Neurology 2 Flashcards

1
Q

Parkinson’s disease is a progressive neurodegenerative condition caused bydegeneration of dopaminergic neurons in ……….

A

the substantia nigra

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2
Q

The symptoms of Parkinson’s disease are characteristically ………..

A

asymmetrical

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3
Q

The most common psychiatric problem is ……. in Parkinson’s disease

A

Depression

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4
Q

Drug-induced parkinsonism has slightly different features to Parkinson’s disease:

A

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

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5
Q

Causes of Parkinsonism

A

Parkinson’s disease

drug-induced e.g.antipsychotics,metoclopramide*

progressive supranuclear palsy

multiple system atrophy

Wilson’s disease

post-encephalitis

dementia pugilistica (secondary to chronic head trauma e.g. boxing)

toxins: carbon monoxide, MPTP

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6
Q

Domperidone does not cause extra-pyramidal side-effects because it …..

A

does not cross the blood-brain barrier

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7
Q

Essential tremor improved by ….

A

alcohol and rest

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8
Q

What is most common cause of titubation (head tremor)?

A

Essential tremor

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9
Q

Management of Essential tremor

A

propranolol is first-line

primidone is sometimes used

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10
Q

(5) Causes and associations of Restless legs syndrome

A
  1. Idiopathic ( +ve FH)
  2. IDA
  3. Uremia
  4. DM
  5. Pregnancy
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11
Q

Management of Restless legs syndrome

A

simple measures: walking, stretching, massaging affected limbs

treat any iron deficiency

dopamine agonists are first-line treatment (e.g. Pramipexole,ropinirole)

benzodiazepines

gabapentin

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12
Q

Chorea is caused by damage to …….

A

the basal ganglia, especially the caudate nucleus

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13
Q

Causes of chorea

A

Huntington’s disease,Wilson’s disease, ataxic telangiectasia

SLE, anti-phospholipid syndrome

rheumatic fever: Sydenham’s chorea

drugs: oral contraceptive pill, L-dopa, antipsychotics

neuroacanthocytosis

pregnancy: chorea gravidarum

thyrotoxicosis

polycythaemia rubra vera

carbon monoxide poisoning

cerebrovascular disease

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14
Q

Huntington’s disease is

A

Autosomal dominant

trinucleotide repeat disorder: repeat expansion of CAG

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15
Q

Huntington’s disease results in degeneration of cholinergic and GABAergic neurons in ……1….

  1. due to defect in huntingtin gene on chromosome ……
A
  1. the striatum of the basal ganglia
  2. chromosome 4
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16
Q

Features of Huntington’s disease

A

Chorea

Dystonia

saccadic eye movements

personality changes (e.g. irritability, apathy, depression) and intellectual impairment

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17
Q

Transient global amnesia is characterized by the acute onset of ……… amnesia

episodes are self-limited and resolve within ……..

A
  1. anterograde amnesia
  2. 24 hours
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18
Q

Avoid drug holiday of parkinson’s medications due to the high risk of ….

A

Acute akinesia or neuroleptic malignant syndrome

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19
Q

Impulse control disorders in parkinson’s
Common with

A

dopamine agonist therapy

a history of previous impulsive behaviours

a history of alcohol consumption and/or smoking

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20
Q

If orthostatic hypotension develops in parkinson’s

A

medication review looking at potential causes should be done.

If symptoms persist then midodrine (acts on peripheral alpha-adrenergic receptors to increase arterial resistance) can be considered.

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21
Q

excessive daytime sleepiness develops in parkinson’s

A

patients should not drive.

Medication should be adjusted to control symptoms.

Modafinil can be considered if alternative strategies fail.

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22
Q

Consider …………………. to manage drooling of saliva in people with Parkinson’s disease.

A

glycopyrronium bromide

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23
Q

prion diseases

A

CJD
kuru

fatal familial insomnia

Gerstmann Straussler-Scheinker disease

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24
Q

Features of Creutzfeldt-Jakob disease

A

Rapid onset of dementia

Myoclonus

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25
Q

Ataxia telangiectasia is an autosomal …… disorder caused by a defect in the ………. gene which encodes for……….

A

autosomal recessive disorder

caused by a defect in the ATM gene

encodes forDNA repair enzymes.

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26
Q

Ataxia telangiectasiais …….. immunodeficiency disorders.

A

combined ( B & T cell) immunodeficiency disorders.

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27
Q

cerebellar ataxia

telangiectasia (spider angiomas)

IgA deficiency resulting in recurrent chest infections

10% risk of developing malignancy, lymphoma or leukaemia, but also non-lymphoid tumours

Features of

A

Ataxia telangiectasia

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28
Q

What are the most common presenting features of Friedreich’s ataxia?

A

Gait ataxia and kyphoscoliosis

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29
Q

Features of Friedreich’s ataxia

A

HOCM
DM
high-arched palate
cerebellar ataxia
optic atrophy
spinocerebellar tract degeneration
absent ankle jerks/extensor plantars

30
Q

Friedreich’s ataxia is an autosomal ……, ……. disorder characterised by aGAA repeat in the …… gene on chromosome …….

A

is an autosomal recessive, trinucleotide repeat disorder characterised by aGAA repeat in the X25 gene on chromosome 9 (frataxin).

31
Q

NF1 is caused by a gene mutation onchromosome …….which encodes ……

A

Chromosome 17

neurofibromin

32
Q

NF2 is caused by gene mutation onchromosome …..

A

onchromosome 22

33
Q

neurofibromatosis, NF1 and NF2. Both are inherited in anautosomal ……..fashion

A

dominant

34
Q

Features of NF2

A

Bilateral vestibular schwannomas

Multiple intracranial schwannomas,

mengiomas

ependymomas

35
Q

Features of NF1

A

Cafe-au-lait spots(>= 6, 15 mm in diameter)

Axillary/groin freckles

Peripheral neurofibromas

Iris hamatomas (Lisch nodules)in > 90%

Scoliosis

Pheochromocytomas

36
Q

Causes of 3rd nerve palsy

A

DM
SLE
Temporal arteritis
MS
Amyloid
Cavernous sinus thrombosis
Weber’s syndrome
Posterior communicating artery aneurysm ( dilated pupils & pain )

Raised ICP due to herniation

37
Q

If CT head is done within 6 hrs of symptoms onset and normal
Next step

If suspected SAH

A

Don’t do LP
Consider alternative diagnosis

38
Q

If CT head is done > 6 hrs of symptoms onset and normal
Next step
If suspected SAH

A

Do LP

39
Q

Bell’s palsy is more common in

A

pregnant women.

40
Q

treatment of Bell’s palsy

A
  1. eye protection
  2. steroids for 7 days
  3. add antivirals for severe facial palsy

if the paralysis shows no sign of improvement after 3 weeks, refer urgently to ENT
a referral to plastic surgery may be appropriate for patients with more long-standing weakness e.g. several month

41
Q

causes of Autonomic neuropathy

A

DM
GBS
multisystem atrophy (MSA), Shy-Drager syndrome
Parkinson’s
infections: HIV, Chagas’ disease, neurosyphilis
drugs: antihypertensives, tricyclics
craniopharyngioma

42
Q

Absent ankle jerks, extensor plantars

A

subacute combined degeneration of the cord
motor neuron disease
Friedreich’s ataxia
syringomyelia
taboparesis (syphilis)
conus medullaris lesion

43
Q

causes of Brain abscesses

A

extension of sepsis from middle ear or sinuses,
trauma or surgery to the scalp, penetrating head injuries and embolic events from endocarditis

44
Q

managment of Brain abscess

A
  1. craniotomy is performed and the abscess cavity debrided
  2. IV antibiotics: IV 3rd-generation cephalosporin + metronidazole
  3. intracranial pressure management: e.g. dexamethasone
45
Q

features of Brown-Sequard syndrome

A
  1. ipsilateral weakness below lesion
  2. ipsilateral loss of proprioception and vibration sensation
  3. contralateral loss of pain and temperature sensation
46
Q

Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL)
patients often present with

A

multi-infarct dementia
migraine

47
Q

Cataplexy describes the sudden and transient loss of ……..

Around two-thirds of patients with ……….. have cataplexy.

A

muscular tone

narcolepsy

48
Q

which drugs can cause cerebellar syndrome ?

A

phenytoin, lead poisoning

49
Q

Subacute combined degeneration of the spinal cord isdue to vitamin B12 deficiency resulting in impairment of

A

the dorsal columns, lateral corticospinal tracts and spinocerebellar tracts.

50
Q

Features of Subacute combined degeneration

A
  1. dorsal column involvement
    - distal tingling/burning/sensory loss is symmetrical and tends to affect the legs more than the arms
  • impaired proprioception and vibration sense
  1. lateral corticospinal tract involvement
    - Weakness, hyperreflexia, spasticity
    - extensor planters, absent ankle jerks, brisk knee reflexes
  2. spinocerebellar tract involvement
    - sensory ataxia
51
Q

Post-lumbar puncture headache, if pain continues for more than 72 hours then specific treatment is indicated, to prevent ……….

A

to prevent subdural haematoma

treatment options include:
blood patch,
epidural saline
intravenous caffeine

52
Q

causes of Peripheral neuropathy, Predominately sensory loss

A

1.DM
2. uraemia
3. vitamin B12 deficiency
4. alcoholism
5. amyloidosis
6. leprosy

53
Q

causes of Peripheral neuropathy, Predominately motor loss

A
  1. GBS
  2. chronic inflammatory demyelinating polyneuropathy (CIDP)
  3. Charcot-Marie-Tooth
  4. porphyria
  5. diphtheria
  6. lead poisoning
54
Q

causes of Peripheral neuropathy, Demyelinating pathology

A
  1. GBS
  2. chronic inflammatory demyelinating polyneuropathy (CIDP)
  3. amiodarone
  4. hereditary sensorimotor neuropathies (HSMN) type I
  5. paraprotein neuropathy
55
Q

causes of Peripheral neuropathy, Axonal pathology

A
  1. alcohol
  2. DM*
  3. vitamin B12 deficiency*
  4. vasculitis
  5. hereditary sensorimotor neuropathies (HSMN) type II

*may also cause a demyelinating picture

56
Q

Nerve conduction studies
Axonal pathology if
conduction velocity:
amplitude:

A

normal conduction velocity
reduced amplitude

57
Q

Nerve conduction studies
demyelinating pathology if
conduction velocity:
amplitude:

A

reduced conduction velocity
normal amplitude

58
Q
  1. parkinsonism
  2. autonomic disturbance
    erectile dysfunction, postural hypotension, atonic bladder
  3. cerebellar signs

features of

A

multiple system atrophy

59
Q

Causes of miosis

A
  1. Horner’s syndrome
  2. Argyll-Robertson pupil
  3. senile miosis
  4. pontine haemorrhage
  5. congenital
  6. drugs
60
Q

Drugs causes of miosis (3)

A

opiates
parasympathomimetics: pilocarpine
organophosphate toxicity

61
Q

There may be a history of frequently sprained ankles
Foot drop
High-arched feet (pes cavus)
Hammer toes
Distal muscle weakness
Distal muscle atrophy
Hyporeflexia
Stork leg deformity

features of

A

Charcot-Marie-Tooth Disease

62
Q

management of Charcot-Marie-Tooth Disease

A

There is no cure, and management is focused on physical and occupational therapy.

63
Q

If a patient is on warfarin who have sustained a head injury with no other indications for a CT head scan, perform a CT head scan within ……. hours of the injury.

A

8 hours

64
Q

Causes of bilateral facial nerve palsy

A
  1. sarcoidosis
  2. GBS
  3. Lyme disease
  4. bilateral acoustic neuromas (as in neurofibromatosis type 2)
65
Q

causes of Foot drop

A
  1. peroneal nerve lesion - the most common cause
  2. L5 radiculopathy
  3. sciatic nerve lesion
  4. superficial or deep peroneal nerve lesion
  5. central nerve lesions (e.g. stroke)
66
Q

Lateral medullary syndrome, also known as Wallenberg’s syndrome, occurs following occlusion of ……

A

posterior inferior cerebellar artery (PICA)

67
Q

Erb-Duchenne paralysis
damage to

A

C5,6 roots

68
Q

Klumpke’s paralysis
damage to

A

T1

69
Q

Cutaneous features of Tuberous sclerosis

A
  1. depigmented ‘ash-leaf’ spots
  2. cafe-au-lait spots
  3. roughened patches of skin over lumbar spine (Shagreen patches)
  4. adenoma sebaceum (angiofibromas): butterfly distribution over nose
  5. fibromata beneath nails (subungual fibromata)
70
Q

Breastfeeding is considered safe for mothers taking anti epileptic medication
Except…….

A

Barbiturates

71
Q

Breastfeeding is considered safe for mothers taking anti epileptic medication
Except…….

A

Barbiturates