Neurology

Question 1

Most common cause of dementia

Answer 1

alzheimers

then
vascular
lewy body

Question 2

What is Alzheimers?

risk factors-

Answer 2

progressive degnerative disease of the brain.

• age
• fhx
• autosomal dominant 5% - amyloid precursor protein (chr21), presenlin 1 (chr 14) , presenilin 2 (chr 1)
• apoprotein E allele E4 - encodes cholestrol transport protein
• caucasian
• downs

Question 3

Pathological Changes of Alzheimers

Answer 3

macroscopic:
- widespread cerebral atrophy, involves more 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 tau protein.
- hyperphosphorylation of tau protein - linked to AD

biochemical:
- deficit of acetylcholine from damage to ascending forebrain projection

Question 4

What are neurofibrillary tangles?
(Alzheimer’s)

Answer 4

• paired helical filaments partly made from tau protein

tau = protein interacts with tubulin stabilising microtubules and promoting tubulin assembly into microtubules.

AD: tau proteins hyperphosphorylated = impaired function

Question 5

How would you manage Alzheimers?

Answer 5

non pharm:
- cognitive stimulation therapy
- cognitive rehab
- group reminiscence therapy

pharm:
- acetylcholinesterase inhibitors (donepezil, galantamine and rivastigmine) - mild - moderate AD

• memantine (NMDA receptor antagonist)- 2nd line

Question 6

when would you give memantine as AD tx?

Answer 6

moderate AF intolerant or CI to acetylcholinesterase inhibitor

as add on drug for mod-severe

monotherapy in severe

Question 7

when can you give an antipsychotic in AD?

Answer 7

to manage noncognitive sx

pt at risk of harming themselves or others.

when agitation hallucination or delusions causing them severe distress

Question 8

how is donepezil contraindicated in and what is the adverse effecct?

AD TX

Answer 8

bradycardic pts

insomnia

Question 9

characteristic pathological feature of lewy body dementia?

where are they found?

Answer 9

alpha-synuclein cytoplasmic inclusions (lewy body)

in substantia nigra, paralimbic and neocortical areas.

Question 10

relationship with alzheimers and lewy body?

Answer 10

upto 40% of of alzheimers have lewy bodies.

Question 11

features of Lewy body dementia?

Answer 11

progressive cognitive impairment: typically before parkinsonism but both occur within a yr of each other.

FLUCTUATING COGNITION

parkinsonisms

visual hallucinations

• poss delusions and nonvisual hallucinations

REM sleep disorder?

Question 12

how can lewy body be differentiated from parkisons disease?

Answer 12

in parkinsons motor symptoms occur at least a yr before cognitive sx.

in lewy body first cognitive impaired then motor.

Question 13

How would you diagnose Lewy body dementia?

Answer 13

clinical

single photon emission computed tomography - SPECT. (called DaTscan)
90% sensitivity 100% specificity

Question 14

How would you manage Lewy Body Dementia?

Answer 14

both acetylchoinesterase inhibtors (donepezil, rivastigmine) and memantine.

NO TO NEUROLEPTICS

Question 15

why should you avoid neuroleptics in Lewy body dementia?

Answer 15

can get irreversible parkinsonism.

if hx of pt with deteriorated after antipsychotic agent

Question 16

What is Vascular Dementia?

Answer 16

second most common form after AD.

group of syndromes of cognitive impairment caused by different mechanisms causing ischaemia or haemorrhage secondary to cerebrovascular disease.

Question 17

subtypes of vascular dementia

Answer 17

stroke related - multi infarct or single infarct dementia

subcortial vd - caused by small vessel disease

mixed dementia - both vd and ad

Question 18

rf of vascular dementia

Answer 18

hx of tia
af
htn
dm
hyperlipidemia
smoking
obesity
coronary heart disease
fhx of storke/cv issue

rare: CADASIL - inherited - cerebral autosomal dominant ateriopathy with subcortical infarcts and leukoencephalopathy

Question 19

pt comes with vascular dementia. how is he presenting?

Answer 19

several months/years of hx of a sudden or stepwise deterioration of cognitive function

Question 20

symptoms of vascular dementia

Answer 20

focal neurological abnormalities: visual disturbance, sensory or motor sx

difficulty with attention and concentration

seizures

memory disturbance
gait disturbance
speech disturbance
emotional disturbance

Question 21

how would you make a diagnosis of vascular dementia?

Answer 21

hx and exam
formal screen for cognitive impairment
med review - exclude medication cause of cognitive decline

MRI - may show infarcts and extensive white matter changes

Question 22

what criteria used for diagnosis of vascular dementia

Answer 22

NINDS-AIREN

presence of cognitive declines interferes with adls not due to secondary effects of cerebrovascular event

cerebrovascular disease - defined by neurological signs/brain imaging

relationship between above 2 disorders inferred by:

onset of dementia within 3 months after recognised stroke
abrupt deterioriation in cognitive function
fluctuating stepwise progressive cognitive deficits

Question 23

how would you mange vascular dementia?

Answer 23

non pharm:
- cognitive stimulation
- multisensory stimulation
-music and art
-animal assisted therapy

pharm:
- no specific ones
- only ache inhibitors or memantine if they have AD too, parkinsons dementia or dementia with lew body.

• aspirin isnt effective in tx of vd PATIENTS.

Question 24

rare causes of dementia

Answer 24

huntingtons
CJD
picks disease - atropy of frontal and temporal lobes
HIV - 50% of aids pts.

Question 25

name some differentials of dementia

Answer 25

hypothyroidism, addisons b12 folate thiamine def syphilis brain tumour normal pressure hydrocephalus subdural haematoma depression chronic drug use - alcohol, barbiturates

Question 26

assessment tools for dementia in non specialist setting

Answer 26

10-point cognitive screener : (10-CS) 6- item cognitive impairment test (6CIT) others: not recommended for non-specialist setting - AMTS -GPCOG -MMSE - 24 or less/30 = suggests dementia

Question 27

what to investigate initially when thinking dementia?

Answer 27

fbc u+e lft calcium glucose esr crp tft vit b12 folate neuroimagine - rule out subdural haematoma, normal pressure hydrocephalus

Question 28

factors favouring delirium over dementia

Answer 28

acute onset impairment of consciousness fluctuation of sx: worse at night, periods of normality abnormal perception - illusions and hallucinations agitation,fear, delusions

Question 29

What is frontotemporal lobar degeneration? 3 types

Answer 29

3 MC cortical dementia after AD and lewy body 3 types: -frontotemporal dementia (picks disease) - progressive non fluent aphasia - chronic progressive aphasia, CPA - semantic dementia

Question 30

common features of frontotemporal lobar dementias

Answer 30

- onset before 65 - insidious onset - relatively preserved memory and visuospatial skills - personality change - social conduct problems

Question 31

What is Picks Disease - frontotemporal dementia?

Answer 31

personality change impaired social conduct. hyperorality disinhibition increased appetite preservation behaviours behaviour speech language can be familial.

Question 32

characteristic appears of picks disease - frontotemporal dementia?

Answer 32

focal gyral atrophy with knife blade appearance

Question 33

tell me the macroscopic changes in picks and the microscopic changes

Answer 33

Macroscopic: atrophy of frontal and temporal lobes Microscopic: Pick Bodies - spherical aggregations of tau protein - silver staining Gliosis Neurofibrillary Tangles Senile Plaques

Question 34

how would you manage picks disease?

Answer 34

AchE inhibitors or memantine

Question 35

features of chronic progressive aphasia

Answer 35

non fluent speech. short utterances that are agrammatic. preserved comprehension

Question 36

features of semantic dementia?

Answer 36

fluent progressive aphasia. fluent speech but empty and conveys little meaning. memory between for recent than remote events unlike AD.

Question 37

name the meds with anticholinergic effect

Answer 37

anticholinergic urological drugs - oxybutynin, solifenacin and tolterodine antihistamine - chlorphenamine and promethazine tricyclic antidepressants - amitriptyline

Question 38

what is ace-III - addenbrooke cognitive examination domains points

Answer 38

assessment tool for memory impairement. 5 domains: attention memory language visuospatial function verbal fluency 100 points 88 or less = possible dementia.

Question 39

name some behavioural and psychological sx of dementia how would you tx?

Answer 39

depression anxiety agitation aggression disinhibition hallucinations delusions slep disturbance ssri antipsychotic - risperidone benzodiazepine - only for crisis.

Question 40

predisposing factors for delirium

Answer 40

age over 65 dementia background significant injury eg- hip fracture frailty or multimorbidity polypharmacy

Question 41

features of delirium

Answer 41

memory disturbances (loss of short> long term) could be agitated or withdrawn disorientation mood change visual hallucinations disturbed sleep cycle poor attention

Question 42

precipitating events of dementia

Answer 42

infection - uti severe pain alcohol withdrawal constipation any cv,resp,neuro,endo condition metabolic: hypercalcaemia, hypoglycaemia, hyperglycaemia, dehydration change of environment

Question 43

how would you manage delirium

Answer 43

tx underlying cause. haloperidol 0.5 mg - 1st line sedative or olanzapine

Question 44

what can antipsychotic prescribing be challenging for parkinson patient?

Answer 44

worsen parkinsonian sx careful reduction of parkinson meds may be helpful if sx need urgent tx : atypical antipsychotic quetiapine and clozapine preffered

Question 45

What is benign essential tremor? genetic most notable where?

Answer 45

autosomal dominant - affected both upper limbs associated with older age. fine tremor affecting all voluntary muscles. hands but also: head (TITUBATION)!!!, jaw and vocal tremors

Question 46

features of benign essential tremor

Answer 46

POSTURAL TREMOR - WORSE IF ARMS OUTSTRETCHED fine tremor - 6-12 hx symmetrical more prominent with voluntary movement worse when tired,stressed or after caffeine improved by alcohol absent during sleep

Question 47

differentials of benign essential tremor

Answer 47

parkinsons MS Huntingtons chorea hyperthyroidism fever dopamine antagonists - eg antipsychotics

Question 48

how would you manage benign essential tremor?

Answer 48

nothing definitive. to improve sx: propranolol 1st line - non selective beta blocker primidone - barbiturate antiepileptic med

Question 49

Name some causes of parkinsonisms

Answer 49

parkinsons drug induced - antipsychotics,metoclopramide progressive supranuclear palsy multiple system atrophy wilsons disease post-encephalitis dementia pugilistica - chronic head trauma cause like boxing toxins: carbon monoxide, MPTP

Question 50

domperidone class of drug function why doesn't it cause extrapyramidal side effects

Answer 50

antisickness dopamine antagonist doesnt cross blood brain barrier

Question 51

What is Parkinson's disease?

Answer 51

progressive neurodegenerative conditions caused by degeneration of dopaminergic neurons in substantia nigra. progressive reduction in dopamine in basal ganglia - so you get disorder of movements ASYMMETRICAL

Question 52

classical triad of features: parkinsons other general picture

Answer 52

REDUCTION IN DOPAMINERGIC OUTPUT CAUSES: bradykinesia - slowness of movement resting tremor - tremor worst at rest rigidity - resisting passive movement ASSYMETRICAL forward tilt stooped posture facial masking shuffling gait

Question 53

epidemiology of parkinsons

Answer 53

twice as common in men mean age: 65

Question 54

talk to me about the triad of sx in parkinsons: bradykinesia

Answer 54

poverty of movement - hypokinesia short shuffling (gait) steps with reduced arm swing difficulty in initiating movement micrographia festinating gait - rapid frequency of steps to comp for small steps and not fall reduced facial movements and expressions(hypomimia) difficulty initiating movement

Question 55

Pathophsyiology of Parkinsons

Answer 55

basal ganglia are group of structures near centre of brain. coordinate habitual movements like walking controlling voluntary movements etc. dopamine plays major role. - in pd you have a drop of dopamine.

Question 56

talk to me about triad of sx in parkinsons: tremor WHAT EXAGGERATES THE PILLROLLING TREMOR

Answer 56

most marked at rest, 3-5 hz worse when stressed/tired, IMPROVES WITH VOLUNTARY MOVEMENT (unilateral) PILL-ROLLING ie in the thumb and index finger. worse at rest better with voluntary movement. worse if pt distracted. miming act of painting a fence

Question 57

tell me about sx in parkinsons: rigidity

Answer 57

resistance to passive movement of a joint lead pipe cogwheel: due to superimposed tremor

Question 58

other general features of parkinsons

Answer 58

mask-like facies flexed posture micrographia drooling of saliva REM sleep behaviour disorder fatigue autonomic dysfunction: postural hypotension due to autonomic failure psychiatric feature: depression is MC feature (40%) - psychosis and sleep disturbances poss impaired olfaction cognitive impairement.

Question 59

how are drug induced parkinsonism presenting features to parkinsons?

Answer 59

motor symptoms are generally rapid onset and bilateral rigidity and rest tremor are uncommon

Question 60

How would you differentiate between essential tremor and Parkinson's?

Answer 60

123 I - FP - CIT single photon emission computed tomography (SPECT)

Question 61

What stain shows alpha synuclein of substantia nigra in parkinsons?

Answer 61

lewy body brown stain positive for alpha synuclein

Question 62

How would you treat parkinsons?

Answer 62

if motor sx affected QoL: levodopa if motor sx not affecting QoL: dopamine agonist (non-ergot derived), levodopa, monoamine oxidase B (MAO-B) inhibitor if optimal levodopa and still sx or developed dyskinesia, add dopamine agonist, MAO-B inhibitor or catechol-o-methyl transferase (COMT) inhibitor as adjunct.

Question 63

out of parkinson treatments rank them for motor symptoms, activities of daily living and motor complications adverse events - what are they too

Answer 63

levodopa - fever adverse events dopamine agonist - more adverse MAO-B inhibitors - fever adverse 1st - more improvement and more motor comps 2nd and 3rd - less improvement and fever motor comps adverse: excessive sleep, hallucination, impulse control disorder

Question 64

when adding a drug as an adjunct in parkinsons you need to make a few decisions based on motor sx adl off time adverse events hallucinations make them for dopamine agonist, MAO-B inhibitors, COMT inhibitors, Amantadine.

Answer 64

Motor Symptoms, ADL : dopamine agonist, MAO-Bi COMTi - improvement in both. Amantadine: no evidence in improvement of either. Off time: dopamine agonist : more off-time reduction, MAO-Bi and COMTi - off-time reduction Amantadine : no evidence Hallucinations: DA - more risk MAO-Bi/COMTi : lower risk of them Amantadine: no evidence Adverse events : DA - intermediate risk MAO-Bi/COMTi : fever adverse events/more adverse events

Question 65

what can happen to a patient if parkinson tx medication is not absorbed/taken? give a reason why this could happen

Answer 65

risk of acute akinesia or neuroleptic malignant syndrome gastroenteritis NO DRUG HOLIDAY

Question 66

what therapy/treatment can impulse control disorder happen with?

Answer 66

dopamine agonist therapy hx of previous impulsive behaviours hx of alcohol consumption and/or smoking

Question 67

parkinson patient is day time sleepy but drives what to do?

Answer 67

modafinil - Atypical dopamine reuptake inhibitor

Question 68

if orthostatic hypotension in parkinson patient what to give? how does it work?

Answer 68

midodrine - acts of peripheral alpha-adrenergic receptors to increase arterial resistance

Question 69

parkinson pt drooling of saliva - how to tx?

Answer 69

glycopyrronium bromide

Question 70

common adverse effects of levodopa reduced effectiveness over what period of time?

Answer 70

dry mouth anorexia palpitations postural hypotension psychosis reddish discolouration of urine upon standing n+V cardiac arrhythmias on off effect dyskinesia by 2 yrs

Question 71

how is levodopa usually prescribed? parkinsons why?

Answer 71

usually with a decarboxylase inhibitor - eg carbidopa or benserazide levo+benserazide : MADOPA levo+carbi: SINEMET prevents peripheral metabolism of levodopa to dopamine outside of brain - reduce side effects

Question 72

can you name some adverse effects of levodopa due to difficulty in achieving a steady dose of levodopa?

Answer 72

end of dose wearing off. - decline of motor activity. on-off phenomenon: large variation in motor performance. - weakness and restricted mobility during off. dyskinesia at peak dose: dystonia,chorea and athetosis (involuntary writhing) effects worsen over time

Question 73

you are at peak dose of levodopa in parkinson tx and develop dyskinesias what are they?

Answer 73

dystonia chorea athetosis - involuntary writhing movements

Question 74

why shouldnt you acutely stop levodopa what to do if they cant take orally

Answer 74

acute dystonia give dopamine agonist patch as rescue medication

Question 75

give me 4 examples of dopamine receptor agonists side effects of ergot derived= what to do if prescribing these?

Answer 75

bromocriptine - ergot derived ropinirole cabergoline - ergot derived apomorphine ergot derived: pulmonary,retroperitoneal and cardiac fibrosis echocardiogram esr creatinine cxr all above before tx and closely monitor during.

Question 76

side effects of dopamine receptor agonists

Answer 76

congestion and postural hypotension in some more likely than levodopa in older pts to cause hallucinations potential: impulse control disorder and excessive daytime somnolence

Question 77

give the the MoA of MAO-B inhibitor and an example

Answer 77

Selegiline/Rasagiline. inhibits breakdown of dopamine secreted by dopaminergic neurons used to delay use of levodopa, then with levodopa to reduce end of dose worsening sx.

Question 78

How does amantadine work and what is its side effects? parkinsons class of drug use

Answer 78

glutamate antagonist - treat dyskinesia associated with levodopa increases dopamine release and inhibits its uptake at dopaminergic synapses ataxia slurred speech confusion dizziness livedo reticularis

Question 79

give me 2 examples of COMTi - catechol-o-methyl transferase inhibitors and what is it? how and when used?

Answer 79

entacapone, tolcapone enzyme breaks down dopamine. use with levodopa esp in established PD patients to slow breakdown of levodopa in brain. extends effective duration of levodopa

Question 80

how do antimuscarinics work in parkinsons? give examples what sx do they help with

Answer 80

block cholinergic receptors used more to tx drug-induced parkinsons help tremor and rigidity procyclidine benzotropine trihexyphenidyl - (benzhexol)

Question 81

what is multiple system atrophy? symptoms

Answer 81

rare neurones of brain degenerate including basal ganglia. - parkinsons presentation but you get autonomic dysfuntcion (postural hypotension,constipation, abnormal sweating and sex dysfunction) too cerebellary dysfunction (ataxia)

Question 82

prolonged use dopamine agonist treat side effect

Answer 82

pulmonary fibrosis

Question 83

What is motor neuron disease and types? with the types theyre main signs

Answer 83

progressive group of diseases affecting motor nerves not sensory. rarely before 40. ALS - MC - 50% pts - LMN signs in arms and UMN signs in legs. if familial : gene responsible lies on chr 21 and codes for superoxide dismutase Primary Lateral Sclerosis: UMN signs only Progressive muscular atrophy: LMN signs only, distal muscles before proximal, best prognosis Progressive Bulbar Palsy: palsy of tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei. Worst prognosis.

Question 84

features of mnd

Answer 84

assymetric limb weakness - ALS mixed lmn and umn signs wasting of small hand muscles/tibialis anterior fasciculations absence of sensory signs/sx: - vague sensory symptoms early in disease poss? - eg limb pain but never sensory sign no cerebellar signs dont affect external ocular muscles abdo reflexes preserved. SPINCTER DYSFUNCTION - LATE FEATURE

Question 85

how to diagnose MND?

Answer 85

clinical nerve conduction studies - normal motor conduction. electromyography - reduced no. of action potentials with increased amplitude. MRI: exclude cervical cord compression and myelopathy

Question 86

how would you manage MND

Answer 86

riluzole - ALS - prolongs life by 3 months - prevents glutamate receptor stimulation Resp care: - non invasive ventilation - BIPAP - at night - 7 month survival benefit Nutrition - PEG - percutaneous gastrostomy tube Prognosis: poor 50% pts die within 3 yrs

Question 87

any associated risk factors for mnd

Answer 87

fhx - 5-10% smoking heavy metals and certain pesticides

Question 88

signs of lower mnd

Answer 88

muscle wasting reduced tone fasciculations reduced reflexes

Question 89

signs of upper mnd

Answer 89

increased tone or spasticity brisk reflexes upgoing plantar reflex

Question 90

What is Multiple Sclerosis?

Answer 90

chronic and progressive autoimmune condition involving demyelination in cns. immune system attacks myelin sheath of myelinated neurones typically under 50 . more in women 20-40 peak.

Question 91

causes of MS

Answer 91

Genetics: monozygotic twin concordance = 30% dizygotic twin concordance = 2% EBV low vit d smoking obesity

Question 92

types of MS

Answer 92

relapsing remitting - mc - 85% acute attacks - last1-2 months followed by periods of remission secondary progressive disease: - relapsing remitting pts deteriorated and got neurological signs and symptoms between relapses. - 65% of RR patients get this within 15 yrs - gait and bladder disorders primary progressive -10% pts - progressive deterioration from onset - mc in older ppl

Question 93

Pathophysiology of MS

Answer 93

affects cns - oligodendrocytes. inflammation and immune cell infiltration damages myelin. pt gets ms attack - episode of optic neuritis. therell be other demyelinating lesions in cns but not causing sx. re-myelination can happen in early disease so sx resolve. later on in disease re-myelination is incomplete for sx get more permanent.

Question 94

characteristic feature of MS onset of sx - ms

Answer 94

disseminated in time and space lesions vary in location - affected sites and sx change over time progress over more than 24hrs. sx last days to weeks at 1st presentation and then improve.

Question 95

how would you investigate MS?

Answer 95

diagnosis need proof of lesions disseminated in time and space MRI: - high signal T2 LESIONS - periventricular plaques - Dawson fingers: often seen on FLAIR images - hyperintense lesions perpendicular to corpus callosum CSF: - oligoclonal bands IgG - not in serum - increased intrathecal synthesis of IgG Visual Evoked Potentials: - delayed, but well preserved waveform

Question 96

features of MS

Answer 96

75% lethargy visual: - optic neuritis - unliateral reduced vision hours to days. -optic atrophy -Uhtoff's phenomenon: worsening of vision (all sx) after body temp rise - internuclear opthalmoplegia - impaired adduction and nystagmus in contralateral abducting eye Sensory - pins/needles -numbness -trigeminal neuralgia - Lhermitte's Syndrome: paresthesiae in limbs on neck flexion Motor: - spastic weakness: mc legs cerebellar: -ataxia : more during acute relapse than presenting sx - tremor Other: - urinary incontinence -sex dysfunction - intellectual deterioration

Question 97

key features of optic neuritis

Answer 97

central scotoma - enlarged central blind spot pain with eye movement impaired colour vision relative afferent pupillary defect

Question 98

explain what a relative afferent pupillary defect is OPTIC NEURITIS

Answer 98

pupil in affected eye constricts more when shining a light in contralateral eye. reduced pupil response to shining light in affected eye - direct pupillary reflex check.

Question 99

in MS , lesion in abducens cnVI causes?

Answer 99

conjugate lateral gaze disorder. conjugate just means connected. lateral gaze where both eyes move laterally to lef tor right. when they look in a direction of affected eye, affected eye cant abduct. eg: lesion in left eye. when looking to left the right eye will adduct ( move towards nose) the left eye will stay middle.

Question 100

MS presents with focal weakness. give examples

Answer 100

incontinence horner syndrome facial nerve palsy limb paralysis

Question 101

ms presents with focal sensory sx. give examples

Answer 101

trigeminal neuralgia numbness paraesthesia lhermittes sign

Question 102

what is lhermittes sign? what does it tell you? how is it caused?

Answer 102

ms electric shock sensation travels down spine and into limbs when flex neck. tells you disease in cervical spinal cord in dorsal column. caused by stretching demyelinated dorsal column

Question 103

MS issue with ataxia - 2 types and differences

Answer 103

ataxia - coordinated movement issue sensory: loss of proprioception. - positive rombergs test. - lose balance when standing with eyes closed. - cause psedoathetosis - involuntary writhing movements. lesion in dorsal columns of spine can cause sensory ataxia. cerebellar: problems with cerebellum coordinating movement, indicating cerebellar lesion.

Question 104

how would you manage an acute relapse of MS?

Answer 104

acute relapse: - high dose steroid: oral/iv methylprednisolone 5 days. - short length of acute relapse

Question 105

when would you give disease modifying drugs for ms ?

Answer 105

relapsing remitting disease + 2 relapses in last 2 yrs + able to walk 100m unaided secondary progressive disease + 2 relapses in past 2 yrs + able to walk 10m (Aided/unaided)

Question 106

drug options for reducing relapse risk - MS

Answer 106

Natalizumab - 1st line - iv ocrelizumab - iv - also used first line fingolimod - oral formula beta-interferon - subcut/IM - not as effective as other drugs glatiramer acetate - subcut - immunomodulating drug - immune decoy

Question 107

natalizumab - ms tx MoA

Answer 107

recombinant monoclonal antibody that antagonises alpha 4 beta 1 integrin found on leucocyte surface. inhibit migration of leucocytes across endothelium across blood brain barrier.

Question 108

ocrelizumab - ms tx MoA

Answer 108

humanised anti-cd20 monoclonal antibody

Question 109

fingolimod - ms tx MoA

Answer 109

sphingosine 1-phosphate receptor modulator (S1P) prevents lymphocytes from leaving lymph nodes

Question 110

if an ms pt experiences fatigue what to give?

Answer 110

exclude anaemia thyroid and depression then give amantadine or mindfulness training and cbt

Question 111

if an ms pt has spasticity what to give?

Answer 111

baclofen and gabapentin: 1st line others: diazepam,dantrolene and tizanidine physio cannabis and botox: under evaluation

Question 112

how would you tx oscillopsia (when visual fields oscillate) in ms pt?

Answer 112

gabapentin : 1st line

Question 113

how would you manage bladder dysfunction in ms pt?

Answer 113

could be urgency incontinence or overflow get uss first to assess bladder emptying - anticholinergic could worsen sx in some pts if significant residual vol: intermittent self-catheterise if not: anticholinergics improve urinary frequency

Question 114

What is duchenne muscular dystrophy?

Answer 114

x linked recessive inherited disorder in the dystrophin genes required for normal muscular function. dystrophinn gene on Xp21

Question 115

features of duchenne muscular dystrophy

Answer 115

progressive proximal muscle weakness from 5 yrs calf pseduohypertrophy gowers sign: child uses arms to stand up from squatted position because muscles around pelvis not strong enough to get body erect. 30% pts - intellectual impairement

Question 116

how would you investigate duchenne muscular dystrophy?

Answer 116

raised creatinine kinase genetic testing replaced muscle biopsy to get definitive diagnosis

Question 117

how would you manage duchenne muscular dystrophy? prognosis

Answer 117

supportive no effective tx prognosis: - most kids cant walk by 12 yrs - survive till 25-30 - have dilated cardiomyopathy!!

Question 118

tell me about becker muscular dystrophy

Answer 118

develops after 10 yrs intellectual impairment much less common also x linked recessive.

Question 119

difference between duchenne muscular dystrophy and becker muscular dystrophy - genetics

Answer 119

duchenne: frameshift mutation = 1 or both of binding sites are lost = severe form becker: non-frameshift insertion in dystrophin gene = both binding sites preserved = milder form

Question 120

Tell me about myotonic dystrophy

Answer 120

inherited myopathy. 20-30 yrs old. skeletal cardiac and smooth muscle affects. 2 types: dm1 and dm2

Question 121

genetic component to myotonic dystrophy

Answer 121

autosomal dominant trinucleotide repeat disorder DM1 caused by CTG repeat at end of DMPK gene on chr 19 DM2 caused by repeat expansion of ZNF9 gene on chr3.

Question 122

key difference between dm1 and dm2 for myotonic dystrophy

Answer 122

dm1: dmpk gene on chr 19. distal weakness more prominent dm2 : znf9 gene on chr3. proximal weakness more. severe congenital form not seen

Question 123

general features of myotonic dystrophy

Answer 123

myotonic facies - long "HAGGARD" appearance frontal balding bilateral ptosis cataracts dysarthria myotonia (tonic spasm of muscle) weakness of arms and legs - initially distal mild mental impairement DM testicular atrophy cardiac: heart block, cardiomyopathy dysphagia PROLONGED MUSCLE CONTRACTION.

Question 124

briefly tell me about facioscapulohumeral muscular dystrophy

Answer 124

in childhood present weakness around face - progress to shoulders and arms sleep with eyes slightly open weakness pursing lips. cant blow cheeks out without air leaking from mouth

Question 125

briefly tell me about oculopharyngeal muscular dystrophy

Answer 125

late adulthood weakness of ocular muscles and pharynx. bilateral ptosis restricted eye movement swallowing problems muscles around limb girdles affected to varying degrees.

Question 126

briefly tell me about limb girdle muscular dystrophy

Answer 126

teenage years progressive weakness around limb girdle - hips and shoulders

Question 127

briefly tell me about emery-dreifuss muscular dystrophy

Answer 127

childhood with contractures - elbows and ankles. contracture: shorterning of muscle and tendons -- restrict range of movement in limb. progressive weakness and wasting of muscle - upper arm and lower legs first

Question 128

What is huntingtons disease?

Answer 128

inherited neurodegenerative condition. progressive incurable death 20yrs after initial sx start. autosomal dominant defect in huntingtin gene on chr 4 - genetic mutation of HTT trinucleotide repeat disorder: repeat expansion of CAG - PHENOMENON OF ANTICIPATION

Question 129

what is the phenomenon of anticipation in huntingtons disease?

Answer 129

disease presents earlier age in sucessive generations

Question 130

features of huntingtons disease age of presentation

Answer 130

typically develop after 35 yrs old chorea - involuntary random irregular abnormal body movements. personality change - irritable, apathy, depression and intellectual impairment dystonia - abnormal muscle tone - abnormal posture saccadic eye movements dysarthria - speech issue dysphagia

Question 131

how would you manage huntingtons?

Answer 131

physio - for contractures speech and language - speech and swallowing difficulty tetrabenazine - chorea

Question 132

why might someone get a brain abscess?

Answer 132

extension of sepsis from middle ear or sinuses trauma or surgery to scalp penetrating head injury embolic event from endocarditis

Question 132

what is someone most likely going to die with when they have huntingtons disease?

Answer 132

aspiration pneumonia. or suicide.

Question 133

presenting sx of brain abscess

Answer 133

depend on site of abscess - if motor cortex will present earlier mass effect on brain and raised icp headache: dull persistent fever: poss absent. not swinging pyrexia though focal neurology: oculomotor nerve palsy or abducens nerve palsy secondary to raised icp nausea papilloedema seizures

Question 134

how would you investigate for brain abscess

Answer 134

ct scanning

Question 135

how would you manage for brain abscess

Answer 135

surgery - craniotomy - debride abscess cavity. abscess can reform because head is closed after abscess drainage iv abx: 3rd gen cephalosporin+ metronidazole icp manage: dexamethasone

Question 136

causative organisms of meningitis

Answer 136

neonatal to 3 months: - group b strept : from mother at birth. mc in low birth weight babies and after prolonged rupture of membranes -ecoli and other gram negative organisms - listeria monocytogenes 1mnth- 6yrs: - neisseria menigitidis - meningococcus - strept pneumoniae - pneumococcus -haemophilus influenzae over6: - Neisseria meningitidis - meningococcus -streptococcus pneumoniae - pneumococcus over 60 : streptococcus pneumonia neisseria meningitidis immunosupresed: listeria monocytogenes

Question 137

meningitis vaccination includes which serotypes and given when

Answer 137

2 months 4 months 12-13 months a and c and b (bexsero)

Question 138

symptoms and signs of meningitis

Answer 138

symptoms: headache fever n+v, photophobia drowsiness seizures signs: neck stiffness purpuric rash - particular with invasive meningococcal disease

Question 139

what stain is used to find tuberculous meningitis ?

Answer 139

ziehl neelsen 20% sensitive

Question 140

csf findings in meningitis bacterial viral tuberculous fungal appearance glucose protein white cells

Answer 140

bacterial: cloudy low - (<1/2 plasma) high (>1 g/l) 10 -5000 polymorphs /mm3 viral: clear/cloudy 60-80% of plasma glucose normal/raised 15-1000 tuberculous: slight cloudy,fibrin web low high 10-1000 cloudy low high 20-200

Question 141

complications of meningitis

Answer 141

neurological sequlae: sensorineural hearing loss - mc seizures focal neurological deficit infective: sepsis, intracerebral abscess pressure: brain herniation, hydrocephalus

Question 142

pt has meningococcal meningitis, risk of what syndrome?

Answer 142

waterhouse-friderichsen adrenal insufficiency secondary to adrenal haemorrhage

Question 143

in what patients would you not do lumbar puncture for suspected meningitis? what to do instead

Answer 143

focal neurological signs papilloedema significant bulging of fontanelle disseminated intravascular coagulation signs of cerebral hernation blood cultures and pcr

Question 144

how would you manage meningitis in kids?

Answer 144

abx under 3 months: iv amox (or ampiciliin) + iv cefotaxime over 3 months: iv cefotaxime - of ceftriaxone steroids - not to under 3 mnths - dexamethasone - if LP shows: frankly purulent csf. csf wbc over 1000/microlitre protein over 1g/l bacteria on gram stain fluids: treat shock eg with colloid cerebral monitoring - mechanical ventilation if resp impairment public health notify

Question 145

what abx would you give as prophylaxis to contacts of meningitis

Answer 145

ciprofloxacin

Question 146

what is meningitis?

Answer 146

inflammation of leptomeninges and csf of subarachnoid space.

Question 147

viral causes of meningitis

Answer 147

non-polio enteroviruses: coxsackie, echovirus mumps hsv, cytomegalovirus, herpes zoster hiv measles

Question 148

risk factors of viral meningitis

Answer 148

pt at extremes of age under 5 and old immunocompromised - pt with renal failure, with dm ivdu

Question 149

clinical features of viral meningitis

Answer 149

common: headache neck stiffness photophobia - milder than bacterial confusion fever NON BLANCHING RASH less common: - focal neurological deficit on exam - seizure: suggest meningoencephalitis

Question 150

how would you investigate viral meningitis

Answer 150

LP - csf opening pressure - 10-20cm3 h20 cell count - 10-300 cells/ul cell differential - lymphocytes glucose - 2.8-4.2 mmol/l protein - 0.5-1 g/dl viral pcr - underlying organism

Question 151

how would you manage viral meningitis?

Answer 151

if thinking bacterial or encephalitis: start broad spect abx with cns pentration: ceftriaxone and aciclovir IV. viral meningitis self limiting - immproves 7-14 days. aciclovir - if pt suspected to have meningitis secondary to hsv.

Question 152

pt with meningitis - hospital approach

Answer 152

a - irway b - reathing c - circulation d - disability : gcs - focal neuro signs, seizures, papilloedema

Question 153

warning signs for acute presentation meningitis

Answer 153

rapidly progressing rash poor peripheral perfusion resp rate under 8 or over 30 or pulse under 40 over 140 ph under 7.3 wbc under 4*10(9) or lactate over 4 mmol/l gcs under 12 or drop of 2 pts poor response to fluid resus

Question 154

in what circumstance would you delay a lumbar puncture - meningitis - bacterial suspected?

Answer 154

signs of severe sepsis or rapidly evolving rash severe resp/cardiac compromise significant bleeding risk signs of raised icp : focal neuro signs,papilloedema,continuous/uncontrolled seizures, gcs 12 or less.

Question 155

how would you manage bacterial meningitis with pt without indication for delayed lp

Answer 155

iv access - blood and blood cultures lp: if not in 1st hr - iv abx after blood cultures taken iv abx: 3mth-50 : cefotaxime - or ceftriaxone over 50: above + amox (or ampicillin) for adults iv dex : adjunctive - esp if pneumoccocal menin in adults - start before or with first dose of antibac, but not later than 12 hours after. NO CT

Question 156

in what case would you avoid dex - meningitis bacterial case?

Answer 156

septic shock, meningococcal septiciaemia immunocompromised or meningitis after surgery.

Question 157

how would you manage a bacterial meningitis case with raised icp? same if severe sepsis or rapidly evolving rash

Answer 157

secure airway + high flow ox iv access - bloods and blood cultures iv dex iv abx neuroimagine

Question 158

what bloods to test for suspected bacterial meningitis? other tests

Answer 158

fbx renal flucose lactate clotting profile crp blood gases throat swab for meningococcal culture

Question 159

bacterial meningitis pt : initial empirical therpay undr 3 months or over 50

Answer 159

iv cefotaxime + amoxicillin

Question 160

bacterial meningitis - initial empiral therapy 3mths - 50 yrs

Answer 160

iv cefotaxime - or ceftriaxone

Question 161

meningococcal meningitis - bactieral scenario mx

Answer 161

iv benzylpenicillin or cefotaxime (or ceftriaxone)

Question 162

pneumococcal meningitis - bacterial meningitis scenario mx meningitis caused by h influenzae

Answer 162

iv cefotaxime (or ceftriaxone)

Question 163

meningitis caused by listeria - mx

Answer 163

iv amox or amp - + gentamicin

Question 164

what to do in bacterial meningitis mx if pt history of immediate hypersensitivity to penicillin to cephalosporins?

Answer 164

chloramphenicol

Question 165

prophylaxis for contacts of bacterial meningitis

Answer 165

if exposed to resp secretion. risk highest in 1st 7 days- lasts for 4 weeks. if confirmed: give if close within 7 days before onset. oral ciprofloxacin or rifampicin. give meningococcal vaccination. - give booster if they had it in infancy. no prophylaxis for pneumococcal.

Question 166

2 tests for meningeal irritation

Answer 166

both pt lies on back kernigs - flex 1 hip and knee to 90 degree. slowly straighten knee keep hip flexed. spinal pain or resistance? brudzinkis - use hand to life head and neck off bed and flex chin to chest. - causes pt to involuntarily flex hips and knees

Question 167

what is guillain barre syndrome? triggers

Answer 167

acute paralytic polyneuropathy affects pns. immune mediated demyelination of pns. acute, symmetrical ascending weakness. sensory symptoms. triggered by infections: - campylobacter jejuni -cytomegalovirus (cmv) -epstein-barr virus (ebv)

Question 168

pathophysiology of guillain barre syndrome

Answer 168

cross reaction of antibodies with gangliosides in pns correlation between anti-ganglioside antibody (anti-gm1) and clinical features seen anti-gm1 antibodies - seen in 25% pts

Question 169

what is miller fisher syndrome?

Answer 169

variant of guillain-barre syndrome opthalmoplegia, areflexia, ataxia. - eye muscles affected first. descending paralysis not ascending. anti-gq1b antibodies in 90% pts

Question 170

initial symptoms of gullain barre syndrome characteristic features other features lesson common findings:

Answer 170

back/leg pain -progressive symmetrical weakness of all limbs. ascending - legs affected first. - reflexes reduced/absent -sensory sx mild - distal paresthesia with very few sensory signs other: - hx of gastroenteritis -respiratory muscle weakness - cranial nerve involvement - diplopia, bilateral facial nerve palsy, oropharyngeal weakness - autonomic involvement: - urinary involvement, diarrhoea less common: - papilloedema : secondary to reduced csf resorption

Question 171

how would you investigate guillain barre syndrome?

Answer 171

lp : rise in protein. normal wbc count - albuminocytologic dissociation nerve conduction studies : decreased motor nerve conduction velocity - due to demyelination prolonged distal motor latency increased f wave latency

Question 172

what criteria to diagnose guillain barre?

Answer 172

brighton criteria

Question 173

how would you manage guillain barre syndrome?

Answer 173

vte prophylaxis - pulmonary embolism iv immunoglobulins - IVIG - 1st line plasmapheresis - alternative to IVIG severe: if resp failure require intubation, ventilation and admit to icu.

Question 174

features of encephalitis

Answer 174

fever headache psychiatric sx , seizures, vomiting focal features: aphasia altered cognition/conciousness. unusual behaviour. peripheral lesions: like cold sores no relationship to presence of HSV encephalitis

Question 175

pathophysiology of encephalitis

Answer 175

hsv-1 - 95% adult cases affects temporal and inferior frontal lobes

Question 176

how would you investigate encephalitis

Answer 176

csf - lymphocytosis, elevated protein pcr: HSV,VSV,ENTEROVIRUSES neuroimaging: CT - medial temporal and inferior frontal changes - eg petechial haemorrhages normal in 1/3 of patients MRI is better hiv test swabs - causative organism, throat and vesicle swab EEG: lateralised periodic discharges at 2Hz

Question 177

how would you manage encephalitis

Answer 177

intravenous aciclovir start in all suspected encephalitis cases - hsv and vsv ganciclovir - cmv

Question 178

complications of encephalitis

Answer 178

lasting fatigue/prolonged recovery headache, chronic pain, learning disability, change to memory, personality, mood , cognition. movement disorder sensory disturbance siezure hormonal imbalance

Question 179

contraindications of lp in encephalitis in kids

Answer 179

gcs below 9 haemodynamiccaly unstable active seizures post-ictal

Question 180

causes of encephalitis in kids

Answer 180

non infective: autoimmune mc : viral bacterial and fungal - rare mc: hsv. hsv-1 from cold sores. in neonates: hsv-2 from genital herpes from birth. vzv: chicken pox,cmv associated with immunodeficiency. ebv: infectious monucleosis, adenovirus, influenza virus. polio, mumps, rubella, measles - all can cause it too.

Question 181

hsv encephalitis - typically affects with lobes

Answer 181

temporal

Question 182

what is shingles herpes zoster?

Answer 182

acute unilateral painful blistering rash caused by reactivation of varicella-zoster virus - vzv. (following vzv infection, chickenpox) virus lies dormant in dorsal root or cranial nerve ganglia.

Question 183

risk factors of shingles

Answer 183

increasing age hiv: strong rf 15* more common other immunosuppressive conoditions: steroids, chemo

Question 184

most common affected dermatomes in shingles

Answer 184

t1-l2

Question 185

features of shingles

Answer 185

prodromal period: - burning pain over affected dermatome for 2-3 days. -pain might be severe and sleep - 20% pts experience fever, headache, lethargy rash - erythematous, macular rash over affected dermatome -quickly becomes vesicular - characteristically well demarcated by dermatome dont cross midline. " bleeding" in adjacent areas might be seen. clinical diagnosis

Question 186

how would you manage shingles - herpes zoster

Answer 186

avoid pregnant women and immunosuppresed - contagious infectious until vesicles crusted over, 5-7 days after onset. covering lesions reduces risk. analgesia: - 1st line para and nsaids - if not : neuropathic agent like amitriptyline - oral corticosteroids - in 1st 2 weeks in immunocompetent adults with localised shingles if pain severe. antivirals - within 72 hrs for most patients, unless pt under 50 and mild truncal rash with mild pain - aciclovir , famciclovir, valaciclovir

Question 187

benefit of prescribing antiviral in shingles - herpes zoster

Answer 187

reduced incidence of post-herpetic neuralgia

Question 188

complications of shingles -herpes zoster

Answer 188

postherpetic neuralgia herpes zoster opthalmicus - ocular division of trigeminal nerve herpes zoster oticus - ear lesions and facial paralysis

Question 189

malaria is caused by?

Answer 189

plasmodium protozoa spread by female anopheles mosquito: 4 diff : plasmodium falciparum - most. plasmodium vivax - 2nd - benign malaria plasmodium ovale plasmodium malariae

Question 190

malaria protects from what diseases?

Answer 190

sickle-cell trait g6pd deficiency hla b53 absence of duffy antigens

Question 191

falciparum malaria - mc - most severe type: features - classic triad why do they occur every 48 hrs?

Answer 191

paroxysms of fever chills and sweating. every 48 hrs because erythrocytic cycle of plasmodium falciparum parasite. fever high intermittent. - possible rigors too. non specific: malaise,headache,myalgia

Question 192

general features of falciparum malaria

Answer 192

fever - cyclical - sweating and sometimes rigors. gi: -anorexia,n+v, abdo pain. diarhoea poss , mc in kids. poss mild jaundice and occasional pruritus resp: cough, poss mild tachypnoea msk: generalised body aches and joint pain neuro: headache. dizziness and sleep disturbance. cv: tachy, hypotension more typical of severe malaria. haem: thrombocytopenia most significant haematological finding. mild anaemia poss renal: aki associated with severe malaria. non severe : mild-moderate increase in creatinine or blood urea nitrogen levels

Question 193

features of severe falciparum malaria how to treat

Answer 193

schizonts on blood film parasitaemia >2% hypogly acidosis temp over 39 severe anaemia iv artesunate if parasite count over 10% then exchange tranfusion shock might show bacterial septicaemia

Question 194

comps of malaria falciparum

Answer 194

cerebral malaria: seizures, coma acute renal failure: blackwater fever, secondary to intravascular haemolysis ards hypogly dic

Question 195

how would you manage falciparum malaria?

Answer 195

uncomplicated falciparum: 1st line: artemisinin-based combo therapy (acts) - artemether+ lumefantrine, artesunat+ amodiaquine, artesunate+ mefloquine, artesunate + sulfadoxine-pyrimethamine, dihydroartemisinin + piperaquine

Question 196

mc non falciparum malaria

Answer 196

vivax - central america and indian subcontinent. then ovale (africa) and malariae. knowlesi - south east asia

Question 197

features of non falciparum malaria

Answer 197

fever headache splenomegaly vivax/ovale: cyclical fever every 48 hrs. malariae: cyclical fever every 72 hours. associated with nephrotic syndrome. ovale and vivax: hypozoite stage : relapse after tx.

Question 198

how would you treat non falciparum malaria?

Answer 198

artemisinin-based combo therapy (act) or chloroquine if chloroquine resistant. act avoid in pregnancy. ovale/vivax: primaquine after acute tx with chloroquine to destroy liver hypnozoites and prevent relapse

Question 199

incubation period of malaria

Answer 199

1-4 weeks after exposure. vivax and ovale can lie dormant upto 4 yrs

Question 200

how do you diagnose malaria

Answer 200

blood film. edta bottle. 3 negative samples over 3 consecutive days needed to exclude.

Question 201

side effect of primaquine

Answer 201

can cause severe haemolysis in g6pd pts

Question 202

doxycline - use in malaria when to give etc side effedcts

Answer 202

broad spect abx se: diarrhoea , thrush. skin sensitivity to sun take 2 days before until 4 weeks after endemic area travel.

Question 203

antimalarial meds

Answer 203

proguanil with atovaquone doxycycline mefloquine - psych side effects. - anxiety,dep,abnormal dreams chloroquine with proguanil

Question 204

general advice preventing malaria

Answer 204

mosquito spray 50% DEET spray nets and barriers antimalarial meds

Question 205

metastatic brain cancer can spread from?

Answer 205

lung - MC breast bowel skin - namely melanoma kidney

Question 206

features of brain tumour

Answer 206

poss asx progressive focal neurological symptoms raised icp

Question 207

features of raised icp

Answer 207

headache contant worse at night or on waking or coughing/straining. vomiting papilloedema on fundoscopy (paton lines) altered mental state ptosis unilateral visual field defect 3rd and 6th nerve palsy

Question 208

with papilloedema what do u see on fundoscopy?

Answer 208

blurring of optic disc margin engorged retinal veins haemorrhages around optic disc paton lines - creases in retina around optic disc loss of venous pulsation elevated optic disc

Question 209

what is a glioma?

Answer 209

tumour of glial cells in brain/spinal cord. support/surround neurones. include: astrocytes,oligodendrocytes, ependymal cells. grade 1-4. 4 (glioblastoma multiforme)

Question 210

3 types of glioma

Answer 210

astrocytoma - mc and aggressive is glioblastoma oligodendroglioma ependymoma

Question 211

what is a meningioma? histology ix tx

Answer 211

tumour of meninges. benign mass effect. = raised icp arise from arachnoid cap and typically located next to dura. spindle cells in concentric whirls and calcified psammoma bodies. CT: contrast enhancement MRI tx: RADIO/ SURGERY

Question 212

gliobastoma multiforme prognosis imaging histology tx

Answer 212

solid tumour with central necrosis and a rim that enhances contrast. pleomorphic tumour cells border necrotic areas surgery and postop chemo and/or radio. dex: oedema

Question 213

what is an acoustic neuroma? classical history of it

Answer 213

vestibular schwannoma (benign tumour of schwann cells surrounding auditory nerve) slow growing benign intracranial tumour. combo of : vertigo hearing loss tinnitus absent corneal reflex

Question 214

features of acoustic neuroma

Answer 214

CN VIII : vertigo, unilateral sensorineural hearing loss, unilateral tinnitus cranial nerve V: absent corneal reflex cranial nerve VII: facial palsy

Question 215

bilateral vestibular schwannoma are seen in what condition?

Answer 215

neurofibromatosis type 2

Question 216

ix of acoustic neuroma mx

Answer 216

MRI of cerebelllopontine angle. audiometry : only 5% of pts will have a normal one surgery, radio.

Question 217

where do acoustic neuromas occur?

Answer 217

cerebellopontine angle

Question 218

unknown primary of metastatic disease what to do?

Answer 218

fbc u+E, lft, calcium, urinalysis, ldh cxr ct chest abdo pelvis AFP , hCG myeloma screen - if lytic bone lesions endoscopy psa - men ca 125 - women with peritoneal malignancy or ascites testicular US - men with germ cell tumour mammography

Question 219

tell me about pituitary tumours. press on what? sx? mx

Answer 219

benign optic chiasm press - bitemporal hemianopia cause hormone def or release excessive hormones lead to: - acromegaly -hyperprolactinaemia - cushings disease - thyroxicosis transphenoidal surgery radio bromocriptine - block excess prolactin somatostatin analogue - block excess GH

Question 220

symptoms of cerebellar disease

Answer 220

D - dysdiadokinesia, dysmetria, "drunk" A - ataxia - limb,truncal N - nystagmus - horizantal = ipsilateral hemisphere I - intention tremor S - slurred staccato speech, scanning dysarthria H - hypotonia

Question 221

unilateral cerebellar lesions cause what signs?

Answer 221

ipsilateral

Question 222

causes of cerebellar disease

Answer 222

friedreichs ataxia neoplastic: cerebellar haemangioma stroke alcohol ms hypothyroid drugS: phenytoin, lead poisoning paraneoplastic: 2 to lung cancer

Question 223

What is normal pressure hydrocephalus?

Answer 223

reversible cause of dementia in elderly. 2 to reduced csf absorption at arachnoid villi. could be due to head injury, subarachnoid haemorrhage or meningitis

Question 224

classic triad of normal pressure hydrocephalus

Answer 224

urinary incontinence dementia and bradyphrenia gait abnormality sx develop over a few months

Question 225

imaging for normal pressure hydrocephalus

Answer 225

hydrocephalus with ventriculomegaly in absence of ,or out of proportion to , sulcal enlargement

Question 226

how would you manage normal pressure hydrocephalus comps of this tx

Answer 226

ventriculoperitoneal shunting seizures infection intracerebral haemorrhage

Question 227

mc cause of hydrocephalus

Answer 227

aqueductal stenosis = insuffiency drainage of csf. chromosomal abnormality arachnoid cysts arnold-chiari malformation

Question 228

presentation of normal pressure hydrocephalus

Answer 228

cranial bones in babies not fused at sutures until 2. outward pressure on cranial bones so have enlarged and rapid increasing head circumference: occipitofrontal circumference bulging anterior fontanelle poor feeding and vomiting poor tone sleepiness

Question 229

how does a ventriculoperitoneal shunt work?

Answer 229

drain csf from ventricles to other body cavity. usually perioneal cavity.

Question 230

what is neurofibromatosis?

Answer 230

genetic condition causing nerve tumours - neuroma benign type 1 type 2

Question 231

neurofibromatosis type 1 gene found on which chromosome? genetic component

Answer 231

chr 17 codes for neurofibromin - tumour suppressor protein. autosomal dominant

Question 232

features of neurofibromatosis type 1

Answer 232

C - cafe au lait spots (more than 15 mm diameter) R - relative with nf1 A - axillary/inguinal freckling BB - bony displasia, bowing of long bone or sphenoid wing dysplasia I - iris hamartomas - lisch nodules - yellow-brown spots on iris N - neurofibromas G - glioma of optic pathway

Question 233

what are neurofibromas? plexiform neurofibroma?

Answer 233

skin coloured raised nodules / papules smooth regular surface. 2 or more. plexiform : larger irregular complex with multiple cell types.

Question 234

neurofibromatosis type 2 on what chr? tx associations genetic component what it codes for

Answer 234

chr 22 codes for merlin - tumour supressor protein in schwann cells. autosomal dominant. associated with acoustic neuroma. bilateral vestibular schwannoma multiple intracranial scwannomas, meningiomas, ependymomas surgery: resect tumour. permanent nerve damage

Question 235

comps of neurofibromatosis

Answer 235

malignant peripheral nerve sheath gi stromal tumour brain tumour spinal cord tumour increased cancer risk - breast and leuk vision loss scoliosis behaviour/ld renal artery stenosis migraine epilepsy

Question 236

What is myasthenia gravis?

Answer 236

autoimmune affecting neuromuscular junction. acetycholine receptor antibody blocks postsynaptic receptor block muscle activity.

Question 237

features of myasthenia gravis link with what condition

Answer 237

worse with activity improves with rest men and women - typically women under 40 men over 60 thymomas!! autoimmune: pernicious anaemia, autoimmune thyroid disorder, rheumtoid, sle thymic hyperplasia in 50-70% more in women

Question 238

2 antibodies that can cause myasthenia gravis

Answer 238

muscle specific kinase (MuSK) antibodies Low density lipoprotein receptor related protein 4 (LRP4) antibody

Question 239

how would you investigate for myasthenia gravis?

Answer 239

single fibre electromyography : high sensitivity ct thorax - exclude thymoma CK normal antibodies to acetylcholine receptors - 85% pts and 40% for anti muscle specific tyrosine kinase antibodies tensilon test: iv edrophonium reduces muscle weakness temp - dont use because of cardiac arrhythmia risk

Question 240

mx of myasthenia gravis

Answer 240

long acting acetylcholinesterase inhibitor: pyridostigmine - 1st line immunosuppression not started at diagnosis, but eventually need: - prednisolone - azathioprine, cyclosporine, mycophenolate mofetil - thymectomy

Question 241

how would you manage myasthenic crisis

Answer 241

plasmapheresis iv immunoglobulins

Question 242

symptoms of myasthenia gravis

Answer 242

affect proximal muscles of limbs and small muscles of head and neck : hard to climb stairs , stand from seat , raise hand above head diplopia ptosis facial movement weakness dysphagia fatigue chewing slurred speech.

Question 243

what examinations could you do for myasthenia gravis?

Answer 243

check for thymectomy scar test for fvc repeat blinking - ptosis prolonged upward gaze - diplopia repeat abduction of 1 arm 20 times - unilateral weakness

Question 244

what drugs can exacerbate myasthenia

Answer 244

penicillamine quinidine, procainamide beta block lithium phenytoin abx: gentamicin, macrolides, quinolones, tetracycline

Question 245

what is narcolepsy

Answer 245

chronic neuro disorder affects brain ability to control sleep wake cycle hla dr2 low levels of orexin (hypocretin) - protein responsible for controlling appetite and sleep patterns early onset of REM sleep

Question 246

features of narcolepsy

Answer 246

teenage hypersomnolence cataplexy - sudden loss of muscle tone often triggered by emotion sleep paralysis vivid hallucinations on going sleep/waking

Question 247

ix for narcolepsy

Answer 247

multiple sleep latency EEG

Question 248

mx for narcolepsy

Answer 248

daytime stimulants - modafinil nightime sodium oxybate

Question 249

What is chronic fatigue syndrome? epidemiology

Answer 249

at least 3 months of disabling fatigue affecting mental and physical function over 50% of time in absence of other disease. more in females past psych hx isnt rf

Question 250

features of chronic fatigue syndrome

Answer 250

sleep problems: insomnia, hypersomina, unrefreshing sleep, disturbed sleep-wake cycle headache muscle/joint pain painful lymph node no enlarged sore throat nausea palpitations dizziness general malaise physical/mental exertion cognitive dysfunction : difficulty thinking, concentration issue, impairement of short term memory.

Question 251

how would you investigate chronic fatigue syndrome

Answer 251

screening bloods fbc u e lft glucose tft esr crp calcium ck ferritin coeliac urinalysis

Question 252

how would you diagnose chronic fatigue syndrome? how would you manage?

Answer 252

if sx persist 3 months refer to cfs energy mx: self management physical activity- if they feel ready. (and if part of cfs only) graded exercise therapy cbt: supportive rather than curative

Question 253

What is wernickes encephalopathy? rarer causes

Answer 253

thiamine deficiency caused by alcoholism. neuropsychiatric persistent vomiting stomach cancer diet def

Question 254

classic triad of wernickes encephalopathy

Answer 254

opthalmoplegia nystagmus ataxia encephalopathy petechial haemorrhage in mamillary bodies and ventricular walls

Question 255

features of Wernickes encephalopathy

Answer 255

oculomotor dysfunction- nystagmus , Opthalmoplegia: lateral rectus palsy, conjugate gaze palsy gait ataxia encephalopathy: confusion, disorientation, indifference, inattentiveness peripheral sensory neuropathy

Question 256

Investigations of Wernicke's Encephalopathy tx

Answer 256

decreased red cell transketolase MRI urgent replacement of thiamine

Question 257

relationship of wernickes with korsakoff

Answer 257

addition of antero and retrograde amnesia and confabulation.

Question 258

calculation of alcohol units

Answer 258

volume ml * alcohol content % /1000 = units of alcohol

Question 259

units of alcohol

Answer 259

no more than 14 spread even over 3 or more days no more than 5 units in single day. binge: 6 or more women. 8 or more men.

Question 260

typical triad of menieres disease

Answer 260

hearing loss vertigo tinnitus

Question 261

what is menieres disease?

Answer 261

long term inner ear disorder. recurrent attacks fullness in ear excessive buildup of endolymph in labyrinth of inner ear - higher pressure than normal. excessive pressure and progressive dilation of endolymphatic system.

Question 262

presentation of menieres disease

Answer 262

typical 40-50 can happen to anyone. unilateral episode of vertigo hearing loss and tinnitus vertigo: episodes. 20 mins. hearing loss: fluctuates at first, then more pernanent. tinnitus: episodes of vertigo. eventually permenant. unilaterla. fullness unexplained falls - without loss of consciousness imbalance - after vertigo. spontaneous nystagmus - during acute attack.

Question 263

how would you diagnose menieres disease how would you manage?

Answer 263

clinical. audiology asessment manage sx prophylaxis acute: buccal/intramuscular prochlorperazine, antihistamines (cyclizine, cinnarizine, promethazine) prophylaxis: betahistine or vestibular rehab

Question 264

what is diabetic neuropathy? how to tx

Answer 264

sensory loss glove and stocking lower legs first. 1st line: amitriptyline, duloxetine, gabapentin or pregabalin tramadol: rescue for neuropathic pain topical capsaicin - localised neuropathic pain - post herpetic neuralgia. pain management clinic

Question 265

what does gastroperesis occur secondary to ? sx? mx?

Answer 265

autonomic neuropathy erratic bg control, bloating, vomiting metoclopramide, domperidone, erythromycin

Question 266

what is trigeminal neuralgia?

Answer 266

pain syndrome. severe unilateral pain. can be idiopathic. compression of trigeminal roots by tumours or vascular problems poss. brief electric shock like pain - abrupt in start and finish.

Question 267

how would you manage trigeminal neuralgia?

Answer 267

1st line: carbamazepine

Question 268

red flag sx suggesting serious illness to do with trigeminal neuralgia

Answer 268

age before 40 fhx of ms optic neuritis deafness sensory changes hx of skin/oral lesions that could spread perineurally pain only in opthalmic division of trigeminal nerve (eye socket, forehead, nose) or bilaterally

Question 269

particularly susceptible areas for trigeminal neuralgia pain evoked by pain remited?

Answer 269

small areas in nasolabial fold light touch washing shaving smoking talking brushing teeth pain remits for variable periods

Question 270

what is horners syndrome?

Answer 270

combo of sx that happen when group of nerves known as sympathetic trunk is damaged. eye and surrounding area on 1 side of face.

Question 271

features of horners syndrome

Answer 271

miosis - small pupil ptosis enophthalmos - sunken eye anhidrosis - loss of sweating one side

Question 272

using anhidrosis to decipher where the lesion is for horners syndrome

Answer 272

face arm and trunk: central lesion face: pre-ganglionic no anhidrosis: post-ganglionic

Question 273

central lesion causes of horners pre ganglionic post ganglionic

Answer 273

stroke, syringomyelia, ms, tumour, encephalitis pancoasts tumour , thryoidectomy, trauma, cervical rib carotid artery dissection, carotid anerusym, cavernous sinus thrombosis, cluster headache

Question 274

heterochronic - difference in iris colour is seen in what?

Answer 274

congenital horners

Question 275

horners - what are apraclonidine drops

Answer 275

alpha adrenergic agonist cause pupillary dilation produces mild pupillary construction in normal pupil.

Question 276

what is gca? temporal arteritis? what condition is it associated with mc in who? key comp?

Answer 276

medium and large artery vasculitis systemic. strong link with polymyalgia rheumatica mc: older white pts vision loss - often irreversible

Question 277

presentation of temporal arteritis - gca

Answer 277

unilateral headache - severe around temple and forehead. scalp tenderness - brushing hair jaw claudication blurred/double vision loss of vision in untx. temporal artery tender and thickened to palpation with reduced or absent pulsation

Question 278

what other features might you have for gca?

Answer 278

sx of pmr: shoulder and pelvic girdle pain and stiffness systemic sx: wt loss, fatigue, low grade fever muscle tenderness carpel tunnel syndrome peripheral oedema

Question 279

how would you diagnose gca?

Answer 279

raised esr - 50mm/hr temporal artery biopsy : multinucleated giant cells (skip lesions) CK and EMG - normal duplex uss - hypoechoic halo sign and stenosis of temporal artery vision testing

Question 280

what should you expect to see in vision testing in gca?

Answer 280

anterior ischaemic optic neuropathy - occlusion of posterior ciliary artery. - FUNDOSCOPY SHOWS SWOLLEN PALE DISC, BLURRED MARGINS poss amaurosis fugax permanent vision loss poss? - sudden diplopia

Question 281

how would you manage gca?

Answer 281

steroids straight away. - 40-60 mg pred daily with no visual sx or jaw claudication - 500mg - 1000mg methylpred if above steroid slowly weaned over 1-2 yrs. aspirin 75mg - decrease vision loss/strokes ppi - omeprazole - gastroprotection on steroids bisphosphonates and calcium and vit d - bone protection

Question 282

What is meralgia paresthetica ?

Answer 282

localised sensory sx of outer thigh caused by compression of lateral femoral cutaneous nerve. mononeuropathy - one nerve

Question 283

presentation of meralgia paresthetica?

Answer 283

abnormal sensation - dysaesthesia and loss of sensation - anaesthesia inlateral femoral cutaneous nerve distribution. skin of upper outer thigh affected. burning numbness pins and needles cold sensation localised hair loss.

Question 284

in meralgia paresthetica , sx aggravated by?

Answer 284

walking standing long time improve when sit down. worse with extension of hip on affected side.

Question 285

how to manage meralgia paresthetica?

Answer 285

rest looser clothing wt loss physio para nsaids neuropathic analgesia - amitriptyline, gabapentin, pregabalin, duloxetine steroid/anaesthetic local nijection surgery: decompression, transection, resection

Question 286

lateral femoral cutaneous nerve originates from?

Answer 286

l1 l2 and l3 nerve roots. being psoas muscle around surface of iliacus muscle under inguinal ligament onto thigh.

Question 287

What is Cerebral Palsy?

Answer 287

non-progressive lesion motor pathways in developing brain. disorder of movement and posture.

Question 288

causes of cerebral palsy antenatal intrapartum postnatal

Answer 288

antenatal 80% - cerebral malformation -congenital infection (rubella, toxoplasmosis, CMV) intrapartum 10% = - birth asphyxia/trauma -preterm birth postnatal 10% - intraventricular haemorrhage -meningitis -head trauma

Question 289

classifying cerebral palsy 4 different types

Answer 289

spastic 80% - hemiplegia, diplegia or quadriplegia -increased tone (hypertonia) and reduced function resulting from damage to UMN dyskinetic: - damage to basal ganglia and substantia nigra -athetoid movements and oro-motor problems . muscle tone problems (hypertonia/hypotonia) causing the athetoid etc. ataxic: - caused by damage to the cerebellum with typical cerebellar signs - problems with coordinated movement due to above. mixed: mixed of spastic,dyskinetic and/or ataxic features.

Question 290

patterns of spastic cerebral palsy

Answer 290

monoplegia: 1 limb affected hemiplegia: one side of body affected diplegia: 4 limbs affected by mostly legs quadriplegia: neck down all 4 limbs - with seizures, speech disturbance and other impairements

Question 291

what non-motor problems might a cerebral palsy patient have?

Answer 291

learning difficulties - 60% epilepsy 30 squints 30% hearing impairement 20

Question 292

How would you manage cerebral palsy?

Answer 292

tx spasticity: -oral diazepam -oral and intrathecal baclofen (muscle relaxant), botulinum toxin type A , orthopaedic surgery and selective dorsal rhizotomy. - anticonvulsants, analgesia prn physio - stretch and strength muscles prevent muscle contractures. OT SLT DIETICIAN - peg feeding poss? ORTHO SURGEON - release contractures or length tendons (tenotomy) glycopyrronium bromide: excessive drooling. anti-epileptic - seizures

Question 293

what condition makes children at risk of developing cerebral palsy?

Answer 293

hypoxic-ischaemic encephalopathy

Question 294

signs and symptoms of cerebral palsy

Answer 294

failure to meet milestones learning difficulty feeding or swallowing problems problems with coordination, speech or walking increased/decreased tone, generally or in specific limbs hand preference below 18 months .

Question 295

based on neuro exam what to each of these indicate: hemi/di plegic gait broad based gait/ataxic gait high stepping gait waddling gait antalgic gait

Answer 295

UMN lesion cerebellar lesion foot drop/lmn lesion pelvic muscle weakness due to myopathy indicates localised pain

Question 296

with a UMN lesion tell me about: inspection tone power reflexes

Answer 296

muscle bulk preserved hypertonia slightly reduced brisk

Question 297

with a lmn tell me about: inspection tone power reflex

Answer 297

reduced muscle bulk with fasciculations hypotonia dramatically reduced reduced

Question 298

complications and associated conditions of cerebral palsy

Answer 298

learning disability epilepsy kyphoscoliosis muscle contractures hearing and visual impairment gastro-oesophageal reflux

Question 299

what is hypoxic ischaemic encephalopathy?

Answer 299

neonates. hypoxia during birth.

Question 300

causes of HIE

Answer 300

anything that leads to asphyxia (deprivation of oxygen) to the brain. eg: - maternal shock -intrapartum haemorrhage -prolapsed cord - causing compression of the cord during birth - nuchal cord - where the cord is wrapped around the neck of the baby

Question 301

grading HIE mild moderate severe

Answer 301

Sarnat Staging mild: - poor feeding, generally irritability and hyper-alert -resolves within 24 hours -normal prognosis moderate: - poor feeding, lethargic, hypotonic, seizures -can take weeks to resolve -up to 40% develop cerebral palsy severe: - reduced gcs, apnoeas, flaccid and redcued or absent reflexes -upto 50% mortality -upto 90% develop cerebral palsy

Question 302

when to suspect HIE

Answer 302

neonate acidosis (ph under 7) on umbilical abg poor Apgar score features of mild,mod,severe HIE evidence of multi organ failure.

Question 303

how would you manage HIE

Answer 303

supportive care with neonatal resus optimal ventilation, circulatory support, nutrition, acid base balance and seizure tx. therapeutic hypothermia - protect brain from hypoxic injury.

Question 304

explain therapeutic hypothermia? how to do it ideal temp intention of it. reduces the risk of ?

Answer 304

baby near/at term that have HIE. cool core temp of baby. transfer baby to neonatal ICU. - cooling blankets and cooling hat. target : 33 and 34 - measure using rectal probe. continue for 72 hours then warm baby to normal temp over 6 hours. reduce inflammation and neurone loss after acute hypoxic injury. reduces risk of cerebral palsy, developmental delay, LD, blindness and death

Question 305

What is Bell's Palsy?

Answer 305

acute unilateral idiopathic facial nerve paralysis. unknown aetiology herpes simplex virus poss? peak incidence: 20-40 yrs. condition mc in pregnant women.

Question 306

features of bell's palsy

Answer 306

lmn facial nerve palsy - forehead affected - in contrast, umn lesions spares the upper face post-auricular pain (may precede paralysis) altered taste dry eyes hyperacusis

Question 307

how would you manage bells palsy?

Answer 307

no tx prednisolone only antivirals + prednisolone all pts: oral pred (50mg for 10 days or 60 mg for 5 days followed by 5 day reducing regime of 10mg a day ) within 72 hrs of onset. debate about adding antiviral: alone not recommendation. - give for severe facial palsy eye care important: prevent exposure keratopathy. - artificial tears and eye lubricant. - if cant close eye at bedtime tape it with microporous tape

Question 308

follow -up for bells palsy

Answer 308

if paralysis shows no improvement after 3 weeks, refer to ent plastic surgery: long standing weakness: several months

Question 309

prognosis of bells palsy

Answer 309

full recovery 3-4 months. if untx 15% - permanent moderate to severe weakness

Question 310

What is epilepsy? types

Answer 310

seizure tendency. transient episodes of abnormal electrical activity in brain. generalised tonic-clonic focal seizures absence atonic myoclonic infantile spasms febrile convulsions

Question 311

What are febrile convulsions? age of onset mc type of seizure here clinical features

Answer 311

seizures provoked by fever in otherwise normal kids. 6 months and 5 years. tonic-clonic usually occur early in viral infection as temp rises rapidly seizures brief: last less than 5 mins

Question 312

types of febrile convulsion simple complex febrile status epilepticus

Answer 312

simple: - less than 15 mins -generalised seizure -typically no recurrence within 24 hours -should be complete recovery within an hr complex: - 15-30 mins -focal seizure -may have repeat seizures within 24 hours febrile status epilepticus : - over 30 mins

Question 313

Mx of Febrile Convulsion following a seizure ongoing

Answer 313

admit if first or features of complex seizure phone ambulance if seizure over 5 mins. benzodiazepine rescue med: rectal diazepam or bvuccal midazolam regular antipyretics: dont reduce chance of them occuring

Question 314

prognosis of febrile convulsions

Answer 314

risk of another one is 1 in 3. depends on: age of onset under 18months fever under 39 shorter duration of fever before seizure fhx of them

Question 315

rf for developing epilepsy post febrile convulsions

Answer 315

fhx of epilepsy complex febrile seizures. background of neurodevelopmental disorder if no rf@ 2.5% risk of developing it. if kids have all 3 features 50% risk

Question 316

what is benign rolandic epilepsy? how to ix and what does it show prognosis features

Answer 316

form of childhood epilepsy occurs between 4 and 12. EEG - centrotemporal spikes excellent prognosis - seizures stop by adolescence seizures at night. partial seizures (paresthesia affect face) but secondary generalisation may occur (parents may only report tonic-clonic movements)

Question 317

Using Contraception in epilepsy what factors are to be considered?

Answer 317

effect of contraceptive on effectiveness of anti-epileptic effect of anti-epileptic on effectiveness of contraceptive potential teratogenic effects of anti-epileptic if women becomes pregnant

Question 318

if women is taking phenytoin carbamazepine barbiturates primidone topiramate oxcarbazepine what to do? for contraception in epileptic pt

Answer 318

ukmec 3 : the COCP and POP ukmec 2 : implant ukmec 1 : depo-provera, IUD,IUS use condom if COCP : min of 30 ug of ethinylestradiol

Question 319

for lamotrigine if women takes what to do? contraceptionin epileptic pt

Answer 319

use condom ukmec 3 : cocp ukmec 1: pop, implant, depo-provera, Iud, ius if COCP: min or 30 ug of ethinylestradiol

Question 320

what conditions are associated with epilepsy?

Answer 320

cerebral palsy: 30% have it tuberous sclerosis mitochondrial diseases

Question 321

what is an alcohol withdrawal seizure? mechanism tx

Answer 321

pt hx of alcohol excess suddenly stopped drinking. 36 hours after drink cessation. give benzodiazepine after stopping. chronic alcohol enhances gaba mediated inhibition in cns and inhibits nmda-type glutamate receptors. alcohol withdrawal dose the opposite

Question 322

what are psychogenic non-epileptic seizures?

Answer 322

pseudoseizures epileptic-like seizures but no electrical discharges. hx of mental health or personality disorder

Question 323

define a focal seizure

Answer 323

partial seizure.- start in temporal lobe. start in a specific area , one 1 side of brain. varied awareness. 30 yr old women told by her husband she wakes at night, grunting sound, lip smacking, non responsive during ep. after minute falls asleep again. focal impaired awareness - explained above. classified into motor: jacksonian march non-motor : deja vu, jamais vu or other features like aura sx: affect hearing speech memory and emotions. - hallucinations -memory flashbacks -deja vu - doing strange things on autopilot

Question 324

generalised seizure define

Answer 324

involve networks on both sides of the brain at onset. lose conciousness immediately. motor: tonic clonic non motor: absence specific types: -tonic clonic - grand mal - tonic -clonic -typical absence - petit mal - myoclonic: brief,rapid muscle jerks - atonic

Question 325

unknown onset seizure - what is it?

Answer 325

unknown origin of seizure

Question 326

focal to bilateral seizure - what is it

Answer 326

starts on 1 side of brain in specific area before spreading to both lobes previously called secondary generalised seizure

Question 327

what is infantile spasms - wests syndrome features eeg: secondary to what? prognosis

Answer 327

brief spasms in first few months of life. 1. flexion of head, trunk, limbs - extension of arms (salaam attack) : 1-2 secs, repeat upto 50 times 2. progressive mental handicap 3. eeg: hypsarrhythmia 4. 2nd to serious neuro abnormality like: TS,encephalitis, birth asphyxia) or cryptogenic poor prognosis

Question 328

what is lennox-gastaut syndrome? onset eeg tx

Answer 328

1-5 years poss extension of infantile spasms - 50% have hx eeg: slow spike ketogenic diet poss 90% moderate-severe mental handicap atypical absences, falls, jerks

Question 329

what is juvenile myoclonic epilepsy (janz syndrome) features tx

Answer 329

typical onset: teens, more in girls 1. infrequent generalised seizures 2. often in morning 3. daytime absences 4. sudden shock like myoclonic seizure responds well to sodium valproate

Question 330

tell me about typical absence seizures: petit mal onset duration eeg tx prognosis

Answer 330

onset: 4-8 years duration : few-30 seconds. no warning, quick recovery: often many per day eeg: 3Hz, generalised, symmetrical sodium valproate, ethosuximide good prognosis: 90-95% become seizure free in adolescence

Question 331

if a focal seizure happens starts in occipital lobe (visual) what happens?

Answer 331

floaters/flashes

Question 332

if a focal seizure happens in parietal lobe what happens ? (sensory)

Answer 332

paresthesia

Question 333

if a focal seizure happens in frontal lobe? (motor)

Answer 333

head/leg movements, posturing post-ictal weakness (todd's paresis), jacksonian march (clonic movements travelling proximally)

Question 334

if a focal seizure starts in temporal lobe what can i expect?

Answer 334

last around 1 min - automatisms (lip smacking/grabbing/plucking) common with/without impairement of conciousness or awareness aura in most: - rising epigastric sensation -psychic or experiental phenomena like deja vu , jamais vu -less common: hallucination (auditory,gustatory,olfactory)

Question 335

sodium valproate is associated with what issue for maternal kids?

Answer 335

neural tube defects risk of neurodevelopmental delay in kids after maternal use of it. DONT USE IN PREGNANCY AND WOMEN OF CHILDBEARING AGE UNLESS NECESSARY.

Question 336

which of the epileptic drugs is seen as least teratogenic or older antiepileptics?

Answer 336

carbamazepine

Question 337

phenytoin is associated with what issue? antiepileptic what to give with it. this is to prevent what?

Answer 337

cleft palate vit k last month of pregnancy prevent clotting disorders in newborn

Question 338

is breast feeding safe for mothers taking anti-epileptics? is there an exception to the rule?

Answer 338

yes NO BARBITURATES

Question 339

women want to get pregnant epileptic what should they take and why?

Answer 339

folic acid 5mg per day well before pregnancy minimise risk of neural tube defects

Question 340

briefly explain what an absence seizure is? tx

Answer 340

in kids. pt becomes blank stares into space and abruptly returns to normal. unaware of surroundings, wont respond. 10-20 seconds. stop when they get older 1st line: ethosuximide 2nd line: m - sodium valproate f: lamotrigine or levetiracetam carbamazepine - exacerbate absence seizures

Question 341

how would you tx focal seizures ?

Answer 341

1st line : lamotrigine or levetiracetam 2nd line: carbamazepine, oxcarbazepine or zonisamide

Question 342

the rules are you start antiepileptics after second seizure. in what case would you start after first?

Answer 342

pt has neuro deficit brain imaging shows structural abnormality EEG shows unequivocal epileptic activity. pt or their fam or carer consider the risk of having a further seizure unacceptable

Question 343

generalised tonic clonic seizure tx?

Answer 343

male: sodium valproate female: lamotrigine or levetiracetam under 10, unlikely to need tx when old enough to have kids or women unable to have children can be given sodium valproate first line.

Question 344

how to atonic seizures present? tx?

Answer 344

drop attacks. brief lapse in muscle tone. no more than 3 minutes. childhood begin. indicative of lennox-gastaut syndrome. mx: - m: sodium valproate - f: lamotrigine

Question 345

how do myoclonic seizures present? tx?

Answer 345

sudden brief muscle contractions - sudden jump. awake during. happen in kids as part of juvenile myoclonic epilepsy. tx: - m - sodium valproate - f : levetiracetam

Question 346

rule for patient who drives on anti-epileptic rule for patient taking other meds women wishing to get pregnant

Answer 346

cant drive for 6 months after seizure. if established epilepsy must be fit free for 12 months. antiepileptics induce/inhibit p450 system - varied metabolism of meds like warfarin. teratogenic esp sodium valproate. breastfeeding safe except barbiturates.

Question 347

sodium valproate MoA uses Adverse effects

Answer 347

increases GABA activity used for generalised seizures in males increased apetite weight gain alopecia: regrowth may be curly p450 enzyme inhibitor ataxia tremor hepatitis pancreatitis thrombocytopenia teratogenic - neural tube defects - dont use in women of reproductive age

Question 348

carbamazepine moa uses adverse effects

Answer 348

binds to sodium channels increasing their refractory period 2nd line for focal seizures p450 enzyme inducer dizziness and ataxia drowsiness leucopenia agranulocytosis SIADH visual disturbances (especially diplopia) aplastic anaemia

Question 349

lamotrigine moa uses adverse effects

Answer 349

sodium channel blocker used for variety of generalised and focal seizures stevens johnson syndrome or DRESS SYNDROME. - life threatening skin rashes. leukopenia

Question 350

phenytoin moa uses adverse effects

Answer 350

binds to sodium channels increasing their refractory period no longer first line due to side effect profile p450 enzyme inducer dizziness and ataxia drowsiness gingival hyperplasia, hirsutism, coarsening of facial features megaloblastic anaemia (folate and vit d def) peripheral neuropathy enhanced vit d metabolism causing osteomalacia (vit d def) lymphadenopathy

Question 351

rescue medication for epilepsy if they dont terminate spontaneously ie after 5-10 mins

Answer 351

benzodiazepines like diazepam administer rectally or intranasally/under tongue. if still fits: status epilepticus. - medical emergency - hospital tx required. mx: further benzodiazepine med, infusion of antiepileptic or general anesthetic.

Question 352

ix for epilepsy

Answer 352

good history eeg: typical patterns of it. after the second simple tonic clonic do. MRI brain: structure of brain. ecg - exclude heart problems blood electrolytes - sodium, potassium, calcium , magnesium bg: hypoglycaemia and diabetes blood cultures, urine cultures and lp where sepsis, encephalitis or meningitis is suspected

Question 353

general advice for epileptic patients

Answer 353

take showers not baths cautious with heights, traffic, heavy/hot/electric equipment be very catious with swimming unless seizures well controlled and they are closely supervised.

Question 354

2 side effects of ethosuximide

Answer 354

night terrors rashes

Question 355

mx of seizures physically

Answer 355

pt in safe position - carpeted floor recovery position if poss something soft under head remove obstacles that could lead to injury note the time at start and end call ambulance if more than 5 mins or 1st seizure.

Question 356

what is status epilepticus? mx: medical options in community ?

Answer 356

med emergency seizure lasting more than 5 mins or 2 or more seizures without regaining consciousness in interim. ABCDE approach secure airway high conc ox assess cardiac and resp function check bg levels iv access - insert cannula iv lorazepam - repeated after 10 mins if the seizure continues. if persist final step is iv phenobarbital or phenytoin. intubation and ventilation to secure airway- transfer to icu. med options in community: - buccal midazolam - rectal diazepam

Question 357

What is an extradural haematoma? caused by? collection of blood most likely where? what artery?

Answer 357

collection of blood between skull and dura. trauma - low impact. - blow to head or fall. in temporal region since the thin skull at pterion overlies the middle meningeal artery - vulnerable to injury

Question 358

classic presentation of extradural haematoma? and why

Answer 358

pt initially loses, briefly regains and then again loses consciousness after low-impact head injury. brief regain in conciousness termed lucid interval. lost due to expanding haematoma and brain herniation. as haematoma expands the uncus of temporal lobe herniates around tentorium cerebelli and pt gets: fixed and dilated pupil due to compression of parasympathetic fibres of 3rd cranial nerve

Question 359

imaging of extradural haematoma

Answer 359

biconvex - lentiform hyperdense collection around surface of brain. limited by the suture lines of the skull.

Question 360

how would you manage extradural haematoma?

Answer 360

if no neurological deficit, cautious clinical and radiological observation is appropriate. definitive: craniotomy and evacuation of the haematoma.

Question 361

what is a subdural haemorrhage?

Answer 361

collection of blood deep to the dural layer of meninges. blood not within substance of brain - extra-axial or extrinsic lesion. can be unilateral or bilateral

Question 362

classifying subdural haemorrhage by age

Answer 362

acute: sx develop within 48 hrs of injury, characterised by rapid neurological deterioration subacute: sx manifest within days to weeks post-injury, with more gradual progression chronic: common in elderly, develop over weeks to months. pts may not recall specific head injury

Question 363

typical presentation of subdural haematoma

Answer 363

hx of head trauma - minor to severe. pt exhibit lucid interval followed by gradual decline in consciousness. this pattern common in chronic sdh. headache confusion lethargy

Question 364

clinical features of subdural haematoma neurological sx other associated features

Answer 364

altered mental status: mild confusion to deep coma. fluctuations in level of conciousness common. focal neuro deficits: weakness on 1 side of body, aphasia, visual field defects, depending on haematoma location headache: often localised to 1 side, worsening over time seizures: may occur, particularly in acute/expanding haematoma.

Question 365

behavioural and cognitive changes in subdural haemorrhage

Answer 365

memory loss: especially in chronic sdh personality change: irritability, apathy, depression cognitive impairement: difficulty with attention, problem-solving and other executive functions

Question 366

physical examination findings in subdural haemorrhage

Answer 366

papilloedema: indicates raised icp pupil changes: unilateral dilated pupil, especialy on side of the haematoma, indicated compression of 3rd cranial nerve. gait abnormalities: including ataxia or weakness in 1 leg hemiparesis or hemiplegia: reflecting the mass effect and midline shift

Question 367

talk to me about acute subdural haematoma ct imaging tx

Answer 367

collection of fresh blood within subdural space caused by high impact trauma. - often other brain underlying injuries. sx depends on size of the compressive acute sdh. could be from indental finding to severe coma and coning due to herniation. ct: 1st line - crescentic collection not limited by suture lines. hyperdense (bright) in comparison to brain. large acute subdural haematomas: push on brain (mass effect) cause midline shift or herniation. tx: - small/incidental: observe surgery: monitor icp and decompressive craniectomy

Question 368

tell me about chronic subdural haematoma who can have it and why? presentation imaging tx

Answer 368

collection of blood within subdural space present for weeks to months. rupture of small bridging veins within subdural sapce rupture, slow bleeding. elderly and alcoholic - they have brain atrophy so fragile/taut bridging veins px: - several week to month progressive hx of either confusion, reduced gcs or neuro deficit. infants have fragile bridging veins to and can rupture in shaken baby syndrome. ct: - crescentic in shape, not restricted by suture lines and compress the brain (mass effect) - hypodense (dark) compared to substance of brain. - small in size and no neuro deficit: conservatively manage - if pt confused and associated neuro def or severe imaging findings: - surgical decompression with burr holes.

Question 369

What is a subarachnoid haemorrhage mc cause

Answer 369

intracranial haemorrhage , blood in subarachnoid space - deep to the subarachnoid layer of meninges. head injury. - traumatic sah. if not trauma its called sponteneous sah

Question 370

causes of spontaneous sah

Answer 370

intracranial aneurysm - saccular berry aneursym - 85% cases - conditions associated with this are - htn -adults pkd - ehlers-danlos -coarctation of aorta - arteriovenous malformation -pituitary apoplexy -mycotic (infective) aneurysms

Question 371

classic presenting features of subarachnoid haemorrhage

Answer 371

headache - sudden onset thunderclap (hit with baseball bat) - severe worst of life occipital - peak in intensity within 1-5 mins - poss hx of a less severe sentinel headache weeks prior to presentation n+ v meningism - photophobia, neck stiffness coma seizures ecg - st elevation - secondary to either autonomic neural stimulation from hypothalamus or elevated levels of circulating catecholamines

Question 372

how would you ix for subarachnoid haemorrhage

Answer 372

non-contrast ct head: 1st line - acute blood (hyperdense/bright on ct) - distributed in basal cisterns, sulci and if severe ventricular system if ct head done within 6 hours of sx onset and is normal : dont do LP consider alternative diagnosis. if ct head done more than6 hrs after sx onset and normal do a LP: should be done at least 12 hrs after sx to allow development of xanthochormia (rbc breakdown) xanthochromia help distinguish true sah from traumatic tap (blood introduced by lp procedure) csf findings: normal or raised opening pressure if ct shows evidence of sah: refer to neurosurgery

Question 373

once i have confirmed a spontaneous sah what other ix doi need to do and why?

Answer 373

to identify causative pathology ct intracranial angiogram - identify vascular lesion eg aneurysm or AVM +/- digital subtraction agiogram - catheter angiogram

Question 374

important predictive factors for SAH

Answer 374

conscious level on admission age amount of blood visible on CT head

Question 375

how would you manage sah?

Answer 375

supportive: - bed rest - analgesia -vte prophylaxis - discontinuation of antithrombotics (reversal of anticoagulation if present) - vasospasm is prevented using : oral nimodipine - intracranial aneurysms at risk of rebleeding so require intervention within 24 hrs - coil by interventional neuroradiologists, minority need craniotomy and clipping

Question 376

complications of aneurysmal sah

Answer 376

re-bleeding: 10% cases within 1st 12 hrs. if suspected - sudden worsening of neuro sx - repeat ct - high mortality 70% hydrocephalus - tx temp with external ventricular drain (csf divert to bag at bedside) or long term ventriculoperitoneal shunt vasospasm - delayed cerebral ischaemia - 7-14 days after osnet - ensure euvolemia - normal blood vol - consider tx with vasopressor if sx persist hyponatremia - due to siadh seizures

Question 377

general risk factors for all intracranial bleeds

Answer 377

head injuries htn aneurysms ischaemic strokes brain tumour thrombocytopenia bleeding disorder s- haemophilia anticoag - doac or warfarin

Question 378

what is the glasgow coma scale? total score min score score that is diagnostic and what to do if so and why?

Answer 378

tool for consciousness based on eyes verbal response and motor response. 15/15 max min 3/15 - 8/15 - needs airway support , risk of airway obstruction or aspiration = hypoxia and brain injury 6 - obeys commands 5 - oriented, localises pain 4 - spontaneous eyes, confused verbal response, normal flexion 3 - speech, inappropriate words, abnromal flexion 2 - pain, incomprehensible sounds, extends 1 - none none none

Question 379

What is cauda equina? presentation main late diagnosis lead to

Answer 379

rare where lumbosacral nerve roots that extend below the spinal cord are compressed. any pt presenting with new/worsening lower back pain. late diagnosis lead to permanent nerve damage results in long term leg weakness and urinary/bowel incontinence.

Question 380

causes of cauda equina

Answer 380

mc cause: central disc prolapse - occurs at l4/l5 or l5/s1 tumours: primary or mets infection: abscess, discitis trauma haematoma

Question 381

presentation of cauda equina

Answer 381

low back pain bilateral sciatica - 50% pts reduced sensation/pins and needles in perianal area decreased anal tone: - good practice to check anal tone in pts with new-onset back pain. - poor sensitivity and specificity urinary dysfunction: - incontinence, reduced awareness of bladder filling, loss of urge to void - incontinence is a late sign that may indicate irreversible damage

Question 382

ix for cauda equina mx of cauda equina

Answer 382

urgent MRI surgent decompression