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Induction of Paralysis and Visual System Injury in Mice by T Cells Specific for Neuromyelitis Optica Autoantigen Aquaporin-4
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Hypocretinergic dysfunction in neuromyelitis optica with coma-like episodes.

B Carlander1, T Vincent, A Le Floch

  • 1Department of Neurology, Hôpital Gui-de-Chauliac, Montpellier, France.

BMJ Case Reports
|June 21, 2011
PubMed
Summary

Devic's neuromyelitis optica (NMO), a CNS demyelinating disease, can present with severe dysautonomia and hypersomnia. A case study revealed a significant drop in hypocretin/orexin levels in cerebrospinal fluid during NMO relapses.

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Published on: November 23, 2013

Area of Science:

  • Neuroimmunology
  • Neurology
  • Sleep Medicine

Background:

  • Devic's neuromyelitis optica (NMO) is a severe autoimmune demyelinating disease targeting the central nervous system (CNS).
  • NMO primarily affects the optic nerves and spinal cord, and is often misdiagnosed as multiple sclerosis.
  • Dysautonomia and hypersomnia are less common but significant symptoms that can occur in NMO patients.

Purpose of the Study:

  • To report a unique case of relapsing NMO with severe dysautonomia and hypersomnia.
  • To investigate the association between NMO relapses and hypocretin/orexin levels in cerebrospinal fluid (CSF).

Main Methods:

  • Case report detailing clinical presentation, diagnostic workup, and treatment of a patient with relapsing NMO.
  • Cerebrospinal fluid (CSF) analysis including hypocretin/orexin level measurement.

Main Results:

  • The patient experienced recurrent episodes of severe dysautonomia and hypersomnia coinciding with NMO relapses.
  • A dramatic decrease in CSF hypocretin/orexin levels was observed during these relapses.
  • Hypocretin/orexin levels showed partial recovery between relapses.

Conclusions:

  • This case suggests a potential link between hypocretin/orexin system dysfunction and NMO relapses, particularly those involving severe dysautonomia and hypersomnia.
  • Further research is warranted to explore the role of hypocretin/orexin in the pathophysiology of NMO.
  • Monitoring hypocretin/orexin levels may offer insights into NMO disease activity and management.