Movement Disorders (revue)

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Parkinsonism and Motor Neuron Diseases: Twenty-Seven Patients with Diverse Overlap Syndromes

Identifieur interne : 002394 ( PascalFrancis/Curation ); précédent : 002393; suivant : 002395

Parkinsonism and Motor Neuron Diseases: Twenty-Seven Patients with Diverse Overlap Syndromes

Auteurs : Rebecca M. Wolf Gilbert [États-Unis] ; Stanley Fahn [États-Unis] ; Hiroshi Mitsumoto [États-Unis] ; Lewis P. Rowland [États-Unis]

Source :

RBID : Pascal:10-0446304

Descripteurs français

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 for parkinsonism. A prospective study may elucidate this possibility.
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C01 01    ENG  @0 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 for parkinsonism. A prospective study may elucidate this possibility.
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C03 05  X  FRE  @0 Démence frontotemporale @2 NM @5 05
C03 05  X  ENG  @0 Frontotemporal dementia @2 NM @5 05
C03 05  X  SPA  @0 Demencia frontotemporal @2 NM @5 05
C03 06  X  FRE  @0 Atrophie multisystématisée @2 NM @5 06
C03 06  X  ENG  @0 Multiple system atrophy @2 NM @5 06
C03 06  X  SPA  @0 Atrofia multisistematizada @2 NM @5 06
C03 07  X  FRE  @0 Pathologie du système nerveux @5 07
C03 07  X  ENG  @0 Nervous system diseases @5 07
C03 07  X  SPA  @0 Sistema nervioso patología @5 07
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C03 08  X  ENG  @0 Human @5 09
C03 08  X  SPA  @0 Hombre @5 09
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C07 01  X  ENG  @0 Degenerative disease @5 38
C07 01  X  SPA  @0 Enfermedad degenerativa @5 38
C07 02  X  FRE  @0 Pathologie de la moelle épinière @5 39
C07 02  X  ENG  @0 Spinal cord disease @5 39
C07 02  X  SPA  @0 Médula espinal patología @5 39
C07 03  X  FRE  @0 Pathologie du système nerveux central @5 40
C07 03  X  ENG  @0 Central nervous system disease @5 40
C07 03  X  SPA  @0 Sistema nervosio central patología @5 40
C07 04  X  FRE  @0 Immunopathologie @5 41
C07 04  X  ENG  @0 Immunopathology @5 41
C07 04  X  SPA  @0 Inmunopatología @5 41
C07 05  X  FRE  @0 Pathologie de l'encéphale @5 42
C07 05  X  ENG  @0 Cerebral disorder @5 42
C07 05  X  SPA  @0 Encéfalo patología @5 42
N21       @1 291
N44 01      @1 OTO
N82       @1 OTO

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<s0>Pathologie du système nerveux central</s0>
<s5>40</s5>
</fC07>
<fC07 i1="03" i2="X" l="ENG">
<s0>Central nervous system disease</s0>
<s5>40</s5>
</fC07>
<fC07 i1="03" i2="X" l="SPA">
<s0>Sistema nervosio central patología</s0>
<s5>40</s5>
</fC07>
<fC07 i1="04" i2="X" l="FRE">
<s0>Immunopathologie</s0>
<s5>41</s5>
</fC07>
<fC07 i1="04" i2="X" l="ENG">
<s0>Immunopathology</s0>
<s5>41</s5>
</fC07>
<fC07 i1="04" i2="X" l="SPA">
<s0>Inmunopatología</s0>
<s5>41</s5>
</fC07>
<fC07 i1="05" i2="X" l="FRE">
<s0>Pathologie de l'encéphale</s0>
<s5>42</s5>
</fC07>
<fC07 i1="05" i2="X" l="ENG">
<s0>Cerebral disorder</s0>
<s5>42</s5>
</fC07>
<fC07 i1="05" i2="X" l="SPA">
<s0>Encéfalo patología</s0>
<s5>42</s5>
</fC07>
<fN21>
<s1>291</s1>
</fN21>
<fN44 i1="01">
<s1>OTO</s1>
</fN44>
<fN82>
<s1>OTO</s1>
</fN82>
</pA>
</standard>
</inist>
</record>

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