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Flashcards in NEURO Deck (78):
1

altered level of consciousness, increased DTR's , weak cry, decreased spent movement

central hypotonia

2

unaffected consciousness, decreased muscle bulk and decreased DTR's?

peripheral hypotonia

3

central hypotonia management

head CT
serum electrolytes
FISH

4

peripheral hypotonia management

CK levels
DNA tests
Nerve conduction
muscle biopsy

5

autosomall recessive inheritence mutations in chromosome 5 w/ hopotonia, weakness and tongue fasciculations?

spinal muscular atrophy

6

werdnig hoffman disease

onset of SMA

7

type II sma onset

6-12 months

8

type III onset

>3 years

9

what is affected in spinal muscular atrophy

degeneration and loss of anterior horn motor neurons

10

clinical features of SMA

weak cry, tongue fasciculations, bell shaped chest, frog leg posture

NORMAL EXTRA OULAR MOVEMENTS AND NORMAL SENSORY EXAM

11

diagnosis of werdnig hoffman?

DNA testing/ muscle biopsy

12

what causes death in werdnigg hoffman?

resp insufficiency or pneumonia

13

prevents the presynaptic release of acetyl choline?

botulinum toxin

14

treatment of botulinum?

botulism immune globulin - NOT ANTIBIOTICS

15

trinucleotide repeat disorder autosomal dominant disease on chromosome 19

MOM IS ALSO AFFECTED?!

congenital myotonic dystrophy

16

atrophy of masseter and temporal is muscles, stiff straight smile and inability to release grip after hand shaking

myotonia

17

true or false

those with congenital myotonic dystrophy will have completely normal academic abilities?

FALSE

all survivors have mental retardation

- IQ of 50-65

18

enlarged ventricles caused by obstruction of CSF flow-- aqueductal stenosis

noncommunicating hydrocephalus

19

increased production of CSF or decreased absorption of CSF

communicating hydrocephalus

20

term used to describe ventricular enlargement caused by brain atrophy

hydrocephalus ex vacuo

21

downward place meant of the cerebellum and medulla through the foramen magnum blocking CSF flow associated w/ a lumbosacral myelomeningocele?

arnold chiari type II

22

absent or hypoplastic vermis w/ cystic enlargement of fourth ventricle

dandywalker malformation

23

when should you suspect hydrocephalus?

large head circumference
large anterior and posterior fontanelles w/ split sutures
sunset sign

24

evaluation of hydrocephalus

urgent head CT scan

25

management of hydrocephalus ?

VP shunt although they may cause infection and obstruction

26

taratogens that cause SB include

valproate, phenytoin, colchicine, vincristine,azathioprine, methotrexate

27

menningocele or myelomeningocele present w/ mental retardation?

myelomeningocele

28

what is the lab marker for neural tube defect?

increased AFP

29

unilateral dilated nonreactive pupil suggests

uncal herniation

30

chenye stokes breathing and decortical posture suggests?

bilateral cortical injury

31

what do you do after ABC"s in a comatose patient?

glucose
urine tox
serum elec
metabolic panel
CT scan
lumbar punch if CT negative
EEG

32

involuntary alteration of conciousnessness, behavior, motor activity, sensation or autonomic function caused by

seizures

33

occurrence of two or more spontaneous seizures w/out an obvious precipitating cause

epilepsy

34

seizure that last more than 30 minutes and the patient doesn't regain conciousness

status epilepticus

35

tonic clonic and absence seizures are both?

generalized afebrile seizures

36

treatment of status epileptics

IV benzo's (lorazapam, diazepam) w/ phenobarbital or phenytoin

37

what must be ruled out in a febrile seizure ?

CSF infection

38

generalized epilepsy tx

valproic acid or phenobarbital

39

absence epilepsy tx

ethosuximide

40

partial epilepsy tx

carbamazepine or phenytoin

41

what are 2 alternative treatments for patients w/ poorly controlled seizures?

vagal nerve stimulator and ketogenic diet

42

if someone has a first febrile seizure do you treat it?

no

43

if someone has recurrent febrile seizures

daily anticonvulsant prophylaxis w/ valproic acid/ phenobarbital

abortive tx w/ rectal diazepam

44

what is the most common cause of infantile spasms?

tuberous sclerosis

45

brief myoclonic jerks lasting 1-2 seconds each occurring in 5-10 seizures over 3-5 minutes?

west syndrome

46

buzz words for west syndrome

jack knife seizure / salaam zeizures

47

how do you treat west syndrome?

ACTH IM injections
valproic acid
vigabatrin ( tuberous sclerosis)

48

most common partial epilepsy during childhood

benign rolandic epilepsy
(benign centreotemporal epilepsy)

49

when do centrotemporal epilepsy occur?

early morning hours when patients are asleep w/ oral buccal manifestations

50

headaches caused by ICP occur when?

in the morning

51

unilateral headaches associated w/ nausea, vomiting or visual changes

migraines

52

migraine associated w/ vertigo, tinnitus, ataxia or dysarthria

basilar artery migraine

53

migraine associated w/ unilateral ptosis or ranial nerve II palsy

opthalmoplegic migraine

54

prophylactic treatment of migraines?

propanolol

55

abortive treatment of migraines

sumatriptan

56

dull aching headaches associated w/ muscle contraction

tension headaches

57

are tension headaches or migraines more common in childhood?

migraines

58

unilateral frontal or facial pain accompanied by conjunctival erythema, lacrimation and nasal congestion

cluster headaches

59

tx for cluster headaches

oxygen or sumatriptan

60

phrophylactic tx of tension headaches

ccb and valproic acid

61

ataxia secondary to a presumed autoimmune / postinfectious cause

acute cerebellar ataxia of childhood

62

what infections cause acute cerebellar ataxia of childhood?

varicella, influenza, EBV and mycoplasma

63

what does head ct scan show in cerebellar ataxia of childhood?

normal ct

64

demyelinating polyneuritis w/ ascendnig weakness, areflexia and normal sensation

guillain barre

65

what is the most common associated infection agen of guillain barre

campylobacter jejuni

66

what kind of immune response is guillain barre

cell mediated immune response

67

what does lumbar puncture show in guillain bar?

albuminocytologic dissociation

68

what it the biggest risk w/ guillain barre?

respiratory muscle paralysis

69

management of guillain barre?

IVIG and plasmapheresis

70

how do you treat syndenhams chorea?

haloperidol valproic acid or phenobarbital

71

what is the drug of choice in turrets?

pimozide

72

autoimmune disorder presents w/ progressive weakness and diploplia?

myasthenia gravis

73

cause of myasthenia gravis

antibodies against eh acetyl choline receptor at neuromuscular junctions

74

what is neonatal myasthenia caused by?

transient weakness in new born from transplacental transfer of maternal AchR antibodies

75

clinical features of myasthenia?

bilateral ptosis
characteristic increasing weakness
diplopia

76

diagnosis of myasthenia

tensilon test ?edrophonium chloride

decremental response

presence of AChR antibody titrs

77

myasthenia gravis treatment of choice

cholinesterase inhibitors
prydostigmide bromide

78

what is the first symptom in botulinum?

constipatio