Antisulfatide antibodies in neuropathy - Clinical and electrophysiologic correlates

Objective: To investigate the clinical and electrophysiologic characteristi cs of the neuropathy associated with elevated serum antisulfatide antibodie s. Methods: Clinical, electrophysiologic, morphologic, and laboratory data of 25 patients with significantly elevated (>25,600) antisulfatide antibodi es were reviewed. Results: Four groups were distinguished based on clinical and electrophysiologic data: Group 1, eight patients with predominantly sm all fiber sensory neuropathy (32%); Group 2, five patients with mixed large and small fiber sensory neuropathy (20%); Group 3, seven patients with axo nal sensorimotor neuropathy (28%); and Group 4, three patients with demyeli nating sensorimotor neuropathy (12%). One additional patient had mononeurit is multiplex and one had ALS. An immunoglobulin M (IgM) monoclonal gammopat hy was found in 30% of the patients tested, but not in any of the Group 1 p atients with small fiber sensory neuropathy. Serum IgM level was elevated i n 12 patients, of whom six had a concomitant monoclonal gammopathy. Morphol ogic studies in five patients showed predominantly axonal degeneration, wit h three of the patients also exhibiting additional features of demyelinatio n. Conclusions: Antisulfatide antibodies are associated with several subtyp es of peripheral neuropathy. Predominantly sensory or sensorimotor axonal n europathies are most common in this series, with the sensory component eith er small fiber or mixed fiber type. A smaller demyelinating group indisting uishable from patients with chronic inflammatory demyelinating polyradiculo pathy was also seen. One third of patients had a concomitant IgM monoclonal gammopathy, and approximately one half had elevated serum IgM.