[Gelastic epilepsy and precocious puberty in hamartoma of the hypothalamus].
Four cases of hypothalamic hamartoma leading to gelastic epilepsy, precocious puberty and behavioural disorders are reported. Cerebral neuroradiologic examinations revealed a tumor-like mass attached to the hypothalamus in the region of the mamillary bodies in all cases. Precocious puberty developed in the two girls at 4 and 13 months but in neither of the two boys, who both suffered behaviour disturbances in the form of aggressive outbursts. A total resection of the tumors of both boys led to histologic confirmation of hamartoma. One boy was free of seizures upon follow-up, whereas seizure frequency in the other boy was reduced, while his aggressivity increased. The cases are discussed in context of current therapeutic conceptions of gelastic epilepsy and central precocious puberty.
Dammann
O
O
Universitätskinderklinik Hamburg-Eppendorf.
Commentz
J C
JC
Valdueza
J M
JM
Christante
L
L
Bentele
K H
KH
D000374:Aggression; D000927:Anticonvulsants / Q000627:therapeutic use; D002675:Child, Preschool; D003952:Diagnostic Imaging; D004569:Electroencephalography; D004827:Epilepsy / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D005260:Female; D006222:Hamartoma / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D007029:Hypothalamic Neoplasms / Q000150:complications* / Q000175:diagnosis; D007223:Infant; D007845:Laughter; D008297:Male; D011629:Puberty, Precocious / Q000209:etiology*
D000927:Anticonvulsants
Gelastische Epilepsie und Pubertas praecox bei Hamartom des Hypothalamus.