Surgical resection of hypothalamic hamartomas for severe behavioral symptoms.
Hypothalamic hamartomas (HHs) are associated with treatment-resistant epilepsy. Many patients also experience severe and sometimes disabling psychiatric problems. The most common behavioral symptoms consist of paroxysms of uncontrolled anger related to poor frustration tolerance. These can include violence, resulting in disrupted family or school relationships, and legal consequences including incarceration. In a large cohort of patients undergoing surgical resection of HHs for refractory epilepsy, 88% of families described an improvement in overall behavioral functioning [1]. Here, we describe four patients (three males, mean age=11.9 years) who underwent surgical resection of HHs largely for behavioral indications. Three patients had relatively well controlled seizures, and one had no history of epilepsy. All patients had striking improvement in their psychiatric comorbidity. HH resection can result in significant improvement in behavioral functioning, even in patients with relatively infrequent seizures. Further investigation under approved human research protocols is warranted.
Division of Pediatric Neurology, Barrow Neurological Institute, Phoenix, AZ 85013, USA.
Yu-tze
YT
Ng
Eric V
EV
Hastriter
Jennifer
J
Wethe
Kevin E
KE
Chapman
Erin C
EC
Prenger
George P
GP
Prigatano
Tracy
T
Oppenheim
Maggie
M
Varland
Harold L
HL
Rekate
John F
JF
Kerrigan
D000293:Adolescent; D001921:Brain / Q000601:surgery*; D002648:Child; D004827:Epilepsy / Q000209:etiology / Q000601:surgery*; D005260:Female; D006222:Hamartoma / Q000150:complications / Q000601:surgery; D006801:Humans; D007027:Hypothalamic Diseases / Q000150:complications / Q000601:surgery; D008297:Male; D001523:Mental Disorders / Q000209:etiology / Q000601:surgery*; D016896:Treatment Outcome