Neuro Flashcards
tension HA
emotional distress, fatigue, lack of sleep, stressors.
eipsodic, worsen throughtout day, band around head, tenderness of posterior M of neck
migraine
can be atypical: cyclical vomiting, abd migraine, paroxysmal vertigo.
throbbing, photophobia, phonophobia, N/V
precipitate by stress, light, odor, food
relieved by sleep
RF: fx
classic- has aura - visual sx, speech changes, sensory abn like paresthesias
common- w/o aura. most common type in children. unilateral, usually frontal or temporal.
basilar artery migran or hemiplegic migrane cause acute ataxia
concerning sx of HA
- relieved by vomiting after lying down- incr ICP
- sudden onset- intracranial hemorrhage
- awaken from sleep- incr ICP
- fever + photophobia- infection
- worsen with cough or valsava- incr ICP
- progressively worsen- serious
ataxia DD
post infectious cerebellitis, infectious cerebellitis, medication or toxin, IC mass, opsoclonus- myoclonus syndrom, migraine HA, hydrocephalus, metabolic disease, neurodegen disease, psych illness
post infectious cerebellitis aka acute cerebellar ataxia
most common cause of ataxia in kids. only in 1-3yo. AI - cerebellar demyelination
occur wks after viral infection
onset- sudden + ataxia, vomit, nystagmus in 50%, dyarthria in some
CSf normal or pleocytosis, protein elevated
most recover in few mo
usually no fever or systemic sx
infectious cerebellitis
viral or bacterial. often febrile, AMS, maps, enterovirus EBV or bacteria that cause meningitis
ataxia localizing tumor
most likely in cerebellum or frontal lobe
opsoclonus-myoclonus syndrome
paraneoplastic syndrome- neuroblastoma. 6mo-3yo. ataxia + myoclonus + opsoclonus( jerky conjugate mvt of eyes)
hydrocephalus ataxia
insidious onset, chronic decr in coordination. HA/V
cerebellar mass
coordination, precision and balance impacted. no impact on initiation of mvt. bc close to 4th ventricle, cause obstructive hydrocephalus.
1) vermis- dysarthria, truncal ataxia, gait abn
2) cerebellar hemispheric lesion-IL lim abn, nystagmus, tremor/ dysmetria, spares speech
fall/look toward side of lesion
3) deep cerebellar nuclei- tremor, myoclonus, opsoclonus
LP + incr ICP
LP is CI bc cause brain herniation
infratentorial lesion
cerebellar signs, incr ICP
brain tumor
most common solid tumor in children and 2nd most common form of childhood ca behind leukemia. highest ca childhood death.
most common 10 is supratentorial.
RF: radioation exposure, genetic syndrome (tuberous sclerosis, NF)
most common medulloblastoma and juvenile pilocytic astrocytoma are each 20%.
brain stem glioma
resection for low grade. prog variable
ependymomas
from 4th ventricle. cause hydrocephlus. resection + rad. 50% 5y survival
astrocytoma of cerebellum
best prog of infratentorial tumor in kids. often cystic. resection. rad if high grad, 90% 5y survival with complete resection
medulloblastoma
most common pediatric tumor. malig that spread through CNS - metastasize to extra cranial site. resection + rad+ chemo.
prog dep on size and spread
hypotonic infant types and PE
1) central hypotonia- dysfunc of UMN
2) peripheral hypotonia- dysfunc of LMN
PE: weak cry, decr spontaneous mvt, frog leg posture, M contractures
peripheral hypotonia
associated with decr fetal mV and breech presentation.
consciousness unaffected. M bulk and DTR decr
etio
1) serum CK
2) DNA test for SMA
3) EMG - myathenic D
4) M bx
central hypotonia
associate with sz in neonatal period, altered lvl of consciousness, incr DTR, ankle clonus
etio:
1) CT to r/o CNS injury or congenital malformation
2) electrolyte: Ca, Mg, MH3, lactate, pruvate to r/o metabolic D
3) high resolution chromosome studies- FISH for genetic D(prader willi)
acute life threatening causes of hypotonia etio
sepsis, meningitis, acute metabolic D
spinal muscular atrophy (SMA)
anterior horn cell degeneration
P: hypotonia, weakness, weak cry, tongue fasiculations, bell shaped chest, frog leg posture, nomral extraocular mvt and sensory
type I-infantile 3yo
etio: AR -C5-survivial MN gene SMN1 mut –> degeneration and loss of anterior horn MN
dx: DNA test -90%, M bx show atrophy
tx: supportive. no cure. gastrotomy tube feeding. physical therapy
prog: survive till type I- 1yo. II-adolescent III-adult.
infantile botulism
bulbar weakness and paralysis. 2/2 ingest C. botulinum spore and absorption of botulinum toxin-in contaminated honey
toxin prevent presynaptic release of ach.
time line
1) onset 12-48 post ingestion.
2) consitmation = 1st sx
3) neuro sx follow- weak cry suck, loss of previous motor milestones, ophthalmoplegia, hyporeflexia
4) paralysis symmetric and descending. diaphragmatic paralysis sometimes
dx: hv, neuro exam, EMG-ancremental response during high freq stimulation
tx: supportive NG feed, ventilation. botulism ig improve course. ab CI = worsen course
prog- excellent
congenital myotonic dystrophy
can’t relax contracted M
AD- C19-trinucleotide repeat, transmit though affected mother in 90% cases
P: antenatal hx- poyhydramnios+ poor swallowing, neonatal-weakness, hypotonia, poor feeding and respiratory problem
PE: facila diplegia (bl weakness), hypotonia, areflexia, arthrogyrposis
myotonia dev after 5yo.
adult-myotonic facies, can’t release rip after handshaking, ptosis
CMD dx, tx, prog
suspect in all infants with hypotonia. examine mom. DNA test
Tx: supportive
prog-MR 50-65. feeding problem improve
hydrocephalus ex vacuo
not true hydrocephalus. ventricular enlargement due to brain atrophy
acquired hydrocephalus
IV hemorrhage- most common in premie, bacterial meningitis, brain tumor
hydrocephalus signs , tx
incr head circumference
1) infants: large anterior and posterior fontanelle split sutures. sunset sing- tonic downward deviation of both eyes caused by P from enlarged 3rd V on upward gaze center in midbrain
2) older kids: sign of ICP: 6CN palsy, brisk DTR, downward plantar
tx: ventriculoperitoneal shunt
Spina bifida
any failure of bone fusion in posterior midline of vertebral column.
decr by prenatal folic acid. cause by valproate, phenytoin, methotrexate
dx: prenatal- AFP elevated in 80%, fetal sonography. after birth- PE, confirm by MRI
myelomingocele
herniation of SC and meninges though bony cleft. usually through lumbosacral area. 20x more common than meningocele
P: fluctuant midline mass, defect dep on lvl of lesion. associated with hydrocephalus /chiari type II in 90%, cervical hydrosyringomyelia, defect in neuronal migration, orthopedic problem, genitourinary defects
tx: urgent survical repair in 24hr to decr morbidity from infection and trauma
meningocele
herniation of only meninges. usually no neural deficits
P: fluctuatnt midline massfillled with CSF
surgical repair
SB occulta
no herniation through cleft.
-hairy pathch or dimple on back. no neural deficit
no tx needed
coma
in
breathing v coma
1) hypovent- opiate or sedative
2) hypervent- ketoacidosis (kussmael), neurogenic pul edema, midbrain injury
3) Cheyne stokes- alternating apnea and hyperpneas- bilateral cortical injruy
4) apneuristic breathing- pause at full inspiration- pons damage
5) ataxic or atonal breathing- irreg respiration no matter. medullary injury, impending death
pupil v coma
1) unilateral dilated, unreactive pupil-uncal herniation
2) b/l diated, UR- irreversible brainstem injury or post ictal
3) bl constricted, R, opiate or pontine
caloric irrigation
deviate toward cold water. if not- pontine injury
febrile sz
non CNS cause. pt 6mo-6yo
simple febrile sz- 15min focal features, or recur w/in 24hr
Dx: hx, nl neuro exam, r/o CNS infection clinically or CSF. LP if suspect meningitis. no imaging or EEG
tx: 1st = none. few- antipyretics. many= daily anticonvulsant or abortive rectal diazepam
infantile spasm
west syndrome. onset 3-8mo. rare in >2yo. tubers sckerisus. brief mycologic jerks, , jackknifing sz or salaam sx.
dx: EEG- hyperarrhythmia pattern.
tx: adrenocortcotropic hormone (ACTH) IM, valproic acid, vigabatrin
prog-poor. severe MR
benign rolandic epilepsy
nocturnal partial sx with secondary generalization. most common partial epilepsy in kids. 3-13yo
AD
early morning- oral buccal manifestation- moaning, fronton, pooling saliva then spread to face and arm then tonic clonic
dx- eeg- spike and sharp wave in mid temporal and central regions
tx- valproic acid
prog- excellent
migraine
unilateral, pounding, start periorbital (kids can be bifrontal). AD. 5HT. w/o aura is most common type in kids
migraine equivalent- no HA but prolong, transient change in behavior, cyclic vomit,/ abd pain and paroxysmal vertigo
opththalmoplegic migraine- unilateral ptosis, CN III palsy
basilar artery migraine- vertigo tinnitus, ataxia, dysarthria
no obvious precipitating cause
improve by sleep. nl neuro exam
propranolol- prophylactic
tension HA
bifrontal, dull, diffuse, aching, M contracture. rare in
acute cerebellar ataxia of childhood
2/2 AI or postinfectious cause (varicella, influenze, EBV, myoplasma)
most common cause of ataxia in kids
onset 18mmo-7y. rare in >10yo
P: truncal ataxia, refuse to walk bc fear of falling, slurred speech and nystagmus, no fever
CT: normal. do to r/o tumor hemorrhage
tx: supportive. take 2-3mo
Guillain barre
ataxia, areflexia, ascending weakness, nl sensation. campylobacter jejuni. prodromal gastroenteritis. cell mediated immune response cross react with schwann cell mem. facial weakness in 50%
dx: LP- albuminocytologic dissociation- incr CSF protein wo incr cell count. EMG- decr N conduction veocity. spinal MRI in
miller fisher syndrome
ophthalmoplegia, ataxia, areflexia
sydenham chorea
st. vitus’ dance
AI + rheumatic fever. self limiting. present with chorea and emotional lability.
onset 5-13yo
cross react with ab to basal ganglia cells
follow 2-7mo after strep pharyngitis. restless, speech jerky, profusion of tongue- chameleon tongue, choleric hand- flexed. milkmaid’s grip- can’t maintain. no effect on gait and cog
DD: SLE, huntington, wilson
dx: elevated antistreptolysin O (ASO) anti DNase (ADB) , MRI - incr signal in caudate and putamen, single photon emission SPECT incr perfusion
tx: haloperidol, valproic acid,
prog- all recover, mo-2yo
gower’s sign
extend each leg then climb up thigh until upright. bc weak pelvic M. in DMD and BMD.
muscular dystrophy dx
CK high. EMG show polyphasic potential. normal conduction.
cardiac involvement in 50%. mild cog impairment in DMD not BMD>
M bx- M dystrophic pattern
DNA testing - deletion in 90% pt
tx: no cure. oral steroid transiently improve strength
myasthenia gravis
AI D progressiv weakness or diplopia
anti ACHR ab at NMJ
neonatal myathenia- transient weakness 2/2 placental transfer of maternal AChR ab in mo with myasthenia graves. see hypotonia, weakness, feeding problem
junile myathenia- 2/2 AChR ab formation. bilateral ptosis - most common presenting sign. incr weakness in day with repetitive activity, diplopia, DTR preserved. may have other AI D.
Dx: tensilon test - IV edrophonium chloride - cholinesterase inhibitor= transient improvement of ptosis. decremental response to N stimulation, AChR ab titers
tx: neonatal- tx sx bc self limiting. juvenile- cholinesterase inhibitor like pyridostigmine bromide. immunotherapy- corticosteroids, plasmapheresis, IVIG, thymectomy
prog: neonatal -resolv in 1-3wk
juvenile- 60% remission after thymectomy
