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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Main Authors: A. Ciammola, J. Sassone, B. Poletti, N. Mencacci, R. Benti, V. Silani
Format: Article
Language:English
Published: Wiley 2011-01-01
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.
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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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