Optic neuromyelitis is characterized by simultaneous or successive necrotizing lesions involving the optic nerves and the spinal cord. We report two females with the disease, aged 30 and 34 years old. In the latter, a neuropathological study was done. Both patients had clinical, neuroradiological and pathological features that differed from those of primary demyelinating syndromes such as multiple sclerosis. These patients illustrate the selectivity of optic nerve and spinal cord lesions. The latter involve mainly pyramidal and Goll tracts while, within the necrotizing lesions of the optic chiasma, the fibers of the unaffected optic nerve are spared. This pattern suggests a selective injury to some population of axons. Blood vessels were not affected in the necrotizing areas and the lesions did not follow a vascular territory, therefore a vascular mechanism causing the disease is unlikely. The clinical and neuropathological features of neuromyelitis optic suggest a selective involvement of some axons.