MovDisordV3, PascalFrancis, Checkpoint, bibRecord, 002511

Clinical overview of the synucleinopathies

Identifieur interne : 002511 ( PascalFrancis/Checkpoint ); précédent : 002510; suivant : 002512

Clinical overview of the synucleinopathies

Auteurs : Maria J. Marti [Espagne] ; Eduardo Tolosa [Espagne] ; Jaume Campdelacreu [Espagne]

Source :

Movement disorders [ 0885-3185 ] ; 2003.

RBID : Pascal:04-0081985

Descripteurs français

Pascal (Inist)
- Parkinson maladie, Démence corps Lewy, Atrophie multisystématisée, Système nerveux autonome pathologie, Symptomatologie, Homme, α-Synucléine.
Wicri :
- topic : Homme.

English descriptors

KwdEn :
- Diseases of the autonomic nervous system, Human, Lewy body dementia, Multiple system atrophy, Parkinson disease, Symptomatology, Synuclein.

Abstract

The term synucleinopathies is used to name a group of neurodegenerative disorders characterized by fibrillary aggregates of α-synuclein protein in the cytoplasm of selective populations of neurons and glia. These disorders include Parkinson's disease (PD), dementia with Lewy bodies (DLB), pure autonomic failure (PAF), and multiple system atrophy (MSA). Clinically, they are characterized by a chronic and progressive decline in motor, cognitive, behavioural, and autonomic functions, depending on the distribution of the lesions. Because of clinical overlap, differential diagnosis is sometimes very difficult. Parkinsonism is the predominant symptom of PD, but it can be indistinguishable from the parkinsonism of DLB and MSA. Autonomic dysfunction, which is an isolated finding in PAF, may be present in PD and DLB, but is usually more prominent and appears earlier in MSA. DLB could be the same disease as PD but with widespread cortical pathological states, leading to dementia, fluctuating cognition, and the characteristic visual hallucinations. The deposition of aggregates of synuclein in neurons and glia suggests that a common pathogenic mechanism may exist for these disorders. Even though synuclein may play an important role in disease development in these disorders, in light of the different symptom complex and prognosis and management issues that characterize each disorder, we think that the term synucleinopathy has little practical value as a diagnostic term for the clinician. Clinicians should attempt to reach standard clinical diagnosis on patients, such as PD, PAF, or MSA.

Affiliations:

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Pascal:04-0081985

Le document en format XML

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<front><div type="abstract" xml:lang="en">The term synucleinopathies is used to name a group of neurodegenerative disorders characterized by fibrillary aggregates of α-synuclein protein in the cytoplasm of selective populations of neurons and glia. These disorders include Parkinson's disease (PD), dementia with Lewy bodies (DLB), pure autonomic failure (PAF), and multiple system atrophy (MSA). Clinically, they are characterized by a chronic and progressive decline in motor, cognitive, behavioural, and autonomic functions, depending on the distribution of the lesions. Because of clinical overlap, differential diagnosis is sometimes very difficult. Parkinsonism is the predominant symptom of PD, but it can be indistinguishable from the parkinsonism of DLB and MSA. Autonomic dysfunction, which is an isolated finding in PAF, may be present in PD and DLB, but is usually more prominent and appears earlier in MSA. DLB could be the same disease as PD but with widespread cortical pathological states, leading to dementia, fluctuating cognition, and the characteristic visual hallucinations. The deposition of aggregates of synuclein in neurons and glia suggests that a common pathogenic mechanism may exist for these disorders. Even though synuclein may play an important role in disease development in these disorders, in light of the different symptom complex and prognosis and management issues that characterize each disorder, we think that the term synucleinopathy has little practical value as a diagnostic term for the clinician. Clinicians should attempt to reach standard clinical diagnosis on patients, such as PD, PAF, or MSA.</div>
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	Movement Disorders (revue)
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Movement Disorders (revue)

Clinical overview of the synucleinopathies

Clinical overview of the synucleinopathies

Source :

Descripteurs français

English descriptors

Abstract

Links toward previous steps (curation, corpus...)

Links to Exploration step

Le document en format XML

Pour manipuler ce document sous Unix (Dilib)

Pour mettre un lien sur cette page dans le réseau Wicri