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

Case Rep Neurol Med

Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Piazzale Brescia 20, 20149 Milan, Italy.

Published: August 2012

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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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3420516PMC
http://dx.doi.org/10.1155/2011/696953DOI Listing

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