Improved Behavior and Neuropsychological Function in Children With ROHHAD After High-Dose Cyclophosphamide.

Abstract

Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) is a rare, generally progressive, and potentially fatal syndrome of unclear etiology. The syndrome is characterized by normal development followed by a sudden, rapid hyperphagic weight gain beginning during the preschool period, hypothalamic dysfunction, and central hypoventilation, and is often accompanied by personality changes and developmental regression, leading to substantial morbidity and mortality. We describe 2 children who had symptomatic and neuropsychological improvement after high-dose cyclophosphamide treatment. Our experience supports an autoimmune pathogenesis and provides the first neuropsychological profile of patients with rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation.

DOI: 10.1542/peds.2015-1080

Cite this paper

@article{Jacobson2016ImprovedBA, title={Improved Behavior and Neuropsychological Function in Children With ROHHAD After High-Dose Cyclophosphamide.}, author={L. A. Jacobson and Shruti Rane and Lisa J McReynolds and Diana A Steppan and Allen Chen and Ido Paz-Priel}, journal={Pediatrics}, year={2016}, volume={138 1} }