Neuro Flashcards
(94 cards)
1
Q
Causes of corticospinal motor disorders
A
- neural migration problem
- ischaemia
- cerebral tumor
- arterial ischaemic stroke
- encephalomyelitis
- hemiplegic migraine
- post-ictal paresis
2
Q
PC corticospinal motor disorders
A
UMN
- weakness: shoulder AD, F elbow, pronation forearm
3
Q
Causes of basal ganglia motor disorders
A
M-WASH Mitochondrial cytopathies Wilsons Acquired: - ischaemia - CO poisoning - post-cardiopulmonary bypass Syndenhams Huntingtons
4
Q
Acquired basal ganglia problems
A
Ischaemia
CO poisoning
post-cardiopulmonary bypass
5
Q
PC basal ganglia motor disorders
A
- dystonia– can’t get going
- dyskinesia
- -> chorea
- -> athetosis
6
Q
Causes of cerebellar motor disordes
A
GAPP
Genetic– ataxia/tel, friederichs, ataxic CP
Acute- drug/alcohol
Posterior fossa tumor
Post-viral-varicella
7
Q
PC cerebellar dysfunction
A
nystagmus dysarthria-- scanning speech dysmetria-- past pointing dysdiadochokinesia \+ Romberg Wide Gait
8
Q
PC Peripheral motor disorders
A
WEAKNESS
- muscle fatiguability
- muscle cramps
- hypotonia
- delayed motor milestones
- waddling gait
- GOWERS SIGN
9
Q
Dx myopathy
A
CPK
Bx
US/MRI
DNA
10
Q
Dx neuropathy
A
nerve conduction studies
Bx (rare)
EMG
DNA
11
Q
Anterior horn cell path
A
- Spinal muscular atrophy
2. Polio
12
Q
Spinal muscular atrophy
A
autosomal recessive
SMN= survival motor neurone–> degen LMN
3 TYPES
13
Q
Type 1 SMA=
A
Werdnig Hoffman
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
15
Q
death werdnig hoffman
A
1 year old– respiratory failure
16
Q
Type 2 SMA
A
CAN SIT :)
17
Q
Type 3 SMA
A
Ku-gel-berg Wel-an-der
CAN WALK :)
18
Q
Peripheral nerve problems
A
- Hereditary motor and sensory neuropathy
- Acute post-infectious polyneuropathy
- Bell’s Palsy
19
Q
HMSN– type 1…
A
Peroneal atrophy= Charcot Marie Tooth
20
Q
Pathophysiology CMT
A
myelin structural problems
– demyelination–> remyelination
= onion bulb formation
21
Q
Inheritance CMT
A
AD
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
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
24
Q
Recovery GBS
A
95% recover 2 years later
25
Dx GBS
1. CSF: albuminocytologic dissasociation
| 2. Nerve conduction studies: decreased
26
Tx GBS
1. SUPPORTIVE
2. IVIG
3. plasma exchange
NOT STEROIDS-- slow down progression
27
Causes of Bells Palsy
IDIOPATHIC
[post-HSV-adults]
```
Compression cp angle
Ramsy Hunt
HTN--> coarct a/w Bells
Sarcoidosis
Lyme disease
```
28
PC age MG
> 10 yo
29
Dx MG
1. Edrophonium= Tensilon Test
2. Antibodies
3. Anti-MUSK= antimuscle Specific Kinase
30
Tx MG
1. Neostigmine, pyridostigmine
2. Steroids
3. Azathioprine
4. Plasma exchange
5. Thymectomy
31
Duchennes genes
del Xp21 DMD gene
32
Symptoms age duchennes
about 3 yo
33
Dx age duchennes
5.5yo
34
Can't walk age duchennes
10-14 yo
35
Complications of duchennes
Scoliosis
DCM
Resp F
36
Dx Duchennes
increase CPK
37
Tx duchennes
1. exercises, passive stretches, splints
2. orthotics
3. lengthen achilles
4. sitting posture
5. truncal brace
6. CPAP, NIPPV
7. support
8. steroids--> ambulatory
38
Beckers
mutation DMD
| milder
39
PC Beckers
10 yo
40
Can't walk beckers
20 yo
41
LE beckers
40 yo
42
Congenital dystorphy
INFANT
- hypotonia
- contractiures
- learning disability
43
Dx congenital dystrophy
RECESSIVE
decrease ECM:
- laminin
- glucosyltransferases
44
Metabolic myopathy infant PC
hypotonia
45
Metabolic myopathy child PC
muscle weakness
| muscle cramps
46
DDx metabolic myopathy
1. glycogen storage disorder
2. lipid smetabolism-- carnitine deficiency
3. mitochondrial cytopathies
47
Benign acute myositis
ACUTE-- post-viral URI-- muscle weakness and pain
| CPK increases
48
Dermatomyositis age
5-10 yo
49
Dx dermatomyositis
1. increase/normal CPK/ESR
| 2. Bx: inflammatory infiltrate, atrophy-- perimysial
50
Tx dermatomyositis
1. physiotx
2. steroids
3. MTX/ cyclosporine
51
Myotonic dystrophy newborn
- hypotonia
- poor feeding
- resp problems
52
Myotonic dystrophy--- WHO ELSE TO EXAMINE?
MOTHERS-- for lock grip
53
Myotonic dystorphy child
- myopathic facies
- learning disabilities
- myotonia
54
Myotonic dystrophy adult
- baldness
- cataracts
- testicular atrophy
55
Death myotonic dystrophy
CARDIOMYOPATHY
56
Causes of central hypotonia
1. Cortical
- malformations
- hIE
2. Genetic
- Downs
- Prader Willi
3. Metabolic
- hypocalcaemia
- hypothyroidism
57
Causes of peripheral hypotonia
1. spinal muscular atrophy
2. myopathy
3. myotonia
4. MG
58
Age PC Friederichs
10-15yo
59
Death Friederichs
40-50yo
- HOCM
- Kyphoscoliosis
60
Age PC Ataxia Telangiectasia
school age
61
Age angiomas in AT
4 yo
- neck
- shoulders
- conjunctiva
62
Deterioration in AT
Cerebellar
| Dystonia
63
Complications of AT
ALL
64
What increases in AT
Alpha feto-protein
65
Dx AT
1. Increase WCC sensitivity irradiation
| 2. ATM gene test
66
Anterior or posterior circulation stroke in kids
ANTERIOR
67
Causes of stroke in kids
1. Cardiac
- CCHD
- IE
2. Haem
- sickle
- def protein S
3. Post- infection- varicella
4. Inflam- SLE
5. Metabolic
6. Vascular
68
Metabolic causes of childhood stroke
1. homocystinuria
2. mitochondrial
3. MELAS
4. CADASIL
69
MELAS
```
Myoclonic
Epilepsy
Lactic
Acidosis
Stroke
```
70
CADASIL
```
Cerebral
Autosomal
Dominant
Arteriopathy with
subcortical
infarcts
Leukonecephalopathy
```
71
Vascular causes of childhood stroke
moyamoya disease
72
PC Extradural haematoma
lucid--> LOC--> seizures
ipsi- pupil dilation
contra- paresis limbs
false localising 6th uni or bi palsy
anaemia
shock
73
Tx extradural haematoma
1. hypovolaemia correction
| 2. evacuate haematoma
74
Phakomata
dense white areas retina
75
Dx Ash leaf spots
UV Woods Lamp
76
Location shagreen
LUMBAR, rough patch
77
Neuro problems in tuberous sclerosis
- infantile spasms
- developmental delay
- focal epilepsy
- intellectual imapirment
78
Dx NFT1= Von Recklinghausen
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
Cafe au lait spots
> 6
before puberty> 5mm
after puberty> 15mm
80
Neurofibromas
compress 2nd and 8th CN
megaencephaly
epilepsy
learning disabilities
81
NFT2
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
Gauchers
beta glucocerebrosidase
crinkle paper
anaemia
thrombocytopenia
infantile-- rapid neuro
adult-- normal life span, normal neuro/IQ
83
Neiman Picks PC age
3-4 months
- difficulty feeding
- FTT
- devpt delay
- hypotonia
- vision and hearing down
84
death in neiman picks
type A--- 3 yo death
85
Tay Sachs
NORMAL- 3-6 MONTHS
- weakness
- exag startle response
- sociall unresponsive-- slow development/ regression
- visual inattention
86
age of febrile seizures
6 months- 5 years old
87
risk factors for febrile seizures
- younger child
- lower temp
- shorter duration illness before
- positive family history
88
non-epileptic seizures
5
1. febrile seizures
2. metabolic
3. head trauma
4. meningitis/ encephalitis
5. poisons/toxins
89
epileptic seizures
1. idiopathic
2. secondary to:
- cerebral malformations
- cerebral vasc occlusion
- triad: asphyxia, infection, IVH
3. cerebral tumor
4. neurodegen
5. neurocutaneous
90
west syndrome
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
Lennox Gastaut
```
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
BECTS: benign epilepsy centrotemporal spikes
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
Early onset benign childhood occipital (panayiotopoulous)
```
1-14 yearsold
younger kids:
- unresponsiveness
- eye deviation
- vomiting
- autonomic
```
older kids:
- headache and hallucinations
EEG- occipital changes
benign :) remission in childhoood
94
juvenile myoclonic
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
