J Korean Neurol Assoc.  1999 Sep;17(5):741-746.

A Case of Paraneoplastic Stiff-Person Syndrome Associated with Breast Cancer

Affiliations
  • 1Department of Neurology, Asan Medical Center, College of Medicine, University of Ulsan.
  • 2Department of Pathology, Asan Medical Center, College of Medicine, University of Ulsan.

Abstract

Stiff-person syndrome (SPS) is a rare CNS disease characterized by progressive fluctuating rigidity and superimposed muscle spasms. We report a woman who developed stiff-person syndrome in association with breast cancer. A 55-year-old woman had progressive rigidity and intermittent muscle spasms, which predominantly occurred in her right shoulder muscles. Her symptoms were aggravated by unexpected stimuli or emotional stress, but relieved during sleep. Needle electromyography showed continuous motor unit activities in affected muscles, which were completely abolished by general anesthesia. An Anti-GAD antibody test was negative. She also disclosed a subacute sensory neuronopathy, confirmed by nerve conduction study and sural nerve biopsy. Chest CT scan revealed an enlargement of the right axillary lymph node, which was proved to be a metastatic ductal carcinoma of the right breast. The authors report the first case of paraneoplastic stiff-person syndrome with sensory neuropathy of axonal type, which is associated with breast cancer.

Keyword

Stiff-person syndrome; Subacute sensory neuronopathy; Breast cancer

MeSH Terms

Anesthesia, General
Axons
Biopsy
Breast Neoplasms*
Breast*
Carcinoma, Ductal
Central Nervous System Diseases
Electromyography
Female
Humans
Lymph Nodes
Middle Aged
Muscles
Needles
Neural Conduction
Shoulder
Spasm
Stiff-Person Syndrome*
Stress, Psychological
Sural Nerve
Tomography, X-Ray Computed
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