pediatric neurology Flashcards
complications of Topiramate in baby
cleft lip
frederich ataxia features
dysarthric speech, head thrusts w/ visual tracking, past-pointing on FNF test, disoragnization of rapid pronation-supination of hand, jerky and illegible handwriting, lurching gate, no DTR. presents at 2-16 yrs old
absence seizure presentation
abrupt onset/offset, w/ automatisms like jerks of arms and eye blinking, less than 30 sec, multiple daily
absence EEG
3 Hz symmetric spike or multiple spike and wave complexes
treatment for absence seizures
ethosuximide, valproate, lamotrigine
characteristic of alternating hemiplegia of childhood
episodic hypotonia, lateralized weakness, episodic dystonia lasting minutes to days with underlying global developmental delay
EEG of alternating hemiplegia of childhood
NORMAL
cause of alternating hemiplegia of childhood
mutation in gene relating to Na/K ATPase channel (ATP1A3); but NOT always, must diagnose clinically. can have spontaneous mutations causing dz
ATPase channel ATP1A3 associated with which dzs
alternating hemiplegia of childhood, rapid onset dystonia/parkinsonism, episodic ataxia, cerebellar ataxia, areflexia, pes cavus, optic atrophy, sensorineural hearing loss syndrome (CAPOS)
7 criteria for alt hemiplegia of childhood
- onset prior to 18mos
- repeated episodes R or L sides of body
- episodes B/L hemiplegia
- other paroxysmal disturbances like tonic/dystonic attacks, nystagmus, strabismus, dyspnea, other autonomic phenomena during hemiplegic attacks or in isolation
- immediate disapearance of sympons when sleeping with recurrence 10-20 min after waking
- underlying developmental delay, learning disability, neuro abnormalities, choreoathetosis, dystonia, ataxia
- no other disorder
symptoms preceding onset of alt hemiplegia of childhood
MC hypotonia, floppiness and abn eye mvmts
treatment for alt hemiplegia of childhood
sleep, can trial benzos (midazolam) or maybe flunarizine (blocks Ca channels)
characteristics of benign neonatal sleep myoclonus
starts in term babies <2 wks old, ONLY occur during sleep, involve ALL extremities. EEG normal. pt will outgrow
characteristics of benign rolandic epilepsy aka BECTS (benign epilepsy w/ centrotemporal spikes)
nocturnal focal or secondarily generalized seizures w/ diurnal partial seizures
onset b/w 7-8 yo, M>F. commonly have motor impairment of face and motor aphasia with no impaired consciousness
seizure characteristics in BRE
most often at night, with clonic movements of mouth w/ salivation and gurgling sounds in throat w/ 2/2 generalization. UNCOMMON to have postictal confusion or amnesia. if they do occur during the day they are typically motor aphasia with no impaired consciousness
seizures are NOT frequent, and do not often go into SE
treatment of BRE
sometimes you dont treat; if you do use: Keppra, lamotrigine, phenobarb, topamax, carbamazepine, oxcarb, valproic acid.
EEG findings BRE (interictal)
high amplitude diphasic spike w/ predominent slow wave. spike or sharp waves appear singly or in groups in midtemporal T3/T4 and central region C3/C4
development of BRE
most kids have remission by teenage years, do have delay in visual, auditory processing and memory deficits; sometimes these improve with remission of seizures
anoxic reflexive epilepsy
kiddos can have breath holding spells and tonic posturing and convulsions (non epileptic) but then can progress to true epilepsy and have anocix reflecive epilepsy which can last longer than minutes
etiology of pallid infantile syncope aka breath holding spells
ocular pressure, you can even reproduce it by giving ocular compression which would then cause bradycardia, asystole and EEG slowing; check for underlying cardiac abnormalities and iron levels (mc in anemic kids)
test to get for breath holding spells
EKG and CBC to check Hgb
panayiotopoulos syndrome
common childhood epilepsy, ages 3-6, M=F, seizures last 20-60 mins, autonomic characteristic (pale, vomit, sweat, drool, pupils dilate) followed by atonic or TC movements, sometimes only one in lifetime or several per yr, MC occur in sleep
EEG findings in panayiotopolus syndrome
often normal otherwise paroxysmal spike and sharp waves MC in occipital region but can be anywhere
late onset version of panayiotopolus
is called gastaut which is brief seizures with mainly visual symptoms (hallucinations, illusions, amaurosis) then hemiclonic convulsions followed by postictal HAs.
