Neuro Flashcards

Question

What is Glatiramer acetate (cop axone) and when is it used?

Answer 1

- synthetic polypeptide containing myelin basic protein - promote proliferation of Th2 cytokines, alters macrophage function. Reduces interferon gamma. Induces Th1 --\> Th2 Adverse - daily injection, rare reaction with palpitations, chest pain dyspnoea, pregnancy category B

Answer 2

- autoimmune inflammatory disease - affects CNS - demyelination and axonal degeneration - 50% need walking stick at 15 yrs

Answer 3

• Beta‐oxidation of VPA saturates at approximately 800 mg/day.\* – Percentage of VPA excreted as glucuronide increases: • from 12.8% at 400 mg/day to 49.4% at 2000 mg/day. – Glucuronide more reactive than products of betaoxidation. - doses above 1000mg are toxic

Answer 4

* cross in the lower medulla * originate from a long cortical strip - posterior frontal lobe * compact in the internal capsule and in the spinal cord * any lesion in the spinal cord will give ipsilateral problems * anything in the brainstem or above will give contralateral problems

Answer 5

inability to encode new memories inability to interact purposefully with environment – test by asking pt to obey commands

Answer 6

it is an oral drug - also used for psoriasis and MS - activates NRF-2 pathway vs oxidative stress and causes mild neutropenia SE: flushing, GIT - but may settle in 1-3/12.

Answer 7

demonstrates spinal cord inflammation - pleocytosis or elevated IgG index IgG index is a measure of intrathecal synthesis of immunoglobulin

Answer 8

Deep * Putamen, thalamus, brainstem, cerebellum * Usually due to hypertension and rupture of deep penetrating arteries Lobar * Superficial * Often secondary to amyloid angiopathy, tumour, arteriovenous malformation, venous sinus thrombosis **melanoma met bleeding most common**

Answer 9

Monoclonal ab to alpha4 integrin (VCAM1) inhibits tissue migration of lymphocytes and monocytes into CNS In other words - binds to alpha4 intern (VLA-4) on lymphocytes stopping them from binding to endothelial VCAM1 and crossing BBB. Used as mono therapy for RELAPSING-REMITTING MS - given monthly IV- relapse risk reduction 67% LFT abnormalities can occur - transaminitis

Answer 10

1. duration of exposure \> 2yrs 2. previous exposure to an immunosupp - methotrexate, aza, cycle 3. JCV serology positive

Answer 11

* primary destruction of the myelin sheath leaving the axon intact * secondary axonal degeneration common late in the disease course * weakness - proximal and distal wasting * tendon reflexes reduced * many causes incl immune/inflammatory, drugs/toxins, metabolic,

Answer 12

1) Flu like symptoms at commencement 2) Injection site reactions - ice skin before injection 3) LFT abnormalities are mild - screen at 1 and 6 months after starting treatment

Answer 13

Subacute onset progressive cortical symptoms/signs behaviours and neuropsych alteration hemiparesis

Answer 14

* dorsal columns responsible for joint position and vibration * input from large sensory fibres in peripheral nerves * cross in medulla * cross over in lower brainstem

Answer 15

Interferon B1 - avonex or 1FNB1b - betaferon It binds to receptors on immune cells - decreases antigen presentation and T cell proliferation, decreases cytokine and MMP expression Major reaction is flu like symptoms and injection site necrosis

Answer 16

 Primary lateral sclerosis * 2‐3.7% of all patients with MND; * pure UMN syndrome * Slow rate of progression 􁈺Pringe et al 1992􁈻

Answer 17

- menstrual irregularities and polycystic ovaries more common -regardless of which AED hyperandrogenism associated with VPA - avoid in child-bearing PCOS prevalence 12.5-26% compared to 6.6% (seems to be associated with VPA)

Answer 18

Pregnancy and epilepsy * proconvulsant effect of oestrogen vs progesterone * background malformation rate – 2% * roughly doubles on one AED * valproate especially more than 1g a day and polypharmacy particularly teratogenic * no agent known to be safe Contraception * combined OCPs – increased oestrogen metabolism with PHT, CBZ, PB, LTG levels lowered * progesterone – anticonvulsant effect

Answer 19

Simple partial – consciousness preserved Motor or sensory incl special sensory Autonomic Psychic Complex partial – consciousness impaired –not lost Simple partial onset – aura Or consciousness impaired from onset

Answer 20

CTB - often normal - occasional areas of hypo density MRI brain - almost always abnormal - very often sub-cortical U fibers, large \>3cm lesions, contrast enhancement in 41% with early PML (60-76% overall) Ix - serology 55% of normal adults have JCV antibodies DNA - JCV PCR of CSF - 74-93% sensitive, 92-100% specific. PCR of PBMC or urine not helpful, CNS biopsy still required frequently

Answer 21

**Symmetric motor & sensory deficits** ## Footnote - Proximal & distal weakness\>wasting - Absent or  reflexes - Distal LL\>UL sensory loss ‐20% painful; autonomic/resp uncommon ** Gradual onset over months‐years; progressive \> 2M**  10% acute onset ** Natural history variable**  Chronic progressive 60%  Relapsing ‐remitting 30%  Monophasic 10%  Spontaneous remission not uncommon but difficult to predict  **M\>F, all ages (mean 50yrs)**  \<10% children

Answer 22

* also known as Lichteim's disease * refers to degeneration of pos and lateral columns of spinal cord * 2\* to Vit B12 deficiency - most common * other causes - vitamin E deficiency, copper deficiency * presents with patchy loss of myelin in dorsal and lateral columns - weakness of legs, arms, trunk, tingling, and numbness that progressively wrosens * made worse with nitric oxide in those with B12 deficiency * can precipitate copper def if taking zinc - displaces zinc

Answer 23

* unconscious collapse and decreased GCS at presentation without cardio/resp arrest * preceding hx helps * Quadriparesis with upgoing plantars * check the eyes - loss of VOR - the vestibulo-ocular reflex * pupillary changes, ptosis * CT angiogram if NCCT excludes brainstem and large hemispheric ICH

Answer 24

- ant spinal aa supplies most of the spine - the pos spine supplied by various arteries - so if infarct then ant spine

Answer 25

* suitable drugs have narrow therapeutic index * close relation of plasma levels to therapeutic/toxic effects * wide inter-individual variation in metabolism * must monitor phenytoin (zero order kinetics) * useful to monitor carbamazepine +- phenobar, ? lamotrigine * valproate levels only useful for monitoring compliance * trough levels important if half life is short – carbamazepine, not phenobarb

Answer 26

* responsible for pain and temperature * input from small sensory fibres in the peripheral nerves * cross in spinal cord just above site of entry

Answer 27

typically seen during complex partial seizures stereotyped automatisms * lip smacking/chewing (operculum) * limb postures – as above reactive automatisms * semi-purposeful interaction with environment – planned actions do not occur

Answer 28

 Diabetes  Alcohol  Uraemia  Toxic / drugs  Paraneoplastic (some)  Nutritional

Answer 29

- on 2 drugs still not achieved remissed --\> refer for surgery if appropriate candidates

Answer 30

## Footnote **Phenytoin** (PHT) - Saturable – zero order kinetics Causes purple glove syndrome and phosphenytoin **Carbamazepine** (CBZ) _Stevens-johnson syndrome in East Asians – 8% carry HLA B\*1502_ Hyponatramia common – but more so with valproate **Valproate** Teratogenicity especially at doses above 800mg day IV form available **Lamotrigine** Danger of SJS – must start slowly – (12.5-25mg daily) Half life halved by CBZ, PHT, doubled by VPA)

Answer 31

* damage to half of the spinal cord * results in paralysis and loss of proprioception on the same (ipsilateral side of the injury) * loss of pain and temperature on the opposite (contra-lateral) side as the lesion * affects dorsal-column-medial lemniscus tract, corticospinal tract, contralateral- - affects spinothalamic. ## Footnote Pure Brown-Séquard syndrome is associated with the following: Interruption of the lateral corticospinal tracts: - Ipsilateral spastic paralysis below the level of the lesion - Babinski sign ipsilateral to lesion - Abnormal reflexes and Babinski sign may not be present in acute injury. Interruption of posterior white column: - Ipsilateral loss of tactile discrimination, vibratory, and position sensation below the level of the lesion Interruption of lateral spinothalamic tracts: - Contralateral loss of pain and temperature sensation. This usually occurs 2-3 segments below the level of the lesion.

Answer 32

NCS‐ Segmental Demyelination  Motor slowing,  Conduction block, dispersion  Long distal/F‐wave latencies  Absent/low ampl. SNAPs (UL\>LL)  CSF protein elevated; \<10 cells/mm  MRI -  Nerve root hypertrophy , enhancement  Nerve biopsy‐ -  inflammation + atrophy -  segmental demyelination /remyelination

Answer 33

Symptomatic stenosis 70‐99% ‐ strong evidence of benefit (NASCET, ECST) NB not if already severely disabled • Symptomatic stenosis 50‐70% ‐ benefit if low surgical morbidity and good life expectancy • Carotid occlusion/trickle flow – low risk of recurrent stroke, generally not suitable for surgery • Asymptomatic carotid stenosis – surgery rarely indicated – risk of stroke on optimal medical therapy (antiplatelet, statin, antihypertensives) estimated ~0.5%pa • Carotid stenting currently has a higher risk of stroke than endarterectomy (possible role in patients aged \<70?)

Answer 34

 Amyotrophic lateral sclerosis * Most common form * Mixed UMN & LMN * Limbs & Bulbar involvement 

Answer 35

enzyme inducing AEDs promote oestrogen and progestin metabolism (CBZ, PHT, PHB, PRM) high dose depot is effective implanon not effective progestin IUD has acceptable failure rate

Answer 36

* reduced/absent sensory potentials lower\>\>upper limbs * **_low amplitude_** distal motor responses, mildly delayed F-waves * conduction velocities: mildly reduced consistent with loss of large myelinated nerve fibres * EMG - neurogenic distal \>\> proximal muscles * motor units: high amplitude, long duration, polyphasic, rapid firing * fibrillations, positive sharp waves

Answer 37

_History & Exam_  Fatigue, variability, pure motor.  Serology‐AChRAb, MuSK Ab _ Electrophysiolog_y  Repetitive stimulation  Single fibre EMG - **most sensitive** _ Pharmacology‐AChEsteras_e Inhibitors  Tensilon test  Trial of neo/pyridostigmine antibiodies sensitive but not specific

Answer 38

most trial pts were pre-treated with alteplase indications in 2015 - \<6hrs from onset, good non contrast CT scan (small core infarct), ICA, basilar or prox MCA occlusion (M1 or M2)

Answer 39

* CPTII deficiency * carnitine palmitoyl transferase assay * muscle bx - moth eaten fibres, Type IIb fibre atrophy and moderate lipid stasis

Answer 40

20-30x higher in young epileptics total death rate - increases by 3 or more in young epileptics – depends on syndrome ? caused by cardiac arrhythmias or apnoea related to frequency and types of seizures 1 = 2/year, absences --\> no increase needs to be raised when discussing treatment and compliance

Answer 41

Provoked seizures – avoid the provoking stimulus – DUH Generalised seizures – valproate à lamotrigine/topiramate/levetiractam Absence seizures à valproate/lamotrigine/ethosuximide/levetiracetam (avoid carbamazepine/tiagabine) Partial seizures à carbamazepine à levetiracetam, topiramate, gabapentin, oxcarbazepine, valproate, tiagabine Phenytoin still used as IV form – but levetiracetam and valproate also IV forms

Answer 42

* protein accumulation in nerve cells * usually involves uml and lmn * progressive degeneratio of motor neurons - * Amyotrophic muscle atrophy, weakness, fasiculation (LMN) * lateral sclerosis: gliosis post degeneration of corticospinal tracts (UMN)

Answer 43

**Total Anterior Cerebral Infarct (TACI)** ## Footnote – Large infarct, poor prognosis – Hemiparesis, hemianopia, cortical signs **Partial Anterior Cerebral Infarct (PACI)** – Smaller, better prognosis, risk of recurrence – 2 of 3 TACI symptoms • Hemiparesis, cortical signs, hemianopia **Lacunar Infarct (LACI)** – No cortical signs, relatively good prognosis – Pure motor hemiparesis, pure sensory stroke, sensorimotor stroke, ataxic hemiparesis, dysarthria‐clumsy hand **Posterior Cerebral Infarct (POCI)** – Stroke with brainstem and/or cerebellar features – Variable prognosis – Ataxia, ophthalmoplegia, quadriparesis, crossed signs

Answer 44

on 2 meds and still have not acheived remission --\> refer for surgery if suitable

Answer 45

It was about BP lowering in ICH - aggressive BP lowering vs standard care - showed no increase in serious adverse events multiple secondary outcomes improved treat BP aggressively to \<140/90 early - some benefit Treat BP aggressively long term - \<130/80

Answer 46

 Progressive bulbar palsy * Presenting sx in ~20% of all px * Most commonly seen 􁈺and worst prognosis in􁈻 in menopausal women * Lower cervical‐thoracic area  rarely confined to bulbar region only

Answer 47

* disorder of synapse antibody blockade, defect of synapse * antibody production against NMJ - AChr or MUSK - ? LRP4, Agrin * thymic hyperplasia * Ab and immune complexes at NMJ * ? associated wiht auto-immune disease - organ specific +- FH * graves, hashimoto's thyroiditis, pernicious anaemia, vitilligo, diabetes, pemphigus, chronic active hepatitis * thymoma 15% - invasive rare * chronic myopathy - long standing/untreated

Answer 48

AEDs are bad, in a dose‐ dependent manner * Valproate is worst * Polytherapy is bad * Folate supplementation is good * No AED exposure ~3% * Monotherapy ~6% * High‐dose valproate ~10% * Conflicting data on polytherapy * Levetiracetam low risk * No proof for folate supplementation

Answer 49

The classic epidemiological definition… – Stroke is the rapid onset of focal (or global, as in subarachnoid haemorrhage) cerebral deficit, lasting \>24 hrs or leading to death, with no apparent cause other than a vascular one – TIAs are the same phenomenon \<24 hours = DEBUNKED - HOWEVER - modern definition -TIAs that last than 1hr usually only a few mins, pt with symptoms have stroke. **TIAs have very high short term risk of stroke - upto 20% within 3 months, HIGHEST risk in first 48hrs!!!**

Answer 50

Endocrine abnormalities (effect of AEDs) – Increased SHBG levels – Hyperandrogenism – Polycystic ovaries – Menstrual irregularities – Anovulatory cycles – Obesity – Insulin resistance

Answer 51

Axonopathy! - most common aeitology is diabetes, alcohol, uraemia, toxic/drugs, nutritonal and occs paraneoplastic they account for 75% of peripheral nerve pathology.

Answer 52

* First line ---\> carbamazepine, phenytoin or valproate * New AEDs - any * third line - clonazepam/clobazam

Answer 53

CBZ vs LTG, TPM, GBP, OXC: LTG best tolerated; CBZ and LTG most efficacious VPA vs TPM, LTG: VPA better tolerated than TPM and more efficacious than LTG

Answer 54

Tx with interferons (avonex) with Glatiramer acetate AND Terifluonomide - delays onset of clinically defined MS and reduces MRI activity markedly. not pubs approved

Answer 55

## Footnote Cervical spine: vertebrae C1-C7, roots C1-C8. Cervical roots emerge above • i.e. C6 emerges at C5/6 Thoracic & lumbar roots emerge below • i.e. L5 emerges at L5/S1

Answer 56

 Distal symmetric polyneuropathy  Lumbosacral polyradiculopathy (caudaequina syndrome; CMV)  Inflammatory demyelinating polyradiculoneuropathy  Sensory neuronopathy  HIV treatment neuropathies eg. ddI, ddC,d4T, lamivudine etc

Answer 57

Oligodendrocytes! - responsible for myelination, one cell can myelinated many fibres in CNS. Patho: 1) inflammation - focal + global infiltration with T cells - CD3, CD4, CD8 and macrophages 2) Demyelination - loss of oligodendrocytes 3) axonal loss - apoptosis and neuronal die-back

Answer 58

lesion with selective, disassociative sensory loss - think central cord lesion with syrinx can have lower moter neuro signs a well as it spreads out Def: A syrinx is a rare, fluid-filled neuroglial cavity within the spinal cord

Answer 59

Study of Osteoporotic Fractures – Accelerated bone loss in patients on AEDs (phenytoin) – Increased risk of hip fracture OR ~2 – Not due to seizures Epilepsy and/or AED use fracture risk OR ~2 – Risk increases with cumulative AED exposure, regardless of type potential mechanisms - balance, bone density, vit D metabolism, calcium absorption, bone modelling need to take measures for bone health - ie smoking cessation, calcium, vit D levels, DEXA

Answer 60

A joke! Benign MS is not benign high chance of further disease activity if not treated. truly benign MS can only be diagnosed after 20yrs and in retrospect

Answer 61

Progressive muscular atrophy * Only develop LMN signs 􁈺without UMN signs􁈻; * Older males 􁈺more commonly􁈻 * Improved survival 􁈺median 48 months cf 36months􁈻

Answer 62

This is for MS - so you need 2+ relapses at least 1 month apart - i.e. new lesion on follow-up MRI brain or simultaneous non enhancing and contrast enhancing lesions

Answer 63

disorders that are like MS but need to be differentiated from them - need to have +ve anti-aquaporin4 (AQP4) igG and 1 core clinical feature - like optic neuritis, acute myelitis. usually bilaterally.

Answer 64

1. focal - 60% then eneralised tonic clonic 35% Characteristics Epileptic seizures rarely exceed 2 minutes Eyes open, slowing of bilaterally synchronous clonic movements Lateral tongue biting is fairly specific

Answer 65

 Flail‐arm syndrome • Symmetric proximal and distal bi‐brachial wasting 􀵅 positive babinski sign 􁈺Hu et al 1998􁈻 * ~ 10% of patients; M:F  9:1 􁈺flail arm group􁈻 * Trend toward improved survival in flail‐arm group 􁈺57 months cf 39months􁈻

Answer 66

ABCD2 – a prognostic score * Age ≥60 years = 1 * BP (systolic \>140 and/or diastolic ≥90) = 1 * Clinical features * unilat weakness =2; speech =1; other =0 * Duration * \>60 mins =2; 10-59 =1; \<10 =0 * Diabetes 2 day ABCD2 risk: 1% if = 0-3 4. 1% if = 4-5 8. 1% if = 6-7 **Although ignores carotid stenosis and AF**

Answer 67

MEDICAL EMERGENCY * Death rate 10% - depends on underlying cause – encephalitis or tumour * \> 1 seizure with incomplete recover between or continuous or recurrent seizures for \> 30mins (\> 5mins medically observed) is a good predictor of CSE development – so TREAT it * ABC – remove danger * Stop seizures – benzo/IV/PR/oral/GEL * Keep seizures stopped – IV phenytoin/levetiracetam/valproate * Transfer to ICU

Answer 68

MRI is gold standard as noted But can do CSF - oligoclonal bands but not in serum - indicates clonal B cells behind BBB Beware if any polymorphs Can do VERs/VEP - visual pattern evoked afferent volleys to occipital cortex - shows optic nerve demyelination

Answer 69

Peroneal @ knee vs sciatic @ hip vs L5 usually sciatic nerve - may lose ankle jerk-tibial nerve

Answer 70

* General increase in most types of birth defect * Specific associations – Neural tube defects and hypospadias (VPA, CBZ) – Congenital heart defects (PHT, VPA, PB) – Craniofacial abnormalities (PHT, VPA, PB, PRM) – Genitourinary defects (PHT,

Answer 71

Not safe - although no proper clinical studies - but in rats - demonstrated teratogenicity

Answer 72

active metabolite of leflunomide - approved to treat RA - selective reversible dihydroorotate -interferes with lymphocyte proliferation by inhibiting pyrimidine synthesis in lymphocytes -reducing tyrosine kinase activity - may prevent interaction of lymphocytes and antigen-presenting cells

Answer 73

* Axonopathy - is the commonest * usually chronic - weeks to months * predilection for large diameter, long nerve fibres * distal symmetric pattern * sensory\>motor areflexia * routine nerve conduciton studies only test for large nerve fibres

Answer 74

* Consider all \<4.5 hrs eligible for alteplase and clot retrieval * Rapidly improving deficit may indicate good collaterals rather than reperfusion – high chance of later worsening • Non‐contrast CT brain + CT angiogram +/‐ CT perfusion **• Key eligibility:** * ischaemic stroke, \<4.5 hr, CT excludes ICH * Are there exclusion criteria for thrombolysis? * Key exclusions: uncontrolled BP \>185/110 at presentation, recent ischaemic stroke, ICH, current anticoagulation, excess bleeding risk

Answer 75

**Progressive inflammatory vs degenerative myopathy** **Most common inflamm disease in older popn** – Onset \>60 yrs, ?1/100,000 – Disabling but not fatal – Wheelchair dep’t in 5‐10yrs – Auto‐immune disease in 15% **• Proximal & distal weakness** – Selective pattern ? Asymmetrical – espQuads, Iliopsoas, FDP – also triceps, biceps, Tibialis ant. – Depressed reflexes – Dysphagia in up to 40%

Answer 76

* Most common cause of flaccid paralysis in the developed world * occurs at all ages, peaks in young adulthood and late life * slight male predominance * increase of 20% with every 10 year rise in age after the first decade * Clinical * ascending, bilateral symmetric weakness progressing over 12hrs to 28days before plateau is reached * variable degree of sensory involvement * generalized hyporeflexia or areflexia – 10% have normal or brisk reflexes * cranial n involvement, bulbar involvement The most common precipitating illness leading to Guillain-Barre syndrome? – CAMPYLOBACTER JEJUNI GASTROENTERITIS

Answer 77

* 3 days to 6 weeks before onset * 30% of infections attributed to C.jejuni and incidence of GBS is estimated to be 0.25-0.65 per 1000 cases of c.jejuni. * associated with acute motor axonal neurppathy * may be secondary to presence of molecular mimicry between gangliosides and antecedent infectious agents – lipooligosaccharide on bacteria mimic gangliosides. * Flu like respiratory illness – in EBV, CMV, VzV, mycoplasma pneumonia

Answer 78

* progression upto 1-3 weeks after symptom onset * recovery over weeks to months * resp insufficiency in 25% patients * mortality 1-2.5% 2\* to pulmonary Cx * 85% full functional recovery * 15% permanent deficit

Answer 79

* more common in eastern asia * clinically – opthalmoplegia, ataxia, areflexia * often distal paresthesia * strong link to GQ!b IgG autoantibodies * disease peaks 1 week – improvement in 2 weeks * excellent prognosis – full recovery by 6 months * likely to make recovery without treatment - IVIG but not plasmapharesis -\_\> slight hastening of recovery from opthalmoplegia or ataxia à but time to complete recovery same. * immune trigger * autoantigens bind to myelin – activation of complement and MAC – macrophages remove myelin debris GM1 and GD1a antigens for axonal neuropathy – nodes of ranvier – abs – then directed against these antigens – disappearance of sodium channels – nerve conduction failure and degeneration.

Answer 80

* preceding illness less common than in GBS * evolution \> 4 weeks, progression over at 2 months * relapsing remitting – more common in young * relatively symmetric upper and lower limbs * usually distal and proximal weakness – if only distal – then think other etiologies * usually sensorimotor – numbness or tingling – sometimes pain * reduced absent reflexes * postural or action tremor

Answer 81

* immune mediated demyelination * T cell activation is predominant factor * Macrophages and humoral factors are also important ## Footnote Nerve conduction studies * most important investigation tool * conduction block most common abnormality * slow conduction velocities * temporal dispersion * increased distal motor latencies * proximal stimulation increases Dx yield * most have some “axonal change”

Answer 82

IV immunoglobulins * improvement in 60% * better earlier response than methylpred – relapse higher * variable maintenance treatment plasma exchange * improvement in 80% * used for pts who are very weak or experience a relapse on another therapy * can be considered as long term therapy steroids * often avoided * traditionally 60-100mg oral pred taken daily or alt day then taper * pulsed cortico similar results with less side effects

Answer 83

* auto-antibodies block VGCC and decrease calcium influx during depolarization of pre synaptic membrane which required for release of acetylcholine from pre-synaptic motor nerve terminals * rare condition * M:F 5-1 * 50% have underlying malignancy * may precede tumour by months-years * usually small cell lung cancer * other paraneoplastic syndromes may exist * HLA- 88, -DR3 asssociated – same as MG

Answer 84

* difficulty walking is initial symptoms in most * bulbar and ocular muscles spared or mildly affected – e.g ptosis * strength improves immediately after exercise * autonomic dysfunction – dry mout, postural hypotension, erective dysfunction - - ie increasing ACH – increasing calcium release. * Unlike MG – symptoms typically out of proportion to degree of weakness on examination * Tendon refelexes absent or reduced – can be increased by brief activation of appropriate muscles or tapping the tendon ## Footnote VGCC auto antibodies - 85-90% positive

Answer 85

* accumulation of alpha synuclein * cytoplasmic lewy bodies * diagnostic criteria - UK brain bank * bradykinesia and one of – rigidity, 4-6Hz rest remor, postural instability not due to other cause. * Supportive criteria (3 criteria) required – unilateral onset, rest tremor, persistent asymmetry, progressive course more than 10yrs, L-dopa response - should be excellent (70-100%), severe L-dopa induced chorea or if response lasts more than 5 yrs – all support parkinsons

Answer 86

* REM sleep behavior disorder – act out their dreams – vivid nightmares, 50% at 10+ years - can use clonazepam or small dose melatonin for tx * Impaired olfaction * Constipation - ? GIT disease pre * Depression * Parkinsons start in Dorsal motor nucleus in the vagus – or gut, olfactory bulb, to brainstem à limbic à cortex

Answer 87

direct dopamine agonists * ergot-derived – cabergoline - problem with fibrosis – cardiac, pulmonary, retroperitoneal * non ergot derived – sifrol – pramipexole - behavior – OCD, gamble, shopaholics, sexual behaviours

Answer 88

Diagnostic criteria All of – onset age over 40, progressive course, bradykinesia, supranuclear gaze palsy + Any three of – dysarthria, dysphagia, minimal/absent tremor, neck/flex worse than extremities rigidity, frequent falls/gait disturbance early And none of – early/prominent ataxia, unexplained neuropathy, prominent dysautonomia – isolated postural BP allowed

Answer 89

autonomic failure – urinary incontinence, + erectile dysfunction in men and poorly L-dopa responsive parkinsonism – bradykinesia + 1 other of rigidity, tremor or postural instability OR cerebellar syndrome

Answer 90

* probable CBS – greater one yr duration, greater than 50yrs of age, insidious onset/gradual progression * asymmetric presentation of 2 of : * limb rigidity or akinesia * limb dystonia * limb myoclonus * plus 2 of * orobuccal or limb apraxia * alient limb – not just limb elevation * cortical sensory deficit * CBD may also present as non-fluent aphasia

Neuro Flashcards

(118 cards)