Gamma Knife Thalamotomy for Essential Tremor: A Retrospective Analysis.

BACKGROUND: Gamma knife (GK) thalamotomy has been used as a treatment option for essential tremor (ET). Numerous studies on GK use in ET treatment have reported more varied responses and complication rates. METHODS: Data from 27 patients with ET who underwent GK thalamotomy were retrospectively analyzed. The Fahn-Tolosa-Marin Clinical Rating Scale for Tremor, handwriting, and spiral drawing were evaluated. Postoperative adverse events and magnetic resonance imaging findings were also evaluated. RESULTS: The mean age at GK thalamotomy was 78.1 ± 4.2 years. The mean follow-up period was 32.5 ± 19.4 months. The preoperative postural tremor, handwriting, and spiral drawing scores were 3.4 ± 0.6, 3.3 ± 1.0, and 3.2 ± 0.8, respectively, all of which showed significant improvements to 1.5 ± 1.2 (55.9% improvement, P < 0.001), 1.4 ± 1.1 (57.6% improvement, P < 0.001), and 1.6 ± 1.3 (50% improvement, P < 0.001), respectively, at the available final follow-up evaluations. Three patients presented with no improvement in tremor. Six patients presented with adverse effects, including complete hemiparesis, foot weakness, dysarthria, dysphagia, lip numbness, and finger numbness, at the final follow-up period. Two patients presented with serious complications, including complete hemiparesis due to massive widespread edema and chronic encapsulated expanding hematoma. One patient died of aspiration pneumonia following severe dysphagia secondary to chronic encapsulated expanding hematoma. CONCLUSIONS: GK thalamotomy is an efficient procedure for treating ET. Careful treatment planning is necessary to reduce complication rates. The prediction of radiation complications will increase the safety and effectiveness of GK treatment.

Selective surgical mamillo-thalamic tract disconnection in hypothalamic hamartoma results in complete disappearance of gelastic seizures.

Hypothalamic hamartoma is a less common condition characterized by the several types of epileptic seizures including the gelastic type. It is reported that gelastic seizures are resistant to medical treatment with anticonvulsants, while stereotactic thermocoagulation or Gamma Knife radiosurgery are effective for seizure control. Here, we report an individual case where direct surgical resection disconnecting hypothalamic hamartoma from mammillothalamic tract resulted in complete disappearance of gelastic seizures without deterioration of cognitive function. A 6-year-old boy developed gelastic seizures at the age of 2 and suffered from precocious puberty. Anticonvulsants including carbamazepine and zonisamide failed to control seizures. The patient underwent direct division of the mammillothalmic tract by removal of hypothalamic hamartoma partially via anterior interhemispheric approach. It was observed that gelastic seizures disappeared completely after the surgical treatment without any endocrine and cognitive dysfunction for a follow-up period of 14 years. The mammillothalamic tract which connects anterior nucleus of thalamus and mammillary bodies plays a key role in gelastic seizures related to hypothalamic hamartoma. In this case, we disconnected the hamartoma specifically from the mammillary bodies and not from the rest of hypothalamus. Effectively, it enabled permanent control of seizures. This result shows that fibers connecting other hypothalamic structures and the dorsomedial nucleus of thalamus are not involved in gelastic seizure propagation from the hypothalamic hamartoma. When surgical treatment of hypothalamic hamartomas is performed it has high morbidity associated with hypothalamic disorders. Therefore, disconnection between hypothalamic hamartoma and mammillary bodies presents a possibility of reducing hypothalamic damage. Surgical disconnection between hamartoma and mammillothalamic tract carries minimal hypothalamic injury risk and our results suggest that it has the potential of seizure control for intractable gelastic seizures with less complications.

Bilateral Radiofrequency Ventral Intermediate Thalamotomy for Essential Tremor.

INTRODUCTION: With the advent of MR-guided focused ultrasound, the importance of the efficacy and safety of bilateral ventral intermediate (Vim) thalamotomy for essential tremor (ET) has increased. However, reports on bilateral Vim thalamotomy for ET remain scarce. METHODS: To review the results and complications of bilateral Vim thalamotomy for the treatment of ET in the upper extremities, we retrospectively analyzed the patients with ET who underwent bilateral Vim thalamotomy with radiofrequency (RF) thermal coagulation. As bilateral simultaneous thalamotomy can cause surgical complications, thalamotomy was performed in stages. The interval between the first and second thalamotomies was 21.3 ± 14.7 months. We evaluated the efficacy using the Clinical Rating Scale for Tremor (CRST) before and after the first and second treatments, respectively. We also evaluated the complications before and after the first and second treatments, respectively. Moreover, we assessed the adverse events. RESULTS: Seventeen patients were included in the study. The mean follow-up period following the second thalamotomy was 29.3 ± 15.0 months. The CRST part A + B scores were 34.9 ± 9.7, 20.8 ± 7.0, and 7.4 ± 6.8 before, following the first (40.4% improvement, p < 0.0001) and second thalamotomies (78.6% improvement, p < 0.0001), respectively. Nine patients presented with prolonged adverse events, including dysarthria, dysgeusia, dysphagia, tongue numbness, unsteady gait, and postural instability at the last available evaluation. All adverse events were mild and did not interfere with the patient's daily activities. DISCUSSION/CONCLUSIONS: Bilateral Vim thalamotomy with RF thermal coagulation was an effective treatment for ET in both upper extremities. Despite most possible complications being mild, additional studies with a larger sample size are required to ensure patient safety.

Precise detection of the germinomatous component of intracranial germ cell tumors of the basal ganglia and thalamus using placental alkaline phosphatase in cerebrospinal fluid.

PURPOSE: The disadvantages of biopsy for lesions in the basal ganglia and thalamus include a risk of various complications, difficulty in selecting the target tissue in some cases due to indistinct neuroimaging findings and limited availability of sample tissue. Placental alkaline phosphatase (PLAP) plays a decisive role in the diagnosis and management of intracranial germ cell tumors (IGCTs) in the basal ganglia and thalamus. The present study aimed to demonstrate the ability, specificity, and optimal use of PLAP values obtained from cerebrospinal fluid (CSF). METHODS: Twenty patients with lesions in the basal ganglia and thalamus were enrolled in this study: 11 had IGCTs and 9 had non-IGCTs. The values of PLAP and other established tumor markers in the CSF were measured in all patients before treatment. RESULTS: The mean follow-up period was 76.0 months (range, 3-168) for all lesions. PLAP was elevated in all 11 patients with IGCTs in the basal ganglia or thalamus, whereas none of the patients with non-IGCT exhibited elevated PLAP. Thus, the sensitivity and specificity of PLAP were both 100%. CONCLUSION: Our data demonstrated that the PLAP value can specifically identify the germinomatous component even in cases of IGCTs in the basal ganglia or thalamus with high sensitivity and specificity. PLAP is undoubtedly beneficial for the safe and timely detection of the germinomatous component of IGCTs in the basal ganglia and thalamus, because reliance on PLAP measurement enables us to avoid invasive surgical procedures and facilitates the prompt initiation of chemoradiation therapy.

Pallidothalamic Tractotomy for Parkinson Disease with 1-Year Follow-Up: A Case Report.

BACKGROUND: The pallidothalamic tract connects the globus pallidus internus and the ventrolateral portion of the thalamus, and ablation of the pallidothalamic tract (pallidothalamic tractotomy [PTT]) reportedly exerts antiparkinsonian effects. However, the detailed clinical course has not yet been elucidated. Here, we present the first single case report of PTT performed in a patient with Parkinson disease, with 1-year follow-up. CASE DESCRIPTION: The patient was a 68-year-old woman with Parkinson disease. After 14 years of receiving oral medication, she experienced the "wearing-off" phenomenon. Levodopa (300 mg/day) was required to maintain daily activities. Rigidity and peak-dose dyskinesia were predominantly observed in the right side of her body. In addition, she demonstrated right foot dystonia with pain. The preoperative Unified Parkinson Disease Rating Scale part 1, 2 (off medication/on medication), 3 (off medication/on medication), and 4 scores were 7, 26/4, 41/23, and 13, respectively. Further, the preoperative Unified Dyskinesia Rating Scale and Parkinson Disease Questionnaire-39 scores were 102 and 46, respectively. She underwent left-sided PTT, and no perioperative complications were observed. At 1 year postoperatively, daily administration of levodopa (200 mg) was maintained without an "off" condition all day. The 1-year Unified Parkinson Disease Rating Scale part 1, 2, 3, and 4 scores were 5, 9/1, 20/12, and 5, respectively. In addition, the 1-year Unified Dyskinesia Rating Scale and Parkinson Disease Questionnaire-39 scores were 20 and 20, respectively. CONCLUSIONS: We present a rare case of PTT performed in a patient with Parkinson disease. PTT might be useful in patients who do not desire device implantation.

Safety and long-term efficacy of ventro-oral thalamotomy for focal hand dystonia: A retrospective study of 171 patients.

OBJECTIVE: To report the safety and long-term efficacy of ventro-oral thalamotomy for 171 consecutive patients with task-specific focal hand dystonia. METHODS: Between October 2003 and February 2017, 171 consecutive patients with task-specific focal hand dystonia underwent unilateral ventro-oral thalamotomy. Etiologies included writer's cramps (n = 92), musician's dystonias (n = 58), and other occupational task-related dystonias (n = 21). The task-specific focal hand dystonia scale was used to evaluate patients' neurologic conditions (range 1-5, high score indicated a better condition). The scores before surgery; at 1 week, 3 months, and 12 months postoperatively; and the last available follow-up period were determined. Postoperative complications and postoperative recurrence were also evaluated. RESULTS: The scores before surgery; at 1 week (1.72 ± 0.57, 4.33 ± 0.85 [p < 0.001]), 3 months (4.30 ± 1.06 [p < 0.001]), and 12 months (4.30 ± 1.13 [p < 0.001]); and the last available follow-up (4.39 ± 1.07 [p < 0.001]) postoperatively improved. The mean clinical follow-up period was 25.4 ± 32.1 months (range: 3-165). Permanent adverse events developed in 6 patients (3.5%). Eighteen patients developed recurrent dystonic symptoms postoperatively. Of these 18 patients, 9 underwent ventro-oral thalamotomy again, of which 7 achieved improvement. CONCLUSION: Ventro-oral thalamotomy is a feasible and reasonable treatment for patients with refractory task-specific focal hand dystonias. Prospective, randomized, and blinded studies are warranted to clarify more accurate assessment of the safety and efficacy of ventro-oral thalamotomy for task-specific focal hand dystonia. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that for patients with task-specific focal hand dystonia, ventro-oral thalamotomy improves dystonia.

Pediatric Optic Pathway/Hypothalamic Glioma.

Optic pathway/hypothalamic gliomas (OP/HGs) are rare astrocytic tumors that appear more commonly among young children and often are unresectable. They comprise approximately 2% of all central nervous system tumors and account for 3-5% of pediatric intracranial tumors. Initial manifestations are often visual disturbances, endocrinopathies and hypothalamic dysfunction such as the diencephalic syndrome, and sometimes hydrocephalus due to cerebrospinal fluid (CSF) outflow obstruction. In many cases, the tumors are diagnosed late in the clinical course because they silently enlarge. These tumors consist mostly of histologically benign, World Health Organization (WHO) grade I tumors represented by pilocytic astrocytomas (PA), the rest being pilomyxoid astrocytomas (PXA) - WHO grade II tumors. In young pediatric patients, however, can be seen PXA that show aggressive clinical course such as CSF dissemination. Our small series of 14 non-Neurofibromatosis type 1 (NF-1) OP/HGs PA patients underwent extended resection without any adjuvant treatments. The median age at initial treatment was 11.5 ± 6.90 years (range, 1-25 years) and median follow up 85.5 ± 25.0 months. Surgical resection for OP/HGs results in acceptable middle-term survival, tumor control and functional outcome equivalent to chemotherapy. There is, however, no longer doubt that chemotherapy with or without biopsy and as-needed debulking surgery remains the golden standard in management of OP/H. Clinical conditions and treatment plans for OP/HGs vary depending on their structure of origin.

Stereotactic Thalamotomy for Hairdresser's Dystonia: A Case Series.

BACKGROUND: Hairdresser's dystonia is a rarely reported form of focal hand dystonia, and the clinical course and treatment remains poorly understood. OBJECTIVES: The aim of this report was to clarify the impact of thalamotomy on hairdresser's dystonia. METHODS: Four consecutive patients with hairdresser's task-specific dystonia evaluated at Tokyo Women's Medical University Hospital between 2008 and 2013 were treated with stereotactic thalamotomy, and were recruited for this case series. RESULTS: The mean age at the onset of symptoms was 37.25 ± 10.64 years, the median duration of symptoms was 4.25 ± 1.3 years, and the mean follow-up period was 17 ± 12.37 months. Two of the 4 patients returned to work with significant improvement following the stereotactic thalamotomy and the beneficial effects persisted for the duration of their clinical follow-up. The other 2 patients experienced transient improvements for up to 3 months. Surgical complications included only dysarthria in 2 patients, and did not interfere with their daily activities. No patients experienced a deterioration of dystonic symptoms after thalamotomy. CONCLUSION: Although the benefits of thalamotomy remain vulnerable to incorrect or insufficient coagulation, stereotactic thalamotomy may be a feasible and effective procedure for patients with hairdresser's dystonia.

Bilateral Stereotactic Thalamotomy for Bilateral Musician's Hand Dystonia.

BACKGROUND: Focal hand dystonia in musicians, also known as musician's dystonia, is a task-specific movement disorder characterized by unwanted involuntary muscle contractions occurring only when playing a musical instrument. CASE DESCRIPTION: Case 1 was a 50-year-old female professional pianist who underwent staged bilateral ventro-oral (Vo) thalamotomy, with an interval between the first and second surgery of 4 years. The first surgery (right Vo thalamotomy) led to significant improvements in dystonic symptoms without any complications. Pre- and postoperative Tubiana's musician's dystonia scale (TMDS) scores were 2 and 5, respectively. The second surgery (left Vo thalamotomy) also led to significant improvements in dystonic symptoms, with dysarthria and verbal recall disturbance resolving within 3 months. Pre- and postoperative TMDS scores were 2 and 5, respectively. The patient was subsequently able to return to live-stage performances. Case 2 was a 48-year-old male clarinet repair technician who underwent staged bilateral Vo thalamotomy, with an interval between the first and second surgery of 13 months. The first surgery (right Vo thalamotomy) led to dramatic improvements in symptoms without any complications. Pre- and postoperative TMDS scores were 2 and 5, respectively. The second surgery (left Vo thalamotomy) also led to significant improvements in symptoms with transient hypophonia. Pre- and postoperative TMDS scores were 2 and 5, respectively. The patient was subsequently able to return to work without difficulty. CONCLUSIONS: The findings in these 2 cases indicate the utility of bilateral stereotactic Vo thalamotomy in the treatment of medically intractable musician's dystonia affecting both hands.

Pallidotomy for Writer's Cramp after Failed Thalamotomy.

BACKGROUND/AIMS: Although many reports have confirmed the effects of stereotactic thalamotomy for writer's cramp, pallidotomy for writer's cramp is yet to be investigated. METHODS: After a 22-year-old woman with writer's cramp had undergone stereotactic thalamotomy twice, symptomatic relief was only temporary. Because her dystonic symptoms spread around the proximal part of the upper limb, she underwent unilateral pallidotomy 21 months after the second thalamotomy. RESULTS: Unilateral pallidotomy improved her dystonic symptoms without any adverse effects immediately after the surgery. During a follow-up observation of 1 year, no recurrent writer's cramp was observed. CONCLUSION: For writer's cramp, when symptoms cannot be improved by thalamotomy, pallidotomy may achieve an effective outcome.

Filtrate of Phellinus linteus Broth Culture Reduces Infarct Size Significantly in a Rat Model of Permanent Focal Cerebral Ischemia.

Phellinus linteus, a natural growing mushroom, has been known to exhibit anti-tumor, anti-inflammatory, anti-allergic and anti-oxidant effects. Aiming to exploit the neuroprotective effects of P. linteus, we evaluated its effects on infarct volume reduction in a rat model of focal cerebral ischemia. Male Sprague-Dawley rats were subjected to right middle cerebral artery occlusion. Filtrate of P. linteus broth culture (various doses), fractionated filtrate (based on molecular weight) or control medium was administered intraperitoneally to rats before or after ischemia induction. Rats were killed at 24 h after the stroke surgery. Cortical and caudoputaminal infarct volumes were determined separately using an image analysis program following staining with 2,3,5-triphenyltetrazolium chloride. Significant cortical infarct volume reductions were found in the pre-treatment groups (30 and 60 minutes before onset of cerebral ischemia) compared with the control group, showing dose dependence. Posttreatment (30 minutes after ischemic onset) also significantly reduced cortical infarct volume. Furthermore, the higher molecular weight (≥12 000) fraction of the culture filtrate was more effective compared with the lower molecular weight fraction. The present findings suggest that P. linteus may be a new promising approach for the treatment of focal cerebral ischemia, with the additional benefit of a wide therapeutic time window since significant infarct volume reduction is obtained by administration even after the ischemic event. Our finding that the higher molecular weight fraction of the P. linteus culture filtrate demonstrated more prominent effect may provide a clue to identify the neuroprotective substances and mechanisms.

Anterior interhemispheric approach for 100 tumors in and around the anterior third ventricle.

OBJECTIVE: We report our experience with anterior interhemispheric approach for tumors in and around the anterior third ventricle, including surgical technique, instrumentation, pre- and postoperative hormonal disturbances, and resection rate. METHODS: One hundred patients with 46 craniopharyngiomas, 12 hypothalamic gliomas, 12 meningiomas, 6 hypothalamic hamartomas, and 24 other lesions were operated on using an anterior interhemispheric approach with or without opening of the lamina terminalis. This surgical approach involves no frontal sinus opening; a narrow (approximately 15-20 mm in width) access between the bridging veins, which is sufficient to remove the tumor totally; and sparing of the anterior communicating artery. Specially designed long bipolar forceps and scissors are necessary for this approach, and concomitant use of angled instruments (endoscope, aspirator, and microforceps) is required frequently. The postsurgical follow-up period varied from 4 months to 18 years. RESULTS: Total removal of the neoplasm was accomplished in 37 of 46 patients with craniopharyngiomas (80.4%), whereas subtotal resection was performed in hypothalamic gliomas. No significant differences in pre- and postoperative hormonal disturbances were observed in 37 craniopharyngiomas and 10 hypothalamic gliomas. There was no operative mortality. Visual acuity was preserved or improved in 68 of 75 patients assessed. The Karnofsky Performance Scale score did not deteriorate in 72 of 75 patients tested. CONCLUSION: The minimally invasive anterior interhemispheric approach, with or without opening of the lamina terminalis, is useful for removal of tumors in and around the anterior third ventricle, such as craniopharyngiomas and hypothalamic gliomas.

Histological findings at the boundary of craniopharyngiomas.

Although a craniopharyngioma is grossly well circumscribed, microscopically the borders are frequently irregular and may be associated with gliosis in the adjacent brain tissue. In the current study, we investigated the histology of the interface between craniopharyngiomas and surrounding normal structures such as the hypothalamus and pituitary gland. Histologically, we classified the findings at the boundary of craniopharyngiomas into three types. In type 1, a relatively thick capsule-like tissue was identified at the boundary between the craniopharyngioma and surrounding normal structure composed of tumor cells and inflammatory changes. In type 2, a craniopharyngioma had a relatively clear cleavage between the surrounding gliosis. In type 3, the boundary had some interdigitation of the tumor in the surrounding gliotic layer adjacent to the craniopharyngioma. In types 1 and 3, surgeons may fail to accomplish complete resection of the tumor. These histological features may result in recurrence of craniopharyngioma even after gross total resection.