Narcolepsy caused by acute disseminated encephalomyelitis.

Abstract

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.

PATIENT:

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.

• Authors: Richard F Gledhill, Peter R Bartel, Yasushi Yoshida, Seiji Nishino, Thomas E Scammell
• Journal: Archives of neurology (Arch Neurol) Vol. 61 Issue 5 Pg. 758-60 (May 2004) ISSN: 0003-9942 [Print] United States
• PMID: 15148155 (Publication Type: Case Reports, Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Carrier Proteins
• Intracellular Signaling Peptides and Proteins
• Neuropeptides
• Orexins

Topics

• Adult
• Brain (diagnostic imaging, pathology)
• Carrier Proteins (cerebrospinal fluid)
• Electroencephalography
• Encephalomyelitis, Acute Disseminated (complications, pathology, physiopathology)
• Female
• Humans
• Intracellular Signaling Peptides and Proteins
• Magnetic Resonance Imaging
• Narcolepsy (etiology)
• Neuropeptides (cerebrospinal fluid)
• Orexins
• Polysomnography
• Radiography