[Vacuolar myelopathy with symmetrical involvement of the fasciculus cuneatus, in a case of amyotrophic lateral sclerosis with multisystemic degeneration]

Abstract

We report on vacuolar myelopathy in a case of sporadic amyotrophic lateral sclerosis (ALS) with multisystemic degeneration. The patient was a 75-year-old woman at death, who had been characterized by severe upper and lower motor neuron disturbance and supranuclear ophthalmoplegia. She became dependent on a respirator 14 months and survived 34 months after the onset of her illness. Neuropathologically, severe degeneration of the basal ganglia, substantia nigra, Clark's dorsal nucleus and spinocerebellar tract were noted together with the prominent alteration of the motor neuron system. The unexpected feature in this patient was the prominent vacuolar change in the white matter of the spinal cord. Especially, the fasciculus cuneatus was entirely occupied by various sizes of vacuoles. These vacuoles tended to be rimmed by thin myelin, which was continuous to disintegrated myelin sheaths. Axons were occasionally noted in the vacuoles. Vacuoles were only rarely present in the corticospinal and spinocerebellar tracts where myelin was severely lost due to the primary disease. In the vacuolar lesions lipid-laden macrophages were occasionally found, suggesting that a postmortem artifact was unlikely. Although the etiology of vacuolar myelopathy is unknown, the selective distribution of vacuolar change as seen in our patient has never been described in the past literature of vacuolar myelopathy associated with various diseases.