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Neuropathological and Biochemical Study of a Case

Catherine Haberland, MD; Eric G. Brunngraber, PhD; Lloyd A. Witting, PhD

Arch Neurol. 1972;26(5):391-402.

Abstract
Neuropathological and biochemical studies were done of a 91/2-year-old girl with a sporadic form of infantile neuroaxonal dystrophy (INAD). The characteristic histopathological features were the following: (1) presence of eosinophilic spheroids in the central, peripheral, and autonomic nervous systems; (2) pallido-striato-nigral iron-containing pigmentary deposits; (3) cerebellar degeneration; and (4) associated neuronal, myelin, and glial changes. The biochemical analysis of the gray matter revealed a normal ganglioside level and composition, a reduction of glycoprotein-carbohydrate content with no alteration in the composition of the heteropolysaccharide chains of higher molecular weight, and a reduced level of glycosaminoglycans. The white matter chemical abnormalities consisted of a marked reduction in cerebrosides and minor abnormalities in the composition of gangliosides and dialyzable glycopeptides. Various views on the origin and possible pathomechanisms underlying the neuroaxonal dystrophy are entertained.

Author Affiliations
Chicago; Elgin, Ill

From the Department of Neuropathology (Dr. Haberland), and Research Laboratory (Dr. Brunngraber), Illinois State Psychiatric Institute, Illinois State Pediatric Institute (Dr. Haberland), Chicago, and Mendel Research Laboratory, Elgin State Hospital, Elgin, Ill (Dr. Witting).

Footnotes
Accepted for publication Sept 14, 1971.

Reprint requests to Department of Neuropathology, Illinois State Psychiatric Institute, 1601 W Taylor St, Chicago 60612 (Dr. Haberland).

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