme inducers, making life more complicated… Carbamazepine is also associated with hyponatraemia.

Question 61

What conditions might be associated with rapid (e.g. 5 weeks) decline to inanition. Also with evidence of myoclonus, and no other signs of trauma, intoxication, fever or any other systemic disease.

Answer 61

A paraneoplastic condition such as limbic encephalitis OR a prion disorder such as Creutzfeldt-Jakob disease. CJD can present with similar syndrome as frontotemporal dementia, but it is much more rapid.

Question 62

Why is metoprolol not used in cocaine-associated intracranial haemorrhage? What is our BP goal for treatment?

Answer 62

The unopposed alpha-action of cocaine can potentially lead to coronary (and possibly) cerebrovascular vaso-constriction. Labetalol is a better choice of the beta blockers. The goal is to bring down to < 160/90

Question 63

What are the behavioural changes that one might see with dopamine-agonist?

Answer 63

Compulsive behaviours are seen, due to dysregulation of the brain’s dopamine-dependent reward systems. These are not the same as Lewy body dementia (dream-enactment, cognitive decline, parkinsonism, visual hallucination). Frontotemporal dementia has features of apathy, impulsivity, perseveration, hoarding, obsessionality

Question 64

What is the first line anti-epileptic for patients with Asian background?

Answer 64

Levetiracetam is usually pretty good. Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and drug rash with eosinophilia and systemic symptoms (DRESS) are rare but severe idiosyncratic reactions, characterized by fever and mucocutaneous lesions that have been associated with the use of carbamazepine, oxcarbazepine, phenytoin, phenobarbital, primidone, zonisamide, lamotrigine, and (less commonly) other AEDs. The period of highest risk is within the first two months of use. The risk may be higher in patients with HLA-B*1502 allele, which occurs almost exclusively in patients of Asian ancestry, including South Asian Indians. The FDA recommends screening such patients for this allele prior to starting carbamazepine, oxcarbazepine, and possibly phenytoin. (There is some specialists who recommend avoiding lamotrigine too) Because cross-hypersensitivity to other AEDs is common, patients who experience this reaction should subsequently be treated with nonaromatic AEDs (eg, valproate, topiramate) which have a lower risk of this reaction. In one case series, the latter medications were well-tolerated when prescribed as alternative AEDs to patients who experienced SJS or TEN in association with an aromatic AED

Question 65

what does ABCD2 stand for?

Answer 65

Age > 60 BP > 140/90 Clinical symptom (1 = speech; 2 = hemiparesis) Duration (1 = 10 - 59 min; 2 >/= 60 min) DM = 1 point

Question 66

What are the side effects of the MS drug, interferon beta?

Answer 66

flulike symptoms after injection elevated AST/ALT lymphopenia worsening of underlying spasticity, depression or migraines

Question 67

how do we treat spasmodic torticolis?

Answer 67

botulinium toxin injections

Question 68

Vitamin B12 deficiency can cause subacute combined degeneration of the cord. Are there any other nutritional deficiencies that can cause a similar picture? Any infections?

Answer 68

Tabes dorsalis can present similarly. Copper deficiency can cause a very similar picture, and can even be associated with a macrocytic anaemia!

Question 69

What is a preventative medication for tension headache?

Answer 69

Amitriptyline