DR MOHAMMED IRFAN SHEIKH over 5 years ago

Hemichoreoathetosis in Neurosarcoidosis

A 39-year-old black man presented with a 2-week history of progressively worsening involuntary movements of the left arm that had been sudden in onset. His medical history consisted of hypertension and poorly controlled diabetes that were now under control. A physical examination revealed chorea of the left upper arm and athetosis of the fingers of the left hand (see video). T1-weighted magnetic resonance imaging (MRI) of the brain was performed after the administration of contrast material and revealed areas of enhancement in both frontal and basal ganglia and the left leptomeningeal ganglia (Panel A, arrows) that suggested an infiltrative process. Further examination revealed small violaceous papules above the eyebrows (Panel B). Biopsy specimens of the papules were obtained, and histopathological analysis revealed dermal non-necrotizing granulomas composed of epithelioid histocytes (Panel C, arrows) that were consistent with sarcoidosis (hematoxylin and eosin). The neurologic symptoms seen in this patient are not typical of patients with sarcoidosis. Hemichoreoathetosis is a rare manifestation of neurosarcoidosis and may be due to the involvement of the basal ganglia. Differential diagnoses include infiltrating lymphoma, gliomatosis cerebri, atypical infections, and ischemic events. Treatment with glucocorticoids and methotrexate was initiated, and at a 6-month follow-up visit the hemichoreoathetosis had resolved. A repeat MRI showed improvement in the regions of the brain that had shown areas of enhancement. The patient continues weekly therapy with methotrexate.