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Atypical Parkinsonism Revealing a Late Onset, Rigid and Akinetic Form of Huntington's Disease

Huntington's disease (HD) is a rare hereditary neurodegenerative disorder characterized in over 90 percent of cases by chorea as the presenting motor symptom. We report a 54-year-old male who presented with Parkinsonism as the initial symptom of the disease. Genetic analysis revealed expansion...

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Autores principales: Ciammola, A., Sassone, J., Poletti, B., Mencacci, N., Benti, R., Silani, V.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3420516/
https://www.ncbi.nlm.nih.gov/pubmed/22937345
http://dx.doi.org/10.1155/2011/696953
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author Ciammola, A.
Sassone, J.
Poletti, B.
Mencacci, N.
Benti, R.
Silani, V.
author_facet Ciammola, A.
Sassone, J.
Poletti, B.
Mencacci, N.
Benti, R.
Silani, V.
author_sort Ciammola, A.
collection PubMed
description Huntington's disease (HD) is a rare hereditary neurodegenerative disorder characterized in over 90 percent of cases by chorea as the presenting motor symptom. We report a 54-year-old male who presented with Parkinsonism as the initial symptom of the disease. Genetic analysis revealed expansion of 40 CAG repeats, and brain MRI showed both severe caudate nuclei and cortical atrophy. Single-photon emission computed tomography (SPECT) imaging of the dopamine transporter showed nigrostriatal pathway degeneration. Here, we also describe his 2 years of clinical followup after ensuing dopaminergic stimulation.
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spelling pubmed-34205162012-08-30 Atypical Parkinsonism Revealing a Late Onset, Rigid and Akinetic Form of Huntington's Disease Ciammola, A. Sassone, J. Poletti, B. Mencacci, N. Benti, R. Silani, V. Case Rep Neurol Med Case Report Huntington's disease (HD) is a rare hereditary neurodegenerative disorder characterized in over 90 percent of cases by chorea as the presenting motor symptom. We report a 54-year-old male who presented with Parkinsonism as the initial symptom of the disease. Genetic analysis revealed expansion of 40 CAG repeats, and brain MRI showed both severe caudate nuclei and cortical atrophy. Single-photon emission computed tomography (SPECT) imaging of the dopamine transporter showed nigrostriatal pathway degeneration. Here, we also describe his 2 years of clinical followup after ensuing dopaminergic stimulation. Hindawi Publishing Corporation 2011 2011-09-07 /pmc/articles/PMC3420516/ /pubmed/22937345 http://dx.doi.org/10.1155/2011/696953 Text en Copyright © 2011 A. Ciammola et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Ciammola, A.
Sassone, J.
Poletti, B.
Mencacci, N.
Benti, R.
Silani, V.
Atypical Parkinsonism Revealing a Late Onset, Rigid and Akinetic Form of Huntington's Disease
title Atypical Parkinsonism Revealing a Late Onset, Rigid and Akinetic Form of Huntington's Disease
title_full Atypical Parkinsonism Revealing a Late Onset, Rigid and Akinetic Form of Huntington's Disease
title_fullStr Atypical Parkinsonism Revealing a Late Onset, Rigid and Akinetic Form of Huntington's Disease
title_full_unstemmed Atypical Parkinsonism Revealing a Late Onset, Rigid and Akinetic Form of Huntington's Disease
title_short Atypical Parkinsonism Revealing a Late Onset, Rigid and Akinetic Form of Huntington's Disease
title_sort atypical parkinsonism revealing a late onset, rigid and akinetic form of huntington's disease
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3420516/
https://www.ncbi.nlm.nih.gov/pubmed/22937345
http://dx.doi.org/10.1155/2011/696953
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