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In this study, we analyzed the outcomes of patients (followed for 5-38 years, average 17.3 years) with craniosynostosis and evaluated their long-term prognosis. In all, 51 patients who underwent surgery for craniosynostosis between 1982 and 2015, including 12 syndromic and 39 non-syndromic cases, were included. The average age at the initial surgery was significantly lower in the syndromic group than that in the non-syndromic group (9.8 months old vs. 19.9 months, respectively). The surgical procedures did not significantly differ between the two groups, but repeat surgery was significantly more common in the syndromic group than in the non-syndromic group (4 children [30.8%] and 3 children [7.7%], respectively). The children requiring repeat surgery tended to be younger at the initial surgery than those who did not. Those patients who required repeat surgery did not have significantly different surgical procedures initially. The incidence of developmental retardation was 49.0% (43.5% in the non-syndromic group and 66.7% in the syndromic group), and only two children in the non-syndromic group displayed recovery. This study is the first to analyze the prognosis for patients who were followed for at least 5 years after cranioplasty. Repeat surgery was common, especially in syndromic patients. Severity of skull deformity and early initial surgery may be important factors determining the need for repeat surgery. Developmental retardation was also common, and improvement was rare even after surgery.

The hanger reflex (HR) is an involuntary head rotation that occurs in response to a clothes hanger encircling the head and compressing the unilateral fronto-temporal area. Here, we developed an elliptical device to induce the HR and examined its utility for the treatment of cervical dystonia (CD). The study included 19 patients with rotational-type CD. The device was applied to each subject’s head for at least 30 min/day for 3 months. Severity scores on part 1 of the Toronto Western Spasmodic Torticollis Rating Scale were evaluated at baseline and after the 3-month trial. Mean scores without and with the device were significantly different both at baseline (16.6 vs. 14.7, respectively; P < 0.05) and after the trial (14.9 vs. 13.6, respectively; P < 0.05). This preliminary trial suggests that our device can improve abnormal head rotation in patients with CD.

(123)I-ioflupane SPECT (DaTscan) is an examination that detects presynaptic dopamine neuronal dysfunction, and has been used as a diagnostic tool to identify degenerative parkinsonism. Additionally, myocardial (123)I-metaiodobenzyl guanidine (MIBG) scintigraphy measures the concentration of cardiac sympathetic nerve fibers and is used to diagnose Parkinson's disease (PD). These exams are used as adjuncts in the diagnosis of parkinsonism, however, the relationship of these two examinations are not well-known. We investigated the relationship of these two scanning results specifically for determining the use of deep brain stimulation therapy (DBS). Subjects were Japanese patients with suspected striatonigral degeneration, including PD; DaTscans and myocardial MIBG scintigraphy were performed. The mean values of the left-right specific binding ratios (SBRs) from the DaTscan, and the early/delayed heart-to-mediastinum ratios (HMRs) from the MIBG scintigraphy were calculated. Using simple linear regression analysis, we compared the SBR and early/delayed HMR values. Twenty-four patients were enrolled in this study. Twenty-one patients were positive via the DaTscan, and the MIBG scintigraphy results showed 14 patients were positive. SBR and both early and delayed HMR were positively correlated in cases of PD, but negative in non-PD cases. A mean SBR value less than 3.0 and a delayed HMR value less than 1.7 indicated a Hoehn-Yahr stage 3 or 4 for PD, which is commonly regarded as a level appropriate for initiating DBS therapy. Our results indicate that performing both DaTscan and MIBG scintigraphy is useful for the evaluation of surgical intervention in PD.

To investigate the working conditions of female neurosurgeons in Japan, two surveys were conducted by The Japan Neurosurgical Society: one involving female neurosurgeons themselves and the other involving the chiefs of neurosurgical departments. The responses were received from 224 (43.8%) female neurosurgeons and 496 (61.2%) departmental chiefs. About half (50.2%) of the female neurosurgeons were married and 39.2% had children (average number of children, 1.27). Their work was full-time in 80.6% of cases; on average, they worked 51.9 h per week, had night duty 2.8 times per month, and had 5.7 days off per month. Many of them stated that they were satisfied with their job status, but about half of them reported difficulty in maintaining a correct work-life balance. Among the institutions surveyed, 29% had female neurosurgeons. The survey of departmental chiefs revealed that the proxies for maternity leave were not available at most institutions, and that there was only limited availability of night child care (41%) or sick child care (39%); female neurosurgeons did not appear to be well-informed of these support systems. These findings suggest that apart from systematic approaches already in place, female neurosurgeons would prefer to have more understanding from their peers and chiefs.

We describe a higher magnifying power operating microscope system to improve one method of high-quality microsurgical clipping for cerebral aneurysm in some cases. This higher magnification is achieved by a new lens design in the optical system, which makes the image of the object very clear at high magnifications (distinctiveness of 7 μm). This higher-resolution operating microscope system provides the surgeon with higher-magnified images (at the maximum of more than 30× magnifications as each working distance) in the operating field. The magnifications can be changed from low power (2.9×) to high power (62.0×) depending on the circumstances in a given procedure. We have used this operating microscope system on 11 patients with microsurgical clipping for cerebral aneurysms. Microsurgical treatment could be performed safely and precisely. All aneurysms were treated without any technical complications. We think that the use of this microscope would have potential benefits for microsurgical treatment for cerebral aneurysms because of better visualization.

Crossed cerebellar diaschisis (CCD) often occurs after ischemic or hemorrhagic stroke that damages the cortico-ponto-cerebellar pathway. However, CCD due to cerebral hyperperfusion following cerebrovascular reconstruction is rare. A 61-year-old woman presented with transient CCD due to cerebral hyperperfusion following bypass surgery for adult moyamoya disease. She developed transient weakness of the right extremities and was diagnosed with moyamoya disease. First, she underwent superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis with indirect synangiosis on the left. Postoperative course was uneventful. Subsequently, she underwent STA-MCA anastomosis with indirect synangiosis on the right. She complained of mild headache on the right, and single photon emission computed tomography (SPECT) performed on the 7th postoperative day demonstrated hyperperfusion in the right frontal and temporal lobes associated with hypoperfusion in the left cerebellum. Magnetic resonance (MR) imaging demonstrated no new lesions and MR angiography showed patent STA-MCA bypass. Subsequent SPECT showed disappearance of both hyperperfusion and CCD. This case strongly suggests that cerebral hyperperfusion after bypass surgery for moyamoya disease may cause transient CCD. Although the clinical significance is still obscure, this phenomenon indicates the cortico-ponto-cerebellar pathway is interrupted due to hyperperfusion, suggesting the development of hyperperfusion syndrome. Careful observation of cerebral hemodynamics after bypass surgery is warranted to avoid hyperperfusion-related complications.

A 28-year-old woman presented with an unusual case of giant thrombosed varix with calcified walls that had mass effects secondary to a pial single-channel arteriovenous fistula (AVF) associated with hereditary hemorrhagic telangiectasia (HHT). She consulted our hospital for chronic headache. She had been diagnosed with HHT based on genetic testing when her 3-year-old son presented with subarachnoid hemorrhage due to spinal AVF. Imaging studies revealed pial single-channel AVF with multiple varices. The varices in the right frontal lobe were over 6 cm in diameter and had laminar thromboses and calcified walls. Because of the mass effect, direct surgical flow disconnection was performed followed by removal of the varices using an internal decompression technique. Postoperatively, the patient was discharged with no neurological symptoms and no longer suffered chronic headache. Intracerebral varices are occasionally associated with high-flow AVF, and usually treated by interrupting the feeding arteries leaving the varices intact. This extremely rare case of intracerebral giant thrombosed varices with calcified wall and mass effect indicates that surgical removal of varices should be considered.

Spontaneous echo contrast (SEC) consists of numerous microechoes swirling in the cardiovascular lumen and is usually seen during blood stasis in dysfunctional left atrium. However, SEC and consecutive local thrombus formation at the carotid artery early after carotid endarterectomy (CEA) have not been reported. This study retrospectively investigated the clinical importance and therapeutic strategy of postoperative SEC and thrombus formation in 113 consecutive patients who underwent CEA between 2001 and 2009. Ultrasonography was routinely performed preoperatively, intraoperatively, and 1 day and 1 week after the operation. If SEC and/or thrombus was detected at any time after the operation, follow-up ultrasonography was performed at short intervals, once a week for inpatients and once every 1-2 months for outpatients. Eight of the 113 patients (7%) had SEC after the operation from Day 1 to 12 (mean 7.2 days), and 6 of these 8 patients developed local de novo thrombus formation at the site of SEC from Day 6 to 33 (mean 14.7 days). The maximum luminal narrowing by the thrombi were 26-62% (mean 37%). After administering anticoagulant therapy, all thrombi disappeared from Day 13 to 190 (mean 57 days) from CEA. SEC seen after CEA is highly associated with consecutive local thrombus formation. Postoperative geometric blood stasis with the absence of intima may be the causative factor for its development.

This review describes the basic concepts of surgical revascularization for moyamoya disease, including direct and indirect bypass surgery. Direct bypass surgery can improve cerebral hemodynamics and reduce further ischemic events immediately after surgery, but may be technically challenging in some pediatric patients. Indirect bypass surgery is simple and has widely been used. However, its beneficial effects can be achieved 3 to 4 months after surgery, and surgical design is quite important to determine the extent of surgical collateral pathways. Combined bypass procedure, especially superficial temporal artery (STA) to middle cerebral artery anastomosis and indirect bypass, encephalo-duro-myo-arterio-pericranial synangiosis, is a safe and effective option to improve the short- and long-term outcome in patients with moyamoya disease. Alternative techniques are also described for specific cases with profound cerebral ischemia in the anterior cerebral artery or posterior cerebral artery territory. Special techniques to safely complete bypass surgery and avoid perioperative complications are presented, including methods to prevent delayed wound healing, to avoid facial nerve palsy after surgery, and to preserve the STA and middle meningeal artery during skin incision and craniotomy. Finally, the importance of careful management of patients is emphasized to reduce the incidence of perioperative complications, including ischemic stroke and hyperperfusion syndrome.

A 47-year-old male presented with asymptomatic mild (45%) internal carotid artery (ICA) stenosis associated with familial hypercholesterolemia. Fluorine-18-fluorodeoxyglucose ([(18)F]FDG) positron emission tomography (PET) showed that the carotid plaque had high uptake of [(18)F]FDG on the initial scan. He was treated with antiplatelet agent, but subsequently developed retinal artery occlusion on the ipsilateral side. The patient safely underwent carotid endarterectomy (CEA). Histological examination revealed that the plaque was rich in lipids and activated macrophages. Previous large randomized clinical trials have clarified that CEA is an effective therapeutic option to reduce the risk for subsequent ischemic stroke in patients with severe (more than 70%) ICA stenosis. The case strongly suggests that [(18)F]FDG PET may be a useful modality to identify inflamed and/or vulnerable carotid plaque and may be useful to predict the risk for subsequent ischemic stroke even in patients with mild to moderate ICA stenosis (less than 70%).

Because of the wide spread of magnetic resonance image (MRI), it may be increasing to find a dissecting aneurysm (DA) of the posterior inferior cerebellar artery (PICA) that causes headache without associated hemorrhage or infarction. Generally, surgical treatment might be considered in cases of DA with hemorrhage. However, the treatment of an unruptured DA with headache or infarction has not been well established. This is the first report regarding the pathology of an intact DA of the PICA that presents with headache only, and the pathological findings showed interesting figures. A 44-year-old man with an unruptured DA of the left PICA presented with sudden left occipital headache. MRI revealed no hemorrhage or infarction. Magnetic resonance angiography (MRA) showed growth of the DA 12 days after the onset of headache. Therefore, surgery was performed to prevent aneurysmal rupture. This DA aneurysm was trapped and removed after an occipital artery (OA)-PICA anastomosis was performed. The surgery was performed without complication. Pathological findings showed folding of the internal elastic lamina, and the true lumen was torn by the false lumen. The dissection reached the adventitia and the wall had numerous macrophages. Pathological findings might help understanding the etiology of DAs and inflammation might play an important role in DAs.

A 78-year-old woman presented with preauricular superficial temporal artery (STA) aneurysm and scalp porocarcinoma, which had both increased in size over 2 years. She had no previous history of head trauma. Three-dimensional (3D) computed tomography (CT) angiography revealed a 4-cm diameter STA aneurysm arising from the main trunk of the left STA and located just lateral to the zygomatic arch. The scalp porocarcinoma was excised by dermatologists. The STA aneurysm was carefully dissected from the surrounding tissues, and was resected after ligation of the proximal STA. Histological examination showed the aneurysm consisted of intima, media, and adventitia, and the diagnosis was atherosclerotic fusiform aneurysm. 3D CT angiography is quite useful to plan surgical strategy for such an unusually large STA aneurysm.

A 47-year-old male underwent stereotactic radiosurgery (25 Gy) for a cerebral arteriovenous malformation located in the right caudate nucleus, using a linear accelerator. Complete obliteration of nidus was confirmed 20 months after radiosurgery. However, a hypointense mass on T2-weighted magnetic resonance imaging developed in the area adjacent to the nidus after approximately 80 months. The mass gradually increased in size and induced severe perifocal edema over 2 years. The mass was successfully excised. Histological examination revealed that the mass consisted of dilated sinusoid vessels attached to the hematoma capsule, and the hematoma included clots in various stages of organization encapsulated by dense collagenous tissue. The histological diagnosis was cavernoma. De novo formation of cavernoma is well known to occur after radiation surgery for intracranial tumor, especially in pediatric patients, but is rare in adults. Based on the radiological and histological findings in the present case, the radiation-induced cavernoma underwent repeated bleedings resulting in chronic encapsulated expanding hematoma.

The present study evaluated the effect of the free radical scavenger edaravone on lesion volume and neurological dysfunction after spinal cord injury (SCI) in mice, and investigated its protective effects on superoxide generation. Female C57BL/6 mice were subjected to SCI using a pneumatic impact device and were treated with 3 mg/kg of edaravone or vehicle 30 minutes before the insult. Motor functions were quantitatively evaluated. Lesion volume was assessed by Dohrmann's two-cone method after one week. In situ detection of superoxide in the injured cord was carried out using the superoxide-sensitive dye dihydroethidium (DHE) staining technique. Pretreatment with edaravone significantly improved motor dysfunction and reduced the lesion volume to about 63% of the control (p < 0.05). Semi-quantitative measurements of red fluorescence emitted from DHE revealed that the superoxide concentration increased in the lesion periphery at 1 and 3 hours after the insult, and that pretreatment with edaravone significantly inhibited the increase of superoxide concentration in the lesion periphery at both time points (p < 0.0001). Double staining with DHE and monoclonal antibody against MAP2 showed that most cells positive for DHE were also positive for MAP2. These findings suggest that edaravone ameliorates tissue damage by scavenging reactive oxygen species, especially in the neurons, after SCI.

Some patients with moyamoya disease treated by conventional surgical procedures may develop postoperative refractory ischemia and perioperative cerebral infarction in the anterior cerebral artery (ACA) territory. We present a novel operative procedure for moyamoya disease to avoid the risk of ischemia in the ACA territory, which consists of simultaneous superficial temporal artery (STA) to middle cerebral artery (MCA) or ACA bypass with pan-synangiosis, encephalo-duro-arterio-myo-synangiosis for the lateral frontal and temporal areas, and encephalo-galeo-arterio-synangiosis for the medial frontal area. This procedure can establish direct bypass to the ACA territory at the first intervention. Simultaneous STA-MCA and STA-ACA bypasses with pan-synangiosis is suitable for patients with moyamoya disease associated with severely impaired perfusion of the ACA territory requiring direct bypass surgery.

A 60-year-old female and a 40-year-old male underwent surgical revascularization for moyamoya disease and suffered small infarction in the ipsilateral frontal lobe 3 or 4 days postoperatively. Neuroimaging suggested that the bypass flow had caused rapid progression of occlusive changes in the carotid forks, a diminishing of moyamoya vessels, and flow reduction in the anterior cerebral artery ipsilateral to surgery, leading to critical ischemia in the frontal lobe. Surgical revascularization improves the outcome of patients with moyamoya disease, but postoperative management such as hydration is important to avoid ischemic complications due to frontal lobe infarction.

Differences in the clinical presentation and angiographic stages of moyamoya disease were studied in 69 patients, 35 children (6.3 +/- 2.9 years old) and 34 adults (44.6 +/- 10.5 years old). The angiographic stage (Suzuki's stage) was compared between childhood and adulthood, ischemic onset and hemorrhagic onset, and female and male. The distribution of the angiographic stage was not very different, but was significantly shifted from stage III to stage IV in adult cases. The angiographic stage in patients with hemorrhagic onset was not significantly different from those with ischemic onset. There was no significant difference between females and males. Angiographic change is not very remarkable between pediatric and adult moyamoya disease. The angiographic stage does not directly correlate with the distinct clinical presentations between pediatric and adult moyamoya disease. Other factors such as cerebral blood flow demand and arteriosclerotic change seem to cause these differences.

The correlation between the drug-induced hypotension somatosensory evoked potential (SEP) test and regional cerebral blood flow changes after acetazolamide administration was studied. Fourteen patients presenting with transient ischemic attack, reversible ischemic neurological deficits, or minor completed stroke were evaluated. All patients had no or only localized low-density areas on computed tomographic scans, and unilateral occlusion or severe stenosis of the internal carotid or middle cerebral artery on cerebral angiograms. The Diamox asymmetry enhancement (DAE) was studied to detect reduced cerebral perfusion reserve in the affected hemispheres. The DAE was 7.9 +/- 5.8% in seven patients positive in the SEP test, significantly higher than -1.5 +/- 2.9% in patients negative in the SEP test. Postoperative SEP tests were negative in all five patients who underwent extracranial-intracranial (EC-IC) bypass surgery, suggesting that the EC-IC bypass improved the cerebral perfusion reserve in the affected hemispheres. The DAE decreased significantly in four of these patients. This study disclosed a significant correlation between the drug-induced hypotension SEP test and DAE. These parameters are considered important for evaluating patients with hemodynamic compromise and/or suitable candidates for EC-IC bypass.