OBJECTIVE: Drug-resistant epilepsy associated with hypothalamic hamartomas
(HHs) can be cured by microsurgical resection of the lesions. Morbidity and
mortality rates for microsurgery in this area are significant. Gamma knife
surgery (GKS) is less invasive and seems to be well adapted for this indic
ation.
METHODS: To evaluate the safety and efficacy of GKS to treat this uncommon
pathological condition, we organized a multicenter retrospective study. Ten
patients were treated in seven different centers. The follow-up periods we
re more than 12 months for eight patients, with a median follow-up period o
f 28 months (mean, 35 mo; range, 12-71 mo). All patients had severe drug-re
sistant epilepsy, including frequent gelastic and generalized tonic or toni
coclonic attacks. The median age was 13.5 years (range, 1-32 yr; mean, 14 y
r) at the time of GKS. Three patients experienced precocious puberty. All p
atients had sessile HHs. The median marginal dose was 15.25 Gy (range, 12-2
0 Gy). Two patients were treated two times (at 19 and 49 mo) because of ins
ufficient efficacy.
RESULTS: All patients exhibited improvement. Four patients were seizure-fre
e, one experienced rare nocturnal seizures, one experienced some rare parti
al seizures but no more generalized attacks, and two exhibited only improve
ment, with reductions in the frequency of seizures but persistence of some
rare generalized seizures. Two patients, now seizure-free, were considered
to exhibit insufficient improvement after the first GKS procedure and were
treated a second time. A clear correlation between efficacy and dose was ob
served in this series. The marginal dose was more than 17 Gy for all patien
ts in the successful group and less than 13 Cy for all patients in the "imp
roved" group. No side effects were reported, except for poikilothermia in o
ne patient. Behavior was clearly improved for two patients (with only sligh
t improvements in their epilepsy). Complete coverage of the HHs did not see
m to be mandatory, because the dosimetry spared a significant part of the l
esions for two patients in the successful group.
CONCLUSION: We report the first series demonstrating that GKS can be a safe
and effective treatment for epilepsy related to HHs. We advocate marginal
doses greater than or equal to 17 Gy and partial dose-planning when necessa
ry, for avoidance of critical surrounding structures.