Description

ROHHAD and ROHHADNET are acronyms for rare pediatric disorders.


ROHHAD:

(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)

(4) hyperprolactinemia

(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.


To read more or access our algorithms and calculators, please log in or register.