Abstract
Prompted by clinical observations in several patients, a cross-sectional study of 18 long-term survivors of disseminated histiocytosis X was made to correlate clinical course, disease status, and extent and severity of neuropsychological disability. Evaluation is complete in 15 patients, 8 boys and 7 girls, ranging in age at diagnosis from 2 months to 12 years. Lahey scores at diagnosis ranged from 1-7 (median 3). No cases of solitary eosinophilic granuloma are included. Diabetes insipidus is present in 7 of 15 patients. Treatment consisted of steroids in 3 patients and multiple drugs sequentially or in combination in 12 patients; cranial irradiation (range 500-1500 rad) was given to 5 patients concomitantly. Current survival from diagnosis is 2-18 years (median 5 years). Thirteen of 15 patients are off therapy for periods of 1 month to 14 years (median 3½ years). Intellectual function was below average (I.Q. < 89) in 7 of 15 patients (.47) using the Wechsler Adult Intelligence Scale or Wechsler Intelligence Scale for Children. Results of the Wide Range Achievement Test revealed below average results in spelling and arithmetic, and average results in word recognition. In the 10 oldest children, results of either Halsted Children's or Reitan Battery indicated cortical dysfunction in 4, equivocal findings in 2, and no abnormality in 4 patients. Emotional difficulties, poor school performance, and delayed developmental milestones were common findings. Two children, surviving 2-4 years with Letterer-Siwe disease have evidence of extrahypothalamic CNS disease: truncal ataxia, pyramidal tract signs, and behavioral changes. We conclude that long-term survivors of histiocytosis X are at high risk for neuropsychological disability. Formal evaluation of the status and developmental progress of each patient should be a part of the longitudinal treatment plan. (Supported by NCI grants CA08480 CA21765 and ALSAC)
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Laurence Ransom, X., Powazek, M., Goff, J. et al. 653 NEUROPSYCHOLOGICAL LATE SEQUELAE OF HISTIOCYTOSIS. Pediatr Res 12 (Suppl 4), 472 (1978). https://doi.org/10.1203/00006450-197804001-00658
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DOI: https://doi.org/10.1203/00006450-197804001-00658
