Hyperekplexia: report of a nonfamilial adult onset case associated with obstructive sleep apnea and abnormal brain nuclear tomography.
We report the unique occurrence of hyperekplexia and obstructive sleep apnea (OSA) in a 48-year-old male. Polysomnography and multiple sleep latency testing excluded cataplexy, which can be confused with startle attacks. A new imaging finding was a nuclear tomography abnormality in the left frontal lobe. We postulate that this finding may represent a functional cortical lesion of a descending pathway that normally inhibits the startle reflex. Serious complications of pharmacotherapy with clonazepam, the drug of choice for hyperekplexia, can be avoided by first evaluating for OSA.
Hochman
M S
MS
Division of Neurology, Baptist Hospital, Miami, Florida.
Chediak
A D
AD
Ziffer
J A
JA
D001921:Brain / Q000000981:diagnostic imaging; D002343:Carotid Artery, Internal / Q000503:physiopathology; D002998:Clonazepam / Q000627:therapeutic use; D004569:Electroencephalography; D005073:Evoked Potentials, Somatosensory; D005625:Frontal Lobe; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D017286:Polysomnography; D011530:Protriptyline / Q000627:therapeutic use; D011859:Radiography; D013216:Reflex, Startle; D012891:Sleep Apnea Syndromes / Q000175:diagnosis* / Q000188:drug therapy; D012894:Sleep Stages; D015899:Tomography, Emission-Computed, Single-Photon
D011530:Protriptyline; D002998:Clonazepam