Tuesday, May 16, 2017

Giggle seizures: No laughing matter

Wilfong AA, Curry DJ. Hypothalamic hamartomas: optimal approach to clinical evaluation and diagnosis. Epilepsia. 2013 Dec;54 Suppl 9:109-14.

Hypothalamic hamartomas (HHs) present a difficult medical problem, manifested by gelastic seizures, which are often medically intractable. Although existing techniques offer modest surgical outcomes with the potential for significant morbidity, the relatively novel technique of magnetic resonance imaging (MRI)-guided stereotactic laser ablation (SLA) offers a potentially safer, minimally invasive method with high efficacy for the HH treatment. We report here on 14 patients with medically refractory gelastic epilepsy who underwent stereotactic frame-based placement of an MR-compatible laser catheter (1.6 mm diameter) through a 3.2-mm twist drill hole. A U.S. Food and Drug Administration (FDA)-cleared laser surgery system (Visualase, Inc.) was utilized to ablate the HH, using real-time MRI thermometry. Seizure freedom was obtained in 12 (86%) of 14 cases, with mean follow-up of 9 months. There were no permanent surgical complications, neurologic deficits, or neuroendocrine disturbances. One patient had a minor subarachnoid hemorrhage that was asymptomatic. Most patients were discharged home within 1 day. SLA was demonstrated to be a safe and effective minimally invasive tool in the ablation of epileptogenic HH. Because use of SLA for HH is being adopted by other medical centers, further data will be acquired to help treat this difficult disorder.

Rolston JD, Chang EF. Stereotactic Laser Ablation for Hypothalamic Hamartoma.Neurosurg Clin N Am. 2016 Jan;27(1):59-67
Stereotactic laser ablation (SLA) is a minimally invasive approach to the treatment of medication-resistant epilepsy that accomplishes ablation of the seizure focus with real-time magnetic resonance thermal mapping. Rates of seizure freedom in early series suggest that SLA approaches and perhaps surpasses the effectiveness of open resection. SLA minimizes the neurocognitive and endocrine adverse effects of open surgery. Secondary benefits of SLA include decreased length of stay, elimination of intensive care unit stay, reduced procedure-related discomfort, and improved access to surgical treatment for patients less likely to consider an open resective procedure.

Kameyama S, Shirozu H, Masuda H, Ito Y, Sonoda M, Akazawa K. MRI-guided stereotactic radiofrequency thermocoagulation for 100 hypothalamic hamartomas. J Neurosurg. 2016 May;124(5):1503-12.

OBJECT The aim of this study was to elucidate the invasiveness, effectiveness, and feasibility of MRI-guided stereotactic radiofrequency thermocoagulation (SRT) for hypothalamic hamartoma (HH). METHODS The authors examined the clinical records of 100 consecutive patients (66 male and 34 female) with intractable gelastic seizures (GS) caused by HH, who underwent SRT as a sole surgical treatment between 1997 and 2013. The median duration of follow-up was 3 years (range 1-17 years). Seventy cases involved pediatric patients. Ninety percent of patients also had other types of seizures (non-GS). The maximum diameter of the HHs ranged from 5 to 80 mm (median 15 mm), and 15 of the tumors were giant HHs with a diameter of 30 mm or more. Comorbidities included precocious puberty (33.0%), behavioral disorder (49.0%), and mental retardation (50.0%). RESULTS A total of 140 SRT procedures were performed. There was no adaptive restriction for the giant or the subtype of HH, regardless of any prior history of surgical treatment or comorbidities. Patients in this case series exhibited delayed precocious puberty (9.0%), pituitary dysfunction (2.0%), and weight gain (7.0%), besides the transient hypothalamic symptoms after SRT. Freedom from GS was achieved in 86.0% of patients, freedom from other types of seizures in 78.9%, and freedom from all seizures in 71.0%. Repeat surgeries were not effective for non-GS. Seizure freedom led to disappearance of behavioral disorders and to intellectual improvement. CONCLUSIONS The present SRT procedure is a minimally invasive and highly effective surgical procedure without adaptive limitations. SRT involves only a single surgical procedure appropriate for all forms of epileptogenic HH and should be considered in patients with an early history of GS.

Buckley RT, Wang AC, Miller JW, Novotny EJ, Ojemann JG. Stereotactic laser ablation for hypothalamic and deep intraventricular lesions. Neurosurg Focus.2016 Oct;41(4):E10.


OBJECTIVE Laser ablation is a novel, minimally invasive procedure that utilizes MRI-guided thermal energy to treat epileptogenic and other brain lesions. In addition to treatment of mesial temporal lobe epilepsy, laser ablation is increasingly being used to target deep or inoperable lesions, including hypothalamic hamartoma (HH), subependymal giant cell astrocytoma (SEGA), and exophytic intrinsic hypothalamic/third ventricular tumors. The authors reviewed their early institutional experience with these patients to characterize clinical outcomes in patients undergoing this procedure. METHODS A retrospective cohort (n = 12) of patients undergoing laser ablation at a single institution was identified, and clinical and radiographic records were reviewed. RESULTS Laser ablation was successfully performed in all patients. No permanent neurological or endocrine complications occurred; 2 (17%) patients developed acute obstructive hydrocephalus or shunt malfunction following treatment. Laser ablation of HH resulted in seizure freedom (Engel Class I) in 67%, with the remaining patients having a clinically significant reduction in seizure frequency of greater than 90% compared with preoperative baseline (Engel Class IIB). Treatment of SEGAs resulted in durable clinical and radiographic tumor control in 2 of 3 cases, with one patient receiving adjuvant everolimus and the other receiving no additional therapy. Palliative ablation of hypothalamic/third ventricular tumors resulted in partial tumor control in 1 of 3 patients. CONCLUSIONS Early experience suggests that laser ablation is a generally safe, durable, and effective treatment for patients harboring HHs. It also appears effective for local control of SEGAs, especially in combination therapy with everolimus. Its use as a palliative treatment for intrinsic hypothalamic/deep intraventricular tumors was less successful and associated with a higher risk of serious complications. Additional experience and long-term follow-up will be beneficial in further characterizing the effectiveness and risk profile of laser ablation in treating these lesions in comparison with conventional resective surgery or stereotactic radiosurgery.

See:  http://childnervoussystem.blogspot.com/2016/12/hypothalamic-hamartoma-treated-with.html

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