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Progressive Dementia and Hypersomnolence With Dream-Enacting Behavior
Oneiric Dementia
Jean E. Cibula, MD;
Stephan Eisenschenk, MD;
Michael Gold, MD;
Thomas A. Eskin, MD;
Robin L. Gilmore, MD;
Kenneth M. Heilman, MD
Arch Neurol. 2002;59:630-634.
Background Sleep disorders are associated with several types of degenerative dementias,
including Alzheimer and prion diseases. Animal models have demonstrated abolition
of rapid eye movement atonia, resulting in dream-enacting complex movements
termed oneiric behavior, and patients with fatal
familial insomnia may have vivid dreams that intrude on wakefulness.
Objective To describe a new form of progressive dementia with hypersomnia and
oneiric behavior.
Methods Neuropsychological and polysomnographic studies of a middle-aged woman
with a progressive dementia, excessive daytime sleepiness, and a vertical
gaze palsy.
Results Neuropsychological testing revealed decreased verbal fluency, impaired
attention and working memory, amnesia, poor recall, and bradyphrenia with
hypersomnia. Polysomnography revealed a rapid eye movement behavioral disorder
with complete absence of slow wave sleep. Prion protein analysis did not reveal
the mutation associated with fatal familial insomnia, and other diagnostic
test findings were unrevealing.
Conclusion Our patient had a previously unreported syndrome of progressive dementia
associated with rapid eye movement behavioral disorder and the absence of
slow wave sleep.
From the Departments of Neurology, University of Kentucky College of
Medicine, Lexington (Dr Cibula), McKnight Brain Institute at the University
of Florida College of Medicine, Gainesville (Drs Eisenschenk, Gilmore, and
Heilman), University of South Florida College of Medicine, Tampa (Dr Gold);
and Department of Pathology, University of Florida College of Medicine (Dr
Eskin), and the Malcolm Randall Veterans Administration Medical Center, Gainesville
(Dr Heilman).
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