Benefit of IVIg for long-standing ataxic sensory neuronopathy with Sjögren’s syndrome

To the Editor: Takahashi et al. have reported on the benefit of IV immunoglobulin (IVIg) in five patients with long-standing ataxic sensory neuronopathy with Sjogren syndrome (SSASN).1 We would like to report a similar experience in two siblings with SSASN. A 62-year-old woman presented with a 1-year history of progressive sensory symptoms with prominent ataxia causing marked gait difficulty, associated with dryness of eyes and mouth (sicca complex) and 40-pound weight loss. Her examination was remarkable for a moderate-to-severe sensory-motor polyneuropathy, with positive Romberg sign and wide-based, ataxic gait. Her 69-year-old brother reported a 5-year history of progressive stocking-glove distribution sensory symptoms. His examination similarly revealed findings of sensory-motor polyneuropathy, with marked ataxia and positive Romberg sign. Prominent lymphocytic infiltration was seen on salivary gland biopsy of both patients. Electrodiagnostic testing demonstrated a predominantly axonal sensorimotor polyneuropathy. The sister had elevated SS-A and SS-B antibodies, but the brother did not. The sister’s sural nerve biopsy demonstrated prominent perivascular inflammation of small epineurial vessels and multifocal myelinated nerve fiber loss. They were treated with IVIg 2 g/kg total dose over 5 days followed by 0.4 g/kg every 3 weeks (sister) or every week (brother). After 3 months of treatment, there was unequivocal improvement in sensory symptoms and dramatic improvement in gait …