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

被引:6
|
作者
Ciammola, A. [1 ,2 ]
Sassone, J. [1 ,2 ]
Poletti, B. [1 ,2 ]
Mencacci, N. [1 ,2 ]
Benti, R. [3 ]
Silani, V. [1 ,2 ,4 ]
机构
[1] IRCCS Ist Auxol Italiano, Dept Neurol, Piazzale Brescia 20, I-20149 Milan, Italy
[2] IRCCS Ist Auxol Italiano, Lab Neurosci, I-20149 Milan, Italy
[3] IRCCS Osped Maggiore, Nucl Med, I-20122 Milan, Italy
[4] Univ Milan, Dino Ferrari Ctr, I-20149 Milan, Italy
关键词
D O I
10.1155/2011/696953
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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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页数:3
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