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MRCPCH Part 2 > Neurology > Flashcards

Neurology Flashcards

1
Q

Presentation of dystrophic myotonia

A 
Mild weakness in the first few years
Progressive wasting of distal muscles
Cataracts
Learning difficulties
Low IgG
Hypothyroidism
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2
Q

Diagnostic tests for dystrophic myotonica

A 
Immunoglobulins
DNA studies
Blood glucose
EMG
TFTs
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3
Q

Features of cingulate herniation (subfalcine - foramen magnum)

A

Usually asymptomatic
Diplegia
Hemiplegia
Visual symptoms

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4
Q

Features of central herniation (tentorial)

A 
Sunsetting sign (upward gaze palsy, dilated pupils)
VI nerve palsy
Decerebrate rigidity
Irregular respiration
Hypertension
Seizures
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5
Q

Features of uncal herniation

A 
Unilateral fixed dilated pupils
III nerve palsy
Contralateral hemiplegia
Extensor plantars
Then ipsilateral hemiplegia
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6
Q

Features of tonsillar herniation

A 
Bulbar palsy
Stridor
Hypotension
Hyperthermia
Neck stiffness
Cardiorespiratory arrest
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7
Q

Causes of hypsarrhythmia on EEG

A

Tuberous sclerosis
Idiopathic
Head injury

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8
Q

EEG pattern associated with infantile spasms

A

Hypsarrhythmia

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9
Q

EEG pattern in absence seizures

A

Spike-wave complexes at a rate of 3 per second

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10
Q

EEG pattern in benign rolandic epilepsy

A

High aptitude spikes in the Centro-temporal region

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11
Q

EEG pattern in herpes simplex encephalitis

A

Periodic complexes clustered in the temporal region OR

Fronto temporal slowing with periodic sharp wave complexes

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12
Q

EEG pattern in tuberous sclerosis

A

High amplitude slow waves and multifocal spikes in a chaotic pattern (hypsarrhythmia)

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13
Q

EEG pattern in space occupying lesion

A

High amplitude slow wave activity seen in a localised region

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14
Q

Structures involved in Erbs palsy

A

Deltoid
Extensor carpi radialis longus
Absent biceps jerk

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15
Q

Features of Landau-Kleffner

A

Infrequent seizures
Acquired receptive aphasia
Behavioural disturbance with hyperactivity

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16
Q

Features of NF1

A 
Cafe au lait patches
Axillary/inguinal freckling
Neurofibromas
Optic glioma
≥2 lisch nodules
Fibroid dysplasia of bone
Macrocephaly (not diagnostic)
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17
Q

Features of Fabry’s disease

A

Cataracts
Early MIs/strokes/renal impairment
Capillary haemangiomas
Burning sensation in hands and feet

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18
Q

Antiepileptic which causes permanent visual field loss in up to 30% patients

A

Vigabatrin

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19
Q

Antiepileptic which causes liver failure

A

Sodium valproate

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20
Q

Antiepileptic which causes dizziness, headache, tremor, gum hypertrophy, coarse face, hirsutism

A

Phenytoin

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21
Q

Antiepileptic which causes nausea, agitation, tremor, increased appetite, weight gain, hair loss

A

Sodium valproate

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22
Q

Features of mumps meningoencephalitis

A

Lymphadenopathy and parotid swelling
Meningitic features with confusion 7-10 days later
Aseptic meningitis on LP

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23
Q

What is Riley Day syndrome also known as?

A

Familial dysautonomia

Hereditary sensory autonomic neuropathy type 3

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24
Q

Diagnostic test for Tay-Sachs

A

Hexoseaminidase levels

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25
Q

Symptoms of Arnold-Chiari malformation

A

Nerve palsies
Atypical facial pain
Respiratory depression
Symptoms due to long spinal tracts being compressed

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26
Q

Best medications for juvenile myoclonic epilepsy?

A

Sodium valproate

Levetiracetam

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27
Q

Diagnostic testing for SMA type 1?

A

Survival motor neurone genotype (molecular genetic testing)

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28
Q

Factors increasing risk of seizures on discontinuation of antiepileptics

A

Older age
Use of multiple anticonvulsants
Myoclonic or tonic clonic seizures
Previous abnormal imaging or EEG

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29
Q

Tumours associated with neurofibromatosis type 1?

A 
Astrocytomas
Wilms tumour
Phaeochromocytoma
Neuroblastoma
Meningioma
Medulloblastoma
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30
Q

Features of Landau-Kleffner syndrome

A

Seizures in approx 70%
Receptive or expressive aphasia
Bitemporal abnormalities on EEG

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31
Q

Features of Friedreich’s ataxia

A 
Ataxia
Posterior column spinal cord dysfunction
Loss of deep tendon reflexes
Motor weakness in lower limbs
Dysarthria
Pes cavus
Reduced visual acuity
Cardiac abnormalities
32
Q

Adjunctive treatments for tonic clonic seizures if valproate/lamotrigine ineffective

A 
Clobazam
Lamotrigine
Levetiraetam
Sodium valproate
Topiramate
33
Q

Features of metachromatic leucodystrophy

A 
Lysosomal storage disease
Gross motor skill regression
Abnormal gait
Loss of deep tendon reflexes
Behavioural difficulties
\+/- seizures
34
Q

Antiepileptic causing hyponatraemia in overdose

A

Carbamazepine

35
Q

Antiepileptic causing rectal irritation

A

Paraldehyde

36
Q

Anti epileptic causing rickets

A

Phenytoin

37
Q

Complications of topiramate

A

Renal stones
Sedation
Anorexias
Paraesthesia

38
Q

Features of neuronal ceroid lipofuscinosis

A

Dementia

Visual difficulties

39
Q

First line tests for NCL

A 
MRI
LP
VEP
ERG
Rectal biopsy - characteristic cytoplasmic inclusions
Skin biopsy - curvilinear bodies
40
Q

Features of internal capsule lesion

A

Contralateral hemiplegia
Sensory loss
UMN cranial nerve deficit in the side opposite the lesion

41
Q

Features of temporal lobe lesion

A

Memory problems

Isolated motor/sensory deficit

42
Q

Features of parietal lobe lesions

A

Speech difficulty

Unilateral limb sensory/motor loss

43
Q

Features of pons lesions

A

LMN ipsilateral cranial nerve deficits

Contralateral limb hemiplegia and loss of sensation

44
Q

Features of lesions in the medulla

A

LMN cranial nerve deficits on the same side of the lesion

Motor deficit in the contralateral limb

45
Q

Tests if suspecting Friedreich’s ataxia

A

Brainstem and somatosensory evoked potential
Echo
Genetic testing

46
Q

Congenital infection causing IUGR, hepatosplenomegaly, jaundice and diffuse intracranial calcification

A

Toxoplasmosis

47
Q

Congenital infection causing IUGR, hepatosplenomegaly, jaundice, purpuric rash, periventricular calcification

A

CMV

48
Q

Features of Menke’s disease

A 
Hypotonia, hypothermia
Myoclonic seizures
Chubby, rosy cheeks
Kinky, colourless, friable hair
Severe mental retardation
Optic atrophy
Low serum copper and ceruloplasmin
49
Q

Features of Moebius syndrome

A 
Congenital facial and abducens nerve palsy
Ectropion
Epiphora
Exposure keratopathy
Abduction weakness
Difficulty chewing, swallowing, coughing
Micrognathia
50
Q

First line treatment for absence seizures

A

Ethosuximide

51
Q

First line treatment for myoclonic epilepsy

A

Sodium valproate
Levetiracetam
Topiramate

52
Q

Useful adjunct in cluster epilepsy

A

Clobazam

53
Q

First line treatment for complex partial seizures

A

Carbamazepine

54
Q

Features of benign rolandic epilepsy

A

2-12 years
Focal seizures occurring in clusters and during sleep
Unilateral facial sensorimotor symptoms common

55
Q

Drugs causing peripheral neuropathy

A 
Phenytoin
Nitrofurantoin
Ethambutol
Azidothymidine (AZT)
Metronidazole
Isoniazid
Amiodarone
Vincristine
Vinblastin
Cysplatin
56
Q

Blood tests for a child with chorea

A

Thyroid function tests
Caeruloplasmin levels
Antinuclear antibodies

57
Q

Causes of pes cavus

A 
Friedreich's ataxia
Tethered spinal cord
Spina bifida
Duchenne
Charcot Marie Tooth

Most things that cause distal lower limb weakness

58
Q

Features of Smith-Lemli-Opitz

A 
Low set ears
Broad, upturned nose
Micrognathia
Syndactyly of toes
Hypospadias
Initial hypotonia, later hypertonia
Cryptorchidism
CVS/renal/GI abnormalities
59
Q

Features of Cornelia de Lange

A 
Learning difficulties
Poor growth
Confluent, bushy eyebrows
Low hairline
Micrognathia
Small hands and feet
Thin lips
Anteverted nose
Down turned mouth
ASD/VSD
60
Q

Congenital infection causing chorioretinitis, thrombocytopenia and blueberry muffin purport, cardiac lesions, deafness, bony involvement, hepatosplenomegaly, jaundice

A

Rubella

61
Q

Conditions causing fasciculations

A

Werdnig-Hoffman
Polio
Syringomyelia
Cervical spondylosis

62
Q

Treatment for Guillain-Barre

A

Physio
Plasmapheresis
IVIG

63
Q

Scoring of eye opening in GCS

A

4 - spontaneous
3 - to verbal command
2 - to painful stimuli
1 - no eye opening

64
Q

Scoring of verbal in GCS

A

Adult/older child

5 - oriented
4 - confused conversation
3 - inappropriate words
2 - incomprehensible sounds
1 - none
65
Q

Scoring of grimace in GCS

A

Young child

5 - spontaneous/normal activity
4 - Less than usual spontaneous, only responsive to touch
3 - vigorous grimace to pain
2 - mild grimace to pain
1 - no response to pain
66
Q

Motor scoring in GCS

A 
6 - obeys commands
5 - localises pain
4 - normal flexion to pain
3 - abnormal flexion to pain (decorticate)
2 - extension to pain (decerebrate)
1 - no response
67
Q

Level of lesion causing absent hip flexion

A

L1-2

68
Q

Features of Leigh’s disease

A 
Swallowing problems
Vomiting
Failure to thrive
Delayed motor milestones
Generalised seizures
Weakness
Sighing or sobbing breathing
Visual abnormalities
Elevated serum lactate
69
Q

Features of glutamic acidaemia

A

Develop normally for first 2 years

Then suddenly develop extra-pyramidal signs during a mild illness

70
Q

Features of Kearns-Sayre syndrome

A

Involves muscles controlling eyelid movement and extra ocular muscles
Bilateral pigmentary retinopathy
Cardiac abnormalities

Also:
Cerebellar ataxia
Proximal muscle weakness
Deafness
Diabetes
GH deficiency
HypoPTH
other endocrine issues
71
Q

Features of dopa-responsive dystonia

A

Age 7ish
Cramps after exercise
Then develop parkinsonism
Symptoms better in the morning and worsen with exertion

72
Q

Features of NCL

A 
Progressive visual loss
Dementia
Motor decline
Therapy resistant epilepsy
Ultimate death
73
Q

Best choice for generalised seizures in teenage girls

A

Lamotrigine

74
Q

EEG pattern in Friedreich’s ataxia

A

Low amplitude compound muscle action potentials, or absent sensory nerve action potentials

75
Q

Treatment for infantile spasms

A

Vigabatrin

76
Q

Skin feature in Fabry’s disease

A

Angiokeratoma

MRCPCH Part 2 (24 decks)

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