Purkinje Cell Cytoplasmic Antibody (PCA-2)-related Chorea–Dystonia Syndrome

Tremor and Other Hyperkinetic Movements

article

Harsh V. Gupta¹  Charles Gervais¹  Mark A. Ross¹  Shyamal H. Mehta¹ 

[1] Department of Neurology, Mayo Clinic

关键词: Paraneoplastic;    PCA-2;    caudate atrophy;    chorea;    dystonia;   

DOI  :  10.5334/tohm.309

学科分类：社会科学、人文和艺术（综合）

来源: Ubiquity Press

【 摘 要 】

There are several causes of adult-onset chorea, with Huntington’s disease (HD) being the most common. We report a patient who developed a complex movement disorder consisting of chorea and dystonia associated with caudate atrophy on brain magnetic resonance imaging (MRI) because of a paraneoplastic etiology that can be easily confused with HD. He was found to have elevated titers of Purkinje cell cytoplasmic antibody (PCA-2) in the serum and cerebrospinal fluid (CSF). A 70-year-old Caucasian male with no pertinent past medical history was referred for complaints of unintentional weight loss, weakness, and involuntary movements. Over the past year, he had experienced progressive weakness in his legs such that he required a walker. As his weakness progressed, he developed bladder and bowel incontinence, further gait decline, and 60-pound (27-kg) weight loss. During the course of his weight loss and gait decline, he developed involuntary movements of the mouth, abnormal posturing of the hands, and dysarthria. His involuntary movements disappeared during sleep and while he was eating or drinking. The tongue did not push food out and he was able to eat and swallow without difficulty. There was no history of medication intake that could cause these involuntary (tardive) movements. The family history was negative for neurodegenerative disorders. He had a 30 pack-year smoking history and prior history of heavy alcohol intake. Clonazepam was prescribed to help with the involuntary movements, which resulted in mild improvement as seen in the latter half of the video (Video 1).

【 授权许可】

CC BY