Preservation of absolute pitch after right amygdalohippocampectomy for a pianist with TLE.

Absolute pitch (AP) ability is a rare musical phenomenon. In the literature, it has been suggested that the relative specialization for pitch processing is in the right temporal lobe in the non-AP population. Since the anatomic basis for absolute pitch is not fully understood and cases of temporal lobe epilepsy of AP possessors are extremely rare, applicability of resection as a treatment of epilepsy in this particular area should be evaluated with caution. In the present study, we examined an AP possessor who suffered from medically refractory temporal lobe epilepsy and underwent right selective amygdalohippocampectomy (SAH). The SAH procedure clearly avoided disturbing important structures for AP, inasmuch as postsurgically she preserved her AP ability and was seizure-free. She did well post-operatively in the test of pure sine wave tones with short reaction time, which could be identified as "true" absolute pitch.

Efficacy of intraoperative irrigation with artificial cerebrospinal fluid in chronic subdural hematoma surgery: study protocol for a multicenter randomized controlled trial.

BACKGROUND: The surgical techniques for treatment of chronic subdural hematoma (CSDH), a common neurosurgical condition, have been discussed in a lot of clinical literature. However, the recurrence proportion after CSDH surgery remains high, ranging from 10 to 20%. The standard surgical procedure for CSDH involves a craniostomy to evacuate the hematoma, but irrigating the hematoma cavity during the procedure is debatable. The authors hypothesized that the choice of irrigation fluid might be a key factor affecting the outcomes of surgery. This multicenter randomized controlled trial aims to investigate whether intraoperative irrigation using artificial cerebrospinal fluid (ACF) followed by the placement of a subdural drain would yield superior results compared to the placement of a subdural drain alone for CSDH. METHODS: The study will be conducted across 19 neurosurgical departments in Japan. The 1186 eligible patients will be randomly allocated to two groups: irrigation using ACF or not. In either group, a subdural drain is to be placed for at least 12 h postoperatively. Similar to what was done in previous studies, we set the proportion of patients that meet the criteria for ipsilateral reoperation at 7% in the irrigation group and 12% in the non-irrigation group. The primary endpoint is the proportion of patients who meet the criteria for ipsilateral reoperation within 6 months of surgery (clinical worsening of symptoms and increased hematoma on imaging compared with the postoperative state). The secondary endpoints are the proportion of reoperations within 6 months, the proportion being stratified by preoperative hematoma architecture by computed tomography (CT) scan, neurological symptoms, patient condition, mortality at 6 months, complications associated with surgery, length of hospital stay from surgery to discharge, and time of the surgical procedure. DISCUSSION: We present the study protocol for a multicenter randomized controlled trial to investigate our hypothesis that intraoperative irrigation with ACF reduces the recurrence proportion after the removal of chronic subdural hematomas compared with no irrigation. TRIAL REGISTRATION: ClinicalTrials.gov jRCT1041220124. Registered on January 13, 2023.

Unique vascular structures of a radicular arteriovenous fistula at the craniocervical junction along the first cervical spinal nerve: A case report.

Background: An arteriovenous fistula (AVF) at the craniocervical junction (CCJ) is a rare vascular malformation. Definitive diagnosis and curative treatment of CCJ AVF are challenging. Case Description: A 77-year-old man presented with subarachnoid hemorrhage. Cerebral angiography showed an AVF at the CCJ, which drained into a radicular vein. The lesion was fed by a vertebral artery, anterior and lateral spinal arteries (LSAs), and the occipital artery (OA). There were two unique structures: the LSA originating from the posterior inferior cerebellar artery of the extracranial V3 segment and the OA feeding the shunt. Curative treatment involved two steps: endovascular embolization of feeders using Onyx and surgical shunt disconnection. Feeding arteries were blackened by Onyx, which helped identify the location of the shunt. The shunt was located behind the first cervical (C1) spinal nerve, and the draining vein was confirmed on the deep side of the nerve. A clip was applied to the draining vein distal to the shunt. Tiny vessels supplying the shunt were then coagulated referring to blackened arteries. Conclusion: A radicular AVF at the CCJ along the C1 spinal nerve had unique vascular structures. Definitive diagnosis and curative treatment were achieved by combining endovascular embolization using Onyx and direct surgery.

Uneven interhemispheric connections between left and right primary sensori-motor areas.

To clarify the characteristics of interhemispheric connections, we investigated cortico-cortical evoked potentials (CCEP) in human. Fourteen patients with temporal lobe epilepsy who underwent invasive EEG monitoring with bilaterally implanted subdural electrodes were studied. Electric pulse stimuli were given in a bipolar fashion at two adjacent electrodes on and around the motor area (MA) or sensory area (SA), and CCEP responses were recorded by averaging electrocorticograms from the contralateral hemisphere. Seventy-two pairs of electrodes were stimulated, and 468 recordings were analyzed. Fifty-one of 468 recordings demonstrated CCEP responses. Of 51 responses, 16 consisted of an initial positive triphasic wave (Type 1), 27 had an initial negative biphasic wave (Type 2), and 8 showed an initial positive biphasic wave (type 3). The mean latencies of the earliest peaks were 13.1, 28.9, and 29.4 ms in Types 1, 2, and 3 responses, respectively. The responses were more frequently evoked by stimulating facial MA (f-MA) and nonfacial MA (nf-MA) than by stimulating SA or noneloquent area. In both f-MA and nf-MA stimulation, the responses were more frequently recorded at the contralateral f-MA than at the contralateral nf-MA or other areas. SA stimulation never evoked CCEP responses at the contralateral MA or SA. The amplitudes were maximal when f-MA was stimulated and responses recorded at the contralateral f-MA. These findings suggest that the interhemispheric connections are uneven. Both f-MA and nf-MA send dense interhemispheric connections to the contralateral f-MA. SA may have no or only rare direct connection with the contralateral MA or SA.

Successful carotid artery stenting with a double-layer micromesh stent for spontaneous extracranial internal carotid artery dissection: a case report.

Extracranial internal carotid artery dissection is a relatively rare disease in Japan. We herein report a case of a 60-year-old woman with spontaneous left internal carotid artery dissection with a dilated and dissected cavity. Following the identification and measurement of the true and false lumens using intravascular ultrasound, a double-layer micromesh stent (Casper stent; Microvention, Terumo, Tustin, CA, USA) was deployed for post-dilation. No perioperative complications were observed, and the patient was discharged on postoperative day 6.

Usefulness of an Electromagnetic Navigation System for Direct Percutaneous Puncture of the Superior Ophthalmic Vein.

Objective: We report a case of dural arteriovenous fistula (dAVF) at the cavernous sinus treated by direct puncture of the superior ophthalmic vein (SOV) using an electromagnetic navigation system. Case Presentation: The case involved a 70-year-old male patient who presented with mild chemosis, proptosis, and abducens palsy of the right eye. In this case, we used an electromagnetic navigation system for direct puncture of the SOV. Angiographic obliteration of the fistula was confirmed and the visual symptoms recovered well after surgery. There were no complications associated with direct puncture of the SOV using the electromagnetic navigation system. Conclusion: Direct puncture of the SOV to obliterate a dAVF is a possible alternative choice of treatment when the usual transvenous access route fails. To reduce the risk of complications, an electromagnetic navigation system is useful.

Brain metastasis from extramammary Paget's disease.

Herein, we present a case of extramammary Paget's disease with brain metastasis that was diagnosed pathologically for the first time in Japan. Moreover, invasive extramammary Paget's disease (with distant metastasis) highly resistant to treatment. Only for brain metastasis, we may control the tumor by surgical resection and stereotactic radiosurgery (SRT) for the treatment of intracranial metastases was assessed. An 76-year-old man was diagnosed with extramammary Paget's disease of the vulva at nearby hospital. Surgical resection and sentinel lymph node dissection were then performed, and the patient received chemotherapy because multiple lymph node metastases were suspected. The patient's response to chemotherapy was poor, and he was in the state of Progressive Disease. He complained of dyslexia and was referred to another hospital when he was 81 years old. Plain magnetic resonance imaging (MRI) was conducted, and two brain tumors in the vicinity of the left cerebellar tent were suspected. In our hospital, gadolinium contrast-enhanced MRI was performed and showed a tumor in the cerebellum (left posterior temporal lobe) and another tumor under the tent (left cerebellar hemisphere). Significant edema was also noted. Based on these findings, the intracranial lesion was diagnosed as metastatic brain tumor. The pathological diagnosis was brain metastasis from extramammary Paget's disease. Postoperative intracranial residual disease was treated with stereotactic radiosurgery. MRI showed that the size of the cerebellar lesions decreased, and no recurrence of cerebral lesions was observed. SRT for extracranial lymph node metastases was performed. Mass reduction and SRT may be the best way to treat brain metastasis from extramammary Paget's disease.

[Radiosurgery of epidermoid tumors with gamma knife: possiblility of radiosurgical nerve decompression].

Long-term results of radiosurgery for epidermoid tumors are reported. There are 7 cases including 2 males and 5 females, ages ranging from 6 to 46 (mean: 33.3 years). At radiosurgery whole tumor was covered in 4 cases and partially covered in 3 cases in attempting to relieve cranial nerve signs like trigeminal neuralgia and facial spasm. The mean maximum and marginal doses were 25.6 Gy and 14.6 Gy respectively. In the mean follow-up of 52.7 months, all the tumors showed good tumor control without any progression and tumor shrinkage has been confirmed in 2 out of the 7 cases. Symptomatic trigeminal neuralgia improved or disappeared in all 4 cases and facial spasm disappeared in one. No neurological deterioration was found in any of the cases after the treatment. In conclusion, it is apparent that epidermoid tumors do respond well to radiosurgery and the accompanying hyperactive dysfunction of cranial nerves is significantly improved by gamma knife treatment with either entire or partial tumor coverage. Therefore the radiosurgical nerve decompression for epidermoid tumor seems to be achieved by gamma-radiosurgery.

Long-term results of gamma knife surgery for growth hormone-producing pituitary adenoma: is the disease difficult to cure?

OBJECT: The authors conducted a study to determine the long-term results of gamma knife surgery for residual or recurrent growth hormine (GH)-producing pituitary adenomas and to compare the results with those after treatment of other pituitary adenomas. METHODS: The series consisted of 67 patients. The mean tumor diameter was 19.2 mm and volume was 5.4 cm3. The mean maximum dose was 35.3 Gy and the mean margin dose was 18.9 Gy. The mean follow-up duration was 63.3 months (range 13-142 months). The tumor resolution rate was 2%, the response rate 68.3%, and the control rate 100%. Growth hormone normalization (GH < 1.0 ng/ml) was found in 4.8%, nearly normal (< 2.0 ng/ml) in 11.9%, significantly decreased (< 5.0 ng/ml) in 23.8%, decreased in 21.4%, unchanged in 21.4%, and increased in 16.7%. Serum insulin-like growth factor (IGF)-1 was significantly decreased (IGF-1 < 400 ng/ml) in 40.7%, decreased in 29.6%, unchanged in 18.5%, and increased in 11.1%, which was almost parallel to the GH changes. CONCLUSIONS: Gamma knife surgery was effective and safe for the control of tumors; however, normalization of GH and IGF-1 secretion was difficult to achieve in cases with large tumors and low-dose radiation. Gamma knife radiosurgery is thus indicated for small tumors after surgery or medication therapy when a relatively high-dose radiation is required.

Statistical parametric mapping of interictal 123I-iomazenil SPECT in temporal lobe epilepsy surgery.

Brain single photon emission computed tomography (SPECT) for epilepsy is divided into two types (using three radionuclide tracers)-perfusion SPECT (123I-IMP or 99 mTc-ECD), identifying epileptogenic foci by detecting abnormality in regional cerebral blood flow, and 123I-iomazenil SPECT, identifying epileptogenic foci based on distribution of central benzodiazepine receptors. This study aimed to statistically evaluate and compare the SPECT effectiveness for the three tracers. Statistical parametric mapping (SPM) analysis was performed on 30 mesial temporal lobe epilepsy (mTLE) patients. The radionuclide and patient data were categorized as follows: abnormality in the medial temporal lobe on the operated hemisphere (AAA), in the entire temporal lobe on the operated hemisphere (AA), in the dominantly affected temporal lobe on the operated hemisphere (A), in bilateral temporal lobes (B), with no abnormalities in bilateral temporal lobes (C), and with abnormality in the temporal lobe on the nonoperated hemisphere (D). For analyses of (AAA), (AA), and (A), examining the hemisphere containing epileptogenic foci, IMP-SPECT was significantly superior to ECD-SPECT (P<0.05). For (AAA), indicating localization, IMZ-SPECT was significantly superior to the other two (P<0.05). IMP-SPECT was superior for lateralizing and IMZ-SPECT was useful for localizing epileptogenic foci in mTLE patients though the applicability of the results in extratemporal lobe epilepsy is unknown.

Clinical significance of ictal high frequency oscillations in medial temporal lobe epilepsy.

OBJECTIVE: To clarify the clinical significance of ictal high frequency oscillations (HFO) in the medial temporal lobe. METHODS: This study included 19 patients who underwent intracranial electrode implantation in bilateral temporal lobes and had at least one seizure recorded at 1kHz sampling rate. The characteristics of ictal HFO in the medial temporal lobe, and the relations between the presence of HFO, pathology, and postoperative seizure outcome were analyzed. RESULTS: Ictal HFO were detected from medial temporal structures in 11 patients with medial temporal lobe epilepsy (MTLE). Among eight patients without HFO, only three were diagnosed with MTLE. Ictal HFO were detected from unilateral medial temporal structures ipsilateral to the side of hippocampal sclerosis (HS). In one patient with bitemporal independent seizure onset, ictal HFO were detected only on the side of HS. HS was detected in all 11 patients with HFO, but in only one of four patients without HFO. Seizure outcome did not differ between patients with and without HFO. CONCLUSIONS: Ictal HFO in the medial temporal lobe may be a specific marker for MTLE with HS. SIGNIFICANCE: Recording of ictal HFO in the medial temporal lobe may be useful for presurgical evaluation of MTLE.

Very high frequency oscillations (over 1000 Hz) in human epilepsy.

OBJECTIVE: High frequency oscillations (HFO) of 100-500Hz have been reported in epileptic human brain. However, the questions of how fast these oscillations can reach, and which frequency range is clinically important remain unanswered. We recorded interictal and ictal very high frequency oscillations (VHFO) of 1000-2500Hz by subdural electrodes using 10kHz sampling rate. We describe the characteristics of VHFO, and discuss their underlying mechanism and clinical significance. METHODS: Five patients with neocortical epilepsy were studied. All patients underwent intracranial EEG monitoring with subdural electrodes. EEG recording with sampling rate of 10kHz was conducted. Histopathology revealed malformation of cortical development in all cases. RESULTS: In four of five patients, very high frequency activities of 1000-2500Hz were detected in highly localized cortical regions (one to four electrodes in individual patient). We named these activities "very high frequency oscillations (VHFO)". Interictally, VHFO appeared intermittently, and were interrupted by spikes. Sustained VHFO without spikes appeared around the start of seizures. CONCLUSIONS: Both interictal and ictal VHFO can be recorded by subdural electrodes. Compared to HFO previously reported, VHFO have much higher frequency, more restricted distribution, smaller amplitude, and different timing of onset. SIGNIFICANCE: Recording of VHFO may be useful for identifying the epileptogenic zone.

Stereotactic radiosurgery for vestibular schwannomas: analysis of 317 patients followed more than 5 years.

OBJECTIVE: Many investigators have reported successful treatment of vestibular schwannomas with gamma knife radiosurgery (GKRS). However, long-term outcomes should be evaluated before concluding that GKRS is truly safe and effective for the treatment of vestibular schwannomas. METHODS: Between May 1991 and December 1998, 346 consecutive patients (excluding those presenting with neurofibromatosis Type 2) were treated with GKRS. Of these, 317 patients were assessed. Twenty-nine patients were lost to follow-up within 5 years. RESULTS: The median follow-up period was 7.8 years. Of 301 patients who underwent serial follow-up imaging, two (1%) experienced complete remission, 184 (61%) experienced partial remission, 93 (31%) had stable tumors, and 22 (7%) experienced treatment failure. The actuarial 5- or 10-year progression-free survival (PFS) rate was 93 and 92%, respectively. Tumors less than 15 cm3 in volume (10-yr PFS, 96%; P < 0.001) or which did not compress the brainstem and deviate the fourth ventricle (10-yr PFS, 97%; P = 0.008) resulted in significantly better PFS rates. Failure of treatment usually occurred within 3 years. When the tumor was treated with a marginal dose of 13 Gy or less, the hearing preservation rate was 68%, transient facial palsy developed at a rate of 1%, and facial numbness developed at a rate of 2%. CONCLUSION: GKRS proved to be a safe and effective treatment for patients followed longer than 5 years who presented with tumors with a volume of less than 15 cm3 and who did not have significant fourth ventricle deviation. Good functional outcomes were observed in this group of patients.