New Onset Seizure Neurology Rotation Lecture Series Last Updated by Lindsay Pagano Summer 2013

Case Presentation 4 year old female presents with first time seizure parents witnessed her playing and then “falling out” and shaking all over for ~20-30 seconds; eyes rolled upward tired and cranky afterwards noted to have URI symptoms that started last night, tactile fever overnight PE: rhinorrhea, congestion; Neuro exam normal differential?

Taking a Seizure History Was it a seizure? –Onset, course of the spell, clinical appearance after the spell –Incontinence, tongue biting, eyes closed or open, how did they fall Where do they localize? –Generalized versus Partial (focal): where is the seizure starting Pick apart what happened first Eye movements After the seizure –clinical changes: VS changes, breathing patterns, mental status –lab changes –Todd’s paralysis/paresis Why did they seize in the first place? –noncompliance with medication –medical illness with known epilepsy –toxins, exposures –trauma –metabolic derangements

Seizure Classification Generalized –tonic-clonic (GTC) –tonic –atonic –absence –myoclonic Partial (focal) –simple –complex –secondary generalization

Epilepsy Syndromes JME, Juvenile Myoclonic Epilepsy –Traid: absence + GTCs + myoclonic –Diagnosis: clinical + EEG (sleep deprived, with photic stim) –Treatment: keppra, depakote BRE, Benign Rolandic Epilepsy –Partial seizures, often nocturnal –Interictal EEG with centrotemporal spikes –+/- treatment Infantile Spasms –Onset at 3-8 months old –new onset seizures: neck/trunk flexion, and shoulder abduction, UE extension; contraction phase followed by tonic phase –etiology: idiopathic, symptomatic (70%), cryptogenic –evaluation: EEG with hypsarrythmia; MRI pending etiology –treatment: vigabatrin (sabril), ACTH, topiramate (topamax) –prognosis: pending etiology (West syndrome, Lennox-Gastaut syndrome) Febrile Seizures

Afebrile Seizure Workup History Full Neurologic exam EEG +/- MRI –MRIbrain with and without contrast, Khalil protocol

EEG to know Absence seizure: 3 Hz spike and wave, generalized

While waiting for the Neurology appointment… Treatment Don’t start until there has been more then 1 unprovoked seizure Prophylactic –Generalized: Keppra Start at 20/kg divided BID for one week, then increase to 30/kg divided BID Side effects –initial fatigue that dissipates –irritability  B6 –Focal: Trileptal Start at 8-10 mg/kg/day divided BID for one week, then increase to 20/kg Side effect: hyponatremia –Absence: ethosuximide Start at 15mg/kg/day for one week, then increase to 20/kg Abortive –Diastat: PRN convulsive seizures > 5 minutes or clusters > 5 minutes 2-5 years old: 0.5 mg/kg 6-11 years old: 0.3 mg/kg ≥ 12 years old: 0.2 mg/kg

PREP question A 6 year old boy presents for evaluation due to an episode of screaming and confusion at night. The boy’s parents heard him scream in his room, and when they went to him, he exhibited rapid twitching of his left arm and hand, stiffening of his left leg, rooled back eyes, and some blinking of both eyes. He was incoherent and minimally responsive for 5 to 10 minutes. After the episode, he was weak on the left side of his body. By morning, he had returned to a normal status. Of the following, the MOST likely diagnosis is: A.Benign rolandic epilepsy B.Juvenile myoclonic epilepsy C.Night terrors D.Nocturnal frontal lobe epilepsy E.Rapid eye movement sleep behavior disorder

A. Benign rolandic epilepsy BRE (aka BECTS) –Partial seizure –Otherwise healthy –Interictal EEG with centrotemporal spikes –+/- treatment (seizures infrequent); if treating, AEDs for partial seizure (eg trileptal) Regarding the other choices: B. Juvenile myoclonic epilepsy: triad of absence + morning myoclonic jerks + morning GTCs C. Night terrors: shouldn’t have focal neuro defecits or repetitive movements D. Nocturnal frontal lobe epilepsy: complex, stereotyped dystonic movements and vocalizations E. REM sleep behavior disorder: loss of typical REM paralysis  acting out dreams; uncommon in children, more common with children with autism