Histopathological and ultrastructural study of a case of infantile metachromatic leukodystrophy

The histopathological and ultrastructural findings on a nerve biopsy specimen and on a CNS necropsy specimen in a case of IML with a typical clinicaL course are reported. Nerve biopsy once again proved to be a sure diagnostic guide even at an early stage of the disease. Some differences in the fine structure of the cytosomes between the nerve biopsy and CNS necropsy material studied four years later may be due to the different rates of catabolism of the constituent lipids as well as to post mortem artefacts.