NEURO Flashcards
altered level of consciousness, increased DTR’s , weak cry, decreased spent movement
central hypotonia
unaffected consciousness, decreased muscle bulk and decreased DTR’s?
peripheral hypotonia
central hypotonia management
head CT
serum electrolytes
FISH
peripheral hypotonia management
CK levels
DNA tests
Nerve conduction
muscle biopsy
autosomall recessive inheritence mutations in chromosome 5 w/ hopotonia, weakness and tongue fasciculations?
spinal muscular atrophy
werdnig hoffman disease
onset of SMA
type II sma onset
6-12 months
type III onset
> 3 years
what is affected in spinal muscular atrophy
degeneration and loss of anterior horn motor neurons
clinical features of SMA
weak cry, tongue fasciculations, bell shaped chest, frog leg posture
NORMAL EXTRA OULAR MOVEMENTS AND NORMAL SENSORY EXAM
diagnosis of werdnig hoffman?
DNA testing/ muscle biopsy
what causes death in werdnigg hoffman?
resp insufficiency or pneumonia
prevents the presynaptic release of acetyl choline?
botulinum toxin
treatment of botulinum?
botulism immune globulin - NOT ANTIBIOTICS
trinucleotide repeat disorder autosomal dominant disease on chromosome 19
MOM IS ALSO AFFECTED?!
congenital myotonic dystrophy
atrophy of masseter and temporal is muscles, stiff straight smile and inability to release grip after hand shaking
myotonia
true or false
those with congenital myotonic dystrophy will have completely normal academic abilities?
FALSE
all survivors have mental retardation
- IQ of 50-65
enlarged ventricles caused by obstruction of CSF flow– aqueductal stenosis
noncommunicating hydrocephalus
increased production of CSF or decreased absorption of CSF
communicating hydrocephalus
term used to describe ventricular enlargement caused by brain atrophy
hydrocephalus ex vacuo
downward place meant of the cerebellum and medulla through the foramen magnum blocking CSF flow associated w/ a lumbosacral myelomeningocele?
arnold chiari type II
absent or hypoplastic vermis w/ cystic enlargement of fourth ventricle
dandywalker malformation
when should you suspect hydrocephalus?
large head circumference
large anterior and posterior fontanelles w/ split sutures
sunset sign
evaluation of hydrocephalus
urgent head CT scan
management of hydrocephalus ?
VP shunt although they may cause infection and obstruction
taratogens that cause SB include
valproate, phenytoin, colchicine, vincristine,azathioprine, methotrexate
menningocele or myelomeningocele present w/ mental retardation?
myelomeningocele
what is the lab marker for neural tube defect?
increased AFP
unilateral dilated nonreactive pupil suggests
uncal herniation
chenye stokes breathing and decortical posture suggests?
bilateral cortical injury
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
involuntary alteration of conciousnessness, behavior, motor activity, sensation or autonomic function caused by
seizures
occurrence of two or more spontaneous seizures w/out an obvious precipitating cause
epilepsy
seizure that last more than 30 minutes and the patient doesn’t regain conciousness
status epilepticus
tonic clonic and absence seizures are both?
generalized afebrile seizures
treatment of status epileptics
IV benzo’s (lorazapam, diazepam) w/ phenobarbital or phenytoin
what must be ruled out in a febrile seizure ?
CSF infection
generalized epilepsy tx
valproic acid or phenobarbital
absence epilepsy tx
ethosuximide
partial epilepsy tx
carbamazepine or phenytoin
what are 2 alternative treatments for patients w/ poorly controlled seizures?
vagal nerve stimulator and ketogenic diet
if someone has a first febrile seizure do you treat it?
no
if someone has recurrent febrile seizures
daily anticonvulsant prophylaxis w/ valproic acid/ phenobarbital
abortive tx w/ rectal diazepam
what is the most common cause of infantile spasms?
tuberous sclerosis
brief myoclonic jerks lasting 1-2 seconds each occurring in 5-10 seizures over 3-5 minutes?
west syndrome
buzz words for west syndrome
jack knife seizure / salaam zeizures
how do you treat west syndrome?
ACTH IM injections
valproic acid
vigabatrin ( tuberous sclerosis)
most common partial epilepsy during childhood
benign rolandic epilepsy
benign centreotemporal epilepsy
when do centrotemporal epilepsy occur?
early morning hours when patients are asleep w/ oral buccal manifestations
headaches caused by ICP occur when?
in the morning
unilateral headaches associated w/ nausea, vomiting or visual changes
migraines
migraine associated w/ vertigo, tinnitus, ataxia or dysarthria
basilar artery migraine
migraine associated w/ unilateral ptosis or ranial nerve II palsy
opthalmoplegic migraine
prophylactic treatment of migraines?
propanolol
abortive treatment of migraines
sumatriptan
dull aching headaches associated w/ muscle contraction
tension headaches
are tension headaches or migraines more common in childhood?
migraines
unilateral frontal or facial pain accompanied by conjunctival erythema, lacrimation and nasal congestion
cluster headaches
tx for cluster headaches
oxygen or sumatriptan
phrophylactic tx of tension headaches
ccb and valproic acid
ataxia secondary to a presumed autoimmune / postinfectious cause
acute cerebellar ataxia of childhood
what infections cause acute cerebellar ataxia of childhood?
varicella, influenza, EBV and mycoplasma
what does head ct scan show in cerebellar ataxia of childhood?
normal ct
demyelinating polyneuritis w/ ascendnig weakness, areflexia and normal sensation
guillain barre
what is the most common associated infection agen of guillain barre
campylobacter jejuni
what kind of immune response is guillain barre
cell mediated immune response
what does lumbar puncture show in guillain bar?
albuminocytologic dissociation
what it the biggest risk w/ guillain barre?
respiratory muscle paralysis
management of guillain barre?
IVIG and plasmapheresis
how do you treat syndenhams chorea?
haloperidol valproic acid or phenobarbital
what is the drug of choice in turrets?
pimozide
autoimmune disorder presents w/ progressive weakness and diploplia?
myasthenia gravis
cause of myasthenia gravis
antibodies against eh acetyl choline receptor at neuromuscular junctions
what is neonatal myasthenia caused by?
transient weakness in new born from transplacental transfer of maternal AchR antibodies
clinical features of myasthenia?
bilateral ptosis
characteristic increasing weakness
diplopia
diagnosis of myasthenia
tensilon test ?edrophonium chloride
decremental response
presence of AChR antibody titrs
myasthenia gravis treatment of choice
cholinesterase inhibitors
prydostigmide bromide
what is the first symptom in botulinum?
constipatio
