Recurrent Hypersomnias


Insufficient Sleep Sleep Fragmentation Narcolepsy Recurrent Hypersomnias

Non-Pharmalogic Strategies Pharmalogic Treatment Sleep Disorder Summary


Kleine-Levin Syndrome: Kleine-Levin Syndrome is a rare disorder, a form of recurrent hypersomnia, occurs primarily in adolescents males.The disorder is characterized by the occurrence of episodes of EDS, usually, but not invariably, accompanied by hyperphagia, aggressiveness, and hypersexuality lasting days to weeks and separated by asymptomatic periods of weeks or months. During symptomatic periods, individuals sleep up to 18 hours per day and are usually drowsy (often to the degree of stupor), confused, and irritable the remainder of the time. During symptomatic episodes, polysomnographic studies show long total sleep time with high sleep efficiency and decreased slow-wave sleep. MSLT studies demonstrate short sleep latencies and SOREMPS. The etiology of the syndrome remains obscure. Treatment with stimulant medication is usually only partially effective. Effects of treatment with lithium, valproic acid, or carbamazepine have been variable but generally unsatisfactory. Fortunately, in most cases, episodes become less frequent over time and eventually subside.

Menstrual-Related Hypersomnia: Another form of recurrent hypersomnia is menstrual-related periodic hypersomnia, in which EDS occurs during the several days before menstruation. The prevalence of this syndrome has not been well defined. Likewise, the etiology is not known, but presumably the symptoms are related to hormonal changes. Some cases of menstrual-related hypersomnia have responded to the blocking of ovulation with estrogen and progesterone (birth control pills).

Idiopathic Recurring Stupor: Numerous cases have been reported in which individuals (predominately middle-aged males) are subject to stuporous episodes lasting from hours to days, in the absence of obvious toxic, metabolic, or structural cause. The individuals are normal between episodes, which occur unpredictably. Some electroencephalogram (EEG) data collected during symptomatic episodes have manifested fast background activity in the 13 to 16 Hz range. Several of these patients have been shown to have elevated plasma and CSF levels of endozepine-4, an endogenous ligand with affinity for the benzodiazepine recognition site at the GABAA receptor. Administration of flumazenil, a benzodiazepine antagonist, has produced transient awakening with normalization of the EEG. In some cases, the episodes resolved spontaneously after several years. Similar cases have been reported in children.

NERVOUS SYSTEM DISORDERS AND EDS: EDS may be associated with various disorders of the central or peripheral nervous systems. It is a clinical feature of many toxic or metabolic encephalopathic processes. These disorders often present with other symptoms and signs, but EDS may dominate the clinical picture, particularly in chronic cases. Structural brain lesions, including strokes, tumors, cysts, abcesses, hematomas, vascular malformations, hydrocephalus, and multiple sclerosis plaques, are known to produce EDS. Somnolence may result either from direct involvement of discrete brain regions (especially the brainstem reticular formation or midline diencephalic structures) or because of effects on sleep continuity (for example, nocturnal seizure activity or secondary sleep-related breathing disorder [SRBD]).