Delayed dystonia with striatal CT lucencies induced by a mycotoxin (3-nitropropionic acid).

We describe a clinical syndrome of delayed dystonia in children subsequent to initial gastrointestinal symptoms and acute noninflammatory encephalopathy. The syndrome was caused by the ingestion of mildewed sugarcane containing the Arthrinium-produced mycotoxin, 3-nitropropionic acid (3-NPA). In the severely affected patients, intoxication usually was heralded by coma, with dystonia appearing 7 to 40 days after recovery from the coma. The dystonia was manifested as choreoathetosis, torsion spasms, or painful paroxysmal spasms of the extremities and was neither progressive nor reversible. CTs of the dystonic patients consistently showed bilateral hypodensities in the lenticular nuclei. The pathogenesis of the selective lenticular lesions induced by 3-NPA is not yet clear.