We describe the neurologic findings in a patient with 
alpha-N-acetylgalactosaminidase deficiency (Kanzaki disease). Clinical and 
electrophysiologic studies revealed sensory-motor polyneuropathy, and sural 
nerve pathology showed decreased density of myelinated fibers with axonal 
degeneration. The patient had mildly impaired intellectual function with 
abnormal brain MRI and sensory-neuronal hearing impairment with repeated 
episodes of vertigo attacks. These findings suggest that Kanzaki disease may 
develop neurologic complications in the CNS and peripheral nervous system.