It is also noted that some symptomatic EDS cases (with Parkinson diseases and the thalamic infarction) appeared, but they are not linked with hypocretin ligand deficiency. Reduced CSF hypocretin-1 levels were seen in most symptomatic narcolepsy cases of EDS with various etiologies and EDS in these cases is sometimes reversible with an improvement of the causative neurological disorder and an improvement of the hypocretin status. CSF hypocretin-1 measurement were also carried out in a limited number of symptomatic cases of narcolepsy/EDS, including narcolepsy/EDS associated with tumors ( n = 5), head trauma ( n = 3), vascular disorders ( n = 5), encephalopathies ( n = 3), degeneration ( n = 30), demyelinating disorder ( n = 7), genetic/congenital disorders ( n = 11), and others ( n = 2). Several cases of symptomatic cataplexy (without EDS) were also reported and in contrast, these cases appear to be often associated with nonhypothalamic structures. These include cases with HLA negative and/or late onset, and cases in which the occurrences of the narcoleptic symptoms are parallel with the rise and fall of the causative disease.Ī review of these cases (especially those with brain tumors) illustrates a clear picture that the hypothalamus is most often involved. Although it is difficult to rule out the comorbidity of idiopathic narcolepsy in some cases, review of the literature reveals numerous unquestionable cases of symptomatic narcolepsy. EDS without cataplexy or any REM sleep abnormalities is also often associated with these neurological conditions, and defined as symptomatic cases of EDS. Of the 116 cases, 10 are associated with multiple sclerosis, one case of acute disseminated encephalomyelitis, and relatively rare cases were reported with vascular disorders ( n = 6), encephalitis ( n = 4) and degeneration ( n = 1), and heredodegenerative disorder (three cases in a family). As, several authors previously reported, inherited disorders ( n = 38), tumors ( n = 33), and head trauma ( n = 19) are the three most frequent causes for symptomatic narcolepsy. To date, we have counted 116 symptomatic cases of narcolepsy reported in literature. We define symptomatic narcolepsy as those cases that meet the International Sleep Disorders Narcolepsy Criteria, and which are also associated with a significant underlying neurological disorder that accounts for EDS and temporal associations. The symptoms of narcolepsy can also occur during the course of other neurological conditions (i.e., symptomatic narcolepsy). Recently, it was discovered that the pathophysiology of (idiopathic) narcolepsy–cataplexy is linked to hypocretin ligand deficiency in the brain and cerebrospinal fluid (CSF), as well as the positivity of the human leukocyte antigen (HLA) DR2/DQ6 (DQB1*0602). The disease is characterized by excessive daytime sleepiness (EDS), cataplexy, and other abnormal manifestations of REM sleep, such as sleep paralysis and hypnagogic hallucinations. Human narcolepsy is a chronic sleep disorder affecting 1:2,000 individuals.
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