Long-Term Outcomes after Multimodal Treatment for Clival Chordoma: Efficacy of the Endonasal Transclival Approach with Early Adjuvant Radiation Therapy.

This study investigates the long-term outcomes of clival chordoma patients treated with the endonasal transclival approach (ETCA) and early adjuvant radiation therapy. A retrospective review of 17 patients (2002-2013) showed a 10-year progression-free survival (PFS) rate of 67.4%, with the ETCA group showing fewer progressions and cranial neuropathies than those treated with combined approaches. The ETCA, a minimally invasive technique, provided a similar extent of resection compared to conventional skull-base approaches and enabled safe delivery of high-dose adjuvant radiotherapy. The findings suggest that ETCA is an effective treatment for centrally located clival chordomas.

Bilateral pallidal stimulation for "sticking-out tongue" feature in patients with primary focal tongue protrusion dystonia.

INTRODUCTION: Tongue protrusion dystonia can cause difficulty with speech, mastication, breathing, and swallowing. Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is a widespread therapeutic alternative for treating medically refractory dystonia. To our knowledge, detailed reports regarding DBS for tongue protrusion dystonia are rare. In this report, we describe two patients with "sticking out" tongue protrusion who had undergone bilateral GPi DBS. METHODS: Operations were performed with surface electromyographic (EMG) monitoring, microelectrode recording, and macrostimulation to identify the point at which tongue kinetic cells respond most effectively. The most effective location for active contacts was identified according to burst EMG response in the posteroventral GPi. RESULTS: Two years after DBS, total Burke, Fahn, and Marsden Dystonia Rating Scale scores of two patients were improved from 12.5 to 1 (92.0%) and from 13 to 1 (92.3%), respectively. One 58-year-old woman who lost 7 kg weight from not eating well improved enough to eat solid food and became free from choking. Another 54-year-old woman who had dysarthria and mumbled could speak more fluently and would not have complained difficulty in reading any more. CONCLUSION: Stimulation on posteroventral GPi for patients with idiopathic "sticking-out" tongue movement changes EMG pattern in orofacial muscles. This fact supports a reason for modulation of unknown circuit connecting tongue-specific area in motor cortex, and basal ganglia.

Simultaneous thalamic and posterior subthalamic electrode insertion with single deep brain stimulation electrode for essential tremor.

OBJECTIVES: The optimal target location of deep brain stimulation (DBS) is the subject of some controversy. We implanted electrodes that could stimulate both posterior subthalamic area (PSA) and ventralis intermedius nucleus of thalamus (Vim), and examined the benefits of bilateral DBS of Vim, PSA, and Vim + PSA in patients with essential tremor (ET). MATERIALS AND METHODS: Electrodes were inserted into the Vim and PSA in ten hemispheres of five consecutive patients. All patients were assessed for action tremor, including postural and kinetic tremors, both preoperatively and at six months and one year postoperatively. RESULTS: The preoperative mean postural tremor score was 1.9 (range 1.0-2.5) and kinetic tremor score was 2.6 (range 2.0-3.0). One year after surgery, these scores had decreased significantly to 0.1 (range 0.0-1.0) and 0.6 (range 0.0-1.5), respectively. The postural and kinetic tremor scores at six-months and one-year post-surgery were similar for Vim, PSA, and Vim + PSA stimulation. CONCLUSIONS: We were able to identify the optimal electrode placement site for each patient based on his or her individualized response to the stimulation. Overall, there was no statistically significant difference among the DBS sites in terms of the benefits afforded by the stimulation. We propose that our technique may be a useful surgical method to treat ET.

Endoscope-assisted microsurgical removal of an epidermoid tumor within the cavernous sinus.

Epidermoid tumor of the cavernous sinus is rare. The aim of this case report is to discuss the role of neuroendoscopes in the removal of such lesions. A 21-year-old man presented with 6-year history of progressive headache, diplopia, and visual disturbance. Work-up revealed an epidermoid tumor located in the right cavernous sinus. An extradural transcavernous approach was utilized via a traditional frontotemporal craniotomy with endoscopic assistance. The postoperative course was uneventful with immediate improvement of the patient's headache. Postoperative magnetic resonance imaging demonstrated complete removal of the tumor. There were no signs of recurrence during a 2-year follow-up period. The endoscope is a useful tool for removing epidermoid tumors from the cavernous sinus and enhances visualization of areas that would otherwise be difficult to visualize with microscopes alone. Endoscopes also help minimize the retraction of neurovascular structures.

Delayed recurrence of hemifacial spasm after successful microvascular decompression: follow-up results at least 5 years after surgery.

BACKGROUND: Microvascular decompression (MVD) for hemifacial spasm (HFS) is regarded as the gold-standard treatment due to its efficacy and durability. However, some patients still suffer from delayed recurrence after initially successful MVD.In this study, we describe our clinical experience in a single institute following up initially successful MVD for HFS 5 or more years after surgery. We analyzed the probability of, risk/predisposing factors for, and timing of delayed recurrence. METHODS: We retrospectively reviewed data from 587 patients meeting our inclusion criteria who underwent MVD for HFS from March, 1999, to June, 2006. We evaluated the time-dependent probability of recurrence and factors affecting delayed recurrence and time of recurrence. RESULTS: The probability of delayed recurrence was 1.0, 1.7, and 2.9 % at 1 year, 2 years, and 5 years after surgery, respectively. Mean time to recurrence was 153.1 months (95 % confidence interval [151.4-154.9]). The probability of late recurrence was increased in patients with co-morbid arterial hypertension (p = 0.036). However, there was a trend towards an association of delayed recurrence with co-existence of young age, male gender, vein or VA offender, and experience of transient facial weakness. CONCLUSIONS: Delayed recurrence of HFS after initially successful MVD is rare; however, there are patients who experience delayed recurrence more than 2 years after MVD, even until 5 years after MVD. Our results suggest that arterial hypertension contributes to late recurrence. We did not find a statistically significant relationship between recurrence and other putative risk/predisposing factors.

Intraoperative vertebral artery angiography to guide c1-2 transarticular screw fixation in a patient with athetoid cerebral palsy.

We present a case of an athetoid cerebral palsy with quadriparesis caused by kyphotic deformity of the cervical spine, severe spinal stenosis at the cervicomedullary junction, and atlantoaxial instability. The patient improved after the first surgery, which included a C1 total laminectomy and C-arm guided righ side unilateral C1-2 transarticular screw fixation. C1-2 fixation was not performed on the other side because of an aberrant and dominant vertebral artery (VA). Eight months after the first operation, the patient required revision surgery for persistent neck pain and screw malposition. We used intraoperative VA angiography with simultaneous fluoroscopy for precise image guidance during bilateral C1-2 transarticular screw fixation. Intraoperative VA angiography allowed the accurate insertion of screws, and can therefore be used to avoid VA injury during C1-2 transarticular screw fixation in comorbid patients with atlantoaxial deformities.

Spontaneous cervical epidural hematoma causing brown-sequard syndrome.

Spontaneous cervical epidural hematoma (SCEH) is a rare clinical entity and has a varied etiology. Urgent surgical decompression should be done to prevent serious permanent neurologic deficits. We describe a 59-year-old female who presented with Brown-Sequard syndrome due to spontaneous cervical epidural hematoma. Initially, she was misdiagnosed as cerebrovascular accident. Cervical magnetic resonance imaging revealed epidural hematoma to the right of the spinal cord extending from C3 to C6. She later underwent surgical evacuation and had complete restoration of neurologic function. The outcome in SCEH is essentially determined by the time taken from onset of the symptom to operation. Therefore, early and precise diagnosis such as careful history taking and MRI evaluation is mandatory.

Outcomes of surgical treatment for hemifacial spasm associated with the vertebral artery: severity of compression, indentation, and color change.

OBJECT: The object of surgical treatment for hemifacial spasm (HFS) is the exclusion of pulsatile neurovascular compression of the root exit zone (REZ). However, spasm persists transiently or permanently in some cases even after complete decompression. In particular, we mainly experience these results when the vertebral artery (VA) is the offender. Hence, we verified color changes of the nerve and indentations from within the operative field in HFS patients with the VA as the offender. So, we reviewed retrospectively the records of those patients who were treated with microvascular decompression (MVD) in order to assess the relationship between operative findings and clinical results. METHODS: A total of 232 patients with HFS associated with the VA were treated with MVD between January 1994 and January 2009 at our institution. The patients were classified into one of the following three categories based on compression severity: Group I, mild; Group II, moderate; Group III, severe. The patients were also classified into one of the following four categories based on the existence of indentation and discoloration of nerve VII: Group A (-/-), B (+/-), C (-/+), or D (+/+). RESULTS: A total of 94.2% and 96.6% of the patients in Groups I and II, respectively, had improved to grades I-II at the last follow-up. The surgical outcomes of Group III were slightly poorer than those of Groups I and II. Group A showed the poorest outcomes with 60% of the patients classified as grades III-IV. In Group B, 98.4% of the patients showed a marked improvement and Groups C and D showed relatively poor outcomes compared with those of Group B. CONCLUSIONS: Severe deviations and color changes of the facial nerves may be the risk factors for poor surgical outcomes. Future studies with larger sample sizes and investigations of the pathophysiology underlying these findings are needed.

Surgery for a case of three-compartment trigeminal schwannoma : technical aspects.

Complete removal of three-compartment trigeminal schwannomas is a challenge to neurosurgeons. To expand exposure of each compartment, the combination and modification of skull base approaches are necessary. The 61-year-old woman was admitted with chronic headache. Preoperative magnetic resonance imaging showed 47×50×40 mm-sized tumor originating primarily in the middle cranial fossa extended to the posterior and the infratemporal fossa. We performed operation in five stage; 1. Zygomatic osteotomy, 2. Inferior temporal fossa plate removal and foramen ovale opening, 3. Cavernous sinus opening, 4. Tailored anterior petrosectomy, 5. Meckel's cave opening. Combination of skull base surgery should be concerned according to the patient. In this study, extradural basal extension with zygomatic osteotomy, interdural posterior extension with tailored anterior petrosectomy, and intracavernous exploration are reasonable options for remodeling three-compartment lesion into a single compartment. Tailoring of bone resection and exploring through natural pathway between meningeal layers accomplish single-stage operation for complete removal of tumors.