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December 1964

Sudanophilic Leukodystrophy and Adrenal Cortical Atrophy

Author Affiliations


Arch Neurol. 1964;11(6):626-631. doi:10.1001/archneur.1964.00460240058008

Introduction  Reports of the association of bronzing of the skin and adrenal atrophy with diffuse cerebral sclerosis have periodically aroused interest in possible metabolic relationships between cerebral white matter destruction and adrenal cortical function. The first such observation was published in 1923 by Siemerling and Creuztfeldt.1 Their patient was a 7-year-old boy in whom bronze discoloration of the hands were first noted at age 4. Increased pigmentation later extended over the entire body. Neurological difficulties did not evolve until eight to nine months prior to death when restlessness, weight loss, and difficulties with speech and gait were noted. There was steady progression to a state of spastic quadriparesis and dysphagia in a matter of a few months. After a seizure he lapsed into coma, and death ensued shortly thereafter. At autopsy, in addition to the finding of diffuse cerebral sclerosis, the adrenals were described as atrophic, with each adrenal