AN AUTOPSY CASE OF SUDANOPHILIC LEUCODYSTROPHY
1974 Volume 24 Issue 1 Pages 47-56
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1974 Volume 24 Issue 1 Pages 47-56
Clinical and neuropathological studies of a sporadic case of sudanophilic leucodystrophy are reported here.
The patient, 48 years old housewife, developed dementia and spastic paraplegia at the age of 45. Her mental and neurological status became worse and finally she fell into decerebrated rigidity or akinetic mutism. General weakness was superimposed and she died after a total clinical course of 3 years and 9 months.
Brain weighed 910 gm and was very atrophic in general appearence. Neuropathologically the diffuse demyelination was observed symmetrically, bilaterally in the cerebral white matter, mostly intense in the frontal lobe. Cerebellum, brain stem and spinal cord were only slightly demyelinated, except for the secondary degeneration of the pyramidal tracts. But pons was partly demyelinated in ascending tracts.
In the demyelinated foci, there was no perivascular inflammatory cell infiltratio but numerous lipid droplets which were stained reddish with Sudan III, were found. The transportation of the myelin breakdown products was retarded. The myelin breakdown products consisted of neutral fat, cholesterol ester and prelipid. The prelipid did not show a metachromasia with acetic acid cresyl-violet (Hirsch-Peiffer) staining and acriflavin reaction was also negative.
Some of the granular cells and glial cells contained lipopigments which showed a yellow color and a positive iron reaction and most of them were found in the frontal lobe.
Gliosis was relatively slight in the deep part of the cerebral white matter because of loss of glial cell. In some areas i.e. cortico-medullary junction where there was slight loss of myelin sheaths, a striking proliferation of glial fibers occurred.
In the middle layer of the cerebral cortex, there were indistinctly limited demyelinated foci accompanied by the spongious state, where loss and shrinkage of nerve cells were found. There was myelin-axonal dissociation in the demyelinated foci and surviving axons were swollen.
Neuropathologically and histochemically, this case was diagnosed as Sudanophilic leucodystrophy of simple form of Hallervorden, van Bogaert-Nyssen type.
