Hypothalamic-midbrain dysregulation syndrome: hypertension, hyperthermia, hyperventilation, and decerebration.
Certain decerebrate lesions of brain stem or hypothalamus induce pharmacologically reversible hypertension and hyperthermia in animals. We observed three young patients with episodic decerebration, hyperthermia, hypertension, and hyperventilation during recovery from comas of different etiologies. The shared pathology on neurologic examinations and computed tomographic scans was hypothalamic-mesencephalic dysfunction, suggesting a diencephalic-brain-stem disconnection syndrome or brain-stem release mechanism. Propranolol was the most effective drug tested, but only two patients responded, one dramatically. This novel clinical syndrome may have localizing and therapeutic significance in pediatric coma that needs to be further defined in future studies.
Pranzatelli
M R
MR
Department of Neurology, Columbia University, New York.
Pavlakis
S G
SG
Gould
R J
RJ
De Vivo
D C
DC
D000293:Adolescent; D000328:Adult; D001930:Brain Injuries / Q000150:complications / Q000503:physiopathology*; D002648:Child; D003128:Coma / Q000209:etiology; D003655:Decerebrate State / Q000209:etiology*; D005334:Fever / Q000209:etiology*; D006801:Humans; D006973:Hypertension / Q000209:etiology*; D006985:Hyperventilation / Q000209:etiology*; D007031:Hypothalamus / Q000503:physiopathology*; D008297:Male; D008636:Mesencephalon / Q000503:physiopathology*; D013577:Syndrome