Narcolepsy caused by acute disseminated encephalomyelitis.
BACKGROUND
Narcolepsy with cataplexy is caused by a selective loss of hypocretin-producing neurons, but narcolepsy can also result from hypothalamic and rostral brainstem lesions.


METHODS
We describe a 38-year-old woman with severe daytime sleepiness, internuclear ophthalmoplegia, and bilateral delayed visual evoked potentials. Her multiple sleep latency test results demonstrated short sleep latencies and 4 sleep-onset rapid eye movement sleep periods, and her cerebrospinal fluid contained a low concentration of hypocretin. Magnetic resonance imaging showed T2 and fluid-attenuated inversion recovery hyperintensity along the walls of the third ventricle and aqueduct, which are consistent with acute disseminated encephalomyelitis.


RESULTS
After treatment with steroids, this patient's subjective sleepiness, hypersomnia, and hypocretin deficiency partially improved.


CONCLUSIONS
Autoimmune diseases such as acute disseminated encephalomyelitis can produce narcolepsy. Most likely, this narcolepsy is a consequence of demyelination and dysfunction of hypocretin pathways, but direct injury to the hypocretin neurons may also occur.
Gledhill
Richard F
RF
Department of Neurology, Medical University of Southern Africa, Ga-Rankuwa Hospital, Pretoria, Republic of South Africa.
Bartel
Peter R
PR
Yoshida
Yasushi
Y
Nishino
Seiji
S
Scammell
Thomas E
TE
D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology*; D002352:Carrier Proteins / Q000134:cerebrospinal fluid; D004569:Electroencephalography; D004673:Encephalomyelitis, Acute Disseminated / Q000150:complications* / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins; D008279:Magnetic Resonance Imaging; D009290:Narcolepsy / Q000209:etiology*; D009479:Neuropeptides / Q000134:cerebrospinal fluid; D000068797:Orexins; D017286:Polysomnography; D011859:Radiography
D002352:Carrier Proteins; D047908:Intracellular Signaling Peptides and Proteins; D009479:Neuropeptides; D000068797:Orexins