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By progression of the disease, motor neurons degenerate in patients with amyotrophic lateral sclerosis (ALS) eventually lose nearly all voluntary muscles in the body. They are awake and aware but cannot move or communicate (locked-in state). Since the function of the brain is preserved, one possible measure to support their communication is to interpret their motor intention by decoding (deciphering) brain signals and present it with external devices. This technology called "brain-machine interface (BMI)" is now close to clinical use in Japan and USA.In our system, we record electrocorticogram (ECoG) obtained with subudural electrodes during their motor imagery, decode it and determine the movement they intended. So far, one patient of ALS with severe paralysis, implanted with this electrodes, successfully operated the PC communication tool only by thinking.

Brain-machine interface (BMI) is a new technology to receive input from the brain which is translated to operate a computer or other external device in real time. After significant progress during the recent 10 years, this technology is now very close to the clinical use to restore neural functions of patients with severe neurologic impairment. This technology is also a strong tool to investigate the mode of neuro-signal processing in the brain and to understand the mechanism of neural dysfunction which leads to the development of novel neurotechnology for the treatment of various sorts of neurological disorders.

The annual incidence of aneurysmal subarachnoid hemorrhage (SAH) is approximately 20 per 100,000/ year in Japan, and it is suggested that approximately 40% of SAH patients have poor outcome. Rebleeding after SAH and delayed cerebral vasospasm are the most important causes of poor outcomes. Particularly, rebleeding significantly worsened the outcome, therefore, its prevention is of utmost importance. Preventive measures against rebleeding include open surgery and endovascular treatment without craniotomy. Selection of the measures to prevent rebleeding should be based on neurological assessment, location and shape of cerebral aneurysm, anticipated difficulties in the treatment, presence of complications, etc. At present, surgical clipping remains the method of choice in Japan. However, there has been growing acceptance that the efficacy of endovascular treatment is comparable to that of surgical treatment, suggesting endovascular treatment should be considered in suitable patients with ruptured cerebral aneurysms. In this report, we describe the proceedures for the standard management of SAH, in particular, treatment of ruptured cerebral aneurysm and selection of treatment measures, according to the Japanese guidelines for the management of aneurysmal subarachnoid hemorrhage.

Lumbar canal stenosis (LCS) and lumbar foraminal stenosis (LFS) are frequently observed in elder patients. For patients with radiculopathy caused by both LCS and LFS, surgical manipulations require nerve root decompression from its exit zone to the lateral part of the vertebral foramen, and often need spinal instrumentation. In the present report, we performed a new strategy of surgical decompression without spinal fixation. An 81-year-old woman suffered from bilateral buttock pain, left leg pain and right leg numbness in the L4 and L5 nerve root distribution. MRI and CT scans showed LCS at L3-5 levels and left LFS at L4-5 level. Although the L5 radiculopathy diminished with conservative treatment for 3 weeks, she could not walk due to residual L4 radiculopathy. We tried to perform surgical decompression as follows; the left L4 nerve root was decompressed by lateral fenestration on the left L4 lamina and simultaneously by bilateral spinal canal decompression through right laminotomy for LCS. This method can achieve decompression of the left L4 nerve root from the spinal canal to the vertebral foramen under direct observation while preserving the continuity of the lamina. The patient was relieved from the radicular pain after the operation, and returned to her previous activity of daily living. One-year after the operation, she was free from the radiculopathy, and radiograms showed no fracture in the preserved left L4 lamina. Lateral fenestration combined with bilateral spinal canal decompression through contralateral laminotomy is potentially a surgical option for radiculopathy caused by both LSC and LFS.

In this paper, we review the current status of immunotherapy targeting Wilms' tumor 1 (WT1) peptide in malignant brain tumors as well as in other hematological and solid malignancies. WT1 is expressed in various kinds of malignancies, and is involved in oncogenesis. The titers of antibodies against WT1 and the frequency of WT1-specific cytotoxic T lymphocytes (CTLs) were higher in cancer patients than in healthy donors, indicating that WT1 protein has immunogenic function. These findings provided us with a rationale for developing cancer immunotherapy that targets the WT1 peptide. Clinical trials of the WT1 peptide vaccination were initiated, and definite immunological and clinical responses were observed. The disease control rate of 57.1% was obtained especially in the case of recurrent glioblastomas, with a median progression-free survival period of 20.0 weeks and progression-free survival rate at 6 months of 33.3%. The trial showed that WT1 vaccination for malignant gliomas, which is generally believed to be an intractable disease, was safe and elicited a favorable clinical response. Further clinical studies of WT1 vaccination in patients with malignant gliomas as well as other cancers are warranted. An enhancement of the efficacy of WT1 vaccination can be expected with a combined treatment using the WT1-specific helper peptide or anti-cancer chemotherapeutic agents. Administration of WT1 vaccination along with other therapeutic modalities during initial treatment or in the case showing minimal residual disease may prolong the survival time of the cancer patients.

We report a case of posttraumatic syringomyelia (PTS) that developed 10 months after spinal cord injury (SCI), A 46-year-old man was involved in a motorcycle accident, in which he received a severe spinal cord injury due to a burst fracture at the T6 level. The patient underwent posterior fixation without decompression at another hospital, and was transferred to our hospital for rehabilitation. Ten months after the SCI, he complained of back and neck pain caused by bending his head backward. MRI showed syringomyelia shaped like a cone extending from the T6 to the C6 level, enlarged by cerebrospinal fluid (CSF) flow toward the syringomyelia at the T6 level. Pain was relieved by syringosubarachnoid shunt implantation and the syrinx disappeared after the operation. PTS emerging 10 months after spinal cord injury is relatively rare. From the radiological and operative findings, PTS was enlarged by the CSF flow, which was hindered at the T6 level by compression of the vertebral body to the spinal cord. In cases of SCI in patients who undergo posterior fixation with insufficient decompression, close attention to PTS is required in the postoperative follow-up.

Drainage by fenestration of the cyst wall via the transsphenoidal apporach is the most commonly used treatment for symptomatic Rathke's cleft cyst (RCC). The same procedure is usually adopted for recurrence of RCC. We have encountered a case of secondary empty sella syndrome presented with visual field defects after repeated surgery for RCC. Secondary empty sella syndrome following the surgery of RCC is rare. The condition was explained by the mechanism that the optic nerve adhered to the cyst wall and it was tethered downward as the cyst shrank after the surgery. We treated the patient via the endonasal endoscopic transsphenoidal approach by placing holed silicone plates under the sellar floor to elevate the sellar contents and the optic nerve. Silicone plate is hard enough to support the sella and the small holes on it would facilitate drainage of the cyst contents. This method has proved useful as chiasmapexy for secondary empty sella syndrome after the surgery of RCC.

A 59-year-old man presented with generalized convulsion. MR imaging demonstrated a homogeneously enhanced dural lesion infiltrating the parenchyma in the right parietal region. He had no history of sinusitis and the lesion a resembled malignant tumors, so surgical treatment was performed. The histopathological studies showed pachymeningitis extending to the cerebral parenchyma, so the diagnosis of pachymeningoencephalitis was made. After the operation, the patient had no neurological deficits and anticonvulsant therapy was continued. We report the third case of idiopathic local pachymenigoencephalitis that we could range extensively in the world. The clinical features and pathogenesis are discussed.

Purpose: Natural history of unruptured cerebral aneurysms is still a matter of discussion. In this study, we investigated the prognosis of unruptured cerebral aneurysms of unoperated cases in a prospective design., Methods: Between September, 1992 and December, 2001, we have encountered a 256 cases of unruptured cerebral aneurysms. Among them, 118 cases were observed and were checked every year for their status. The endpoint was designed as their death and aneurysm rupture. Their rupture rate, mortality due to aneurysm death, and the cause of death other than aneurysm were investigated. Univariate analysis, chi-square test was used as statistics. A p-value less than 0.05 was considered as significant., Results: Annual rupture rate of unoperated unruptured cerebral aneurysms of size below 5 mm, between 5-15 mm, and over 15 mm increased according to the aneurysm size, 0.4%, 3.3% and 9.9% respectively. The sole risk factor for the feasibility of rupture of unruptured aneurysms was their size (p < 0.001). Aneurysm related mortality, however, was high in posterior circulation aneurysms. In patients under 70 years of age, 45% of patients died of cerebral aneurysms, but this rate decreased to 17% for patients over 70 years of age., Conclusion: The rupture rate of unruptured cerebral aneurysms over 5 mm in size is not low. Unruptured aneurysms of the posterior circulation may have a much higher risk of rupture, so further investigation is necessary.

To clarify difference of significant risk factors and that of prevalence with increasing age between white matter lesion (WML) and silent lacunar infarction (SLI), 946 brain check-up subjects were investigated. Twelve risk factors including age, sex, and hypertension were analyzed using multiple logistic regression analysis. A case of WML, which changed into infarction, was presented. Significant (P < 0.01) risk factors for WML were aging (odds ratio; 1.12/year) and hypertension (3.66), while those for SLI were aging (1.10/year), hypertension (4.89), and retinal arteriosclerosis (3.46). Both WML and SLI emerged in middle age and thereafter the prevalence rates increased with age, although WML emerged earlier and was more frequent throughout than SLI. The results indicated that WML was essentially aging phenomenon, while SLI was strongly affected by vascular risk factors, although WML might possibly chang into infarction.

Recently developed synthetic aperture magnetometry (SAM) is a new MEG signal analysis introducing a high performance spatial filtering technique. SAM is not an inverse solution like dipole analysis, but rather an adaptive beamformer for estimating source activity at each selected voxel inside of the brain. SAM can estimate source changes as a function of time, or power changes subjected to statistical analysis from the non-averaged raw MEG data. Thus this method enables to display the regional currentodensitogram of the arbitrary selected brain tissue as if depth electrodes were inserted (SAM virtual sensor), and to detect the origin of epileptic discharges and their spread. By applying statistical discrimination, SAM can also demonstrate the spatial distribution of event-related changes of brain rhythm (SAM statistical method), in other words activated cerebral cortex during task performance. We will present the usefulness of noninvasive SAM methods in epilepsy surgery detecting the epileptogenic zone by SAM virtual sensor method as well as eloquent brain by SAM statistical method.

We developed a simple system of an "extracorporeal" ventriculoatrial (VA) shunt using a one-way ball valve (Acty valve II, Kaneka Medix) to release the patient from postoperative constraint during the ventricular drainage. The system is constructed in such a way that the ventricular drainage tube is connected to the central venous catheter via a one-way valve. The CSF is regulated by using the valve and is diverted into the systemic circulation as in the conventional ventriculoatrial shunt. After 2 or 3 weeks of CSF diversion through the extracorporeal VA shunt, a ventriculoperitoneal shunt is placed if hydrocephalus is apparent by temporary occlusion of the system. We applied this system to 4 patients with hydrocephalus, and we found it useful and free from adverse effects. The patient was freed from physical constraint involved in conventional ventricular drainage and an effective program of early rehabilitation was able to be started.