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We report the case of a 60-year-old man who first presented with transient difficulty of word recall. Subsequent MRI revealed an invasive brain tumor in the left frontal lobe. The patient underwent open biopsy, and diffuse astrocytoma(WHO grade II)was diagnosed. However, the malignant potential of this tumor was not particularly low because of a few enhancement on preoperative evaluation, and radiation therapy was initially performed. Four months after ending irradiation, temozolomide treatment was introduced for tumor regrowth. After another 2 months, combined chemotherapy with bevacizumab was also started due to tumor enlargement, which was evaluated as malignant transformation to glioblastoma. Two focal lesions with signal hyperintensity on DWI appeared in the frontal and temporal lobes at different locations 3 months after starting bevacizumab. The left temporal lesion subsequently changed to a ring-enhanced tumor, and glioblastoma(WHO grade IV)was finally diagnosed at decompressive surgery. Another frontal lesion, however, continued to maintain a favorable course without any changes in signal despite appearing as similar signal-hyperintense lesions. The temporal hyperintense lesion may undergo malignant transformation into glioblastoma with typical radiological appearance. Recent studies on image changes following bevacizumab treatment have attracted widespread attention, and the clinical significance of such hyperintense lesions has gained attention. This present case was thought to be valuable because of the contradistinctive aspects at the same time, in which the hyperintense lesions of the frontal and temporal lobes seemed to represent antitumor activity or drug refractory effects based on bevacizumab treatment.

Pleomorphic carcinoma is an uncommon neoplasm that is known to arise in diverse sites, including the lung, breast, pancreas, gallbladder and prostate. We encountered a rare case of pleomorphic carcinoma of undetected origin associated with production of granulocyte-colony stimulating factor (G-CSF). The patient was a 62-year-old man who presented with cough and general fatigue. Chest computed tomography (CT) demonstrated a mass lesion in the mediastinum, so mediastinoscopic tumor biopsy was performed. However, making the diagnosis was difficult despite the suspected malignant potential. CT-supported positron emission tomography showed multiple uptake lesions in the small intestine, duodenum and right adrenal gland, in addition to the mediastinum. The patient subsequently presented with right-side weakness and sensory impairment. Brain CT showed multiple intracerebral lesions, indicating metastatic tumors. For the definitive diagnosis and to clarify options for chemotherapeutic management, the patient underwent surgical craniotomy. The resected specimen was considered to represent sarcomatoid carcinoma with positive immunoreactive staining for cytokeratin as an epithelial marker, indicating pleomorphic carcinoma. Furthermore, laboratory data showed leukocytosis without any infections and elevated serum G-CSF levels during hospitalization. The patient developed complications of occlusive intussusception in the small intestine caused by probably metastatic lesions and died from disease progression 82 days after initial admission. Although the presence of G-CSF-producing pleomorphic carcinoma was confirmed, the primary origin of this pleomorphic carcinoma could not be indentified clearly even at autopsy.

Background: Traumatic acute epidural hematoma following skull fracture may result from a direct head injury and develops unilaterally at the impact site. Contrecoup injury that occurs in head trauma has rarely been reported to result in a simultaneous contralateral epidural hematoma. When bilateral epidural hematoma does occur, the contralateral hematoma may be missed unless head computed tomographic images are requested. Case presentation: A 25-year-old man was involved in a road traffic accident and was struck on his right temporal region, rendering him unconscious. On admission to hospital, he had a swelling of the right temporal region of the scalp. An initial computed tomography scan demonstrated bilateral acute epidural hematoma of the middle cerebral fossa, with a linear fracture at the right temporoparietal region. Follow-up imaging showed the enlargement of the left-sided hematoma, and urgent hemai?¾toma evacuation of the left side was successful performed; the cause of bleeding was the left middle meningeal artery at the base of the left middle cerebral fossa. A small right-sided hematoma, believed to be due to rupture of the right posterior middle cerebral artery, was treated conservatively. The patient’s postoperative course was uneventful, and he was discharged from hospital 30 days following his initial admission, with no residual neurological deficit. Conclusions: Contrecoup epidural hematoma due to skull fracture and detachment of the dura mater from the base of the skull may be associated with rupture of the posterior middle cerebral artery causing a contralateral epidural hematoma. This case demonstrates an interesting mechanism to develop bilateral Epidural Hematoma (EDH).

A 70-year-old man presented to our hospital because of difficulty with discrete movement of the right upper limb and dysarthria. Computed tomography(CT)of the head revealed a chronic subdural hematoma(CSDH)on the left side. The patient underwent single burr-hole irrigation and drainage on the same day. In addition to the burr hole, a cross-shaped dural incision was made which revealed a thick outer membrane and solidified hematoma. We removed as much of the clotted hematoma as possible using a curved suction tube under neuroendoscopy. The postoperative CT revealed that the hematoma was partially removed and the mass effect was reduced. As a result, the patient's neurological deficits improved. We reached a diagnosis of organizing CSDH following histologic examination of the removed hematoma that showed inflammatory cell infiltration and multiplication of fibroblasts. Neuroendoscopic hematoma evacuation via a burr hole is minimally invasive and may be a useful procedure in the treatment of some cases of organizing CSDH.

The incidence of vitamin K deficiency in infancy has decreased markedly, due to prophylactic administration of vitamin K during the neonatal period. However, vitamin K deficiency bleeding may occur during or after the neonatal period despite prophylactic administration in Japan. Two cases are reported of intracranial hemorrhage associated with coagulopathy in full-term infants who had received prophylactic administration of vitamin K. More reliable methods for prophylactic administration should be established.

A 23-year-old man was admitted with a rare case of radiation-induced astrocytoma manifesting as 3-month history of unstable gait. He had received 50 Gy of irradiation therapy for a germ cell tumor in the right basal ganglia 13 years earlier. Magnetic resonance (MR) imaging on admission showed a non-enhanced mass lesion in the right cerebellar hemisphere with expansion to the vermis. The histological diagnosis of the stereotaxic biopsy specimen was grade II astrocytoma. Two months later, he developed drowsiness, and MR imaging demonstrated that the tumor had enlarged and was enhanced after gadolinium injection. The clinical diagnosis was high-grade glioma resulting from malignant transformation. The tumor had compressed the mesencephalic aqueduct, leading to obstructive hydrocephalus. Endoscopic third ventriculostomy was performed to improve the cerebrospinal fluid circulation. He underwent chemotherapy with temozolomide postoperatively, but died 8 months after the initial diagnosis of astrocytoma. The clinical course of radiation-induced astrocytoma is not benign. The potential for malignant transformation necessitates careful postoperative follow up for patients with this tumor.

Symptomatic Rathke’s cleft cyst is usually accompanied by a long history of headache, visual disturbance, and hypopituitarism; however, rare cases present with acute onset and the clinical features in such cases remain uncertain. We report herein the clinical features of Rathke’s cleft cyst with acute onset and discuss the clinical significance. In this study, we defined acute onset as the clinical course with clinical symptoms within a 7-day history. From among 35 cases of symptomatic Rathke’s cleft cyst that were pathologically diagnosed at Fukuoka University Hospital between 1990 and 2009, five cases presented with acute onset. The symptoms, endocrinological findings, MR image findings, and pathological findings of these cases were analyzed retrospectively. Mean age was 56.8 years. Initial symptoms included headache (n = 3), general malaise (n = 2), polyuria (n = 2), and fever (n = 1). MR imaging revealed an intrasellar cystic lesion with suprasellar extension in all cases and showed rim enhancement in three cases. All cases were treated by transsphenoidal surgery. Pathological findings included hemorrhage (n = 2), hypophysitis (n = 2), and abscess formation in the cyst (n = 1). Postoperatively, all symptoms, except for hypopituitarism, improved in all cases. Rathke’s cleft cysts sometimes present with acute onset, and the presentation is consistent with the features of pituitary apoplexy caused by pituitary adenoma. Although pituitary apoplexy due to hemorrhage, inflammation, or infection due to an underlying Rathke’s cleft cyst is difficult to diagnose pre-operatively, Rathke’s cleft cyst should be included in the differential diagnosis, and early surgical treatment is needed, as for pituitary apoplexy caused by pituitary adenoma.

A 37-year-old man involved in a motor vehicle accident was admitted to our hospital with disturbed consciousness. Computed tomography (CT) showed an acute, right-sided epidural hematoma and pneumocephalus. Emergency evacuation of the acute epidural hematoma was carried out, and his condition gradually recovered. However, cerebrospinal fluid (CSF) rhinorrhea continued despite conservative treatment. Three-dimensional reconstructed CT revealed a bone defect, which was approximately 20 mm in size, in the planum sphenoidale and tuberculum sellae, and magnetic resonance imaging demonstrated a traumatic meningo-encephalocele through the defect, with CSF collection in the sphenoid sinus. Endoscopic endonasal transsphenoidal surgery was performed. The 9-mm diameter dural defect was clearly visualized in the sphenoid sinus and was reconstructed with a combination of suturing and overlay with abdominal fascia, and absorbable polyglycolic acid felt. The CSF leakage from a traumatic meningo-encephalocele through the planum sphenoidale was successfully repaired by endoscopic endonasal surgery. Thorough preoperative evaluation of the feasibilities of the endoscopic and transcranial approaches should be based on the preoperative identification of the fistula, the bone defect, and vital structures.

A 13-year-old girl presented with an exceedingly rare case of primary yolk sac tumor located within the lateral ventricle, manifesting as headache, nausea, and diplopia. Magnetic resonance imaging revealed a 4-cm-diameter solid enhanced mass within the left inferior horn of the lateral ventricle. The tumor was removed subtotally via left middle temporal corticotomy. The histological and immunohistochemical diagnosis was pure yolk sac tumor. The serum alpha-fetoprotein (AFP) level was elevated at 1957.2 ng/ml and the serum beta-human chorionic gonadotropin level was 4 mIU/ml after surgery. The patient underwent radiotherapy (whole brain, 30 Gy; tumor bed, 21 Gy; whole spinal axis, 30 Gy) and chemotherapy (ifosfamide, cisplatin, etoposide). After three treatment cycles, the serum AFP level had decreased to 4.5 ng/ml. However, the tumor recurred with cerebrospinal fluid dissemination after nine cycles of chemotherapy. She died 18 months after surgery. The possibility of germ cell tumor should be considered in pediatric patients with brain tumors occurring outside the pineal or suprasellar region.

Ruptured aneurysms of the distal anterior cerebral artery (ACA) are relatively rare and surgical management provides some unique technical challenges. This retrospective analysis of 20 patients with distal ACA aneurysms evaluated the clinical features and surgical strategies. The characteristic findings were small and common concurrent aneurysms, and frequent intracerebral hematoma (ICH). Aneurysms were divided by location on the genu (n = 13), infracallosal (n = 5), and supracallosal portions (n = 2). All patients except one underwent surgery via an interhemispheric route. Unilateral craniotomy was performed for aneurysms on the genu portions without massive ICH. Bilateral craniotomies were selected for aneurysm located on the infracallosal portion or combined with massive ICH. No intraoperative rupture was observed. Favorable outcomes were achieved in 15 of 20 patients, and only one patient died. The preoperative Hunt and Kosnik grade was closely correlated with the outcome.

We report 3 patients who developed subarachnoid hemorrhage (SAH) from the vertebral dissecting aneurysm after head-nuchal pain. All 3 patients developed sudden head-nuchal pain, at the onset of which no subarachnoid hemorrhage was observed on CT (n=2) or on CT and MRI (n=1). Subsequently, SAH occurred within 1 or 2 days. All 3 patients were men aged 46 to 55 years, and had a Hunt and Kosnik Grade of III (n=1) or V (n=2) at admission. Vertebral angiography revealed aneurysmal dilatation with narrowing, but no posterior inferior cerebeller artery (PICA) involvement in any of the patients. All patients were treated for proxymal occlusion, including aneurysm, using GDC coils. In 1 patient, decompressive craniectomy was performed due to progression of a thrombosis to the PICA, and cerebeller and brain stem infarction. The Glasgow Outcome Scale (GOS) was good recovery in 2 patients. The patient who underwent decompressive craniectomy presented quadriplegia and had a score of Vegetative State. Although dissecting vertebral aneurysm presenting with head-nuchal pain and/or ischemic symptoms generally carry a favorable prognosis, we should be careful that it might associated with SAH as shown in the present case.

A 71-year-old male presented with left hemiparesis and confused conversation. Computed tomography showed a mass lesion with rim enhancement in the right parietal lobe. He developed meningeal irritation the day after admission. Emergent fluid-attenuated inversion recovery (FLAIR) magnetic resonance (MR) imaging revealed a clear hyperintense component in the right lateral ventricle and niveau formation inside the intracerebral lesion, indicating intraventricular rupture of the brain abscess. The patient underwent aspiration of the abscess and ventricular drainage with antibiotic administration. Nocardia asteroides was isolated from the aspirated pus, so systemic and direct administration of effective antibiotics was subsequently commenced. These procedures resulted in gradual improvement of his clinical course, and he left our hospital. Several days after discharge, he developed acute pan-peritonitis due to malignant lymphoma. He appeared to be progressively deteriorating after an exploratory laparotomy, and died on the 17th day after the laparotomy. Intraventricular rupture of nocardia brain abscess can be successfully treated after early definitive diagnosis with FLAIR MR imaging.

A 5-year-old boy was struck by a pickup truck, and admitted with Glasgow Coma Scale score of 14. Initial computed tomography (CT) showed no evidence of intracerebral lesions except for a skull fracture. Repeat CT 5 hours later showed hyperdense middle cerebral artery (MCA) sign, but he did not show any focal signs. Eighteen hours after the accident, he developed left hemiparesis. CT revealed a fresh infarction in the right MCA territory, associated with definite hyperdense MCA sign. He was immediately transferred to our hospital for further evaluation. Emergent angiography revealed a rat tail-shaped occlusion of the right internal carotid artery at the C-3 level. Cross-filling of the right MCA territory was insufficient for collateral circulation. He was treated conservatively because of the significant risk of hemorrhagic change from the established infarction. His hemiparesis improved gradually, and he was discharged on foot. Hyperdense MCA sign in a child is an important clinical sign for the early detection of cerebral ischemia after blunt carotid injury, before any focal signs appear.

A 49-year-old man presented with symptomatic cavernous malformation in the ventrolateral portion of the medulla oblongata manifesting as left-sided numbness and gait disturbance. Neurological examination disclosed sensory disturbance on the left, cerebellar ataxia, nystagmus, dysphagia, and right hypoglossal nerve paresis. Magnetic resonance imaging revealed a cavernous malformation with hemorrhage occupying the right paramedian field of the medulla oblongata. The patient underwent complete removal of the lesion through vertical incision of the bulging surface of the ventrolateral medulla, anatomically coinciding with the inferior olive. The neurological deficits improved without additional postoperative deficits. This unusual microsurgical approach through a ventrolateral medullary incision permits direct resection of a subpial intrinsic lesion, even on the ventral medulla.

We report a case of ruptured tectal arteriovenous malformation (AVM) that was demonstrated angiographically only after removal of an unruptured occipital AVM. A 57-year-old man presented with sudden onset of diplopia and tinnitus. Computed tomography revealed a small hemorrhage in the right tectum mesencephali with intraventricular hemorrhage. Magnetic resonance imaging and angiography disclosed AVM in the right occipital lobe which was separate from the hemorrhagic lesion. Angiography demonstrated that the right occipital AVM was fed by the parieto-occipital artery and drained into the superior sagittal sinus and vein of Galen. However, no abnormal vascular lesion was detected near the tectum mesencephali. As venous hypertension was considered the reason for hemorrhage, the occipital AVM was completely resected. Postoperative angiography demonstrated disappearance of the occipital AVM, but it also disclosed a small tectal AVM fed by branches from the superior cerebellar artery, which had not been detected on preoperative angiography. This was considered the true cause of hemorrhage, and gamma knife surgery was accordingly performed. Even if an AVM is demonstrated, if the lesion does not correspond to the hemorrhage we recommend serial angiographical evaluation so that a small AVM is not missed.

A 64-year-old man underwent microvascular decompression of the left superior cerebellar artery (SCA) for left trigeminal neuralgia (TN) using a sling of Teflon tape fixed to the tentorium with fibrin glue. The TN disappeared immediately after surgery, but recurred unusually rapidly at 2 weeks later at the same intensity as before. Second surgery revealed the SCA was suspended from the tentorium, but the trigeminal nerve was stretched and displaced superolaterally because of adhesion to the superior petrosal vein. The adhesion was thought to involve the fibrin glue used during the sling retraction procedure. The nerve was meticulously dissected from the adhesion, and the trigeminal nerve was placed in the correct position. The postoperative course was uneventful, and the TN disappeared completely. We recommend that the smallest amount of the fibrin glue possible be used to avoid adhesion to the surrounding neurovascular elements.

Pilomyxoid astrocytoma (PMA) is a variant of pilocytic astrocytoma (PA). However, PMA shows a higher rate of recurrence and dissemination in cerebrospinal fluid (CSF) than does pilocytic astrocytoma (PA). PMA occurs predominantly in the hypothalamic/chiasmic region, and thus it is usually treated with chemotherapy following surgical biopsy. We discuss the treatment of PMA. Materials and Methods: Between 1992 and 2009, the authors treated 5 patients, 2 males and 3 females, ranging in age from 3 months to 11 years. Results: Three patients showed CSF dissemination on the initial radiographic examination. All patients received chemotherapy; the most commonly used drug combination was that of cisplatin (CDDP)/carboplatin (CBDCA) and etoposide. If these drugs were unsuccessful, they were changed, or other drugs were added to the combination. After chemotherapy, four patients showed remarkable tumor regression. Nevertheless, one patient died due to tumor progression 22 months after initial diagnosis. Conclusion: Although our series comprised a small number of patients, treatment of PMA with chemotherapy appeared to be of value. Even if initial chemotherapy is ineffective, we recommend continued CDDP/CBDCA-based chemotherapy with new drug combinations.

A 23-year-old man was admitted with a rare case of radiation-induced astrocytoma manifesting as 3-month history of unstable gait. He had received 50 Gy of irradiation therapy for a germ cell tumor in the right basal ganglia 13 years earlier. Magnetic resonance (MR) imaging on admission showed a non-enhanced mass lesion in the right cerebellar hemisphere with expansion to the vermis. The histological diagnosis of the stereotaxic biopsy specimen was grade II astrocytoma. Two months later, he developed drowsiness, and MR imaging demonstrated that the tumor had enlarged and was enhanced after gadolinium injection. The clinical diagnosis was high-grade glioma resulting from malignant transformation. The tumor had compressed the mesencephalic aqueduct, leading to obstructive hydrocephalus. Endoscopic third ventriculostomy was performed to improve the cerebrospinal fluid circulation. He underwent chemotherapy with temozolomide postoperatively, but died 8 months after the initial diagnosis of astrocytoma. The clinical course of radiation-induced astrocytoma is not benign. The potential for malignant transformation necessitates careful postoperative follow up for patients with this tumor.

The incidence of vitamin K deficiency in infancy has decreased markedly, due to prophylactic administration of vitamin K during the neonatal period. However, vitamin K deficiency bleeding may occur during or after the neonatal period despite prophylactic administration in Japan. Two cases are reported of intracranial hemorrhage associated with coagulopathy in full-term infants who had received prophylactic administration of vitamin K. More reliable methods for prophylactic administration should be established.

We report a case of intramedullary cervical metastasis from cerebellar anaplastic oligodendroglioma without any MR appearance for CSF dissemination or recurrence in the primary tumor site. The case was a 73-year-old female who underwent tumor resection for cerebellar anaplastic oligodendroglioma following irradiation and TMZ chemotherapy. One year and a half later, a small nodular lesion developed at the temporal lobe. While treatment with TMZ was restarted during the course, another intramedullary cervical lesion produced additionally without any CSF dissemination or recurrence at the primary site. The histological examination obtained in spinal surgery revealed evidence of similar features consistent with a previous cerebellar tumor with anaplastic oligodendroglioma. Although CSF dissemination by malignant glioma with leptomeningeal enhancement is relatively well recognized at their terminal stage, cases with intramedullary cervical metastasis without any leptomeningeal enhancement have only been rarely described. We discussed the mechanisms of intramedullary cervical metastasis from intracranial malignant glioma.

A 13-year-old girl presented with an exceedingly rare case of primary yolk sac tumor located within the lateral ventricle, manifesting as headache, nausea, and diplopia. Magnetic resonance imaging revealed a 4-cm-diameter solid enhanced mass within the left inferior horn of the lateral ventricle. The tumor was removed subtotally via left middle temporal corticotomy. The histological and immunohistochemical diagnosis was pure yolk sac tumor. The serum alpha-fetoprotein (AFP) level was elevated at 1957.2 ng/ml and the serum beta-human chorionic gonadotropin level was 4 mIU/ml after surgery. The patient underwent radiotherapy (whole brain, 30 Gy; tumor bed, 21 Gy; whole spinal axis, 30 Gy) and chemotherapy (ifosfamide, cisplatin, etoposide). After three treatment cycles, the serum AFP level had decreased to 4.5 ng/ml. However, the tumor recurred with cerebrospinal fluid dissemination after nine cycles of chemotherapy. She died 18 months after surgery. The possibility of germ cell tumor should be considered in pediatric patients with brain tumors occurring outside the pineal or suprasellar region.

Numerous studies have reported the clinical efficacy of temozolomide (TMZ) treatment for high-grade glioma, but information on Japanese populations has been limited. This study assessed the safety and early outcomes of TMZ treatment, with or without combination therapy.The subjects comprised ten patients with high-grade glioma [glioblastoma multiforme (GBM), n=3, gliosarcoma (GS), n=1, anaplastic oligodendroglioma (AO), n=3, anaplastic mixed oligoastrocytoma (AOA), n=1, and anaplastic ependymoma (AE), n=2]. All the patients were initially treated with conventional radiotherapy following surgical resection with or without adjuvant chemotherapy. As second- or third-line chemotherapy, patients received TMZ for recurrence or tumor progression. As combination therapy, the local administration of tumor necrosis factor-alpha and the addition of carboplatin and etoposide were included for three patients during the course of oral TMZ treatment.Partial response (PR) to TMZ therapy was achieved by four out of the ten patients (objective response rate, 40%), while three patients displayed stable disease (SD) and three showed disease progression (PD). One of the patients receiving combination therapy has continued to show shrinkage of the relapsed tumor. Despite prior radio- and chemotherapy, most patients experienced only grade 1-2 hematotoxicity that was well-controlled by conservative therapy.TMZ chemotherapy is effective for the treatment of high-grade glioma in some patients without serious toxicity. Assessing the true efficacy of TMZ will require a larger study with comparison of long-term outcomes between other agents or combined therapeutic modalities.

Pilomyxoid astrocytoma (PMA) shows a higher rate of recurrence and cerebrospinal fluid (CSF) dissemination than does pilocytic astrocytoma (PA). In this article, we discuss the treatment of PMA.Between 1992 and 2007, the authors treated 5 patients. Two of these were male, three female. Their ages ranged from 3 months to 11 years.Three patients showed CSF dissemination on the initial radiographic examination. All patients received chemotherapy; the most commonly used combination drugs were cisplatin (CDDP)/carboplatin (CBDCA) and etoposide. When these drugs were unsuccessful, they were changed or other drugs added to the combination. After chemotherapy, four patients showed remarkable tumor regression. Nevertheless, one patient died 22 months after initial diagnosis, due to tumor progression.While our series was limited to a small number of patients, we have a positive impression of the value of chemotherapy. Even if initial chemotherapy is ineffective, we recommend continued CDDP/CBDCA-based chemotherapy with new drug combinations.

We report a case of ruptured tectal arteriovenous malformation (AVM) that was demonstrated angiographically only after removal of an unruptured occipital AVM. A 57-year-old man presented with sudden onset of diplopia and tinnitus. Computed tomography revealed a small hemorrhage in the right tectum mesencephali with intraventricular hemorrhage. Magnetic resonance imaging and angiography disclosed AVM in the right occipital lobe which was separate from the hemorrhagic lesion. Angiography demonstrated that the right occipital AVM was fed by the parieto-occipital artery and drained into the superior sagittal sinus and vein of Galen. However, no abnormal vascular lesion was detected near the tectum mesencephali. As venous hypertension was considered the reason for hemorrhage, the occipital AVM was completely resected. Postoperative angiography demonstrated disappearance of the occipital AVM, but it also disclosed a small tectal AVM fed by branches from the superior cerebellar artery, which had not been detected on preoperative angiography. This was considered the true cause of hemorrhage, and gamma knife surgery was accordingly performed. Even if an AVM is demonstrated, if the lesion does not correspond to the hemorrhage we recommend serial angiographical evaluation so that a small AVM is not missed.

We present an extremely rare case of an orbital melanocytoma that occurred in a 51-year-old man. The patient suffered from diplopia and mild exophthalmos of the right eye for 2 months. Brain magnetic resonance imaging showed a well-demarcated round mass 3.5 cm in diameter in the right orbit. We performed total resection of this tumor. Histological findings revealed a proliferation of large polygonal cells with fine pigment granules in the cytoplasm and prominent nucleoli. Although these tumor cells revealed immunohistochemical reactivity in HMB-1, there was no S-100 or Melan A antibody reactivity. Also, there were no malignant findings of nuclear polymorphism, mitoses, or necrosis. The brown pigments were confirmed to be melanin by bleaching and the Fontana-Masson silver stain method. The MIB-1 labeling index was less than 1%. This tumor also consisted of 50% melanophages, which revealed immunohistochemical reactivity in CD68, CD163, and in (1-AT antibodies. These histological findings led us to diagnose an orbital melanocytoma with partial tumor regression.

Hypointense signal appearance of metastatic adenocarcinoma on T2-weighted imaging (T2-WI) has been infrequently documented. The purpose of this report was to evaluate the degree to which mucin content affects signal manifestations on conventional MR imaging.This series of 24 cases with intracerebral metastatic adenocarcinoma was assessed retrospectively, focusing on the association between hypointense appearance on T2-WI and intratumoral mucin content.Among the 24 metastatic adenocarcinomas, intratumoral mucin was histopathologically confirmed in 8 lesions. Of these, 4 masses were demonstrated as hyperintense signal on T2-WI. The other 4 masses were depicted as isointensity. No cases were identified with hypointense signals in mucin-containing metastatic adenocarcinoma. Conversely, only 2 metastatic tumors originating from the stomach exhibited hypointense signal lesions on T2-WI. No histological or magnetic resonance imaging evidence of blood products or other forms of iron were identified as causes of the hypointense appearance on T2-WI.This hypointense signal may simply reflect the relatively shorter T2 relaxation time of the primary tissue from which metastases arose. Intratumoral mucin itself may be considered to demonstrate the masses as hyper- or isointense signals in the brain.

A 64-year-old man underwent microvascular decompression of the left superior cerebellar artery (SCA) for left trigeminal neuralgia (TN) using a sling of Teflon tape fixed to the tentorium with fibrin glue. The TN disappeared immediately after surgery, but recurred unusually rapidly at 2 weeks later at the same intensity as before. Second surgery revealed the SCA was suspended from the tentorium, but the trigeminal nerve was stretched and displaced superolaterally because of adhesion to the superior petrosal vein. The adhesion was thought to involve the fibrin glue used during the sling retraction procedure. The nerve was meticulously dissected from the adhesion, and the trigeminal nerve was placed in the correct position. The postoperative course was uneventful, and the TN disappeared completely. We recommend that the smallest amount of the fibrin glue possible be used to avoid adhesion to the surrounding neurovascular elements.

Advanced magnetic resonance (MR) techniques provide physiological and metabolic information that complements the anatomical information available from conventional MR imaging. The purpose of this study was to evaluate the clinical usefulness of proton MR spectroscopy (1H-MRS) in preoperative quantitative assessment of intracranial gliomas.Eight patients with histologically verified gliomas, comprising 2 cases with glioblastoma multiforme (GBM, grade 4), 5 cases with anaplastic oligodendroglioma (AO, grade 3; high-grade glioma), and 1 case with fibrillary astrocytoma (FA, grade 2; low-grade glioma) were evaluated using the 1H-MRS protocol following conventional MR imaging, diffusion-weighted imaging (DWI), and perfusion-weighted imaging (PWI) preoperatively.High-grade gliomas tended to demonstrate signal hyperintensity by DWI and higher relative cerebral blood volume (rCBV) by PWI. Increased ratios of choline (Cho) to N-acetylaspartate (NAA) (Cho/NAA) and Cho to creatine (Cr) (Cho/Cr) correlated highly with tumor malignancy. The presence of lactate and lipid was predominately detected in patients with high-grade glioma.The combination of multiple MR parameters, based on DWI, PWI and 1H-MRS, appears valuable for preoperatively predicting the degree of malignancy in glioma.

We report an unusual case of subarachnoid hemorrhage (SAH) due to ruptured aneurysm originating at the caudal loop of posterior inferior cerebellar artery (PICA). The patient was a 77-year-old female presented with a sudden onset of headache and vomiting. Initial CT scan demonstrated a SAH with thick hematoma mainly in the cisterna magna. Vertebral angiogram revealed a saccular aneurysm arising from the tonsillomedullary segment of the left PICA, and communicating artery with supplying a territory of contralateral vermis as an anastmotic vessel. At surgery, no vessel branches were confirmed in the vicinity of the aneurysm, and this aneurysm was successfully clipped. In the fetus, numerous basilar and vertebral arteries are organized in plexiform formations around the brain stem. It has been suggested that the pathogenesis of such aneurysm or communicating artery could be related with a remnant of a primitive vertebrobasilar anastomosis. Based on these considerations, congenital vessel-wall weakness and hemodynamic stress associated with communicating artery may contribute to the development of distal PICA aneurysms.

We report a case of a nonfunctioning pancreatic endocrine tumor arising from intracranial ectopic pancreatic tissue.An 11-year-old girl was admitted to our hospital with a brain tumor. Hydrocephalus and brain malformation were apparent at birth. We first identified a mass-like lesion in the child's brain at age 4 months. We monitored the lesion yearly by magnetic resonance imaging (MRI) until she reached age 11 years. Gadolinium-enhanced MRI showed the lesion to be a tumor, which was resected.Examination of surgical specimens revealed a mature pancreatic tissue. We also identified monotonous neoplastic cells with round nuclei and positive immunoreactivity for synaptophysin, chromogranin A, and neurospecific enolase. However, these cells were negative for pancreatic endocrine markers. We diagnosed nonfunctioning pancreatic endocrine tumor arising from intracranial ectopic pancreatic tissue.Migrating pancreatic elements may have induced brain malformation during embryonic development and subsequently become malignant.

This study assessed safety and efficacy for intratumoral administration of tumor necrosis factor-a (TNF-SAM2) into the post-operative tumor cavity through an Ommaya reservoir for patients with malignant glioma.Seven patients with malignant glioma, comprising 3 cases with glioblastoma multiforme (GBM), 3 cases with anaplastic astrocytoma (AA) and 1 case with malignant ependymoma (ME) were included in the study. All patients were pathologically diagnosed and initially treated with adjuvant therapy (radiation and/or ranimustine and/or systemic TNF-SAM2 infusion) following surgical resection. TNF-SAM2 was administrated into the post-operative tumor cavity through a reservoir at a concentration of 1x10(4) U/body when recurrence was detected, or as initial induction therapy concomitant with radiotherapy.Partial response to this regional immunotherapy was seen in 4 out of 7 patients, and 1 patient with GBM has remained clinically stable for184 weeks without tumor progression. With AA, 2 cases appeared to display slowed advance and longer times to tumor recurrence or regrowth. No serious adverse effects, such as brain edema, hemorrhage or seizure were observed, nor systemic toxicities.Local immunotherapy with TNF-SAM2 may safely contribute to therapeutic efficacy in some patients with malignant glioma.

We report a case with a unilateral sudden sensorineural hearing loss caused by an infarction of brainstem and cerebellum. The patient was a 74-year-old male presented with a sudden onset of hearing loss and tinnitus in the right ear and dizziness. Steroid was administered on suspicion of idiopathic sudden deafness. However, the initial symptoms were deteriorated approximately 2 weeks later. He newly complained of the numbness of the right face and double vision, and he was transferred to our hospital for further evaluation. Neurological examination demonstrated horizontal nystagmus, diminution in the right facial sensation, right peripheral facial palsy, right hearing loss and cerebellar ataxia. Urgent MRI disclosed fresh infarctions of the right middle cerebellar peduncle and cerebellum localized in the territory of anterior inferior cerebellar artery. In general, idiopathic sudden deafness and Meniere's disease are frequent diagnosis in cases of sudden hearing loss with vertigo, but these symptoms may rarely be caused by cerebrovascular disorder. In patients with risk factors for arteriosclerosis, cerebrovascular disorder should be taken into consideration even if idiopathic sudden deafness may be suspected clinically. We emphasize the diagnostic importance of careful observation on neurological findings and early detection of radiological abnormalities on MRI.

This study assessed the safety, tolerance and preliminary efficacy of a combination chemotherapy regimen consisting of ranimustine (MCNU) and recombinant human mutant tumor necrosis factor-alpha (TNF-SAM2) for patients with newly diagnosed supratentorial malignant astrocytomas.The initial regimens were prescribed as adjuvant therapy in conjunction with radiotherapy following standard surgical treatment. Ranimustine (MCNU) was administered intravenously at 100 mg/m2 on Day 1, i.e., at the onset of radiation therapy, and was followed by 80 x 10(4) U/m2 TNF-SAM2 intravenously from Day 3. TNF-SAM2 was prescribed weekly for up to 5 injections during the postoperative period concurrent with radiation therapy. TNF-SAM2 and MCNU were given each 8- to 12-week cycle until tumor progression was evident, or for a total of four cycles over a 1-year period. The primary end-points were safety and tolerability and the secondary end-point was overall survival.Twenty-six consecutive eligible patients, including 5 with anaplastic astrocytoma (3 men and 2 women) and 21 with glioblastoma (13 men and 8 women), were treated. All of the 3 evaluable patients with anaplastic astrocytoma partially responded to treatment (PR), with a time to tumor progression (TTP) of 107 weeks and an estimated median survival time of 330 weeks. Of the 15 evaluable patients with glioblastoma, 8 (53.3%) showed no change in response to the treatment (NC), while 7 (46.7%) had progressive disease (PD), with a time to tumor progression (TTP) of 36 weeks and an estimated median survival time of 69 weeks. Although this regimen appeared to be safe, there was no improvement in response or survival time compared with a historical control of patients who received chemotherapy with MCNU alone in conjunction with radiotherapy for glioblastoma. Neither neurological toxicity, severe or life-threatening hematological toxicity, nor fatal toxicity (WHO Grade 4) was experienced.These results suggest that combined chemotherapy with mutant TNF-alpha (TNF-SAM2) in this patient population seems to be safe and tolerable and may benefit those with anaplastic astrocytoma. These intriguing clinical observations warrant further evaluation to determine whether this approach can provide therapeutic benefits and improve survival.

We report a case of spontaneous intracranial hypotension (SIH) manifesting as sudden deafness followed by chronic subdural hematoma in the course of the disease. The patient was a 31-year-old female presenting with an orthostatic headache. Judging from her characteristic symptoms, SIH was strongly suggested. MR images with gadolinium were consistent with the diagnosis of SIH with a diffuse dural enhancement. Her symptoms improved gradually by conservative therapies with bed rest and oral steroid, but she developed poor hearing at 3 weeks after the onset of the symptoms. She was diagnosed as having sudden deafness. After altering the therapeutic modality to intravenous steroid administration, her perceptive deafness resolved earlier, and her symptoms of headache disappeared. However, she was readmitted to our hospital at 4 months after the onset, complaining of the aggravation of headache with a different type, which was worse in the recumbent posture. Follow-up MR images revealed a development of subdural hematoma with mass effect, and the hematoma had to be treated by burr hole drainage. In this case, the site of CSF leakage along the spinal axis was not identified by spinal MR images. This case is considered to be a rare one, complicating sudden deafness followed by chronic subdural hematoma during the clinical course of the SIH.

We report two adult cases of unilateral schizencephaly manifesting as minor neurological signs. Case 1 was a 52-year-old female with an attack of partial seizure. CT demonstrated a closed cleft in the left frontal lobe. MRI revealed that the cleft was covered with gray matter, and was extending to the ventricular wall. Her epileptic seizure was successfully controlled by medication. Case 2 was a 58-year-old female with a history of mild right hemiparesis from her birth. CT demonstrated a closed cleft in the left peri-Rolandic area. MRI revealed a cortical infolding which extended to the lateral ventricle, and complication with polymicrogyria. Two patients were diagnosed as having normal intelligence, and unilateral schizencephalies with closed-lip. There has appeared to be more reports in the recent literature dealing with unilateral schizencephaly with mildly neurologic dysfunction. It is sufficiently capable of making such a diagnosis for schizencephaly by initial CT evaluation of a detailed radiographic assessment, in which characteristic findings with focal enlargement of cortical sulci or appearance of cortical infolding extending to deep white matter will be detected, though MRI is considered to be useful for a more detailed neuroanatomical evaluation.

We report a case of subarachnoid hemorrhage (SAH) from vertebral dissecting aneurysm 4 days after first nuchal pain. The patient was a 46-year-old man with a sudden onset of nuchal pain. There were no obvious abnormalities detected on MR images in another hospital. Four days later, however, he was admitted to our hospital because of severe re-attack of nuchal pain. CT demonstrated moderate SAH and cerebral angiograms revealed right vertebral dissecting aneurysm. Proximal occlusion of the vertebral artery including its aneurysmal dilatation was performed using detachable coils. We strongly suspected that his initial symptom of nuchal pain was due to dissection of the vertebral artery itself, since the aneurysmal dilatation accompanied by intramural hematoma had been observed retrospectively in the initial MR imaging. The incidence of the vertebral dissecting aneurysm presenting with nuchal pain alone due to dissection is reported to be 7% in the literature. The prognosis of non-hemorrhagic vertebral dissecting aneurysm followed by delayed SAH is considered to be fatal. Therefore, careful investigations for differential diagnosis should be taken into account since the diagnostic possibility exists that non-hemorrhagic vertebral dissecting aneurysm would be manifested by a symptom of headache/nuchal pain alone.

We report a 74-year-old man with an ischemic lesion in the ventral midbrain. He presented with contralateral ptosis and marked upward gaze paresis of the right eye. Neurological examination revealed partial oculomotor nerve palsy caused by impairment of the right levator palpebrae, superior rectus and inferior oblique muscles. This finding is highly suggestive of a possible lesion in the midbrain affecting the oculomotor fascicular fibers. Magnetic resonance images showed an ischemic lesion in the paramedian area of the right midbrain tegmentum. The coronal view of T 2-weighted imaging clearly demonstrated to be the site of lesions below the red nucleus. It seemed to be coincidental with the impaired site of involving the caudal part of oculomotor fascicular fibers emerging from the nucleus. This report is considered to be a typical case of partial fascicular oculomotor paresis based on impairment of the caudal part of oculomotor fascicles for the levator palpebrae, superior rectus, and inferior oblique muscles. This is a valuable case to be documented in which neurological site of lesions are consistent with those found in radiological study.

A 5-year-old boy was struck by a pickup truck, and admitted with Glasgow Coma Scale score of 14. Initial computed tomography (CT) showed no evidence of intracerebral lesions except for a skull fracture. Repeat CT 5 hours later showed hyperdense middle cerebral artery (MCA) sign, but he did not show any focal signs. Eighteen hours after the accident, he developed left hemiparesis. CT revealed a fresh infarction in the right MCA territory, associated with definite hyperdense MCA sign. He was immediately transferred to our hospital for further evaluation. Emergent angiography revealed a rat tail-shaped occlusion of the right internal carotid artery at the C-3 level. Cross-filling of the right MCA territory was insufficient for collateral circulation. He was treated conservatively because of the significant risk of hemorrhagic change from the established infarction. His hemiparesis improved gradually, and he was discharged on foot. Hyperdense MCA sign in a child is an important clinical sign for the early detection of cerebral ischemia after blunt carotid injury, before any focal signs appear.

A 49-year-old man presented with symptomatic cavernous malformation in the ventrolateral portion of the medulla oblongata manifesting as left-sided numbness and gait disturbance. Neurological examination disclosed sensory disturbance on the left, cerebellar ataxia, nystagmus, dysphagia, and right hypoglossal nerve paresis. Magnetic resonance imaging revealed a cavernous malformation with hemorrhage occupying the right paramedian field of the medulla oblongata. The patient underwent complete removal of the lesion through vertical incision of the bulging surface of the ventrolateral medulla, anatomically coinciding with the inferior olive. The neurological deficits improved without additional postoperative deficits. This unusual microsurgical approach through a ventrolateral medullary incision permits direct resection of a subpial intrinsic lesion, even on the ventral medulla.

This study assesses the safety, tolerance and preliminary efficacy of combined treatment with carboplatin, etoposide and recombinant human mutant tumor necrosis factor-alpha (TNF-SAM2) for recurrent malignant supratentorial astrocytomas at first relapse.Carboplatin was administered intravenously at a dose of 400 mg/m2 on Day 1, while etoposide was administered intravenously at a dose of 100 mg/m2 from Day 1 to Day 3 for 3 days. From Day 7, 80x10(4) U/m2 TNF-SAM2 was given intravenously for up to 5 injections for 2 weeks. Treatment was repeated every 8 to 12 weeks.Ten patients previously treated with surgery, radiation therapy and chemotherapy with a nitrosourea (ranimustine: MCNU) for malignant astrocytomas received this regimen for up to four cycles. Three patients with anaplastic astrocytomas, one patient with anaplastic oligoastrocytoma and 6 patients with glioblastomas (3 men and 7 women), aged 27 to 69 years, were eligible and were evaluated for response and toxicity. Grade 2 and 3 hematological toxicities occurred in 4 (40%) and 2 patients (20%), respectively. Grade 2 hepatic toxicity was observed in two patients. Of 9 evaluable patients, three (33%), including one glioblastoma, partially responded to the treatment (PR) with time to tumor progression (TTP) of 231, 121 and 57 weeks, respectively. Two patients had stable disease (SD), while 4 glioblastomas (44%) had progressive disease (PD) with TTP values of 11, 15, 6 and 12 weeks, respectively.These results suggest that combined therapy with carboplatin, etoposide and recombinant mutant TNF-alpha in this patient population seems to be safe and acceptable and may benefit those with recurrent anaplastic astrocytomas. These intriguing clinical observations warrant a properly stratified randomized trial to determine whether this approach can provide therapeutic benefits and improve survival.

Little is known about modulation by cytokines of major histocompatibility complex (MHC) antigen expression on intracranial tumors in vivo. The ability of cytokines to up-regulate MHC class-1 (MHC-1) antigen expression was investigated first in vitro using three rat glioma cell lines. Immunohistochemistry showed that incubation with recombinant rat interferon-gamma (rrIFN-gamma) increased MHC-1 antigen expression in RG2, C6, and 9L cell lines. Flow cytometric analysis revealed different baseline levels of MHC-1 antigen expression in each line (RG2 lowest, C6 highest), and that these levels increased in all lines after stimulation with 100 U ml(-1) or more of rrIFN-gamma. The antitumor effect of rrIFN-gamma in vivo was evaluated by assessing survival of rats with implanted intracerebral RG2 gliomas after intracarotid infusion of rrIFN-gamma. A high dose of rrIFN-gamma (2.4 x 10(5) U kg(-1)) significantly increased the survival, compared to control (p < 0.02). Intracarotid pre-treatment with the bradykinin analogue RMP-7 did not further increase survival. Immunohistochemical staining of tumor sections after in vivo rrIFN-gamma, infusion showed no clear increase in MHC-1 antigen expression on tumor cells but increased staining for ED2 antigen within tumor tissue, presumably from perivascular cells with MHC class-2 antigen.