Painful torticollis with tongue atrophy--a different neck-tongue syndrome.

Two children were referred with neck pain, torticollis, dysarthria, and atrophy of the tongue. The erythrocyte sedimentation rate was markedly elevated in both cases. Radiologic appearances were similar. In both cases the cervical spine was stable. Computerised tomography showed soft-tissue swelling surrounding the odontoid process and distorting the theca. Magnetic resonance imaging showed gadolinium enhancement of the soft tissue overlying the clivus and around the odontoid, impinging on the medulla and high cervical cord. Biopsy in the second case was not diagnostic. Steroids led to a dramatic and sustained improvement in symptoms and a marked decrease in soft tissue mass. The history, elevated inflammatory indices, radiologic appearance and response to steroids, are consistent with an inflammatory process.