Paraneoplastic "stiff person syndrome" with metastatic adenocarcinoma and anti-Ri antibodies

• Published in: Neurology Vol. 62; no. 8; p. 1402
• Main Authors: McCabe, D J H, Turner, N C, Chao, D, Leff, A, Gregson, N A, Womersley, H J, Mak, I, Perkin, G D, Schapira, A H V
• Format: Journal Article
• Language: English
• Published: United States 27.04.2004
• Subjects: Adenocarcinoma - diagnostic imaging; Adenocarcinoma - immunology; Adenocarcinoma - secondary; Adult; Carcinoembryonic Antigen - blood; Deglutition Disorders - etiology; Dysarthria - etiology; Female; Fluorodeoxyglucose F18; Humans; Lung Neoplasms - diagnostic imaging; Lung Neoplasms - immunology; Lymphatic Metastasis - diagnostic imaging; Lymphatic Metastasis - immunology; Muscle Weakness - etiology; Muscle Weakness - physiopathology; Neurons - immunology; Palliative Care; Paraneoplastic Syndromes, Nervous System - diagnosis; Paraneoplastic Syndromes, Nervous System - immunology; Radionuclide Imaging; Spinal Cord - immunology; Stiff-Person Syndrome - diagnosis; Stiff-Person Syndrome - immunology; Upper Extremity - physiopathology
• ISSN: 1526-632X
• DOI: 10.1212/01.WNL.0000123694.64121.D5

A 43-year-old woman presented with clinical and electrophysiologic features of stiff person syndrome (SPS), without abdominal or lumbar paraspinal muscle involvement. Investigations revealed metastatic adenocarcinoma of the lung with positive anti-Ri antibodies. Her clinical condition improved with diazepam, baclofen, tizanidine, and palliative chemotherapy. Screening for an underlying malignancy and anti-Ri antibodies should be considered in patients with SPS when clinical presentation is atypical.