Neuro Flashcards
Cracked pot sound on percussion of the skull indicating hydrocephalus
MacEwen sign
Malformation consisting of cystic expansion of the 4th ventricle int eh posterior fossa and midline cerebellar hypoplasia, resulting from a developmental failure of the roof of the 4th ventricle during embryogenesis
Dandy-walker malformation
Brain weight:volume ratio >98th percentile for age (≥ 2 SD above the mean)
Megalencephaly
Most common megalencephalic syndrome; cerebral gigantism
Sotos syndrome
Cerebral hemispheres are absent or represented by membranous sacs with remnants of frontal, temporal, or occipital cortex dispersed over the membrane
Hydranencephaly
EEG findings in typical absence seizure
3 Hz spike-and-slow wave discharges
EEG findings in atypical absence seizure
1-2 Hz spike-and-slow wave discharges
__ seizures often occur at night and can be very numerous and brief
frontal lobe
EEG in patients with partial seizures usually show __ in the lobe where the seizure originates
focal spikes or sharp waves
Most common benign epilepsy syndrome with partial seizures
Child wakes up at night owing to simple partial seizures causing buccal and throat tingling and tonic or clinic contractions of one side of the face, with drooling and inability to speak but with preserved consciousness
Benign childhood epilepsy with centrotemporal spikes (BECTS)
EEG findings in BECTS
broad-based centrotemporal spikes that are markedly increased in frequency during drowsiness and sleep
Most common cause of surgically remediable partial epilepsy in adolescents and adults
Preceded by febrile seizures
Mesial temporal sclerosis
Form of chronic encephalitis that manifests with unilateral intractable partial seizures, epilepsia partialis continua, and progressive hemiparesis of the affected side, with progressive atrophy of the contralateral hemisphere
Rasmussen encephalitis
Typical absence seizures usually start at
5-8 yr
Consists of myoclonic and other seizures during the 1st yr of life, with generalized 3 Hz spike-and-slow wave discharges
Benign myoclonic epilepsy of infancy
Most common generalized epilepsy in young adults
Starts in adolescence with one or more of the ff: myoclonic jerks in the morning, often causing the patient to drop things; generalized tonic clonic or clonic-tonic-clonic seizures upon awakening; and juvenile absences
EEG shows generalized 4-5 Hz polyspike-and-slow wave discharges
Juvenile myoclonic epilepsy (Janz syndrome)
Starts during the first 2 mo of life with severe myoclonic seizures and burst suppression pattern on EEG
Early myoclonic infantile encephalopathy (EMIE)
Starts during the first 2 mo of life
Manifests as tonic seizures and is usually caused by brain malformations or syntaxin binding protein 1 mutations
Same EEG pattern as EMIE
Early epileptic infantile encephalopathy (EEIE) or Ohtahara syndrome
Starts as focal febrile status epilepticus and later manifests myoclonic and other seizure types
Severe myoclonic epilepsy of infancy or Dravet syndrome
Starts between the ages of 2 and 12 mo and consists of a triad of infantile spasms that usually occur in clusters, developmental regression and a typical EEG picture called hypsarrhythmia
West syndrome
Typically starts between the age of 2 and 10 yr and consists of a triad of:
- Developmental delay
- Multiple seizure types that as a rule include atypical absences and myoclonic, astatic and tonic seizures
- EEG findings 1-2 Hz spike-and-slow waves, polyspike bursts in sleep and a slow background in wakefulness
Lennox-Gestaut Syndrome
First line treatment of absence seizures
Ethosuximide
or valproic acid
First line treatment of complex partial seizures
oxcarbazepine
or carbamazepine
Drug of choice for juvenile myoclonic epilepsy
valproate
lamotrigine
Drug of choice for Lennox-Gestaut syndrome
valproate
topiramate
lamotrigine
rufinamide
Drug of choice for infantile spasms
ACTH
Drug of choice for West syndrome
ACTH
The most important factor for seizure relapse
abnormal EEG before medication is discontinued
Seizures that occur __ after AEDs are completely discontinued indicate relapse, and resumption is usually warranted
> 2-3 mo
EEG manifestations of status epilepticus Stage I Stage II Stage III Stage IV
Stage I: initial distinct electrographic seizures
Stage II: waxing and waning electrographic seizures
Stage III: continuous electrographic seizures
Stage IV: Continuous ictal discharges punctuated by flat periods
Periodic epileptiform discharges on flat background