Gamma Knife Surgery for Epilepsy Related to Hypothalamic Hamartomas

Neurosurgery ◽  
2000 ◽  
Vol 47 (6) ◽  
pp. 1343-1352 ◽  
Author(s):  
Jean Régis ◽  
Fabrice Bartolomei ◽  
Bertrand de Toffol ◽  
Pierre Genton ◽  
Tatsuya Kobayashi ◽  
...  
2005 ◽  
Vol 102 ◽  
pp. 53-55 ◽  
Author(s):  
Marco A. Barajas ◽  
Maria G. Ramírez-Guzmán ◽  
Carlos Rodríguez-Vázquez ◽  
Vinicio Toledo-Buenrostro ◽  
Abel Cuevas-Solórzano ◽  
...  

Object.Hypothalamic hamartoma is a nonneoplastic malformative mass of neurons and glia in the region of the hypothalamus. Because of its location, open surgery is associated with high morbidity and mortality rates. Gamma knife surgery (GKS) may be an efficient and safe treatment approach, which produces little morbidity. The authors describe the results of GKS in three patients with hypothalamic hamartomas.Methods.All patients were male, aged 3, 12, and 15 years. The lesions were classified according to the Valdueza scale: one was Type IIb and two were Type IIa. The patients presented with gelastic seizures (15–20 per day), generalized epilepsy, behavioral abnormalities, and alterations of the sleep cycle. Precocious puberty was present in one patient. The Type IIb tumor had a volume of 1.8 cm3, and the Type IIa tumors were 597 mm3and 530.1 mm3. The lesions received 12.5 Gy, 14 Gy, and 15 Gy, respectively, to the 50% isodose line. The patients were followed for 30 to 50 months. After 3 months, all patients showed improvement of their sleep, behavior, and epilepsy. At the present time, these patients are receiving low-dose antiepileptic agents and have achieved adequate social development and school integration.Conclusions.Gamma knife surgery appears to be a good, safe, and effective option for the treatment of selected hypothalamic hamartomas. No morbidity or mortality was associated with these three cases.


2005 ◽  
Vol 102 (Special_Supplement) ◽  
pp. 53-55 ◽  
Author(s):  
Marco A. Barajas ◽  
Maria G. Ramírez-Guzmán ◽  
Carlos Rodríguez-Vázquez ◽  
Vinicio Toledo-Buenrostro ◽  
Abel Cuevas-Solórzano ◽  
...  

Object. Hypothalamic hamartoma is a nonneoplastic malformative mass of neurons and glia in the region of the hypothalamus. Because of its location, open surgery is associated with high morbidity and mortality rates. Gamma knife surgery (GKS) may be an efficient and safe treatment approach, which produces little morbidity. The authors describe the results of GKS in three patients with hypothalamic hamartomas. Methods. All patients were male, aged 3, 12, and 15 years. The lesions were classified according to the Valdueza scale: one was Type IIb and two were Type IIa. The patients presented with gelastic seizures (15–20 per day), generalized epilepsy, behavioral abnormalities, and alterations of the sleep cycle. Precocious puberty was present in one patient. The Type IIb tumor had a volume of 1.8 cm3, and the Type IIa tumors were 597 mm3 and 530.1 mm3. The lesions received 12.5 Gy, 14 Gy, and 15 Gy, respectively, to the 50% isodose line. The patients were followed for 30 to 50 months. After 3 months, all patients showed improvement of their sleep, behavior, and epilepsy. At the present time, these patients are receiving low-dose antiepileptic agents and have achieved adequate social development and school integration. Conclusions. Gamma knife surgery appears to be a good, safe, and effective option for the treatment of selected hypothalamic hamartomas. No morbidity or mortality was associated with these three cases.


Neurosurgery ◽  
2000 ◽  
Vol 47 (6) ◽  
pp. 1343-1352 ◽  
Author(s):  
Jean Régis ◽  
Fabrice Bartolomei ◽  
Bertrand de Toffol ◽  
Pierre Genton ◽  
Tatsuya Kobayashi ◽  
...  

ABSTRACT 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 indication. 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 were more than 12 months for eight patients, with a median follow-up period of 28 months (mean, 35 mo; range, 12–71 mo). All patients had severe drug-resistant epilepsy, including frequent gelastic and generalized tonic or tonicoclonic attacks. The median age was 13.5 years (range, 1–32 yr; mean, 14 yr) at the time of GKS. Three patients experienced precocious puberty. All patients had sessile HHs. The median marginal dose was 15.25 Gy (range, 12–20 Gy). Two patients were treated two times (at 19 and 49 mo) because of insufficient efficacy. RESULTS All patients exhibited improvement. Four patients were seizure-free, one experienced rare nocturnal seizures, one experienced some rare partial seizures but no more generalized attacks, and two exhibited only improvement, 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 observed in this series. The marginal dose was more than 17 Gy for all patients in the successful group and less than 13 Gy for all patients in the “improved” group. No side effects were reported, except for poikilothermia in one patient. Behavior was clearly improved for two patients (with only slight improvements in their epilepsy). Complete coverage of the HHs did not seem to be mandatory, because the dosimetry spared a significant part of the lesions 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 necessary, for avoidance of critical surrounding structures.


2010 ◽  
Vol 113 (Special_Supplement) ◽  
pp. 215-221 ◽  
Author(s):  
David Mathieu ◽  
Charles Deacon ◽  
Catherine-Andrée Pinard ◽  
Brendan Kenny ◽  
Julie Duval

Object Hypothalamic hamartomas (HHs) are congenital lesions typically presenting with medically refractory epilepsy. Open or endoscopic surgical procedures to remove or disconnect the hamartoma have been reported to be effective but are associated with significant morbidity. The authors of studies on Gamma Knife surgery for HHs have reported an encouraging rate of epilepsy resolution with minimal side effects. At the Centre Hospitalier Universitaire de Sherbrooke, the authors have undertaken a prospective observational study of the outcomes of patients who underwent radiosurgery for HHs. Methods Patients were included in the study if they had an HH, refractory epilepsy, and no other suspected seizure focus. After radiosurgery, seizure status was assessed every 3 months and reported using the Engel Classification. Quality of life evaluation was performed annually using a standardized questionnaire, and neuropsychological evaluation was performed after 2 years. Results Nine patients were included in the study. They ranged in age from 12 to 57 years. Epilepsy began in infancy in all cases and was refractory to standard antiepileptic drugs. The patients received an average of 2 antiepileptic drugs before undergoing radiosurgery. Using the Régis Classification, 6 patients had smaller hamartomas (Grade I–III) and underwent treatment of the entire lesion, using a margin dose of 14–20 Gy. Treatment volume ranged from 0.3 to 1.0 ml. Three patients had larger lesions (Grade IV–VI) for which a radiosurgical disconnection was attempted, targeting the area of attachment to the hypothalamus. For those patients, the margin dose was 15 or 16 Gy, with treatment volume ranging from 0.8 to 1.8 ml. In all patients, the radiation dose received by the optic pathways was kept below 10 Gy. Disconnection led to no improvement in epilepsy (Engel Class IV). Four patients in whom the entire lesion was treated are now seizure free (Engel Class I), with another having only rare seizures (Engel Class II). Quality of life and verbal memory were improved in those patients with more than 3 years of follow-up. No adverse event occurred after radiosurgery. Conclusions Radiosurgery safely and effectively controlled the epileptic disorder in patients with HHs when the entire lesion could be targeted. Radiosurgical disconnection is ineffective and cannot be recommended.


2016 ◽  
Vol 127 (9) ◽  
pp. e166
Author(s):  
Véronique Martel ◽  
David Mathieu ◽  
Catherine-Andrée Pinard ◽  
Pascale Bourgeois ◽  
Julie Duval ◽  
...  

2013 ◽  
Vol 18 ◽  
pp. S345
Author(s):  
N. Martínez Moreno ◽  
N. Lorite Díaz ◽  
E. Kusak ◽  
R. Martínez Álvarez

2007 ◽  
Vol 14 (2) ◽  
pp. 73-79 ◽  
Author(s):  
Jean Régis ◽  
Didier Scavarda ◽  
Manabu Tamura ◽  
Nathalie Villeneuve ◽  
Fabrice Bartolomei ◽  
...  

Neurosurgery ◽  
2000 ◽  
Vol 47 (2) ◽  
pp. 502-502
Author(s):  
Jean Régis ◽  
Fabrice Bartolomei ◽  
Tatsuhia Takakura ◽  
Tomokatsu Hori ◽  
Oskar Schrottner ◽  
...  

2010 ◽  
Vol 113 (Special_Supplement) ◽  
pp. 207-214 ◽  
Author(s):  
Adib A. Abla ◽  
Andrew G. Shetter ◽  
Steve W. Chang ◽  
Scott D. Wait ◽  
David G. Brachman ◽  
...  

Object The authors present outcomes obtained in patients who underwent Gamma Knife surgery (GKS) at 1 institution as part of a multimodal treatment of refractory epilepsy caused by hypothalamic hamartomas (HHs). Methods Between 2003 and 2010, 19 patients with HH underwent GKS. Eight patients had follow-up for less than 1 year, and 1 patient was lost to follow-up. The 10 remaining patients (mean age 15.1 years, range 5.7–29.3 years) had a mean follow-up of 43 months (range 18–81 months) and are the focus of this report. Five patients had undergone a total of 6 prior surgeries: 1 transcallosal resection of the HH, 2 endoscopic transventricular resections of the HH, 2 temporal lobectomies, and 1 arachnoid cyst evacuation. In an institutional review board–approved study, postoperative complications and long-term outcome measures were monitored prospectively with the use of a proprietary database. Seven patients harbored Delalande Type II lesions; the remainder harbored Type III or IV lesions. Seizure frequency ranged from 1–2 monthly to as many as 100 gelastic seizures daily. The mean lesion volume was 695 mm3 (range 169–3000 mm3, median 265 mm3). The mean/median dose directed to the 50% isodose line was 18 Gy (range 16–20 Gy). The mean maximum point dose to the optic chiasm was 7.5 Gy (range 5–10 Gy). Three patients underwent additional resection 14.5, 21, and 32 months after GKS. Results Of the 10 patients included in this study, 6 are seizure free (2 after they underwent additional surgery), 1 has a 50%–90% reduction in seizure frequency, 2 have a 50% reduction in seizure frequency, and 1 has observed no change in seizure frequency. Overall quality of life, based on data obtained from follow-up telephone conversations and/or surveys, improved in 9 patients and was due to improvements in seizure control (9 patients), short-term memory loss (3 patients), and behavioral symptoms (5 patients); in 1 patient, quality of life remains minimally affected. Incidences of morbidity were all temporary and included poikilothermia (1 patient), increased depression (1 patient), weight gain/increased appetite (2 patients), and anxiety (1 patient) after GKS. Conclusions Of the approximately 150 patients at Barrow Neurological Institute who have undergone treatment for HH, the authors have reserved GKS for treatment of small HHs located distal from radiosensitive structures in patients with high cognitive function and a stable clinical picture, which allows time for the effects of radiosurgery to occur without further deterioration. The lack of significant morbidity and the clinical outcomes achieved in this study demonstrated a low risk of GKS for HH with results comparable to those of previous series.


2004 ◽  
pp. 33-50 ◽  
Author(s):  
J. Régis ◽  
M. Hayashi ◽  
L. P. Eupierre ◽  
N. Villeneuve ◽  
F. Bartolomei ◽  
...  

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