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May 1959

Biochemical Alterations in a Case of Heller's Disease

Author Affiliations

Division of Neurology, Department of Medicine, Albert Einstein College of Medicine, Yeshiva University, New York.; Department of Pediatrics and Department of Medicine, University of Western Reserve School of Medicine, Western Reserve University, Cleveland.

AMA Am J Dis Child. 1959;97(5_PART_II):668-675. doi:10.1001/archpedi.1959.02070010670004

In 1909 Heller1,2,2a identified a group of mentally retarded children with common symptomatic and historical manifestations. His findings have been confirmed by Zappert, Corberi and others.3,4 The patients described by Heller were affected between the ages of two to six years in a subacute and progressive manner. Previously normal children manifested signs of impaired conceptualization. This impairment was particularly obvious in the sphere of language. Although the ability to speak words might remain intact, language disintegrated and within a year of the onset of symptoms it was reduced to monoverbal irrelevancies or replaced by sounds and noises. Behavioral changes occurred early. Psychomotor agitation, occasionally associated with apparent hallucinatory episodes, was followed by apathy or a variably excited state of idiocy. In several instances convulsions appeared both prior to the clinical recognition of the syndrome as well as during its course. However epilepsy has not been considered an essential