RT Journal
A1 KOREY SR, WINOGRAD H
T1 BIochemical alterations in a case of heller's disease
JF A.M.A. Journal of Diseases of Children
JO A.M.A. Journal of Diseases of Children
YR 1959
FD May 1
VO 97
IS 5_PART_II
SP 668
OP 675
DO 10.1001/archpedi.1959.02070010670004
UL http://dx.doi.org/10.1001/archpedi.1959.02070010670004
AB 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