Open-loop deep brain stimulation for the treatment of epilepsy: a systematic review of clinical outcomes over the past decade (2008-present).

OBJECTIVE The field of deep brain stimulation (DBS) for epilepsy has grown tremendously since its inception in the 1970s and 1980s. The goal of this review is to identify and evaluate all studies published on the topic of open-loop DBS for epilepsy over the past decade (2008 to present). METHODS A PubMed search was conducted to identify all articles reporting clinical outcomes of open-loop DBS for the treatment of epilepsy published since January 1, 2008. The following composite search terms were used: ("epilepsy" [MeSH] OR "seizures" [MeSH] OR "kindling, neurologic" [MeSH] OR epilep* OR seizure* OR convuls*) AND ("deep brain stimulation" [MeSH] OR "deep brain stimulation" OR "DBS") OR ("electric stimulation therapy" [MeSH] OR "electric stimulation therapy" OR "implantable neurostimulators" [MeSH]). RESULTS The authors identified 41 studies that met the criteria for inclusion. The anterior nucleus of the thalamus, centromedian nucleus of the thalamus, and hippocampus were the most frequently evaluated targets. Among the 41 articles, 19 reported on stimulation of the anterior nucleus of the thalamus, 6 evaluated stimulation of the centromedian nucleus of the thalamus, and 9 evaluated stimulation of the hippocampus. The remaining 7 articles reported on the evaluation of alternative DBS targets, including the posterior hypothalamus, subthalamic nucleus, ventral intermediate nucleus of the thalamus, nucleus accumbens, caudal zone incerta, mammillothalamic tract, and fornix. The authors evaluated each study for overall epilepsy response rates as well as adverse events and other significant, nonepilepsy outcomes. CONCLUSIONS Level I evidence supports the safety and efficacy of stimulating the anterior nucleus of the thalamus and the hippocampus for the treatment of medically refractory epilepsy. Level III and IV evidence supports stimulation of other targets for epilepsy. Ongoing research into the efficacy, adverse effects, and mechanisms of open-loop DBS continues to expand the knowledge supporting the use of these treatment modalities in patients with refractory epilepsy.

Cost of Deep Brain Stimulation Infection Resulting in Explantation.

BACKGROUND: Deep brain stimulation (DBS) hardware infection is a serious complication, often resulting in multiple hardware salvage attempts, hospitalizations, and long-term antibiotic therapy. OBJECTIVES: We aimed to quantify the costs of DBS hardware-related infections in patients undergoing eventual device explantation. METHODS: Of 362 patients who underwent 530 electrode placements (1 January 2010 to 30 December 2014), 16 (4.4%) had at least 2 hardware salvage procedures. Most (n = 15 [93.8%]) required complete explantation due to recurrent infection. Financial data (itemized hospital and physician costs) were available for 13 patients and these were analyzed along with the demographic data. RESULTS: Each patient underwent 1-5 salvage procedures (mean 2.5 ± 1.4; median 2). The mean total cost for a patient undergoing the median number of revisions (n = 2), device explantation, and subsequent reimplantation after infection clearance was USD 75,505; just over half this cost (54.2% [USD 40,960]) was attributable to reimplantation, and nearly one-third (28.9% [USD 21,816]) was attributable to hardware salvage procedures. Operating-room costs were the highest cost category for hardware revision and explantation. Medical and surgical supplies accounted for the highest reimplantation cost. CONCLUSIONS: DBS infection incurs significant health care costs associated with hardware salvage attempts, explantation, and reimplantation. The highest cost categories are operating-room services and medical and surgical supplies.

Hypothalamic hamartomas: neuropathological features with and without prior gamma knife radiosurgery.

BACKGROUND: The neuropathological consequences of Gamma Knife radiosurgery (GK) on hypothalamic hamartoma (HH) are unknown. OBJECTIVE: In a cohort of patients undergoing surgery for treatment-resistant epilepsy, we compared surgically resected HH tissue from patients without (group I; n = 19) and with (group II; n = 10) a history of GK (median dose 16 Gy to the 50% isodose margin). METHODS: Techniques included thick-section stereology for total nucleated and total neuron cell counts, and thin-section immunohistochemistry. Normal human hypothalamus derived from age-matched autopsy material was used as control tissue for CD68 immunohistochemistry. Qualitative scoring of tissue sections was performed by a neuropathologist who was blind to the GK treatment history. RESULTS: GK is associated with decreased total cell density (p < 0.02). A dose-dependent association of GK with decreased total neuron density approached significance (p = 0.06). Group II HH tissue had significantly more (1) reactive gliosis, (2) thickened capillary endothelium and (3) microglial activation. Degenerative features, including karyorrhexis and pyknotic nuclei, were infrequent in group II and absent in group I HH tissue. CONCLUSIONS: Nonnecrotizing doses of GK radiosurgery decrease cell density in human HH tissue. Cell loss resulting from GK may contribute to decreased excitation in the neuronal networks responsible for seizure onset in HH tissue.

Electrical stimulation of the anterior nucleus of thalamus for treatment of refractory epilepsy.

PURPOSE: We report a multicenter, double-blind, randomized trial of bilateral stimulation of the anterior nuclei of the thalamus for localization-related epilepsy. METHODS: Participants were adults with medically refractory partial seizures, including secondarily generalized seizures. Half received stimulation and half no stimulation during a 3-month blinded phase; then all received unblinded stimulation. RESULTS: One hundred ten participants were randomized. Baseline monthly median seizure frequency was 19.5. In the last month of the blinded phase the stimulated group had a 29% greater reduction in seizures compared with the control group, as estimated by a generalized estimating equations (GEE) model (p = 0.002). Unadjusted median declines at the end of the blinded phase were 14.5% in the control group and 40.4% in the stimulated group. Complex partial and "most severe" seizures were significantly reduced by stimulation. By 2 years, there was a 56% median percent reduction in seizure frequency; 54% of patients had a seizure reduction of at least 50%, and 14 patients were seizure-free for at least 6 months. Five deaths occurred and none were from implantation or stimulation. No participant had symptomatic hemorrhage or brain infection. Two participants had acute, transient stimulation-associated seizures. Cognition and mood showed no group differences, but participants in the stimulated group were more likely to report depression or memory problems as adverse events. DISCUSSION: Bilateral stimulation of the anterior nuclei of the thalamus reduces seizures. Benefit persisted for 2 years of study. Complication rates were modest. Deep brain stimulation of the anterior thalamus is useful for some people with medically refractory partial and secondarily generalized seizures.

Radiosurgery for multiple sclerosis-related trigeminal neuralgia: retrospective review of long-term outcomes.

OBJECTIVE: The objective of this study was to assess long-term outcomes of facial pain and numbness after radiosurgery for multiple sclerosis (MS)-related trigeminal neuralgia (MS-TN). METHODS: The authors conducted a retrospective review of their Gamma Knife radiosurgeries (GKRSs) to identify all patients treated for MS-TN (1998-2014) with at least 3 years of follow-up. Treatment and clinical data were obtained via chart review and mailed or telephone surveys. Pain control was defined as a facial pain score of I-IIIb on the Barrow Neurological Institute (BNI) Facial Pain Intensity Scale. Kaplan-Meier analysis was performed to determine the rates of pain control after index and first salvage GKRS procedures. Patients could have had more than 1 salvage procedure. Pain control rates were based on the number of patients at risk during follow-up. RESULTS: Of the 50 living patients who underwent GKRS, 42 responded to surveys (31 women [74%], median age 59 years, range 32-76 years). During the initial GKRS, the trigeminal nerve root entry zone was targeted with a single isocenter, using a 4-mm collimator with the 90% isodose line completely covering the trigeminal nerve and the 50% isodose line abutting the surface of the brainstem. The median maximum radiation dose was 85 Gy (range 50-85 Gy). The median follow-up period was 78 months (range 36-226 months). The rate of pain control after the index GKRS (n = 42) was 62%, 29%, 22%, and 13% at 1, 3, 5, and 7 years, respectively. Twenty-eight patients (67%) underwent salvage treatment, including 25 (60%) whose first salvage treatment was GKRS. The rate of pain control after the first salvage GKRS (n = 25) was 84%, 50%, 44%, and 17% at 1, 3, 5, and 7 years, respectively. The rate of pain control after the index GKRS with or without 1 salvage GKRS (n = 33) was 92%, 72%, 52%, 46%, and 17% at 1, 3, 5, 7, and 10 years, respectively. At last follow-up, 9 (21%) of the 42 patients had BNI grade I facial pain, 35 (83%) had achieved pain control, and 4 (10%) had BNI grade IV facial numbness (very bothersome in daily life). CONCLUSIONS: Index GKRS offers good short-term pain control for MS-TN, but long-term pain control is uncommon. If the index GKRS fails, salvage GKRS appears to offer beneficial pain control with low rates of bothersome facial numbness.

Resection and permanent intracranial brachytherapy using modular, biocompatible cesium-131 implants: results in 20 recurrent, previously irradiated meningiomas.

OBJECTIVE: Effective treatments for recurrent, previously irradiated intracranial meningiomas are limited, and resection alone is not usually curative. Thus, the authors studied the combination of maximum safe resection and adjuvant radiation using permanent intracranial brachytherapy (R+BT) in patients with recurrent, previously irradiated aggressive meningiomas. METHODS: Patients with recurrent, previously irradiated meningiomas were treated between June 2013 and October 2016 in a prospective single-arm trial of R+BT. Cesium-131 (Cs-131) radiation sources were embedded in modular collagen carriers positioned in the operative bed on completion of resection. The Cox proportional hazards model with this treatment as a predictive term was used to model its effect on time to local tumor progression. RESULTS: Nineteen patients (median age 64.5 years, range 50-78 years) with 20 recurrent, previously irradiated tumors were treated. The WHO grade at R+BT was I in 4 (20%), II in 14 (70%), and III in 2 (10%) cases. The median number of prior same-site radiation courses and same-site surgeries were 1 (range 1-3) and 2 (range 1-4), respectively; the median preoperative tumor volume was 11.3 cm3 (range 0.9-92.0 cm3). The median radiation dose from BT was 63 Gy (range 54-80 Gy). At a median radiographic follow-up of 15.4 months (range 0.03-47.5 months), local failure (within 1.5 cm of the implant bed) occurred in 2 cases (10%). The median treatment-site time to progression after R+BT has not been reached; that after the most recent prior therapy was 18.3 months (range 3.9-321.9 months; HR 0.17, p = 0.02, log-rank test). The median overall survival after R+BT was 26 months, with 9 patient deaths (47% of patients). Treatment was well tolerated; 2 patients required surgery for complications, and 2 experienced radiation necrosis, which was managed medically. CONCLUSIONS: R+BT utilizing Cs-131 sources in modular carriers represents a potentially safe and effective treatment option for recurrent, previously irradiated aggressive meningiomas.

Role of Gamma Knife surgery in the management of pineal region tumors.

OBJECT: Increasingly, radiosurgery is used to treat pineal region tumors, either as a primary treatment or as an adjunct to conventional radiation therapy. The authors report their experience with Gamma Knife surgery (GKS) for the treatment of pineal region tumors. METHODS: The authors retrospectively reviewed the charts of all patients undergoing GKS at their institution between 1997 and 2005. Seventeen patients underwent GKS for nonmetastatic tumors of the pineal region. All patients were treated using Leksell Gamma Plan treatment planning software (versions 4.12::5.34). The mean treatment volume was 7.42 cm(3) (range 1.2-32.5 cm(3)). Prescribed doses ranged from 12 to 18 Gy. All doses were prescribed to the 50% isodose line. Independent neuroradiologists reviewed all follow-up imaging studies for evidence of progression of disease. RESULTS: One patient (Case 10) died 6 days after GKS. Mean clinical and imaging follow-up in the remaining 16 cases was 31 months. Local control was established during a mean neuroimaging follow-up period of 31 months (range 1-95) in 16 patients (100%). In 2 of these 16 patients (one with an anaplastic astrocytoma, the other with a primitive neuroectodermal tumor), leptomeningeal and spinal spread of tumor developed despite control of the pineal lesions. There were no new neurological deficits attributable to GKS. Three patients died (including the one who died 6 days after GKS) during the follow-up period. Conclusions Excellent control of pineal region brain tumors can be obtained with GKS when it is used in conjunction with surgery, conventional radiation therapy, or both. Patient survival and quality of life can be optimized through the use of multimodal treatment, including surgery, conventional radiation therapy and/or radiosurgery, and chemotherapy, when applicable.

Microvascular decompression after gamma knife surgery for trigeminal neuralgia: intraoperative findings and treatment outcomes.

OBJECT: The authors sought to determine whether the results of trigeminal microvascular decompression (MVD) are influenced by prior gamma knife surgery (GKS). METHODS: Gamma knife surgery is an established procedure for treating medically intractable trigeminal neuralgia but failures do occur. The authors assessed six patients (two men and four women; mean age 52 years) who experienced pain recurrence after GKS and elected to undergo trigeminal MVD via retrosigmoid craniotomy. Three patients underwent a single GKS to a maximal dose of 80 Gy, whereas three others underwent a second GKS to total of 120 to 135 Gy. At surgery, none of the six patients demonstrated excess arachnoid thickening, grossly apparent changes in the nerve itself, or any other tissue alterations that made successful mobilization of a blood vessel from the trigeminal root entry zone technically more difficult. A single individual had a small atherosclerotic plaque in the superior cerebellar artery near its contact point with the trigeminal nerve. Follow up at a mean of 25.4 months (range 7.5-42 months) indicated that five patients were pain free. One patient had improved but still relied on medications for pain control. CONCLUSIONS: In the authors' experience, trigeminal MVD can be performed without added difficulty in patients who have previously undergone GKS. The success rates seem similar to those normally associated with MVD. Patients who elect the less invasive option of GKS can be assured that trigeminal MVD remains a viable alternative at a later date if further surgery is required.

Radiation Necrosis Secondary to Trigeminal Nerve TomoTherapy: A Cautionary Case Study.

New radiation delivery modalities have recently challenged Gamma Knife surgery as the historic gold standard in the treatment of trigeminal neuralgia (TN). TomoTherapy, a relative newcomer, has been approved by the U.S. FDA for various intracranial pathologies but is currently off label for the treatment of TN. A 73-year-old female presented with gait instability, intermittent headaches, and confusion. She was treated with TomoTherapy for refractory TN at an outside facility, which failed to reduce her symptoms. Magnetic resonance imaging demonstrated a lesion in the right mesial temporal lobe. A standard right anterior temporal lobectomy was performed and the final pathological report was notable for necrosis, gliosis, and edema consistent with a remote radiation injury. The patient improved postoperatively, but at her two-year follow up, she continued to have persistent bilateral TN and new onset seizures. Imaging revealed no new mass in the resection field. Stereotactic radiosurgery (SRS) is an evolving field with broadening indications, which makes it ever more important for physicians to be aware of differences between various SRS modalities. This case report highlights a cautionary example, and emphasizes the need for a more systematic evaluation of novel SRS methods before clinical application.

Physiology and pathophysiology of cortico-basal ganglia-thalamocortical loops: theoretical and practical aspects.

A new theoretical framework is used to analyze functions and pathophysiological processes of cortico-basal ganglia-thalamocortical loops and to demonstrate the hierarchical relationships between various loops. All hierarchical levels are built according to the same functional principle: Each loop is a neural optimal control system (NOCS) and includes a model of object behavior and an error distribution system. The latter includes dopaminergic neurons and is necessary to tune the model to a controlled object (CO). The regularities of pathophysiological processes in NOCSs are analyzed. Mechanisms of current functional neurosurgical procedures like lesioning and deep brain stimulation (DBS) of various basal ganglia structures and neurotransplantation are described based on proposed theoretical ideas. Parkinson's disease (PD) is used to exemplify clinical applications of the proposed theory. Within the proposed theoretical framework, PD must be considered as a disease of the error distribution system. The proposed theoretical views have broad fundamental and clinical applications.

Gamma knife radiosurgery for recurrent trigeminal neuralgia.

OBJECT: Pain may fail to respond or may recur after initial gamma knife radiosurgery (GKS) for trigeminal neuralgia (TN). The authors examined their experience with performing a second GKS procedure in these patients. METHODS: Twenty-nine patients underwent repeated GKS for TN at our institution between March 1997 and March 2002. Questionnaires were mailed to patients to assess the degree of their pain relief and the extent of facial numbness. Nineteen patients responded. All patients underwent repeated GKS involving a single 4-mm isocenter directed at the trigeminal nerve as it exited the brainstem (mean maximum dose 23.2 Gy). At a mean follow up of 13.5 months after the second procedure, 10 patients (53%) were pain free and medication free. Four patients (21%) were pain free but elected to continue medication in reduced dose, and two patients (11%) had incomplete but satisfactory pain control and were still taking medication. There was new-onset facial numbness in eight patients (42%), rated as tolerable in all instances. CONCLUSIONS: Patients with facial numbness had a greater likelihood of being pain free than those with no sensory loss. The authors observed no cases of corneal anesthesia, keratitis, or deafferentation pain.

Gamma knife radiosurgery for trigeminal neuralgia associated with multiple sclerosis.

OBJECT: The authors assessed the efficacy and complications from gamma knife radiosurgery (GKS) for multiple sclerosis (MS)-associated trigeminal neuralgia (TN). METHODS: There were 15 patients with MS-associated TN (MS-TN). Treatment involved three sequential protocols, 70 to 90-Gy maximum dose, using a single 4-mm isocenter targeting the ipsilateral trigeminal nerve at its junction with the pons with the 50% isodose. Pain was appraised by each patient by using Barrow Neurological Institute (BNI) Scores I through IV: I, no pain; II, occasional pain not requiring medication; IIIa, no pain but continued medication; IIIb, some pain, controlled with medication; IV, some pain, not controlled with medication; and V, severe pain/no pain relief. With a mean follow up of 17 months (range 6-38 months), 12 (80%) of 15 patients experienced pain relief. Three patients (20%) reported no relief (BNI Score V). For responders, the mean latency from treatment to the onset of pain relief was 13 days (range 1-61 days). Maximal relief was achieved after a mean latency of 56 days (range 1-157 days). Five patients underwent a second GKS after a mean interval of 534 days (range 231-946 days). The mean maximum dose at this second treatment was 48 Gy. The target was unchanged from the first treatment. All five patients who underwent repeated GKS improved. Complications were limited to delayed facial hypesthesias. Two (13%) of 15 patients experienced onset of numbness after the first GKS, as well as two of five patients following a second GKS. The patients found this mild and not bothersome. Each patient who developed hypesthesias also experienced complete pain relief. CONCLUSIONS: Gamma knife radiosurgery is an effective treatment for MS-TN. Radiosurgery carries an acceptable small risk of mild facial hypesthesias, and hypesthesia appears predictive of a favorable outcome.

Seizure outcome and complications following hypothalamic hamartoma treatment in adults: endoscopic, open, and Gamma Knife procedures.

OBJECT: This study aimed at identifying outcomes with respect to seizures, morbidity, and mortality in adult patients undergoing resective or Gamma Knife surgery (GKS) to treat intractable epilepsy associated with hypothalamic hamartoma (HH). METHODS: Adult patients undergoing surgical treatment for HH-related epilepsy were prospectively monitored at a single center for complications and seizure outcome by using a proprietary database. Preintervention and postintervention data for patients 18 years of age and older, and with at least 1 year of follow-up, were analyzed, with specific attention to seizure control, complications, hormonal status, and death. RESULTS: Forty adult patients were found in the database (21 were women). The median HH volume was 0.54 cm(3). In 70% of patients, it was located inside the third ventricle, attached unilaterally and vertically to the hypothalamus (Delalande Type II). Most patients (26) underwent an endoscopic resection, 10 patients had a transcallosal or other type of open (pterional or orbitozygomatic) resection, and 4 patients chose GKS. Twenty-nine percent became seizure free in the long term, and overall a majority of patients (55%) reported at least > 90% seizure improvement. Only 3 patients were ultimately able to discontinue anticonvulsants, whereas most patients were taking an average of 2 antiepileptic drugs pre- and postoperatively. The only factor significantly correlated with seizure-free outcome was the absence of mental retardation. The HH volume, HH type, and amount of resection or disconnection were not correlated to seizure freedom. A total of 4 patients (10%) died, 2 immediately after surgery and 2 later. All of them had undergone a resection, as opposed to GKS, and still had seizures. Postoperatively, persistent neurological deficits were seen in 1 patient; 34% of patients had mild hormonal problems; and 59% experienced weight gain of at least 6.8 kg (average gain 12.7 kg). CONCLUSIONS: Surgical or GKS procedures in adults with HH provided seizure freedom in one-third of patients. The only significant favorable prognostic factor was the absence of mental retardation. The overall mortality rate was high, at 10%. Other important morbidities were persistent hormonal disturbances and weight gain.

Long-term radiosurgical control of subtotally resected adult pineocytomas.

OBJECT: The optimal management of pineocytomas remains controversial. Although the value of complete microsurgical removal is well accepted, gross-total resection is not always feasible. Data regarding the role of postoperative adjuvant stereotactic radiosurgery (SRS) for residual disease is limited and conflicting. Here, the authors review the largest single-institution experience with multimodal pineocytoma management in an effort to quantify the utility of adjuvant radiosurgical treatment of residual disease. METHODS: The medical records and radiographic studies for all patients with histologically confirmed pineocytoma at the Barrow Neurological Institute between 1999 and 2011 were retrospectively reviewed. Clinical and radiographic data, including the volumetric extent of resection, were collected retrospectively, and Kaplan-Meier analysis was used to identify progression-free survival. RESULTS: Fourteen adults with newly diagnosed pineocytomas were surgically treated in the period from 1999 to 2011. The median clinical and radiographic follow-ups were 44 and 53 months, respectively. Twelve patients (86%) underwent microsurgical removal and 2 (14%) underwent endoscopic biopsy. Five patients (36%) had complete resections and 9 (64%) demonstrated residual disease. Three patients (21%) presented with radiographic recurrence at a median interval of 43 months after initial treatment (range 13-83 months). At the time of recurrence, the median preoperative tumor volume was 2.6 cm(3). Adjuvant SRS was used to treat 3 subtotally resected tumors (33%) following initial presentation and 2 (66%) at the time of recurrence. Among patients with subtotally resected tumors, progression-free survival was significantly longer (p < 0.05) for those who did as compared with those who did not undergo adjuvant radiosurgery. To date, no patient who underwent adjuvant radiosurgery has demonstrated radiographic or clinical evidence of disease progression. CONCLUSIONS: Microsurgical removal remains the definitive treatment for pineocytomas, yet residual disease can be effectively controlled using adjuvant SRS.

Gamma Knife surgery combined with resection for treatment of a single brain metastasis: preliminary results.

OBJECT: Resection and whole-brain radiation therapy (WBRT) have classically been the standard treatment for a single metastasis to the brain. The objective of this study was to evaluate the use of Gamma Knife surgery (GKS) as an alternative to WBRT in patients who had undergone resection and to evaluate patient survival and local tumor control. METHODS: The authors retrospectively reviewed the charts of 150 patients treated with a combination of stereotactic radiosurgery and resection of a cranial metastasis at their institution between April 1997 and September 2009. Patients who had multiple lesions or underwent both WBRT and GKS were excluded, as were patients for whom survival data beyond the initial treatment were not available. Clinical and imaging follow-up was assessed using notes from clinic visits and MR imaging studies when available. Follow-up data beyond the initial treatment and survival data were available for 68 patients. RESULTS: The study included 37 women (54.4%) and 31 men (45.6%) (mean age 60 years, range 28-89 years). In 45 patients (66.2%) there was systemic control of the primary tumor when the cranial metastasis was identified. The median duration between resection and radiosurgery was 15.5 days. The median volume of the treated cavity was 10.35 cm(3) (range 0.9-45.4 cm(3)), and the median dose to the cavity margin was 15 Gy (range 14-30 Gy), delivered to the 50% isodose line (range 50%-76% isodose line). The patients' median preradiosurgery Karnofsky Performance Scale (KPS) score was 90 (range 40-100). During the follow-up period we identified 27 patients (39.7%) with recurrent tumor located either local or distant to the site of treatment. The median time from primary treatment of metastasis to recurrence was 10.6 months. The patients' median length of survival (interval between first treatment of cerebral metastasis and last follow-up) was 13.2 months. For the patient who died during follow-up, the median time from diagnosis of cerebral metastasis to death was 11.5 months. The median duration of survival from diagnosis of the primary cancer to last follow-up was 30.2 months. Patients with a pretreatment KPS score ≥ 90 had a median survival time of 23.2 months, and patients with a pretreatment KPS score < 90 had a median survival time of 10 months (p < 0.008). Systemic control of disease at the time of metastasis was not predictive of increased survival duration, although it did tend to improve survival. CONCLUSIONS: Although the debate about the ideal form of radiation treatment after resection continues, these findings indicate that GKS combined with surgery offers comparable survival duration and local tumor control to WBRT for patients with a diagnosis of a single metastasis.

Gamma Knife surgery for hypothalamic hamartomas and epilepsy: patient selection and outcomes.

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 mm(3) (range 169-3000 mm(3), median 265 mm(3)). 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.

Salvage gamma knife stereotactic radiosurgery for surgically refractory trigeminal neuralgia.

PURPOSE: To evaluate the clinical outcome of patients with surgically refractory trigeminal neuralgia (TN) treated with rescue gamma knife radiosurgery (GKRS). METHODS AND MATERIALS: Seventy-nine patients with typical TN received salvage GKRS between 1997 and 2002 at the Barrow Neurological Institute (BNI). All patients had recurrent pain following at least one prior surgical intervention. Prior surgical interventions included percutaneous destructive procedures, microvascular decompression (MVD), or GKRS. Thirty-one (39%) had undergone at least two prior procedures. The most common salvage dose was 80 Gy, although 40-50 Gy was typical in patients who had received prior radiosurgery. Pain outcome was assessed using the BNI Pain Intensity Score, and quality of life was assessed using the Brief Pain Inventory. RESULTS: Median follow-up after salvage GKRS was 5.3 years. Actuarial analysis demonstrated that at 5 years, 20% of patients were pain-free and 50% had pain relief. Pain recurred in patients who had an initial response to GKRS at a median of 1.1 years. Twenty-eight (41%) required a subsequent surgical procedure for recurrence. A multivariate Cox proportional hazards model suggested that the strongest predictor of GKRS failure was a history of prior MVD (p=0.029). There were no instances of serious morbidity or mortality. Ten percent of patients developed worsening facial numbness and 8% described their numbness as "very bothersome." CONCLUSIONS: GKRS salvage for refractory TN is well tolerated and results in long-term pain relief in approximately half the patients treated. Clinicians may reconsider using GKRS to salvage patients who have failed prior MVD.

Cranial migration of a baclofen pump catheter associated with subarachnoid hemorrhage: case report.

OBJECTIVE: Cephalad migration of an indwelling intrathecal catheter within the spinal canal has rarely been described. Cranial subarachnoid hemorrhage (SAH) related to movement of this type of catheter has not been described. We report a case of SAH coincident with the migration of a free fragment of a baclofen pump catheter into the prepontine cistern. CLINICAL PRESENTATION: A baclofen pump system was removed from a 47-year-old man with spasticity related to multiple sclerosis. A section retained in the spinal canal extended up to the T9 level. Ten days after the pump and lower portion of the catheter were removed, the patient presented with a severe headache and a classic aneurysmal pattern of SAH. The patient's catheter was found to have migrated adjacent to the basilar artery at the level of the superior cerebellar artery. An extensive evaluation, including computed tomography angiography, digital subtraction angiography performed twice, magnetic resonance imaging, and magnetic resonance angiography, showed no apparent cause for the hemorrhage. Initially, the catheter was left in place. However, 5 months after the SAH, the patient elected to have the catheter removed. INTERVENTION: The catheter was pulled out from below through a C6-C7 laminoplasty without complications. The patient made an excellent recovery. DISCUSSION: Cephalad catheter migration is a rare phenomenon. The mechanism of rostral migration remains unclear. The forces that propel a free fragment of catheter under these circumstances seem to be sufficient to cause a small vessel to rupture and bleed. Given the lack of an observed arterial injury, we postulate that venous bleeding caused this hemorrhage.

Long-term pain response and quality of life in patients with typical trigeminal neuralgia treated with gamma knife stereotactic radiosurgery.

OBJECTIVE: The long-term outcome of patients treated with gamma knife radiosurgery (GKRS) for typical trigeminal neuralgia has not been fully studied. We evaluated 185 patients who underwent their first GKRS treatment between 1997 and 2003 at the Barrow Neurological Institute. METHODS: Follow-up was obtained by surveys and review of medical records. Outcomes were assessed by the Barrow Neurological Institute Pain Intensity Score and Brief Pain Inventory. The most common maximum dose was 80 Gy targeted at the root entry zone. Outcomes are presented for the 136 (74%) patients for whom more than 4 years of clinical follow-up data were obtained. RESULTS: Treatment failed in 33% of the cohort within 2 years, but only an additional 1% relapsed after 4 years. Actuarial analysis demonstrated that 32% of patients were pain-free off medication and 63% had at least a good outcome at 7 years. When GKRS was used as the primary treatment, 45% of the patients were pain-free at 7 years. In contrast, 10% of patients in whom previous treatment had failed were pain-free. When needed, salvage therapy with repeat GKRS, microvascular decompression, or percutaneous lesioning was successful in 70%. Posttreatment facial numbness was reported as very bothersome in 5%, most commonly in patients who underwent another invasive treatment. After GKRS, 73% reported that trigeminal neuralgia had no impact on their quality of life. CONCLUSION: GKRS is a reasonable long-term treatment option for patients with typical trigeminal neuralgia. It yields durable pain control in a majority of patients, as well as improved quality of life with limited complications and it does not significantly affect the efficacy of other surgical treatments, should they be needed.