Neurology 2 Flashcards

(71 cards)

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
Ataxia telangiectasia is an autosomal ...... disorder caused by a defect in the .......... gene which encodes for ..........
autosomal recessive disorder caused by a defect in the ATM gene encodes for DNA repair enzymes.
26
Ataxia telangiectasia is ........ immunodeficiency disorders.
combined ( B & T cell) immunodeficiency disorders.
27
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
Ataxia telangiectasia
28
What are the most common presenting features of Friedreich's ataxia?
Gait ataxia and kyphoscoliosis
29
Features of Friedreich's ataxia 
HOCM DM high-arched palate cerebellar ataxia optic atrophy spinocerebellar tract degeneration absent ankle jerks/extensor plantars
30
Friedreich's ataxia is an autosomal ......, ....... disorder characterised by a GAA repeat in the ...... gene on chromosome .......
is an autosomal recessive, trinucleotide repeat disorder characterised by a GAA repeat in the X25 gene on chromosome 9 (frataxin). 
31
NF1 is caused by a gene mutation on chromosome ....... which encodes ......
Chromosome 17 neurofibromin
32
NF2 is caused by gene mutation on chromosome .....
on chromosome 22
33
neurofibromatosis, NF1 and NF2. Both are inherited in an autosomal ........ fashion
dominant
34
Features of NF2
Bilateral vestibular schwannomas Multiple intracranial schwannomas, mengiomas ependymomas
35
Features of NF1
Cafe-au-lait spots (>= 6, 15 mm in diameter) Axillary/groin freckles Peripheral neurofibromas Iris hamatomas (Lisch nodules) in > 90% Scoliosis Pheochromocytomas
36
Causes of 3rd nerve palsy
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
If CT head is done within 6 hrs of symptoms onset and normal Next step If suspected SAH
Don’t do LP Consider alternative diagnosis
38
If CT head is done > 6 hrs of symptoms onset and normal Next step If suspected SAH
Do LP
39
Bell's palsy is more common in
pregnant women.
40
treatment of Bell's palsy
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
causes of Autonomic neuropathy
DM GBS multisystem atrophy (MSA), Shy-Drager syndrome Parkinson's infections: HIV, Chagas' disease, neurosyphilis drugs: antihypertensives, tricyclics craniopharyngioma
42
Absent ankle jerks, extensor plantars
subacute combined degeneration of the cord motor neuron disease Friedreich's ataxia syringomyelia taboparesis (syphilis) conus medullaris lesion
43
causes of Brain abscesses
extension of sepsis from middle ear or sinuses, trauma or surgery to the scalp, penetrating head injuries and embolic events from endocarditis
44
managment of Brain abscess
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
features of Brown-Sequard syndrome
1. ipsilateral weakness below lesion 2. ipsilateral loss of proprioception and vibration sensation 3. contralateral loss of pain and temperature sensation
46
Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) patients often present with
multi-infarct dementia migraine
47
Cataplexy describes the sudden and transient loss of ........ Around two-thirds of patients with ........... have cataplexy.
muscular tone narcolepsy
48
which drugs can cause cerebellar syndrome ?
phenytoin, lead poisoning
49
Subacute combined degeneration of the spinal cord is due to vitamin B12 deficiency  resulting in impairment of
the  dorsal columns, lateral corticospinal tracts and spinocerebellar tracts.
50
Features of Subacute combined degeneration
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 2. lateral corticospinal tract involvement - Weakness, hyperreflexia, spasticity - extensor planters, absent ankle jerks, brisk knee reflexes 3. spinocerebellar tract involvement - sensory ataxia
51
Post-lumbar puncture headache, if pain continues for more than 72 hours then specific treatment is indicated, to prevent ..........
to prevent subdural haematoma treatment options include: blood patch, epidural saline intravenous caffeine
52
causes of Peripheral neuropathy, Predominately sensory loss
1.DM 2. uraemia 3. vitamin B12 deficiency 4. alcoholism 5. amyloidosis 6. leprosy
53
causes of Peripheral neuropathy, Predominately motor loss
1. GBS 2. chronic inflammatory demyelinating polyneuropathy (CIDP) 3. Charcot-Marie-Tooth 4. porphyria 5. diphtheria 6. lead poisoning
54
causes of Peripheral neuropathy, Demyelinating pathology
1. GBS 2. chronic inflammatory demyelinating polyneuropathy (CIDP) 3. amiodarone 4. hereditary sensorimotor neuropathies (HSMN) type I 5. paraprotein neuropathy
55
causes of Peripheral neuropathy, Axonal pathology
1. alcohol 2. DM* 3. vitamin B12 deficiency* 4. vasculitis 5. hereditary sensorimotor neuropathies (HSMN) type II *may also cause a demyelinating picture
56
Nerve conduction studies Axonal pathology if conduction velocity: amplitude:
normal conduction velocity reduced amplitude
57
Nerve conduction studies demyelinating pathology if conduction velocity: amplitude:
reduced conduction velocity normal amplitude
58
1. parkinsonism 2. autonomic disturbance erectile dysfunction, postural hypotension, atonic bladder 3. cerebellar signs features of
multiple system atrophy
59
Causes of miosis
1. Horner's syndrome 2. Argyll-Robertson pupil 3. senile miosis 4. pontine haemorrhage 5. congenital 6. drugs
60
Drugs causes of miosis (3)
opiates parasympathomimetics: pilocarpine organophosphate toxicity
61
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
Charcot-Marie-Tooth Disease
62
management of Charcot-Marie-Tooth Disease
There is no cure, and management is focused on physical and occupational therapy.
63
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.
8 hours
64
Causes of bilateral facial nerve palsy
1. sarcoidosis 2. GBS 3. Lyme disease 4. bilateral acoustic neuromas (as in neurofibromatosis type 2)
65
causes of Foot drop
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
Lateral medullary syndrome, also known as Wallenberg's syndrome, occurs following occlusion of ......
posterior inferior cerebellar artery (PICA)
67
Erb-Duchenne paralysis damage to
C5,6 roots
68
Klumpke's paralysis damage to
T1
69
Cutaneous features of Tuberous sclerosis
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
Breastfeeding is considered safe for mothers taking anti epileptic medication Except…….
Barbiturates
71
Breastfeeding is considered safe for mothers taking anti epileptic medication Except…….
Barbiturates