ABSTRACT

Case Presentation ................................................................................................... 273 Differential Diagnosis ............................................................................................ 274 Diagnostic Approach ............................................................................................. 275 Treatment Strategy ................................................................................................. 275 Long-Term Outcome .............................................................................................. 275 Pathophysiology/Neurobiology of Disease ............................................................ 278 Clinical Pearls ........................................................................................................ 278 Suggested Readings ............................................................................................... 279

A 10-year-old right-handed male without a signicant past medical history was referred for evaluation of possible nocturnal seizures. His seizures began at 7 years of age and have occurred almost exclusively during sleep, usually within 2 hours of falling asleep. The stereotyped episode involves rocking and bicycling motions, gagging, rolling of the eyes, and profuse sweating lasting approximately 2 minutes. These episodes have occurred on average three times per week, but recently have become more frequent. He has had two similar episodes that have occurred while awake. The patient’s previous evaluation included two routine sleep-deprived EEGs and a 1.5T noncontrast MRI of the brain, all of which were interpreted as normal. Other laboratory tests included a complete blood count and comprehensive metabolic prole, which were also normal. His physical and neurologic examination is unremarkable, but his family history is signicant for a maternal aunt and brother with epileptic seizures since childhood that were primarily nocturnal.