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From the Sleep Disorders Center (Drs. Boeve, Silber, and Olson), Department of Neurology (Drs. Boeve, Silber, Kokmen, and Petersen), Department of Psychology and Psychiatry (Drs. Ferman, Smith, and Ivnik), Department of Laboratory Medicine and Pathology (Dr. Parisi), and Division of Pulmonology and Internal Medicine (Dr. Olson), Mayo Clinic, Rochester, MN.
Address correspondence and reprint requests to Dr. Bradley F. Boeve, Mayo Clinic, 200 First Street SW, Rochester, MN 55905.
Background: REM sleep behavior disorder (RBD) has been reported with various neurodegenerative disorders, most frequently in disorders with Lewy body pathology. RBD often precedes the onset of PD, and a recent prospective study showed that 38% of patients with RBD eventually developed PD.
Methods: We identified 37 patients with degenerative dementia and a history of bursts of vigorous movement of the arms and legs with vocalization during sleep and associated with dream recall. Patients with and without two or more signs of parkinsonism were compared. Clinical, laboratory, and neuropsychometric features were analyzed, and criteria for the clinical diagnosis of dementia with Lewy bodies (DLB) were applied to all patients.
Results: Thirty-four of the 37 patients were male with mean age at onset of 61.5 years for RBD and 68.1 years for cognitive decline. RBD commenced before or concurrently with dementia in all patients but two. Parkinsonism (two or more signs) occurred in 54% of the sample (20/37), with a mean age at onset of 69.1 years. Polysomnography (PSG) confirmed RBD in all patients studied. Neuropsychological testing demonstrated impaired perceptual-organizational skills, verbal fluency, and marked constructional dyspraxia in more than one-half the patients. There were no statistically significant differences in the frequency of clinical features or in neuropsychological performance between patients with and without parkinsonism. Thirty-four patients (92%) met criteria for clinically possible or probably DLB. Three patients were autopsied; all had limbic with or without neocortical Lewy bodies.
Conclusions: We report a group of predominantly male patients with a characteristic association of RBD and degenerative dementia. The clinical and neuropsychometric features of the groups of patients with and without parkinsonism are similar. We hypothesize that the underlying pathology in these patients is DLB.
Supported in part by National Institute on Aging grant AG08031.
Presented in part at the 49th annual meeting of the American Academy of Neurology, Boston, MA, April 1997.
Received November 21, 1997. Accepted in final form April 1, 1998.
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