[NARCOLEPSY AND LOSS OF EMOTIONAL TONUS IN CHILDREN].
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We report on a 45-year-old man with a history of multiple psychiatric admissions for a gait disorder and episodic weakness thought to be psychogenic who was subsequently diagnosed with status cataplecticus due to narcolepsy. The gait difficulties resolved with venlafaxine. This case demonstrates that status cataplecticus can be misdiagnosed as a psychogenic gait disorder.
In 1989, we described a new autosomic-recessive myelin-mutant rat that develops a progressive motor syndrome characterized by tremor, ataxia, immobility episodes (IEs), epilepsy, and paralysis. taiep is the acronym of these symptoms. The rat developed a hypomyelination, followed by demyelination. At an age of 7-8 months, taiep rats developed IEs, characterized electroencephalographically by REM sleep-like cortical activity. In our study, we analyzed the ontogeny of gripping-induced IEs between 5 and 18 months, their dependence to light-dark changes, sexual dimorphism, and susceptibility to mild stress. Our results showed that IEs start at an age of 6.5 months, with a peak frequency between 8.5 and 9.5 months. IEs have two peaks, one in the morning (0800-1000 h) and a second peak in the middle of the night (2300-0100 h). Spontaneous IEs showed an even distribution with a mean of 3 IEs every 2 h. IEs are sexually dimorphic being more common in male rats. The IEs can be induced by gripping the rat by the tail or the thorax, but most of the IEs were produced by gripping the tail. Mild stress produced by i.p. injection of physiological saline significantly decreased IEs. These results suggested that IEs are dependent on several biological variables, which are caused by hypomyelination, followed by demyelization, which causes alterations in the brainstem and hypothalamic mechanisms responsible for the sleep-wake cycle regulation, producing emergence of REM sleep-like behavior during awake periods.
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We report two cases of fibromyalgia occurring during the course of a long-standing narcolepsy/cataplexy. This association is probably not fortuitous and we propose an easy clinical procedure to search for narcolepsy/cataplexy in these patients. Narcolepsy/cataplexy is a treatable condition requiring drugs that in turn may improve in the long-term symptoms of fibromyalgia.
Lipocalin-type prostaglandin D synthase (L-PGDS) is a brain enzyme, which produces prostaglandin D(2), a substance with endogenous somnogenic effects. Using a standardized protocol for immunonephelometric determination of cerebrospinal fluid (CSF) L-PGDS levels, we show that CSF L-PGDS levels are significantly lower in 34 patients with excessive daytime sleepiness when compared with levels in 22 healthy controls. Thus, L-PGDS may represent the first neurochemical measure of excessive daytime sleepiness.
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This study determined the test-retest reliability of the polysomnographic findings in narcolepsy. The diagnosis of narcolepsy was based on clinical symptoms and polysomnographic signs. Control subjects were screened before participation and were split based on their screening multiple sleep latency test (MSLT) into high- and low-MSLT groups. Subjects completed two polysomnographic evaluations with at least 5 days between laboratory tests. Narcoleptics had lower sleep efficiencies and high stage 1% when compared to the low MSLT control group. They had more awakenings and less stage 2% than the control groups. Narcoleptics had a shorter latency to 1 when compared to the high-MSLT group but comparable to that of the low-MSLT group. Narcoleptics had a higher number of sleep-onset rapid eye movement periods (SOREMPs) than both control groups. The MSLT scores were stable across the two evaluations and showed a statistically significant correlation. Twenty-eight of the 30 narcoleptic subjects had two or more SOREMPs on reevaluation. None of the controls had multiple SOREMPs. Thus, multiple SOREMPs were shown to be a reliable finding in patients with narcolepsy.
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