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Neuro Flashcards

1
Q

Causes of corticospinal motor disorders

A
  1. neural migration problem
  2. ischaemia
  3. cerebral tumor
  4. arterial ischaemic stroke
  5. encephalomyelitis
  6. hemiplegic migraine
  7. post-ictal paresis
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2
Q

PC corticospinal motor disorders

A

UMN

- weakness: shoulder AD, F elbow, pronation forearm

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3
Q

Causes of basal ganglia motor disorders

A 
M-WASH
Mitochondrial cytopathies
Wilsons
Acquired:
- ischaemia
- CO poisoning 
- post-cardiopulmonary bypass
Syndenhams
Huntingtons
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4
Q

Acquired basal ganglia problems

A

Ischaemia

CO poisoning

post-cardiopulmonary bypass

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5
Q

PC basal ganglia motor disorders

A
  • dystonia– can’t get going
  • dyskinesia
  • -> chorea
  • -> athetosis
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6
Q

Causes of cerebellar motor disordes

A

GAPP

Genetic– ataxia/tel, friederichs, ataxic CP
Acute- drug/alcohol
Posterior fossa tumor
Post-viral-varicella

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7
Q

PC cerebellar dysfunction

A 
nystagmus
dysarthria-- scanning speech
dysmetria-- past pointing
dysdiadochokinesia
\+ Romberg
Wide Gait
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8
Q

PC Peripheral motor disorders

A

WEAKNESS

  • muscle fatiguability
  • muscle cramps
  • hypotonia
  • delayed motor milestones
  • waddling gait
  • GOWERS SIGN
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9
Q

Dx myopathy

A

CPK
Bx
US/MRI
DNA

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10
Q

Dx neuropathy

A

nerve conduction studies
Bx (rare)
EMG
DNA

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11
Q

Anterior horn cell path

A
  1. Spinal muscular atrophy

2. Polio

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12
Q

Spinal muscular atrophy

A

autosomal recessive
SMN= survival motor neurone–> degen LMN

3 TYPES

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13
Q

Type 1 SMA=

A

Werdnig Hoffman

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14
Q

PC Werdnig Hoffman

A

IN UTERO: decrease fetal movements
arthrogryphosis

CANNOT SIT

LMN- HYPOTONIA:
tongue fasciculations
decrease DTR’s

lack anti gravity movement hip flexors
intercostal recessions

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15
Q

death werdnig hoffman

A

1 year old– respiratory failure

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16
Q

Type 2 SMA

A

CAN SIT :)

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17
Q

Type 3 SMA

A

Ku-gel-berg Wel-an-der

CAN WALK :)

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18
Q

Peripheral nerve problems

A
  1. Hereditary motor and sensory neuropathy
  2. Acute post-infectious polyneuropathy
  3. Bell’s Palsy
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19
Q

HMSN– type 1…

A

Peroneal atrophy= Charcot Marie Tooth

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20
Q

Pathophysiology CMT

A

myelin structural problems
– demyelination–> remyelination
= onion bulb formation

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21
Q

Inheritance CMT

A

AD

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22
Q

PC CMT + age

A

1 - 10 year old

  • symmetrical DISTAL
  • motor: distal atorphy
  • sensory: distal paraesthesia
  • pes cavus and hammer toes–> gait problems

rarely– unable to walk

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23
Q

PC GBS

A

2-3 weeks post…
viral URI
campy

---> ascending paralysis 
\+ loss reflexes
\+weakness
\+ autonomic
\+ BULBAR--> can't swallow, rest depression= death
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24
Q

Recovery GBS

A

95% recover 2 years later

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25
Q

Dx GBS

A
  1. CSF: albuminocytologic dissasociation

2. Nerve conduction studies: decreased

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26
Q

Tx GBS

A
  1. SUPPORTIVE
  2. IVIG
  3. plasma exchange

NOT STEROIDS– slow down progression

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27
Q

Causes of Bells Palsy

A

IDIOPATHIC
[post-HSV-adults]

Compression cp angle
Ramsy Hunt
HTN--> coarct a/w Bells
Sarcoidosis
Lyme disease
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28
Q

PC age MG

A

> 10 yo

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29
Q

Dx MG

A
  1. Edrophonium= Tensilon Test
  2. Antibodies
  3. Anti-MUSK= antimuscle Specific Kinase
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30
Q

Tx MG

A
  1. Neostigmine, pyridostigmine
  2. Steroids
  3. Azathioprine
  4. Plasma exchange
  5. Thymectomy
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31
Q

Duchennes genes

A

del Xp21 DMD gene

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32
Q

Symptoms age duchennes

A

about 3 yo

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33
Q

Dx age duchennes

A

5.5yo

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34
Q

Can’t walk age duchennes

A

10-14 yo

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35
Q

Complications of duchennes

A

Scoliosis
DCM
Resp F

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36
Q

Dx Duchennes

A

increase CPK

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37
Q

Tx duchennes

A
  1. exercises, passive stretches, splints
  2. orthotics
  3. lengthen achilles
  4. sitting posture
  5. truncal brace
  6. CPAP, NIPPV
  7. support
  8. steroids–> ambulatory
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38
Q

Beckers

A

mutation DMD

milder

39
Q

PC Beckers

40
Q

Can’t walk beckers

41
Q

LE beckers

42
Q

Congenital dystorphy

A

INFANT

  • hypotonia
  • contractiures
  • learning disability
43
Q

Dx congenital dystrophy

A

RECESSIVE

decrease ECM:

  • laminin
  • glucosyltransferases
44
Q

Metabolic myopathy infant PC

45
Q

Metabolic myopathy child PC

A

muscle weakness

muscle cramps

46
Q

DDx metabolic myopathy

A
  1. glycogen storage disorder
  2. lipid smetabolism– carnitine deficiency
  3. mitochondrial cytopathies
47
Q

Benign acute myositis

A

ACUTE– post-viral URI– muscle weakness and pain

CPK increases

48
Q

Dermatomyositis age

49
Q

Dx dermatomyositis

A
  1. increase/normal CPK/ESR

2. Bx: inflammatory infiltrate, atrophy– perimysial

50
Q

Tx dermatomyositis

A
  1. physiotx
  2. steroids
  3. MTX/ cyclosporine
51
Q

Myotonic dystrophy newborn

A
  • hypotonia
  • poor feeding
  • resp problems
52
Q

Myotonic dystrophy— WHO ELSE TO EXAMINE?

A

MOTHERS– for lock grip

53
Q

Myotonic dystorphy child

A
  • myopathic facies
  • learning disabilities
  • myotonia
54
Q

Myotonic dystrophy adult

A
  • baldness
  • cataracts
  • testicular atrophy
55
Q

Death myotonic dystrophy

A

CARDIOMYOPATHY

56
Q

Causes of central hypotonia

A
  1. Cortical
    - malformations
    - hIE
  2. Genetic
    - Downs
    - Prader Willi
  3. Metabolic
    - hypocalcaemia
    - hypothyroidism
57
Q

Causes of peripheral hypotonia

A
  1. spinal muscular atrophy
  2. myopathy
  3. myotonia
  4. MG
58
Q

Age PC Friederichs

59
Q

Death Friederichs

A

40-50yo

  • HOCM
  • Kyphoscoliosis
60
Q

Age PC Ataxia Telangiectasia

A

school age

61
Q

Age angiomas in AT

A

4 yo

  • neck
  • shoulders
  • conjunctiva
62
Q

Deterioration in AT

A

Cerebellar

Dystonia

63
Q

Complications of AT

64
Q

What increases in AT

A

Alpha feto-protein

65
Q

Dx AT

A
  1. Increase WCC sensitivity irradiation

2. ATM gene test

66
Q

Anterior or posterior circulation stroke in kids

67
Q

Causes of stroke in kids

A
  1. Cardiac
    - CCHD
    - IE
  2. Haem
    - sickle
    - def protein S
  3. Post- infection- varicella
  4. Inflam- SLE
  5. Metabolic
  6. Vascular
68
Q

Metabolic causes of childhood stroke

A
  1. homocystinuria
  2. mitochondrial
  3. MELAS
  4. CADASIL
69
Q

MELAS

A 
Myoclonic
Epilepsy
Lactic
Acidosis
Stroke
70
Q

CADASIL

A 
Cerebral
Autosomal
Dominant
Arteriopathy with
subcortical
infarcts
Leukonecephalopathy
71
Q

Vascular causes of childhood stroke

A

moyamoya disease

72
Q

PC Extradural haematoma

A

lucid–> LOC–> seizures

ipsi- pupil dilation
contra- paresis limbs
false localising 6th uni or bi palsy

anaemia
shock

73
Q

Tx extradural haematoma

A
  1. hypovolaemia correction

2. evacuate haematoma

74
Q

Phakomata

A

dense white areas retina

75
Q

Dx Ash leaf spots

A

UV Woods Lamp

76
Q

Location shagreen

A

LUMBAR, rough patch

77
Q

Neuro problems in tuberous sclerosis

A
  • infantile spasms
  • developmental delay
  • focal epilepsy
  • intellectual imapirment
78
Q

Dx NFT1= Von Recklinghausen

A

2/7

  1. optic glioma
  2. lisch nodules
  3. cafe au lait
  4. neurofibromas
  5. axillary freckling
  6. sphenoid dysplasia–> eye protrusion
  7. FDR- NF1
79
Q

Cafe au lait spots

A

> 6
before puberty> 5mm
after puberty> 15mm

80
Q

Neurofibromas

A

compress 2nd and 8th CN

megaencephaly

epilepsy

learning disabilities

81
Q

NFT2

A

6

  1. acoustic neuromas: hearing/ataxia/CN7
  2. phaeo
  3. pul HTN
  4. renal artery stenosis—HTN
  5. gliomatous change cns
  6. rarely—> malignant sarcomas
82
Q

Gauchers

A

beta glucocerebrosidase
crinkle paper
anaemia
thrombocytopenia

infantile– rapid neuro
adult– normal life span, normal neuro/IQ

83
Q

Neiman Picks PC age

A

3-4 months

  • difficulty feeding
  • FTT
  • devpt delay
  • hypotonia
  • vision and hearing down
84
Q

death in neiman picks

A

type A— 3 yo death

85
Q

Tay Sachs

A

NORMAL- 3-6 MONTHS

  • weakness
  • exag startle response
  • sociall unresponsive– slow development/ regression
  • visual inattention
86
Q

age of febrile seizures

A

6 months- 5 years old

87
Q

risk factors for febrile seizures

A
  • younger child
  • lower temp
  • shorter duration illness before
  • positive family history
88
Q

non-epileptic seizures

A

5

  1. febrile seizures
  2. metabolic
  3. head trauma
  4. meningitis/ encephalitis
  5. poisons/toxins
89
Q

epileptic seizures

A
  1. idiopathic
  2. secondary to:
    - cerebral malformations
    - cerebral vasc occlusion
    - triad: asphyxia, infection, IVH
  3. cerebral tumor
  4. neurodegen
  5. neurocutaneous
90
Q

west syndrome

A

age: 1-3 months
- F spasms of neck, limbs
- followed by extensor spasms (salaam position)

EEG:

  • hypsarrhythmia
  • chaotic high voltage
  • multifocal sharp wave discharges

neurodevelopment and learning disability

tx: vigabatrin
+ corticosteroids

91
Q

Lennox Gastaut

A 
1-3 year old
DROP ATTACKS
astatic seizures
tonic
atypical seizures

a/w neurodevelopment and behaviour

POOR PROGNOSIS
hx of infantile/west syndrome spasm

92
Q

BECTS: benign epilepsy centrotemporal spikes

A

4-10 yo
tonic clonic seizures while sleeping

and simple focal seizures with awareness of abnormal feelings of tongue and facie

sharp focal waves from rolandic (Centrotemporal area)

benign :) remission in childhood

93
Q

Early onset benign childhood occipital (panayiotopoulous)

A 
1-14 yearsold
younger kids:
- unresponsiveness
- eye deviation
- vomiting
- autonomic

older kids:
- headache and hallucinations

EEG- occipital changes

benign :) remission in childhoood

94
Q

juvenile myoclonic

A

adolescent/adulthood

myoclonic with TC’s and absence

shortly after walking

hx: throwing cereal and drinks in the morning

ok learning

good tx– but LIFELONG

genetic

PAEDS SC1 (13 decks)

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