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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Wiley
2011-01-01
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Series: | Case Reports in Neurological Medicine |
Online Access: | http://dx.doi.org/10.1155/2011/696953 |
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author | A. Ciammola J. Sassone B. Poletti N. Mencacci R. Benti V. Silani |
author_facet | A. Ciammola J. Sassone B. Poletti N. Mencacci R. Benti V. Silani |
author_sort | A. Ciammola |
collection | DOAJ |
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. |
format | Article |
id | doaj-art-add838a3bfcd409a9d02be34f58ccaf9 |
institution | Kabale University |
issn | 2090-6668 2090-6676 |
language | English |
publishDate | 2011-01-01 |
publisher | Wiley |
record_format | Article |
series | Case Reports in Neurological Medicine |
spelling | doaj-art-add838a3bfcd409a9d02be34f58ccaf92025-02-03T06:11:07ZengWileyCase Reports in Neurological Medicine2090-66682090-66762011-01-01201110.1155/2011/696953696953Atypical Parkinsonism Revealing a Late Onset, Rigid and Akinetic Form of Huntington's DiseaseA. Ciammola0J. Sassone1B. Poletti2N. Mencacci3R. Benti4V. Silani5Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Piazzale Brescia 20, 20149 Milan, ItalyDepartment of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Piazzale Brescia 20, 20149 Milan, ItalyDepartment of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Piazzale Brescia 20, 20149 Milan, ItalyDepartment of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Piazzale Brescia 20, 20149 Milan, ItalyNuclear Medicine, IRCCS-Ospedale Maggiore, 20122 Milan, ItalyDepartment of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Piazzale Brescia 20, 20149 Milan, ItalyHuntington'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.http://dx.doi.org/10.1155/2011/696953 |
spellingShingle | A. Ciammola J. Sassone B. Poletti N. Mencacci R. Benti V. Silani Atypical Parkinsonism Revealing a Late Onset, Rigid and Akinetic Form of Huntington's Disease Case Reports in Neurological Medicine |
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 |
url | http://dx.doi.org/10.1155/2011/696953 |
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