[A Case of Suprasellar Germinoma that Spontaneously Regressed before Endoscopic Biopsy].

We present a very rare case of suprasellar germinoma that spontaneously regressed before endoscopic biopsy. An 8-year-old boy was admitted to our hospital due to headache, polydipsia, and polyuria. Neurological examination performed on admission revealed bitemporal hemianopia. Enhanced MRI demonstrated a homogeneously enhanced tumor mass in the suprasellar region obstructing the foramen of Monro. Tests for all serum tumor markers were negative. Plain X-ray and CT scan were performed once and twice, respectively, for seven days between admission and endoscopic biopsy. The total dose of diagnostic radiation exposure before surgery was 110 mGy. Endoscopic observation during surgery revealed that the tumor had markedly decreased in size and the foramen of Monro had reopened. A very small piece of the tumor was harvested during surgery. Plain CT scan just after surgery showed a marked decrease in the tumor size, compared with the preoperative CT scan. The pathological diagnosis of the tumor was pure germinoma. The patient then underwent chemotherapy(four courses of CARE regimen)followed by radiation therapy with a dose of 24 Gy/15 fr. The tumor completely disappeared after treatment, and no recurrence was observed in the past four years.

Headache attack followed by rapid disease progression in pediatric moyamoya disease--how should we manage it?

CASE REPORT: A 4-year-old female was presented at our hospital with frequent right frontal headache attack. She was diagnosed with moyamoya disease and was conservatively followed up. One year later, the frequency of headache gradually decreased. However, follow-up MR imaging revealed that the disease stage markedly progressed in the right side and cerebral infarction occurred in the temporal lobe with atrophy of the right frontal lobe. She underwent direct and indirect revascularization on the right side. CONCLUSION: Aware of this case, we would like to emphasize that headache may be one subtype of ischemic attacks and require frequent MR follow-up to see the disease course. If there is any sign of disease progression, immediate surgical intervention should be indicated to avoid irreversible brain damage.

[Repeat bypass surgery for intracranial hemorrhage 30 years after indirect bypass for moyamoya disease].

A 39-year-old man had been diagnosed with moyamoya disease and underwent a bilateral encephalo-duro-arterio-synangiosis(EDAS)intervention at the age of 9 years. During the 30 years after his bilateral EDAS, he experienced no cerebrovascular events. However, at age 39, he suddenly presented with mild consciousness disturbance and vomiting and was transferred to a local hospital. Brain CT showed an intracerebral hemorrhage associated with ventricular hematoma. He was referred to our hospital for further investigation and treatment. Cerebral angiography showed faint collaterals through the site of the bilateral EDAS and development of basal moyamoya vessels. SPECT showed decreased cerebral blood flow(CBF)and cerebrovascular reactivity(CVR)in the right frontal lobe. We diagnosed him with delayed cerebral hemorrhage due to delayed rupturing of fragile moyamoya vessels after indirect bypass. The patient underwent a repeat bypass surgery(STA-MCA anastomosis and encephalo-duro-myo-arterio-pericranial synangiosis;EDMAPS)on the right side. He showed improvement in cerebral hemodynamics after surgery, and has since remained free from cerebrovascular events. Hemorrhagic events occurring a very long time after indirect bypass surgery in pediatric-onset moyamoya disease are rare. In such cases, a lifelong follow-up strategy may be necessary. Repeat bypass surgery may be a powerful tool to prevent such hemorrhagic events.

[Neuroendoscopic surgery for intra- and para-ventricular tumors:impacts on biopsy and hydrocephalus treatment].

In this report, the authors retrospectively review and discuss their results for neuroendoscopic surgery for intra- and para-ventricular tumors. This study included 28 patients who were admitted to our hospital for intra- or para-ventricular tumors between January 2005 and March 2013. There were 17 males and 11 females, and their age varied widely from 1 to 75 years. Using a neuroendoscopic technique, tumor biopsy was attempted in all 28 patients. Biopsy was possible in 25 patients(89.3%)but not in the other 3(10.7%)because of hypervascularity or tumor bleeding. Of these 25 patients, pathological diagnosis was possible in 23(92%), but not in other 2(8%)because of incomplete sampling. In 18 of 28 patients, various forms of hydrocephalus were observed. Neuroendoscopic procedures were quite useful to improve cerebrospinal fluid dynamics in 13 of these patients, including endoscopic third ventriculostomy(ETV)in 9(except for 1 patient with ETV failure), septostomy with ventriculoperitoneal shunting(VPS)in 3, and cystostomy in 1. Simple VPS was performed in the other 4 patients. These results suggest that neuroendoscopic surgery is valuable as a minimally invasive procedure for pathological diagnosis and hydrocephalus treatments in patients with intra- and para-ventricular tumors. Further development of neuroendoscopic equipment is warranted to accurately diagnose tumors with hypervascularity and subependymal tumors.

Walk-In Hospital Admission of Patients with Subarachnoid Hemorrhage: Clinical Presentation and Outcome.

OBJECTIVE: Neurosurgeons occasionally encounter cases of subarachnoid hemorrhage (SAH) in admitted walk-in patients, termed "walk-in SAH." However, their clinical characteristics have not been fully understood. We thus, aimed to investigate several characteristics of patients with walk-in SAH and compare them with those of patients with good grade SAH who arrived at the hospital by ambulance. METHODS: Between January 2011 and January 2018, consecutive patients with World Federation of Neurosurgical Society (WFNS) grade I and II aneurysmal SAH were enrolled. They were dichotomized into walk-in and ambulance groups, and their demographic and disease-related characteristics were compared. Furthermore, predictors associated with unfavorable outcomes were investigated in patients with walk-in SAH. RESULTS: Of 171 patients with World Federation of Neurosurgical Society grade I and II SAH, 68 (39.8%) were categorized as walk-in SAH. The mean time for diagnosis in patients with walk-in SAH was significantly longer than that in patients who arrived by ambulance (P < 0.01). Multivariate analysis demonstrated that a lower rate of hypertension, high grades on the Barrow Neurological Institute scale, and Early Brain Edema Score were significantly associated with walk-in SAH (odds ratio [OR] 0.44, 95% confidence interval [CI] 0.21-0.91, P = 0.03; OR 0.32, 95% CI 0.13-0.76, P = 0.007; OR 0.11, 95% CI 0.02-0.51, P < 0.0001, respectively). Additionally, severe angiographic vasospasm was a significant predictor of unfavorable outcomes in walk-in SAH (OR 37.7, 95% CI 1.10-1290.90, P = 0.04). CONCLUSIONS: Patients with walk-in SAH exhibit radiological characteristics associated with a more favorable outcome among patients with good grade SAH. Therefore, these patients may have a positive prognosis.

Bilateral subthalamic deep brain stimulation for camptocormia associated with Parkinson's disease.

BACKGROUND: Few multiple case studies of the effects of deep brain stimulation for camptocormia associated with Parkinson's disease have been reported. Although deep brain stimulation was in some cases not effective against camptocormia, it is unclear in which types of patients it was effective in treating camptocormia. OBJECTIVE: We treated 4 Parkinson's disease patients with camptocormia and evaluated their paraspinal muscle status by computed tomography to specify the characteristics of cases of effective treatment. METHODS: The 2 female and 2 male patients in this study were 60-69 years old, with a disease duration from onset to surgery of 7-13 years and a follow-up period of 18-40 months. The electrodes were implanted bilaterally in the subthalamic nuclei. RESULTS: Camptocormia was improved in 3 cases, and was unchanged in the remaining case although other parkinsonian symptoms improved. The computed tomography number of paraspinal muscle in the unimproved patient was much smaller than that in the improved patients. CONCLUSIONS: A relationship may exist between improvement of camptocormia and severity of paraspinal muscle degeneration.

[Cerebral infarction suspected due to dissection of intracranial carotid artery in 2 children].

Intracranial cerebral artery dissection in children is very rare. We report 2 children who were diagnosed as having cerebral infarction which was suspected to be due to dissection of intracranial cerebral artery. Case 1: An 11-year-old girl presented with conscious disturbance and hemiparesis after seizure. Computed tomography (CT) showed cerebral infarction in the right frontal lobe. Her symptoms did not change, but angiography demonstrated reversible change. She received conservative therapy. Case 2: A 10-year-old boy complained of headache, and presented nausea and monoparesis of the left upper extremity. CT showed cerebral infarction in the right frontal lobe. He received superficial temporal artery-middle cerebral artery anastomosis because his symptoms fluctuated. In both cases, cerebral angiography showed string sign from the supraclinoid portion of the internal carotid artery to the middle and the anterior cerebral artery. Follow-up angiography showed improvement of stenosis. In pediatric cases of cerebral artery dissection, improvement with conservative therapy is frequently reported. It is necessary to follow-up such patients closely using magnetic resonance angiography or conventional angiography to determine whether or not surgery is indicated.

Malignant transformation of supratentorial clear cell ependymoma.

Recurrence of clear cell ependymoma is not a rare condition, but malignant transformation of clear cell ependymoma has not yet been well presented. The authors report a 44-year-old man who presented with progressive right hemiparesis. A brain tumor in the left frontal premotor area was removed and an initial pathological diagnosis of oligodendroglioma was made. The tumor recurred 4 months later, and reoperation of the tumor and adjuvant local radiotherapy were performed. The patient subsequently underwent surgical removal of recurrent tumors on another four occasions (6 times in total) during a period of 11 years and finally died of the original disease. Histopathological studies of all surgical and autopsy specimens were carried out. The first and second surgical specimens did not contain any ependymal rosettes or pseudorosettes, and thus a diagnosis of oligodendroglioma was made. However, the third surgical specimen showed pseudorosettes. At this time, the tumor had an ultrastructural appearance compatible with ependymoma. Thereafter, the recurrent tumors showed anaplastic features such as nuclear pleomorphisms and necrosis with pseudopallisading. The autopsy specimens resembled a feature of glioblastoma but the tumor was sharply demarcated from the surrounding parenchyma.

Folliculostellate cell tumor in pituitary gland.

A 33-year-old woman presented with visual field defects. Magnetic resonance imaging demonstrated a pituitary tumor with suprasellar extension. The tumor was partially removed by trans-sphenoidal surgery. Histologically, the tumor was composed of fascicles of spindle cells which were immunohistochemically positive for S-100 protein, Galectin-3, vimentin and EMA. Based on the morphology and immunostaining pattern, diagnosis of folliculostellate cell tumor in the pituitary gland was given.

A newly designed training tool for microvascular anastomosis techniques: Microvascular Practice Card.

BACKGROUND: We report a newly designed training card (Microvascular Practice Card) that is a non-animal practice tool for surgical training and practicing the skills for microvascular anastomosis techniques. METHODS: The Microvascular Practice Card is a pocketbook-sized card that has silicone tubes affixed to it. On the card, 6 small-diameter, 4-cm-long tubes side by side are arranged in 4 directions with both ends secured. The tubes are available in diameters of 2.0, 1.0, 0.5, and 0.3 mm. The thickness of the tube wall is 0.05 or 0.1 mm. The card includes a record area that allows records to be written. Four directional tubes are arranged on one card, making it possible to practice various directional suturing and anastomosing. RESULTS: Beginners begin to practice suturing with larger diameter tubes (2.0 mm) and refine their skills using 1.0 mm diameter tubes as they get used to the practice. For vascular anastomosis, the card provides for end-to-end anastomosis, end-to-side anastomosis, and side-to-side anastomosis. Furthermore, superfine diameter tubes (0.5 and 0.3 mm) help microsurgeons to gain experience at higher magnifications. Training on this card is performed through a plastic box with a small hole using long microinstruments. CONCLUSION: Microvascular Practice Card is a new training tool for repeatedly practicing microvascular anastomosis in various situations. This non-animal practice tool would help trainees practice under safe and hygienic conditions and reduce the number of laboratory animals used during technical training.

A development of atheromatous plaque is restricted by characteristic arterial wall structure at the carotid bifurcation.

BACKGROUND: It is said atheromatous plaque is located very focally, but there have been few reports regarding this matter. Various aspects of the pathogenesis of the development of atheromatous plaque at the carotid bifurcation have previously been discussed. We have noted the correlation of plaque localization with characteristics of the cervical carotid artery wall. METHODS: Morphological and histopathologic changes in the carotid bifurcation were examined in 72 cadaver cases with or without atheromatous plaque. We determined the level at which the wall structure changed to muscular artery from elastic artery and analyzed its influence on the development of atheromatous plaque. RESULT: Atheromatous plaques at the distal site of the ICA extended within 0 to 37 mm from the carotid bifurcation. The proximal side of the CCA more than 5 mm away from the bifurcation was elastic artery, whereas the distal side of the ICA more than 15 mm from the bifurcation was muscular artery. The area of the carotid bifurcation between elastic artery and muscular artery was a transitional zone. Approximately 80% of them were located within 15 mm, and these areas were coincident with the transitional zone. CONCLUSION: Most atheromatous plaque was located in the transitional zone. The arterial wall structure is related to the development of atheromatous plaque at the cervical carotid bifurcation.

A new training method to improve deep microsurgical skills using a mannequin head.

Neurosurgeons need fine and special microsurgical techniques, such as the ability to suture deep microvasculature. Intensive training is required to perform microsurgery, especially in deep microvascular anastomosis. There have been many previous reports of training methods for typical microsurgical techniques, including suturing of surgical gloves, Silastic tubes, living animals, and chicken wing arteries. However, there have been no reports of training methods to improve deep microsurgical skills under the various hand positions specific to neurosurgical operation. Here, we report a new training method using a mannequin head, water balloons, and clay to mimic actual deep microsurgery in the brain. This method allows trainees to experience microsurgery under various hand positions to approach the affected areas located at various depths in the brain from various angles.

Neuroprotective role of transgenic PAF-acetylhydrolase II in mouse models of focal cerebral ischemia.

BACKGROUND AND PURPOSE: Platelet-activating factor (PAF) and oxidized unsaturated free fatty acids have been postulated to aggravate neuronal damage in the postischemic brain. Type II PAF-acetylhydrolase (PAF-AH II) not only terminates signals by PAF by its PAF-hydrolyzing activity but also protects cells against oxidative stress. We examined whether PAF-AH II can rescue cerebral neurons against ischemic insults. METHODS: Transgenic mice overexpressing human PAF-AH II in neurons were generated and enzyme expressions were examined biochemically and histochemically. The mice were subjected to 60 minutes of transient middle cerebral artery occlusion followed by reperfusion for 24 hours. The infarction and apoptosis were estimated by TTC staining and fluorescence TUNEL staining, respectively. RESULTS: Overexpression of PAF-AH II was found in brains of transgenic mice by Western blot and enzymatic activity analyses. In immunohistochemistry, human PAF-AH II expression was found throughout the central nervous system, especially in neurons of neocortex, hippocampus, and basal ganglia. The neurological deficit scores, cerebral edema index, and relative infarction volume were all significantly (P<0.05) lower in transgenic mice (1.30+/-0.72, 1.12+/-0.04, and 14.0+/-7.7%, respectively) than in wild-type mice (2.56+/-0.93, 1.23+/-0.12, and 31.9+/-9.7%, respectively). Percentages of apoptotic cells were also significantly (P<0.001) lower in transgenic mice (cortex, 5.2+/-3.3%; hippocampus, 3.4+/-7.0%) than in wild-type mice (cortex, 41.1+/-16.9%; hippocampus, 58.9+/-15.3%). CONCLUSIONS: These results indicate that PAF-AH II exerts strong neuroprotective effects against ischemic injury and suggest a possibility for clinical use of this enzyme in cerebral ischemia.

Clinical value of constructional skill testing in patients with secondary normal pressure hydrocephalus--two case reports.

Cognitive functions are frequently impaired in patients with normal pressure hydrocephalus (NPH). Two patients with NPH initially had dysfunctional constructional skill but exhibited improvements after shunt surgery. Dysfunction of constructional skill should be added to the important clinical features of NPH. The geometric test can be used as a practical tool for evaluation of parietal lobe function in patients with NPH.

Basal encephalocele associated with hypoplasia of the internal carotid artery.

A 22-year-old woman presented with a basal encephalocele associated with hypoplasia of the internal carotid artery (ICA) manifesting as a 6-year history of decreased vision in the right eye. She underwent encephalo-arterio-synangiosis under a diagnosis of hypoplasia of the ICA at age 6 years. Magnetic resonance imaging showed the encephalocele medial to the right temporal lobe. Frontotemporal craniotomy was performed for resection of the encephalocele and repair of the defect of the skull base. The pathogeneses of these developmental anomalies were probably related to developmental failure of the embryonic primordium during the 4th and 10th weeks.

[A Case of Cervical Internal Carotid Artery Occlusion Presenting with Subarachnoid Hemorrhage Caused by Rupture of Leptomeningeal Anastomosis].

A 71-year-old man was admitted to our hospital with a diagnosis of subarachnoid hemorrhage (SAH). Angiographies revealed neither aneurysms nor vascular anomalies. However, these images elucidated the occlusion of the left cervical internal carotid artery as well as developed leptomeningeal anastomoses through the ipsilateral posterior cerebral artery, which resulted in blood perfusing the ipsilateral middle and anterior cerebral artery territories. Because the localization of SAH coincided with the developed leptomeningeal anastomosis, we speculated that the rupture of the developed leptomeningeal anastomosis in the basal cistern was the cause of SAH. We performed superficial temporal and middle cerebral artery bypass surgery to prevent rebleeding and ischemic stroke. In patients with occlusion of the internal carotid artery, SAH induced by the rupture of aneurysm formed by hemodynamic stress was recognized. However, rupture of developed leptomeningeal anastomosis should be considered as a possible cause of SAH of unknown origin. (Received March 7, 2016; Accepted August 31, 2016; Published January 1, 2017).

Transparent endoscopic sheath and rigid-rod endoscope used in endoscopic third ventriculostomy for hydrocephalus in the presence of deformed ventricular anatomy.

OBJECT: Endoscopic third ventriculostomy (ETV) has been widely performed for the treatment of noncommunicating hydrocephalus. In cases of hydrocephalus in conjunction with deformed and complex ventricular anatomy, it is preferable to use a rigid-rod endoscope for ETV, because the excellent visibility provided by this instrument yields a smooth and correct orientation in the ventricle. The authors report on ETV procedures in which they used a transparent endoscopic sheath that has a common channel in which a rigid-rod endoscope and an instrument can be inserted. METHODS: In 15 cases of noncommunicating hydrocephalus, a transparent endoscopic sheath and a rigid endoscope were used for ETV. In 11 of the 15 patients, the diameter of the foramen of Monro and the width of the third ventricle were greater than 5 mm, and thus a transparent endoscopic sheath and a rigid endoscope could be smoothly introduced through the foramen of Monro and an ETV successfully performed. Four patients had congenital or acquired narrowing of the foramen of Monro and an anatomically deformed ventricular system. In three of the patients, opening of the narrowed foramen and an ETV were successfully performed using the transparent endoscopic sheath under direct visualization through the rigid-rod endoscope. CONCLUSIONS: A transparent endoscopic sheath increases safety by offering a corridor to the third ventricle. It also provides excellent visibility without troublesome bleeding from tissues surrounding the foramen of Monro during endoscopic procedures in which a rigid endoscope is used.

Acute dural venous sinus thrombosis without brain parenchymal abnormality: assessment with cerebral blood volume using dynamic susceptibility contrast magnetic resonance imaging.

We report results applying the dynamic susceptibility contrast (DSC) magnetic resonance (MR) technique to a patient with dural venous sinus thrombosis (DVST) of the right transverse-sigmoid sinus without brain parenchymal abnormality. The DSC-MR technique clearly demonstrated increased regional cerebral blood volume of the right temporo-parieto-occipital region adjacent to a right transverse-sigmoid sinus thrombosis in a patient with DVST without cerebral edema or hemorrhage.

Decrease in platelet count as an independent risk factor for symptomatic vasospasm following aneurysmal subarachnoid hemorrhage.

OBJECT: Increased platelet consumption is expected in patients with cerebral vasospasm, according to data from clinical and experimental studies. The authors investigated sequential changes in platelet counts in patients with subarachnoid hemorrhage (SAH) and the difference in platelet consumption between patients with and those without symptomatic vasospasm (SV). Variables related to platelet count as well as other clinical and radiological variables were analyzed as independent predictors of SV. METHODS: One hundred consecutive patients who had undergone surgery within 48 hours after SAH onset were entered in the study. Clinical and radiological variables and blood cell counts, including red blood cells, white blood cells, and platelets, after SAH were retrospectively examined. Twenty of these variables were entered into univariatete and multivariate analyses to determine predictors for SV. After SAH, the platelet count decreased to a minimum and then increased rapidly to levels greater than those recorded on admission. This change was specific to SAH, and platelet consumption was more severe in patients with SV than in those without. There were three independent predictors of SV: a ratio of the lowest platelet count and the admission count greater than 0.7 (odds ratio [OR] 0.322, 95% confidence interval [CI] 0.124-0.834, p = 0.0196) and a history of hypertension (OR 0.338, 95% CI 0.126-0.906, p = 0.0311) were negatively significant (that is, decreases the occurrence of SV), and a Fisher Grade 3 (OR 4.42, 95% CI 1.48-13.2, p = 0.0077) was positively significant (that is, increases the occurrence of SV). CONCLUSIONS: The association between a decrease in platelet count and the occurrence of SV indicates the important role of platelets in the pathophysiology of vasospasm following SAH.

Multiple epidermoid cysts located in the pineal and extracranial regions treated by neuroendoscopy.

A 22-year-old woman presented with a rare case of multiple epidermoid cysts located in the pineal and extracranial regions. Magnetic resonance (MR) imaging showed a lesion in the pineal region as hypointense on the T(1)-weighted image and hyperintense on the T(2)-weighted image, without enhancement. Neuroendoscopic treatment was performed under a diagnosis of pineal cyst. However, the cyst wall was too thick to perforate, although third ventriculostomy was performed. Diffusion-weighted MR imaging demonstrated the lesions in the pineal and extracranial regions as marked hyperintensity. The diagnosis was epidermoid cyst. Subsequently, neuroendoscopic treatment of the pineal epidermoid cyst was performed. Careful preoperative diagnosis of epidermoid cysts based on diffusion-weighted MR imaging is required.