Subacute Necrotizing Encephalomyelopathy (LEIGH)

Subacute Necrotizing Encephalopathy (Leigh) in an Adult

“…In this respect the disease of the adult is very similar to that in childhood. In none of the adult cases and in only one of the six juveniles was there an indication of a familial occurrence, which is known to occur in nearly 50% of the cases in childhood (Jellinger and Seitelberger, 1970).…”

“…Our own case therefore, would be the third. All the three were males, which corresponds to the general male prepon derance in this disease (Montpetit et al, 1971;Jellinger and Seitelberger, 1970). The longest course was 2 years (Solheid et al, 1971), the shortest 2 months (Martin, 1972 -present case).…”

“…About 100 cases of this disease have been described now {Jellinger and Seitelberger, 1970;Montpetit et al, 1971). Thus, pediatricians are usually famil iar with it.…”

“…Necrotic foci of several millimeters in diameter with prolif erated capillaries, fat granule cells, preserved axons and neurons. Some authors (Sipe, 1973;Jellinger and Seitelberger, 1970;Gullotta, 1968;Guazzi et al, 1968) also point out a proliferation of astrocytes and glial fibrils, which may go beyond the region of the actual lesions. Myelin sheaths within their area are destroyed.…”

“…Neuropathological ex amination shows symmetrical necrotizing and intensely vascularized lesions in which the nerve cells and axons are relatively spared. To date about 100 cases have been reported and reviewed by Jellinger and Seitelberger (1970), Montpetit et al (1971), Pincus et al (1973) and Sipe (1973). While most patients were infants and very young children, some juvenile and even adult cases were also observed {Hard man, 1968;Feigin and Goebel, 1969;Martin, 1972;Sipe, 1973).…”