Spontaneous improvement of subacute exacerbation in a case of sensory ataxic neuropathy associated with Sjögren's syndrome

We reported a 66-year-old man with Sjögren's syndrome (sicca syndrome) presenting a sensory ataxic neuropathy, which showed spontaneous remission. He developed difficulty in standing and walking during recent several months. Neurological examinations showed sensory ataxia with areflexia in all extremities and mild distal-dominant decrease in the superficial sensation. Laboratory examinations of blood, urine, and cerebrospinal fluid were all unremarkable; the blood levels of vitamin B₁, B₂, B₆ and B₁₂ were low normal. Sensory nerve action potentials and somatosensory evoked potentials were absent. Cervical cord MRI revealed no abnormal signals. Severe loss of myelinated fibers and scattered myelin ovoids were seen in sural nerve biopsy. Tentative diagnosis at admission was subacute sensory neuropathy associated with malignancy. Screening examinations for malignancy were undertaken and all revealed negative. Because of coexisting sicca symptoms and positive Shirmer test (0 mm), a lip biopsy was performed and a diagnosis of Sjögren's syndrome was confirmed. Sensory ataxia improved gradually. Two years later, blood B₁ levels were low but he remained able to walk. This case is an uncommon example of spontaneous symptomatic remission in sensory ataxic neuropathy associated with Sjögren's syndrome.