Synucleinopathy pathology and REM sleep behavior disorder plus dementia or parkinsonism

Bradley F Boeve, M. H. Silber, Joseph E Parisi, Dennis W Dickson, Tanis Jill Ferman, E. E. Benarroch, A. M. Schmeichel, G. E. Smith, Ronald Carl Petersen, J. E. Ahlskog, J. Y. Matsumoto, David S Knopman, C. H. Schenck, M. W. Mahowald

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Abstract

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 α-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). Conclusion: In the setting of degenerative dementia or parkinsonism, RBD often reflects an underlying synucleinopathy.

Original languageEnglish (US)
Pages (from-to)40-45
Number of pages6
JournalNeurology
Volume61
Issue number1
StatePublished - Jul 8 2003

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REM Sleep Behavior Disorder
Parkinsonian Disorders
Dementia
Pathology
Lewy Body Disease
Multiple System Atrophy
Vascular Dementia
Synucleins
Naphazoline
Motor Neuron Disease
Polysomnography
Ubiquitin
Neurodegenerative Diseases
Immunohistochemistry

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Boeve, B. F., Silber, M. H., Parisi, J. E., Dickson, D. W., Ferman, T. J., Benarroch, E. E., ... Mahowald, M. W. (2003). Synucleinopathy pathology and REM sleep behavior disorder plus dementia or parkinsonism. Neurology, 61(1), 40-45.

Synucleinopathy pathology and REM sleep behavior disorder plus dementia or parkinsonism. / Boeve, Bradley F; Silber, M. H.; Parisi, Joseph E; Dickson, Dennis W; Ferman, Tanis Jill; Benarroch, E. E.; Schmeichel, A. M.; Smith, G. E.; Petersen, Ronald Carl; Ahlskog, J. E.; Matsumoto, J. Y.; Knopman, David S; Schenck, C. H.; Mahowald, M. W.

In: Neurology, Vol. 61, No. 1, 08.07.2003, p. 40-45.

Research output: Contribution to journalArticle

Boeve, BF, Silber, MH, Parisi, JE, Dickson, DW, Ferman, TJ, Benarroch, EE, Schmeichel, AM, Smith, GE, Petersen, RC, Ahlskog, JE, Matsumoto, JY, Knopman, DS, Schenck, CH & Mahowald, MW 2003, 'Synucleinopathy pathology and REM sleep behavior disorder plus dementia or parkinsonism', Neurology, vol. 61, no. 1, pp. 40-45.
Boeve, Bradley F ; Silber, M. H. ; Parisi, Joseph E ; Dickson, Dennis W ; Ferman, Tanis Jill ; Benarroch, E. E. ; Schmeichel, A. M. ; Smith, G. E. ; Petersen, Ronald Carl ; Ahlskog, J. E. ; Matsumoto, J. Y. ; Knopman, David S ; Schenck, C. H. ; Mahowald, M. W. / Synucleinopathy pathology and REM sleep behavior disorder plus dementia or parkinsonism. In: Neurology. 2003 ; Vol. 61, No. 1. pp. 40-45.
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abstract = "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 α-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). Conclusion: In the setting of degenerative dementia or parkinsonism, RBD often reflects an underlying synucleinopathy.",
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T1 - Synucleinopathy pathology and REM sleep behavior disorder plus dementia or parkinsonism

AU - Boeve, Bradley F

AU - Silber, M. H.

AU - Parisi, Joseph E

AU - Dickson, Dennis W

AU - Ferman, Tanis Jill

AU - Benarroch, E. E.

AU - Schmeichel, A. M.

AU - Smith, G. E.

AU - Petersen, Ronald Carl

AU - Ahlskog, J. E.

AU - Matsumoto, J. Y.

AU - Knopman, David S

AU - Schenck, C. H.

AU - Mahowald, M. W.

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N2 - 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 α-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). Conclusion: In the setting of degenerative dementia or parkinsonism, RBD often reflects an underlying synucleinopathy.

AB - 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 α-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). Conclusion: In the setting of degenerative dementia or parkinsonism, RBD often reflects an underlying synucleinopathy.

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