Immunoglobulin therapy for idiopathic chronic sensory ataxic neuropathy.

Abstract

Chronic sensory ataxic neuropathy (CSAN) is characterized by its insidious onset and slowly progressive sensory impairment. Deep sensation leading to sensory ataxia, various degrees of mild autonomic symptoms, and well-preserved motor function have been reported.1,2 Loss of the sensory and autonomic ganglion neurons, accompanied by inflammatory cell infiltration and sensory and autonomic axon loss, are the major pathology.3 An immune-mediated mechanism is believed to be in the pathogenic background. Some CSAN cases are associated with Sjogren’s syndrome1,4 or malignancy, but others are not associated with any background diseases; as such, they are designated as idiopathic chronic sensory ataxic neuropathy (ICSAN). An effective treatment for this disease has not been established. We performed an open trial of IV immunoglobulin (IVIg) therapy for four patients with ICSAN who did not respond …