GIANT AXONAL NEUROPATHY - COMBINED CENTRAL AND PERIPHERAL NERVOUS-SYSTEM DISEASE

The presence of onion bulb formations, not reported previously, suggested a remyelinating process after segmental demyelinating changes. The involvement of the CNS was emphasized in this case, as evidenced by clinical findings and by electronystagmography and the auditory brain stem response, which suggested lesions in connections between brain stem and cerebellum, and between the cochlear nucleus and the inferior colliculus, respectively. CNS function should be examined carefully in this disease.