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Calcification of basal ganglia and cerebellar roof nuclei in mentally defective patient with hidrotic ectodermal dysplasia

Analysis of intracranial concretions by electron microprobe

Department of Pathology, Duke University Medical Center, Durham, North Carolina.

This report describes, for the first time, an analysis by electron microprobe of concretions in the brain of an individual with striopallidodentate calcification. We also report the unique association of this intracranial syndrome with hidrotic ectodermal dysplasia. An institutionalized male with impaired intellectual function and hidrotic ectodermal dysplasia was known since the age of 3 years to have bilateral radiopaque densities in the region of the basal ganglia on skull roentgenogram. He died at age 29 in congestive heart failure from rheumatic pancarditis. At autopsy, concretions were identified in globus pallidus, caudate nuclei, thalamus, and dentate nuclei. Mineral deposits within the brain, analyzed by energy dispersive x-ray microanalysis, consisted predominately of calcium and phosphorus. Trace amounts of magnesium, iron, and silicon also were detected.

Dr. Copelands address is Department of Pathology, Duke University Medical Center, Durham, NC 27710.

This study was supported in part by USPHS grants No. CA05437 and EY00146.

Accepted for publication April 1, 1977.

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				W. C. Robertson Jr, C. Viseskul, Y. E. Lee, and R. V. Lloyd Basal Ganglia Calcification in Kearns-Sayre Syndrome Arch Neurol, November 1, 1979; 36(11): 711 - 713. [Abstract] [PDF]