Flashcards in NEURO Deck (78):
altered level of consciousness, increased DTR's , weak cry, decreased spent movement
unaffected consciousness, decreased muscle bulk and decreased DTR's?
central hypotonia management
peripheral hypotonia management
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
type III onset
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?
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
true or false
those with congenital myotonic dystrophy will have completely normal academic abilities?
all survivors have mental retardation
- IQ of 50-65
enlarged ventricles caused by obstruction of CSF flow-- aqueductal stenosis
increased production of CSF or decreased absorption of CSF
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
when should you suspect hydrocephalus?
large head circumference
large anterior and posterior fontanelles w/ split sutures
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?
what is the lab marker for neural tube defect?
unilateral dilated nonreactive pupil suggests
chenye stokes breathing and decortical posture suggests?
bilateral cortical injury
what do you do after ABC"s in a comatose patient?
lumbar punch if CT negative
involuntary alteration of conciousnessness, behavior, motor activity, sensation or autonomic function caused by
occurrence of two or more spontaneous seizures w/out an obvious precipitating cause
seizure that last more than 30 minutes and the patient doesn't regain conciousness
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 ?
generalized epilepsy tx
valproic acid or phenobarbital
absence epilepsy tx
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?
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?
brief myoclonic jerks lasting 1-2 seconds each occurring in 5-10 seizures over 3-5 minutes?
buzz words for west syndrome
jack knife seizure / salaam zeizures
how do you treat west syndrome?
ACTH IM injections
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
migraine associated w/ vertigo, tinnitus, ataxia or dysarthria
basilar artery migraine
migraine associated w/ unilateral ptosis or ranial nerve II palsy
prophylactic treatment of migraines?
abortive treatment of migraines
dull aching headaches associated w/ muscle contraction
are tension headaches or migraines more common in childhood?
unilateral frontal or facial pain accompanied by conjunctival erythema, lacrimation and nasal congestion
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?
demyelinating polyneuritis w/ ascendnig weakness, areflexia and normal sensation
what is the most common associated infection agen of guillain barre
what kind of immune response is guillain barre
cell mediated immune response
what does lumbar puncture show in guillain bar?
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?
autoimmune disorder presents w/ progressive weakness and diploplia?
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?
characteristic increasing weakness
diagnosis of myasthenia
tensilon test ?edrophonium chloride
presence of AChR antibody titrs
myasthenia gravis treatment of choice