Dancelike Movements in a Patient With a History of Rash

A 67-year-old man visited the neurology clinic for new-onset, generalized, uncontrollable movements. His wife noticed onset of some unusual facial expressions and facial movements. This then evolved to him having some writhing movements of the left upper and left lower extremity. His speech and swallowing also became affected. He noted a tendency to bite his tongue, which was moving uncontrollably. Shortly before his neurology clinic visit, the same writhing movements of right-sided limbs developed. No cognitive or behavioral changes were reported. He had been diagnosed with cutaneous lupus erythematosus 5 years previously after a malar rash of his face developed after sun exposure. The patient had a strong family history of autoimmunity, with 3 sisters having systemic lupus erythematosus. On physical examination, he had marked chorea, hyperkinetic movements that were unpredictable. When he walked in the hallway, he had a narrow-based gait, with some mild upper extremity hyperkinetic movements. Because of the time course and the personal and family history of autoimmunity, autoimmune chorea was suspected. His cerebrospinal fluid demonstrated normal protein concentration, blood cell count, immunoglobulin G index and synthesis rate, and oligoclonal bands. Indirect immunofluorescence assays using HEp-2 substrate were positive for antinuclear antibody and Sjö‎gren syndrome-A antibody (anti-Ro). Autoimmune chorea was diagnosed in the context of a known history of a limited form of systemic lupus erythematosus. The patient received intravenous methylprednisolone infusions. Trimethoprim-sulfamethoxazole, double strength was given for Pneumocystis jirovecii prophylaxis. A rash developed, and the patient was determined to be sulfa allergic. Instead, he received atovaquone as prophylaxis. Three years later, the patient remained in remission from his chorea except for mild occasional hyperkinetic movements of his tongue. In adults, autoimmune chorea is the most common form of chorea after levodopa-induced dyskinesias and Huntington disease. Patients have a subacute onset of symptoms and rapid progression. Patients may have accompanying neuropsychiatric symptoms.