Synucleinopathy pathology and REM sleep behavior disorder plus dementia or parkinsonism.
OBJECTIVE
To determine if synucleinopathy pathology is related to REM sleep behavior disorder (RBD) plus dementia or parkinsonism.


METHODS
The clinical and neuropathologic findings were analyzed on all autopsied cases evaluated at Mayo Clinic Rochester from January 1990 to April 2002 who were diagnosed with RBD and a neurodegenerative disorder. Ubiquitin and/or alpha-synuclein immunocytochemistry was used in all cases. The clinical and neuropathologic diagnoses were based on published criteria.


RESULTS
Fifteen cases were identified (14 men). All had clear histories of dream enactment behavior, and 10 had RBD confirmed by polysomnography. RBD preceded dementia or parkinsonism in 10 (66.7%) patients by a median of 10 (range 2 to 29) years. The clinical diagnoses included dementia with Lewy bodies (DLB) (n = 6); multiple-system atrophy (MSA) (n = 2); combined DLB, AD, and vascular dementia (n = 1); dementia (n = 1); dementia with parkinsonism (n = 1); PD (n = 1); PD with dementia (n = 1); dementia/parkinsonism/motor neuron disease (n = 1); and AD/Binswanger's disease (n = 1). The neuropathologic diagnoses were Lewy body disease (LBD) in 12 (neocortical in 11 and limbic in 1) and MSA in 3. Three also had argyrophilic grain pathology. In the LBD cases, concomitant AD pathology was present in six (one also with Binswanger's pathology, and one also with multiple subcortical infarcts).


CONCLUSIONS
In the setting of degenerative dementia or parkinsonism, RBD often reflects an underlying synucleinopathy.
Boeve
B F
BF
Department of Neurology, Mayo Clinic, Rochester, Minneapolis, MN 55905, USA. bboeve@mayo.edu
Silber
M H
MH
Parisi
J E
JE
Dickson
D W
DW
Ferman
T J
TJ
Benarroch
E E
EE
Schmeichel
A M
AM
Smith
G E
GE
Petersen
R C
RC
Ahlskog
J E
JE
Matsumoto
J Y
JY
Knopman
D S
DS
Schenck
C H
CH
Mahowald
M W
MW
D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D003704:Dementia / Q000150:complications / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000378:metabolism*; D020734:Parkinsonian Disorders / Q000150:complications / Q000473:pathology*; D020187:REM Sleep Behavior Disorder / Q000150:complications / Q000378:metabolism* / Q000473:pathology*; D012189:Retrospective Studies; D012737:Sex Factors; D051843:Synucleins; D051844:alpha-Synuclein
D009419:Nerve Tissue Proteins; C497604:SNCA protein, human; D051843:Synucleins; D051844:alpha-Synuclein