[Ataxic sensory and autonomic neuropathies associated with primary Sjögren's syndrome: a case report]

A 49-year-old woman had xerostomia and foreign body sensation of eye since 27 years old. Since the age of 30 she developed occasional fever attack released by cooling body without medication. Three years later she began to complain difficulty to maintain standing position with closed eyes. Upon admission axillary lymph node swelling, anisocoria and Adie's pupils were evident. She had reddish and atrophic dry tongue. Peripheral reflexes of upper extremities were decreased. Patellar and achilles tendon reflexes were diminished. Perception of pain and temperature were reduced on the right peripheral upper and lateral lower extremities. Deep sensation of four extremities were also decreased. Romberg's sign was positive. She had ataxic gait, orthostatic hypotension, Valsarlva abnormalities and hypohidrosis. Laboratory findings revealed elevated erythrocyte sedimentation rate, high IgG and rheumatoid factor levels, leukopenia, positive anti SS-A/Ro antibody and positive minor salivary gland biopsy. Axillary skin biopsy, CT scan cerebrospinal fluid were normal. Sensory nerve conduction velocity and amplitude were decreased, but motor nerve conduction velocity was normal. Sural nerve biopsy was characterized by loss of large and small myelinated fibers. All findings found in our patient were consistent with those of thirteen patients reported by Griffin et al in 1990 who postulated that T-cell inflammation of the dorsal root ganglion was an extraglandular site of autoimmune attack in Sjögren's syndrome.