To highlight the diagnostic challenges created by a rare neurological manifestation of Sjögren’s disease.

Sensory ganglionopathy is an uncommon and often under-recognized complication of Sjögren’s disease, characterized by a distinct clinical presentation that differs from length-dependent peripheral neuropathies. The diagnosis can be challenging due to its insidious onset, non-length–dependent sensory symptoms, and overlap with other neurological or structural conditions. 

We present a case of Sjögren’s disease-related sensory ganglionopathy manifesting as severe sensory ataxia and pseudoathetosis. 

A 73-year-old male was referred for involuntary movements, lower extremity weakness, and generalized pain. Exam revealed choreiform movements, dystonic posturing in his right hemibody, and axial and extremity ataxia. These symptoms worsened significantly with eyes closed. He had severely diminished sensation to all modalities with almost absent proprioception. Lower extremity deep tendon reflexes were absent. Gait was ataxic with unsteady station. EMG revealed severe peripheral polyneuropathy with segmental demyelination and axonal loss with normal needle exam. CSF protein was elevated to 83 mg/dL and serum was positive for Sjogren’s antibody. He was initially treated with IVIG monthly and hydroxychloroquine for presumed CIDP associated with Sjogren’s disease. On further evaluation of exam and progressive worsening despite treatment, his clinical picture was more suggestive of Sjogren’s sensory ganglionopathy.