Resection of the lesion in patients with hypothalamic hamartomas and catastrophic epilepsy.
BACKGROUND
Patients with hypothalamic hamartomas (HH) often have severe refractory epilepsy, incapacitating behavioral abnormalities, and cognitive decline. Attempts to control the seizure disorder by resection of apparently epileptogenic mesial temporal or other cortical structures have failed consistently.


OBJECTIVE
To report a series of 13 patients in whom the hamartoma itself was resected.


METHODS
All patients underwent preoperative evaluation between ages 2 and 33 years and had subtotal or complete resection of the hamartoma. Follow-up ranged from 1 to 5.5 years (mean: 2.8 y).


RESULTS
Preoperatively, all patients had variable combinations of gelastic, complex partial, and generalized seizures. Eight had drop attacks. In addition, all had marked behavior abnormalities and cognitive impairment. Postoperatively, two patients are completely seizure-free and 11 are either seizure-free or have achieved a greater than 90% reduction of drop attacks and generalized tonic-clonic seizures. However, minor gelastic, complex partial, and atypical absence seizures have persisted in 11 patients, although at significantly reduced rates. In addition, there has been a dramatic improvement in behavior and cognition. Three patients had an anterior thalamic and one a capsular infarct, which left only minimal long-term deficits. Exact location of the lesion in relation to the interpeduncular fossa and the walls of the third ventricle correlated with extent of excision, seizure control, and complication rate.


CONCLUSIONS
Resection can alleviate both the seizures and the behavioral and cognitive abnormalities of hypothalamic hamartomas, but complications are frequent.
Palmini
A
A
Porto Alegre Epilepsy Surgery Program, Hospital São Lucas da PUCRS, Brazil. apalmini@conex.com.br
Chandler
C
C
Andermann
F
F
Costa Da Costa
J
J
Paglioli-Neto
E
E
Polkey
C
C
Rosenblatt
B
B
Montes
J
J
Martínez
J V
JV
Farmer
J P
JP
Sinclair
B
B
Aronyk
K
K
Paglioli
E
E
Coutinho
L
L
Raupp
S
S
Portuguez
M
M
D000293:Adolescent; D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000209:etiology; D004569:Electroencephalography; D004827:Epilepsy / Q000209:etiology* / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D006222:Hamartoma / Q000150:complications* / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D007029:Hypothalamic Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000601:surgery*; D000077213:Lamotrigine; D008279:Magnetic Resonance Imaging; D008297:Male; D019635:Neurosurgical Procedures / Q000009:adverse effects; D010291:Paresis / Q000209:etiology; D020127:Recovery of Function; D012074:Remission Induction; D014055:Tomography, Emission-Computed; D016896:Treatment Outcome; D014227:Triazines / Q000627:therapeutic use
D014227:Triazines; D000077213:Lamotrigine