2014 年3月18日 山中克郎教授による教育症例カンファレンス
ナルコレプシー 脱力発作 睡眠発作 悪夢
諏訪中央病院 佐藤泰吾先生 総合診療部
小川朋子ら 炭酸リチウムが奏効した反復性過眠症の 1 例. 臨床神経, 2010, 50: 700-703.
DAUVILLIERS, Y., et al. CSF hypocretin-1 levels in narcolepsy, Kleine-Levin syndrome, and other hypersomnias and neurological conditions. J Neurol Neurosurg Psychiatry, 2003, 74.12: 1667-1673.
ARNULF, I., et al. Kleine–Levin syndrome: a systematic review of 186 cases in the literature. Brain, 2005, 128: 2763-2776.
The details of 186 patients from 139 articles were compiled. Primary KLS cases (n = 168) were found mostly in men (68%) and occurred sporadically worldwide. The median age of onset was 15 years (range 4–82 years, 81% during the second decade) and the syndrome lasted 8 years, with seven episodes of 10 days, recurring every 3.5 months (median values) with the disease lasting longer in women and in patients with less frequent episodes during the first year. It was precipitated most frequently by infections (38.2%), head trauma (9%), or alcohol consumption (5.4%). Common symptoms were hypersomnia (100%), cognitive changes (96%, including a specific feeling of derealization), eating disturbances (80%), hypersexuality (43%), compulsions (29%), and depressed mood (48%). In 75 treated patients (213 trials), somnolence decreased using stimulants (mainly amphetamines) in 40% of cases, while neuroleptics and antidepressants were of poor benefit. Only lithium (but not carbamazepine or other antiepileptics) had a higher reported response rate (41%) for stopping relapses when compared to medical abstention (19%).
ARNULF, Isabelle, et al. Kleine–Levin syndrome: a systematic study of 108 patients. Ann Neurol, 2008, 63: 482-493.
Novel predisposing factors were identified including increased birth and developmental problems (odds ratio, 6.5). Jewish heritage was overrepresented, and five multiplex families were identified. Human leukocyte antigen typing was unremarkable. Patients were 78% male (mean age at onset, 15.7 6.0 years), averaged 19 episodes of 13 days, and were incapacitated 8 months over 14 years. The disease course was longer in men, in patients with hypersexuality, and when onset was after age 20. During episodes, all patients had hypersomnia, cognitive impairment, and derealization; 66% had megaphagia; 53% reported hypersexuality (principally men); and 53% reported a depressed mood (predominantly women). Patients were remarkably similar to control subjects between episodes regarding sleep, mood, and eating attitude, but had increased body mass index. We found marginal efficacy for amantadine and mood stabilizers, but found no increased family history for neuropsychiatric disorders.
SCHENCK, Carlos H.; ARNULF, Isabelle; MAHOWALD, Mark W. Sleep and sex: what can go wrong? A review of the literature on sleep related disorders and abnormal sexual behaviors and experiences. Sleep, 2007, 30: 683.
In our meta-analysis, we observed that the changes in sexual behavior shared some similarities with the disturbed eating behavior, such as increased quantities (increased frequency of masturbation or of sexual intercourse, demanding intercourse several times daily45), compulsions (with active and uncontrolled research of sex), lack of judgment in the choice of sexual partner (sexual advances were made to religious sisters, to the patient’s daughter46 or sister,47-49 to a nurse “who is said to have been old enough to be his grandmother,” 44 and in three cases to other males by otherwise non-
homosexual male patients46,50,51), inattention to the environment (such as masturbating in public), and absence of self-awareness of the inappropriateness of the behavior.