Gamma Knife for Giant Hypothalamic Hamartomas—Not the Only Effective Tool in the Kit

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.

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Gamma Knife Radiosurgery for Refractory Epilepsy Caused by Hypothalamic Hamartomas

Stereotactic and Functional Neurosurgery ◽

10.1159/000094036 ◽

2006 ◽

Vol 84 (2-3) ◽

pp. 82-87 ◽

Cited By ~ 45

Author(s):

David Mathieu ◽

Douglas Kondziolka ◽

Ajay Niranjan ◽

John Flickinger ◽

L. Dade Lunsford

Keyword(s):

Gamma Knife ◽

Refractory Epilepsy ◽

Gamma Knife Radiosurgery ◽

Hypothalamic Hamartomas

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Gamma knife surgery for hypothalamic hamartomas accompanied by medically intractable epilepsy and precocious puberty: experience in Mexico

Journal of Neurosurgery ◽

10.3171/sup.2005.102.s_supplement.0053 ◽

2005 ◽

Vol 102 (Special_Supplement) ◽

pp. 53-55 ◽

Cited By ~ 10

Author(s):

Marco A. Barajas ◽

Maria G. Ramírez-Guzmán ◽

Carlos Rodríguez-Vázquez ◽

Vinicio Toledo-Buenrostro ◽

Abel Cuevas-Solórzano ◽

...

Keyword(s):

Gamma Knife ◽

Precocious Puberty ◽

Intractable Epilepsy ◽

Gamma Knife Surgery ◽

Sleep Cycle ◽

Hypothalamic Hamartoma ◽

High Morbidity ◽

Content Type ◽

Hypothalamic Hamartomas ◽

Fine Print

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.

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Gamma Knife Radiosurgery for Hypothalamic Hamartomas: Systematic Review

10.1055/s-0041-1725545 ◽

2021 ◽

Author(s):

Aditya M. Kondajji ◽

Audree Evans ◽

Ansley Unterberger ◽

Daniel Kulinich ◽

Kevin Ding ◽

...

Keyword(s):

Systematic Review ◽

Gamma Knife ◽

Gamma Knife Radiosurgery ◽

Hypothalamic Hamartomas

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Hypothalamic Hamartomas: Neuropathological Features with and without Prior Gamma Knife Radiosurgery

Stereotactic and Functional Neurosurgery ◽

10.1159/000341076 ◽

2013 ◽

Vol 91 (1) ◽

pp. 45-55 ◽

Cited By ~ 7

Author(s):

John F. Kerrigan ◽

Angela Parsons ◽

Stephen G. Rice ◽

Kristina Simeone ◽

Andrew G. Shetter ◽

...

Keyword(s):

Gamma Knife ◽

Gamma Knife Radiosurgery ◽

Hypothalamic Hamartomas

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Gamma Knife Surgery for Epilepsy Related to Hypothalamic Hamartomas

Neurosurgery ◽

10.1097/00006123-200012000-00014 ◽

2000 ◽

Vol 47 (6) ◽

pp. 1343-1352 ◽

Cited By ~ 110

Author(s):

Jean Régis ◽

Fabrice Bartolomei ◽

Bertrand de Toffol ◽

Pierre Genton ◽

Tatsuya Kobayashi ◽

...

Keyword(s):

Gamma Knife ◽

Pathological Condition ◽

Gamma Knife Surgery ◽

Clear Correlation ◽

Drug Resistant ◽

Hypothalamic Hamartomas ◽

Generalized Seizures ◽

Successful Group ◽

Drug Resistant Epilepsy

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.

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Gamma Knife Surgery for Epilepsy Related to Hypothalamic Hamartomas

Neurosurgery ◽

10.1093/neurosurgery/47.6.1343 ◽

2000 ◽

Vol 47 (6) ◽

pp. 1343-1352 ◽

Cited By ~ 22

Author(s):

Jean Régis ◽

Fabrice Bartolomei ◽

Bertrand de Toffol ◽

Pierre Genton ◽

Tatsuya Kobayashi ◽

...

Keyword(s):

Gamma Knife ◽

Gamma Knife Surgery ◽

Hypothalamic Hamartomas

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Gamma Knife surgery for hypothalamic hamartomas causing refractory epilepsy: preliminary results from a prospective observational study

Journal of Neurosurgery ◽

10.3171/2010.8.gks101059 ◽

2010 ◽

Vol 113 (Special_Supplement) ◽

pp. 215-221 ◽

Cited By ~ 27

Author(s):

David Mathieu ◽

Charles Deacon ◽

Catherine-Andrée Pinard ◽

Brendan Kenny ◽

Julie Duval

Keyword(s):

Quality Of Life ◽

Observational Study ◽

Antiepileptic Drugs ◽

Gamma Knife ◽

Prospective Observational Study ◽

Refractory Epilepsy ◽

Gamma Knife Surgery ◽

Hypothalamic Hamartomas ◽

Treatment Volume

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.

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Gamma knife radiosurgery for hypothalamic hamartomas in patients with medically intractable epilepsy and precocious puberty

Journal of Neurosurgery ◽

10.3171/jns.2000.92.4.0726 ◽

2000 ◽

Vol 92 (4) ◽

pp. 726-731 ◽

Cited By ~ 50

Author(s):

Frank Unger ◽

Oskar Schröttner ◽

Klaus Haselsberger ◽

Eva Körner ◽

Robert Ploier ◽

...

Keyword(s):

Mr Imaging ◽

Gamma Knife ◽

Precocious Puberty ◽

Seizure Control ◽

Gamma Knife Radiosurgery ◽

Content Type ◽

Hypothalamic Hamartomas ◽

Gelastic Epilepsy ◽

Fine Print

✓ Hamartoma of the hypothalamus represents a well-known but rare cause of central precocious puberty and gelastic epilepsy. Due to the delicate site in which the tumor is located, surgery is often unsuccessful and associated with considerable risks. In the two cases presented, gamma knife radiosurgery was applied as a safe and noninvasive alternative to obtain seizure control.Two patients, a 13-year-old boy and a 6-year-old girl, presented with medically intractable gelastic epilepsy and increasing episodes of secondary generalized seizures. Abnormal behavior and precocious puberty were also evident. Magnetic resonance (MR) imaging revealed hypothalamic hamartomas measuring 13 and 11 mm, respectively. After general anesthesia had been induced in the patients, radiosurgical treatment was performed with margin doses of 12 Gy to 90% and 60% of isodose areas, covering volumes of 700 and 500 mm3, respectively.After follow-up periods of 54 months in the boy and 36 months in the girl, progressive decrease in both seizure frequency and intensity was noted (Engel outcome scores IIa and IIIa, respectively). Both patients are currently able to attend public school. Follow-up MR imaging has not revealed significant changes in the sizes of the lesions.Gamma knife radiosurgery can be an effective and safe treatment modality for achieving good seizure control in patients with hypothalamic hamartomas.

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