A Patient with Intractable Attacks of Cataplexy

Abstract

A 29-year-old white man presents for evaluation of refractory daily generalized convulsions and drop attacks previously diagnosed as cataplexy. The patient's typical convulsive events started at 8 years of age, occur daily, and consist of jerking in all extremities with preserved consciousness. He has been on multiple anti-epileptic drugs (AEDs) including phenobarbital, valproic acid, and levetiracetam without decrease in event frequency. At age 17 he started having daily drop attacks consisting of full body weakness followed by collapse. The drop attacks, referred to as "cataplexy" by the patient, always occurred in public, have no clear trigger and have never resulted in injury to the patient. A typical drop attack is shown in Video 1.Magnetic resonance imaging of the brain, and 3 routine electroencephalograms (EEGs) without video monitoring were normal. A polysomnogram (PSG) followed by multiple sleep latency testing (MSLT) performed at an outside institution while on AEDs was interpreted as showing 5 out 5 sleep onset REM sleep periods (SOREMPs), with an average sleep latency of 1.2 minutes (data from PSG was not available). With complaints of daytime sleepiness, the patient was diagnosed with narcolepsy with cataplexy and started on sodium γ-hydroxybutyrate (GHB) with no improvement in drop attack frequency. Clomipramine and venlafaxine were added with no improvement noted.

An 18-Year-Old Woman with Prolonged Eyes Closed Unresponsiveness during Multiple Sleep Latency Testing

“…4,5 Presence of intact DTRs in all four extremities helped exclude generalized cataplexy, given that in typical cataplexy DTRs are absent. In addition, this patient's unresponsiveness lasted for nearly an hour, and typical cataplexy is not so prolonged.…”