MovDisordV3, PascalFrancis, Checkpoint, bibRecord, 002614

Selective loss of striatal preprotachykinin neurons in a phenocopy of Huntington's disease

Identifieur interne : 002614 ( PascalFrancis/Checkpoint ); précédent : 002613; suivant : 002615

Selective loss of striatal preprotachykinin neurons in a phenocopy of Huntington's disease

Auteurs : Eric K. Richfield [États-Unis] ; Jean-Paul Vonsattel [États-Unis] ; Marcy E. Macdonald [États-Unis] ; ZHIQIANG SUN [États-Unis] ; Yun-Ping P. Deng [États-Unis] ; Anton Reiner [États-Unis]

Source :

Movement disorders [ 0885-3185 ] ; 2002.

RBID : Pascal:02-0309341

Descripteurs français

Pascal (Inist)
- Chorée Huntington, Immunohistochimie, Tachykinine, Corps strié, Perte, Neurone, Substance P, Physiopathologie, Homme, Préprotachykinine.
Wicri :
- topic : Homme.

English descriptors

KwdEn :
- Corpus striatum, Human, Huntington disease, Immunohistochemistry, Loss, Neuron, Pathophysiology, Substance P, Tachykinin.

Abstract

Phenocopies of Huntington's disease (HD) are individuals with a family history, clinical symptoms, and occasionally pathological evidence of HD but without an expanded CAG repeat within the HD gene. We report on an HD phenocopy with selective loss of preprotachykinin (PPT) neurons, dysfunction of surviving PPT neurons, preservation of preproenkephalin (PPE) neurons within the striatum, and greater loss of immunohistochemical staining for substance P in terminals of striatal neurons projecting to the substantia nigra, than in those projecting to the internal pallidal segment. This case demonstrates the existence of one type of striatal lesion that may produce a clinical picture similar to HD, and raises the possibility of a rare hereditary disease that mimics HD.

Affiliations:

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Pascal:02-0309341

Le document en format XML

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<term>Neuron</term>
<term>Pathophysiology</term>
<term>Substance P</term>
<term>Tachykinin</term>
</keywords>
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<term>Immunohistochimie</term>
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<term>Neurone</term>
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<front><div type="abstract" xml:lang="en">Phenocopies of Huntington's disease (HD) are individuals with a family history, clinical symptoms, and occasionally pathological evidence of HD but without an expanded CAG repeat within the HD gene. We report on an HD phenocopy with selective loss of preprotachykinin (PPT) neurons, dysfunction of surviving PPT neurons, preservation of preproenkephalin (PPE) neurons within the striatum, and greater loss of immunohistochemical staining for substance P in terminals of striatal neurons projecting to the substantia nigra, than in those projecting to the internal pallidal segment. This case demonstrates the existence of one type of striatal lesion that may produce a clinical picture similar to HD, and raises the possibility of a rare hereditary disease that mimics HD.</div>
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<region><li>Massachusetts</li>
<li>Tennessee</li>
<li>État de New York</li>
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<name sortKey="Deng, Yun Ping P" sort="Deng, Yun Ping P" uniqKey="Deng Y" first="Yun-Ping P." last="Deng">Yun-Ping P. Deng</name>
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<name sortKey="Reiner, Anton" sort="Reiner, Anton" uniqKey="Reiner A" first="Anton" last="Reiner">Anton Reiner</name>
<name sortKey="Vonsattel, Jean Paul" sort="Vonsattel, Jean Paul" uniqKey="Vonsattel J" first="Jean-Paul" last="Vonsattel">Jean-Paul Vonsattel</name>
<name sortKey="Zhiqiang Sun" sort="Zhiqiang Sun" uniqKey="Zhiqiang Sun" last="Zhiqiang Sun">ZHIQIANG SUN</name>
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	Movement Disorders (revue)
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Movement Disorders (revue)

Selective loss of striatal preprotachykinin neurons in a phenocopy of Huntington's disease

Selective loss of striatal preprotachykinin neurons in a phenocopy of Huntington's disease

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