Diffuse degeneration of cerebral white matter resembling so-called Binswangers disease and symmetrical necrosis of the globus pallidus associated with acute porphyria and cerebral atherosclerosis

摘要

A 59-year-old woman admitted to hospital in coma also showed generalized muscular rigidity and porphyria. After two days she became fully conscious but the muscular rigidity increased and persisted until death occurred 12 weeks later.The brain showed diffuse degeneration of the white matter; symmetrical necrosis of the globus pallidus was associated with severe localized atherosclerosis.The lesions probably resulted from the combined effects of porphyria and atherosclerosis. It is suggested that in later life unusual cerebral lesions may occur with porphyria because of associated atherosclerosis. The porphyria may be overlooked clinically.