A Case of Ganglionopathy Presenting with Hyperkinetic Movements

Sensory ganglionopathy is a rare, often underrecognized neurological manifestation of Sjogren's disease, presenting with non-length-dependent sensory symptoms that may mimic other neuropathies.

We report a case of Sjogren's-related sensory ganglionopathy presenting as severe sensory ataxia and pseudoathetosis. A 73-year-old man developed involuntary movements, sensory loss, and gait ataxia. Exam showed absent proprioception, areflexia, and worsening ataxia with eye closure. MRI revealed degenerative spine disease; EMG showed demyelinating and axonal polyneuropathy. CSF protein was elevated and Sjogren's antibodies were positive.

Progressive sensory ataxia despite immunotherapy confirmed Sjogren's-associated sensory ganglionopathy emphasizing the importance of early recognition.