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Parkinsonism and motor neuron diseases: Twenty‐seven patients with diverse overlap syndromes

Identifieur interne : 001188 ( Main/Corpus ); précédent : 001187; suivant : 001189

Parkinsonism and motor neuron diseases: Twenty‐seven patients with diverse overlap syndromes

Auteurs : Rebecca M. Wolf Gilbert ; Stanley Fahn ; Hiroshi Mitsumoto ; Lewis P. Rowland

Source :

RBID : ISTEX:7C83527D711E75B3604D4B60C7490EC5DD3EA55C

English descriptors

Abstract

It has long been recognized that signs of motor neuron disease (MND) may accompany clinical evidence of parkinsonism in different neurodegenerative conditions. By using the Columbia University Division of Movement Disorders database, we reviewed data from 5,500 cases of parkinsonism and recorded the presence of upper motor neuron (UMN) dysfunction, lower motor neuron (LMN) dysfunction, or both. Among the 27 patients so identified, we counted those with autonomic dysfunction, cerebellar dysfunction, or dementia. Among the 27 cases, seven had UMN signs and LMN signs as well as parkinsonism and were diagnosed with amyotrophic lateral sclerosis (ALS)‐parkinsonism (Brait‐Fahn disease). Three of the seven had dementia that was not deemed to be frontotemporal dementia (FTD). Six other patients had no LMN signs but had UMN signs and parkinsonism and were classified as having primary lateral sclerosis (PLS)‐parkinsonism. Four patients had both UMN and LMN signs with parkinsonism as well as the characteristic dementia of FTD; they were diagnosed with FTD‐parkinsonism‐ALS. Seven patients had MND, parkinsonism, and autonomic or cerebellar dysfunction, a combination compatible with multiple system atrophy (MSA). Three patients had syndromes compatible with hereditary spastic paraplegia (HSP). In sum, we found that MND occurs in association with diverse parkinsonian syndromes; some are heritable, others sporadic and causes are uncertain. Having MND may be a risk factor forparkinsonism. A prospective study may elucidate this possibility. © 2010 Movement Disorder Society.

Url:
DOI: 10.1002/mds.23200

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ISTEX:7C83527D711E75B3604D4B60C7490EC5DD3EA55C

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<p>It has long been recognized that signs of motor neuron disease (MND) may accompany clinical evidence of parkinsonism in different neurodegenerative conditions. By using the Columbia University Division of Movement Disorders database, we reviewed data from 5,500 cases of parkinsonism and recorded the presence of upper motor neuron (UMN) dysfunction, lower motor neuron (LMN) dysfunction, or both. Among the 27 patients so identified, we counted those with autonomic dysfunction, cerebellar dysfunction, or dementia. Among the 27 cases, seven had UMN signs and LMN signs as well as parkinsonism and were diagnosed with amyotrophic lateral sclerosis (ALS)‐parkinsonism (Brait‐Fahn disease). Three of the seven had dementia that was not deemed to be frontotemporal dementia (FTD). Six other patients had no LMN signs but had UMN signs and parkinsonism and were classified as having primary lateral sclerosis (PLS)‐parkinsonism. Four patients had both UMN and LMN signs with parkinsonism as well as the characteristic dementia of FTD; they were diagnosed with FTD‐parkinsonism‐ALS. Seven patients had MND, parkinsonism, and autonomic or cerebellar dysfunction, a combination compatible with multiple system atrophy (MSA). Three patients had syndromes compatible with hereditary spastic paraplegia (HSP). In sum, we found that MND occurs in association with diverse parkinsonian syndromes; some are heritable, others sporadic and causes are uncertain. Having MND may be a risk factor forparkinsonism. A prospective study may elucidate this possibility. © 2010 Movement Disorder Society.</p>
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<p>Potential conflict of interest: Dr. Wolf Gilbert's fellowship while performing this work was supported by the Parkinson's Disease Foundation. She has no conflict of interest related to this research. Drs. Fahn, Mitsumoto, and Rowland did not receive funding for this project and have no conflicts of interest related to this research.</p>
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<title>Parkinsonism and motor neuron diseases: Twenty‐seven patients with diverse overlap syndromes</title>
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<namePart type="given">Rebecca M. Wolf</namePart>
<namePart type="family">Gilbert</namePart>
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<affiliation>Department of Neurology, NYU Langone Medical Center, New York, New York, USA</affiliation>
<description>Correspondence: 145 East 32nd Street, 2nd floor, New York, NY 10016</description>
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<affiliation>Department of Neurology, Columbia University Medical Center, New York, New York, USA</affiliation>
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<namePart type="given">Hiroshi</namePart>
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<affiliation>Department of Neurology, Columbia University Medical Center, New York, New York, USA</affiliation>
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<abstract lang="en">It has long been recognized that signs of motor neuron disease (MND) may accompany clinical evidence of parkinsonism in different neurodegenerative conditions. By using the Columbia University Division of Movement Disorders database, we reviewed data from 5,500 cases of parkinsonism and recorded the presence of upper motor neuron (UMN) dysfunction, lower motor neuron (LMN) dysfunction, or both. Among the 27 patients so identified, we counted those with autonomic dysfunction, cerebellar dysfunction, or dementia. Among the 27 cases, seven had UMN signs and LMN signs as well as parkinsonism and were diagnosed with amyotrophic lateral sclerosis (ALS)‐parkinsonism (Brait‐Fahn disease). Three of the seven had dementia that was not deemed to be frontotemporal dementia (FTD). Six other patients had no LMN signs but had UMN signs and parkinsonism and were classified as having primary lateral sclerosis (PLS)‐parkinsonism. Four patients had both UMN and LMN signs with parkinsonism as well as the characteristic dementia of FTD; they were diagnosed with FTD‐parkinsonism‐ALS. Seven patients had MND, parkinsonism, and autonomic or cerebellar dysfunction, a combination compatible with multiple system atrophy (MSA). Three patients had syndromes compatible with hereditary spastic paraplegia (HSP). In sum, we found that MND occurs in association with diverse parkinsonian syndromes; some are heritable, others sporadic and causes are uncertain. Having MND may be a risk factor forparkinsonism. A prospective study may elucidate this possibility. © 2010 Movement Disorder Society.</abstract>
<note type="content">*Potential conflict of interest: Dr. Wolf Gilbert's fellowship while performing this work was supported by the Parkinson's Disease Foundation. She has no conflict of interest related to this research. Drs. Fahn, Mitsumoto, and Rowland did not receive funding for this project and have no conflicts of interest related to this research.</note>
<subject lang="en">
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<topic>motor neuron disease</topic>
<topic>amyotrophic lateral sclerosis</topic>
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<topic>frontotemporal dementia</topic>
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<topic>parkinsonism</topic>
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<identifier type="ISSN">0885-3185</identifier>
<identifier type="eISSN">1531-8257</identifier>
<identifier type="DOI">10.1002/(ISSN)1531-8257</identifier>
<identifier type="PublisherID">MDS</identifier>
<part>
<date>2010</date>
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<caption>vol.</caption>
<number>25</number>
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<number>12</number>
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