ROHHAD and ROHHADNET are acronyms for rare pediatric disorders.
(1) rapid-onset obesity (RO) secondary to hyperphagia
(2) hypothalamic dysfunction (H)
(3) hypoventilation (H)
(4) autonomic dysfunction (AD; dysautonomia)
ROHHADNET is ROHHAD plus a neuroendocrine tumor (NET).
Additional clinical features may include:
(1) growth failure (pituitary)
(2) glucocorticoid insufficiency (pituitary)
(3) congenital hypopituitarism (hypothalamic)
(5) behavioral disorders (depression, psychosis, obsessive-compulsive disorder, Tourette's)
(6) cardiopulmonary arrest
(7) fixed pupils and/or anisocoria
(8) temperature dysregulation with fever (hypothalamic)
(9) electrolyte abnormalities
(10) gastrointestinal dysmotility
In the patient described by Sethi et al the patient had a hypothalamic encephalitis.
Neuroendocrine tumors may show neurological paraneoplastic syndromes.
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