Hypersomnolence and increased REM sleep with low cerebrospinal fluid hypocretin level in a patient after removal of craniopharyngioma.
Here we report a hypersomnolent girl with extensive hypothalamic damage after removal of a craniopharyngioma. The presence of a short sleep latency, sleep onset REM periods during a multiple sleep latency test (MSLT) and negative HLA DQB1*0602 typing suggested a diagnosis of symptomatic narcolepsy. Low cerebrospinal fluid hypocretin-1 level indicated destruction of hypocretin-producing neurons in the hypothalamus. An increased amount of REM sleep and a lack of REM sleep cyclicity documented by all-night polysomnography were different findings from previous reports of hypocretin-deficient idiopathic symptomatic narcolepsy. A more global hypothalamic lesion demonstrated by brain magnetic resonance imaging (MRI) after surgery seemed to cause marked disinhibition of REM sleep as well as hypersomnolence in this patient.
Tachibana
Naoko
N
Osaka Medical Center for Health Sciences and Promotion, Japan. nanaosaka@aol.com
Taniike
Masako
M
Okinaga
Takeshi
T
Ripley
Beth
B
Mignot
Emmanuel
E
Nishino
Seiji
S
D002648:Child; D003397:Craniopharyngioma / Q000601:surgery*; D006970:Disorders of Excessive Somnolence / Q000134:cerebrospinal fluid* / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins / Q000134:cerebrospinal fluid*; D009479:Neuropeptides / Q000134:cerebrospinal fluid*; D000068797:Orexins; D010911:Pituitary Neoplasms / Q000601:surgery*; D011183:Postoperative Complications; D012895:Sleep, REM / Q000502:physiology
C000596229:HCRT protein, human; D047908:Intracellular Signaling Peptides and Proteins; D009479:Neuropeptides; D000068797:Orexins