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The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://www.neurology.org/content/72/15/1296.full.html
Neurology ® is the official journal of the American Academy of Neurology. Published continuously
since 1951, it is now a weekly with 48 issues per year. Copyright © 2009 by AAN Enterprises, Inc. All
rights reserved. Print ISSN: 0028-3878. Online ISSN: 1526-632X.
ARTICLES
GLOSSARY
DSM-IV ⴝ Diagnostic and Statistical Manual of Mental Disorders, 4th edition; LBD ⴝ Lewy body dementia; MMSE ⴝ Mini-
Mental State Examination; MSA ⴝ multiple system atrophy; PD ⴝ Parkinson disease; PSG ⴝ polysomnogram; RBD ⴝ REM
sleep behavior disorder; UPDRS ⴝ Unified Parkinson’s Disease Rating Scale.
REM sleep behavior disorder (RBD) is characterized by a loss of the normal muscle atonia that
accompanies REM sleep.1,2 Affected patients have excessive motor activity such as punching,
kicking, or crying out in association with dream content. RBD is commonly associated with
neurodegenerative disorders characterized by ␣-synuclein deposition, including PD, multiple
system atrophy (MSA), and Lewy body dementia (LBD).2-5 Recent studies have suggested that
it may be related to ␣-synuclein-mediated degeneration of sleep-regulating nuclei in the brain-
stem, especially in the pontine tegmentum.6,7
In a substantial proportion of cases, RBD can occur before the development of dementia or
parkinsonism.5,8,9 This has major implications in the understanding of the pathophysiology of
PD and LBD, and suggests that an opportunity exists for neuroprotective measures in the
Remained Developed
Total disease-free disease Parkinsonism Dementia
No. 93 67 26 15 11
Mean age at 65.4 ⫾ 9.3 64.5 ⫾ 9.9 67.6 ⫾ 7.3 p⫽0.15 67.0 ⫾ 8.1 p⫽0.37 68.1 ⫾ 6.8 p⫽0.25
polysomnogram, y
Men/women (% female) 75/18 (19.6) 52/15 (22.4) 23/3 (11.6) p⫽ 0.38 12/3 (20.0) p⫽ 1.0 11/0 (0) p⫽ 0.11
Duration since 4.8 ⫾ 3.6 4.5 ⫾ 3.5 5.5 ⫾ 3.9 p⫽0.23 4.6 ⫾ 3.3 p⫽0.92 6.7 ⫾ 4.6 p⫽0.20
polysomnogram to last visit
well or disease onset
(mean ⴞ SD)
Duration since symptom 12.0 ⫾ 9.6 12.2 ⫾ 10.4 11.5 ⫾ 6.6 p⫽0.75 11.1 ⫾ 5.6 p⫽0.73 12.5 ⫾ 8.7 p⫽0.93
onset to last visit well or
disease (mean ⴞ SD)
cluded, 78 (83.9%) met the strictest inclusion crite- ism and dementia.2,8 Obtaining an accurate picture
ria of a full research evaluation follow-up of the risk of developing a neurodegenerative disor-
examination. Of the remaining 15 patients, 6 had an der is essential for accurate counseling of patients and
in-person follow-up supplemented by a more recent for planning of any potential neuroprotective trials.
telephone follow-up, 3 had in-person clinical However, definition of the risk of developing a neu-
follow-up only (with J.M.), and 6 had telephone rodegenerative condition has been published only in
follow-up only.
The mean age of participants was 65.4 years and
Figure Survival curve of all patients with
75 patients (80.4%) were men (table). The mean du- idiopathic REM sleep behavior
ration of disease from PSG diagnosis to last evalua- disorder (RBD) (A), and the survival
tion was 5.2 years. The mean duration between RBD curve of patients with idiopathic RBD
for whom a systematic research-
symptom onset and PSG diagnosis was 7.2 years.
based examination was performed by
Of the 93 patients included, 26 developed neuro- a movement disorders specialist (B)
degenerative disease, 15 developed parkinsonism,
and 11 developed dementia. Of the patients with
parkinsonism, 14 had a diagnosis of idiopathic PD,
and 1 was diagnosed with MSA. Of the patients with
dementia, 7 met clinical criteria for LBD, and 4 had
no clinical hallmarks of LBD and met clinical criteria
for AD. There were no significant differences in age
or sex between those who did or did not develop
disease, although there may have been a tendency
toward decreased risk of dementia in women (0/11
dementia patients, p ⫽ 0.11). There was no differ-
ence in RBD duration between those who did or did
not develop disease.
The life table survival curve is presented in figure
1. The estimated 5-year risk of developing neurode-
generative disease (parkinsonism or dementia) was
17.7%. The estimated 10-year risk of disease was
40.6%, and the 12-year risk was 52.4%. Linear re-
gression of the disease risk vs time suggested that
the risk of developing disease remained constant over
the follow-up period (R2 ⫽ 0.038, p ⫽ 0.54). In the
strict-inclusion cohort (n ⫽ 78), the estimated risk
was similar; 5-year risk was 19.5%, 10-year risk was
38.0%, and 12-year risk was 55.0%.
DISCUSSION It has been commonly noted that Disease outcome is defined as the development of parkin-
RBD often precedes the development of parkinson- sonism or dementia.
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