The Journal of Neuroscience, January 1, 1999, 19(1):248-257
Neuronal Degeneration in Canine Narcolepsy
J. M.
Siegel1,
R.
Nienhuis1,
S.
Gulyani1,
S.
Ouyang1,
M. F.
Wu1,
E.
Mignot2,
R. C.
Switzer3,
G.
McMurry1, and
M.
Cornford4
1 Veterans Administration Medical Center Sepulveda
and Department of Psychiatry and Brain Research Institute, University
of California Los Angeles School of Medicine, Neurobiology Research
151A3, Sepulveda, California 91343, 2 Department of
Psychiatry and Behavioral Sciences, Sleep Research Center, Richard
Lucas/Lab Surge Building, Palo Alto, California 94304, 3 NeuroScience Associates, Knoxville, Tennessee 37922, and
4 Department of Pathology, Harbor University of California
Los Angeles Medical Center, Torrance, California 90509
Narcolepsy is a lifelong illness characterized by persistent
sleepiness, hypnagogic hallucinations, and episodes of motor paralysis
called cataplexy. We have tested the hypothesis that a transient
neurodegenerative process is linked to symptom onset. Using the
amino-cupric silver stain on brain sections from canine narcoleptics,
we found elevated levels of axonal degeneration in the amygdala, basal
forebrain (including the nucleus of the diagonal band, substantia
innominata, and preoptic region), entopeduncular nucleus, and
medial septal region. Reactive neuronal somata, an indicator of
neuronal pathology, were found in the ventral amygdala. Axonal
degeneration was maximal at 2-4 months of age. The number of reactive
cells was maximal at 1 month of age. These degenerative changes precede
or coincide with symptom onset. The forebrain degeneration that we have
observed can explain the major symptoms of narcolepsy.
Key words:
narcolepsy; REM sleep; amygdala; basal forebrain; canine; amino-cupric silver; degeneration; cataplexy
Copyright © 1999 Society for Neuroscience 0270-6474/99/191248-10$05.00/0
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