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Case Reports in Neurological Medicine
Volume 2011, Article ID 696953, 3 pages
http://dx.doi.org/10.1155/2011/696953
Case Report

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

1Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Piazzale Brescia 20, 20149 Milan, Italy
2Nuclear Medicine, IRCCS-Ospedale Maggiore, 20122 Milan, Italy
3“Dino Ferrari” Center, University of Milan, 20149 Milan, Italy

Received 30 May 2011; Accepted 11 July 2011

Academic Editors: P. Berlit, A. E. Cavanna, S. T. Gontkovsky, P. Mir, D. J. Rivet, and P. Sandroni

Copyright © 2011 A. Ciammola et al. 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.

Abstract

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.