Subacute Necrotizing Encephalomyelopathy: Report of a Case with Etiopathogenetic Considerations

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Abstract
A case of rapidly-fatal, necrotizing encephalomyelopathy in a 13-month-old baby boy is reported. From the morphologic point of view the process was characterized by bilateral necrotizing foci in the brachia ponds, in the dorsal columns of the spinal cord, in the facial nerve nuclei, and in one supraoptic nucleus. A review of the literature shows that a large percentage of cases have a familial incidence. The disease is believed to be due to a genetically transmitted metabolic disturbance that prevents the normal utilization of thiamine.

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