tional Certifications in Sleep
and Clinical Neurophysiology),
Sleep Medicine, and Clinical
Neurophysiology (EEG/Epilepsy
Monitoring). He was renamed
in 2013 to the Best Doctors in
America, selected as a Fellow
of the American Academy of
Neurology, and serves on the
ABRET Board of Directors and
AAN Continuum and Frontiers
in Epilepsy Editorial Boards.
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AASM Membership Sections Newsletter
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Issue # 4
American Academy
of Sleep Medicine
20
colepsy, Huntington disease, and spinocerebellar atrophy type 3, and
most previous pathologic reports of RBD patients have been derived
from single tertiary centers, or been limited by relatively small numbers,
leading to concerns about selection/reporting biases.
Boeve and colleagues from the International RBD Study Group and
a consortium of other North American, European, and Asian academic
centers recently reported on neuropathological data in 172 RBD patients
(the largest series to date) in Sleep Medicine. Autopsied RBD Patients
with or without concurrent neurological disorders were included. 143
(83%) were men, with mean age in the sixth decade. 82 (48%) had poly-
somnogram-confirmed RBD, while 52% had probable RBD with either
a typical dream enactment behavior history or positive validated RBD
questionnaire screening. RBD preceded cognitive, motor, or autonomic
impairments in 87 (51%) of patients. Pathologically, neurodegeneration
was seen in 170 (98.8%) of the cases. Synucleinopathy was by far the
most common neuropathology, seen in 160 (94%) patients, including
Lewy body disease in 77 (44.7%), combined Lewy body disease with
Alzheimer disease in 59 (34.3%), MSA in 19 (11%), and a small number
of cases with mixed neurodegenerative features (6 cases), PSP (2 cases),
brain iron accumulation type-1 with LBD (1 case), CJD/ALS (1 case),
and hypothalamic inflammatory or indeterminate lesions (2 cases).
In this pivotal work, Boeve and colleagues have confirmed that the
overwhelming majority of RBD patients have underlying synucleinop-
athy neurodegeneration, and further expanded the range of neuropa-
thologies known to underlie RBD. Importantly, this work supports the
hypothesis that RBD is a biomarker for synucleinopathy, and underscores
the importance of regular, longitudinal neurological follow-up and
appropriate counseling of RBD patients.
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