Cerebral Paragonimiasis With Hemorrhagic Stroke in a Developed Country.

Paragonimiasis is a food-borne parasitic disease caused by Paragonimus lung flukes, which are epidemic in Asia. Cerebral paragonimiasis accounts for <1% of symptomatic paragonimiasis but is the most common extrapulmonary infection. Cerebral paragonimiasis often mimics stroke and sometimes causes severe neurological sequelae. A 61-year-old woman was admitted to the hospital for severe headache. A head computed tomography scan revealed intracerebral hemorrhage with subarachnoid hemorrhage. The patient also had lesions in the lungs. She frequently ate Japanese mitten crab. Peripheral blood examination results of increased eosinophilia and immunological testing results confirmed the diagnosis of Paragonimus westermani infection. The patient was successfully treated with praziquantel as the first-line agent. Cerebral paragonimiasis is currently rare in developed countries; however, it is an important disease to consider.

Anaplastic astrocytoma cells not detectable on autopsy following long-term temozolomide treatment: A case report.

We herein present an autopsy case of a glioma patient who received long-term treatment with temozolomide (TMZ). The patient, a 35-year-old man with a hypointense tumor of the left frontal lobe, without contrast enhancement following gadolinium (Gd) administration on T1-weighted images, underwent tumor removal surgery, after which the tumor was diagnosed as anaplastic astrocytoma. By the third round of surgery, the tumor had progressed to anaplastic astrocytoma with contrast enhancement following Gd administration, and the patient received 60 Gy of external beam radiotherapy and nimustine hydrochloride (ACNU)-based chemotherapy. After the fifth tumor removal surgery, TMZ was substituted with ACNU chemotherapy, which suppressed tumor progression. Following the 41st TMZ treatment, hemorrhage was observed in the residual tumor, and the hematoma had been replaced by a hemangioma. The hemangioma and surrounding brain tissue was removed during the sixth surgery. The patient survived for 14 years and 9 months after the initial surgery, but succumbed to hydrocephalus due to bleeding from hemangiomas. The histopathological specimens of the first to the sixth surgeries revealed mutant isocitrate dehydrogenase 1 (IDH1; R132H point mutation) and p53-positive tumor cells, but cells positive for the R132H mutation or p53 could not be detected by immunohistochemistry in the autopsy specimens of the brain after 108 courses of TMZ treatment. Mutant IDH1 (R132H) cells were also not detected in the autopsy specimens of the brain by polymerase chain reaction analysis.

Clinical presentation and treatment of distal posterior inferior cerebellar artery aneurysms.

Aneurysms located at the distal portion of the posterior inferior cerebellar artery (PICA) are rare, and their clinical features are not fully understood. We report the clinical features and management of 30 distal PICA aneurysms in 28 patients treated during the past decade at Kagoshima University Hospital and affiliated hospitals. Our series includes 20 women and eight men. Of their 30 aneurysms, 24 were ruptured, and six were unruptured; there were 27 saccular and two fusiform aneurysms; one was dissecting. Their location was at the anterior-medullary (n = 4), lateral-medullary (n = 9), tonsillomedullary (n = 7), telovelotonsillar (n = 6), and cortical (n = 4) segment of the PICA. In 18 patients, angiographic features suggested hemodynamic stress including an absent contralateral PICA or ipsilateral anterior inferior cerebellar artery, termination of the vertebral artery (VA) at the PICA, and hyperplasia or occlusion of the contralateral VA. As three patients died before surgery, 27 aneurysms in 25 patients were surgically treated. Of these, 6 were unruptured aneurysms; 20 were clipped via midline or lateral suboccipital craniotomy, and 5 were embolized with Guglielmi coils; in one, the PICA flow was reconstructed by OA-PICA anastomosis, and in the other one, the PICA was resected. Of the 25 surgically treated patients, 22 (88%) had good outcomes. The predominant contributor to the development of distal PICA aneurysms is thought to be increased hemodynamic stress attributable to anomalies in the PICA and related posterior circulation. Both direct clipping and coil embolization yielded favorable outcomes in our series. However, considering the difficulties that may be encountered at direct clipping in the acute stage and the availability of advanced techniques and instrumentation, aneurysmal coiling is now the first option to address these aneurysms.

[Three pediatric cases with choroid plexus papilloma in the fourth ventricle].

Choroid plexus papillomas are rare; they comprise less than 1% of all intracranial tumors. In children, most of these neoplasms arise in the lateral ventricle while in adults they are primarily located in the fourth ventricle. We report 3 children with choroid plexus papilloma in the fourth ventricle; they were one 5-month-old girl and 2 boys aged 8-and 15-years. The baby girl presented with macrocephaly and signs of raised intracranial pressure, the 2 boys exhibited cerebellar signs. On magnetic resonance imaging (MRI) the tumors were well-enhanced with a cauliflower-like contour and hydrocephalus. In one case, diffusion weighted images (DWIs) showed an isointense tumor signal clearly different from the well-known hyperintensity of medulloblastomas, Via suboccipital craniotomy we succeeded in the total or subtotal surgical resection of these tumors and there were no sequela. None of the tumors have recurred in the intervening 2-9 years. In conclusion, choroid plexus papilloma should be included as a differential diagnosis in children presenting with tumors in the fourth ventricle. The contour of the tumor on MRI and its intensity on DWI may be clues for a correct preoperative diagnosis.

Transethmoidal meningoencephalocele involving the olfactory bulb with enlarged foramina of the lamina cribrosa--case report.

A 3-year-old girl presented with a transethmoidal meningoencephalocele manifesting as recurrent rhinorrhea. Initially, she developed meningitis, but after treatment she experienced rhinorrhea. Two months later, she again presented with rhinorrhea. Neuroimaging studies revealed a small protrusion (15 mm x 10 mm) at the roof of the ethmoidal sinus. Nasal endoscopy confirmed the diagnosis of meningoencephalocele. The operative findings revealed a small hole in the left olfactory bulb, which had descended into an enlarged foramen along with the arachnoid membrane. The left olfactory bulb was removed, and the enlarged foramina of the lamina cribrosa were covered with a frontal pericranial flap. The defect in the bone was very small, but contributed to the development of meningitis and leakage of the cerebrospinal fluid. Basal cephalocele should be considered in a patient with recurrent rhinorrhea and intracranial infections, even in the absence of any apparent anomaly.

Changes in tissue factor and the effects of tissue factor pathway inhibitor on transient focal cerebral ischemia in rats.

INTRODUCTION: To determine the contribution of tissue factor (TF) to focal cerebral ischemia/reperfusion injury, we investigated the changes in TF in rat brains with transient focal cerebral ischemia and also assessed the effect of TF pathway inhibitor (TFPI). MATERIALS AND METHODS: Spontaneous hypertensive rats were subjected to 90-min of middle cerebral artery occlusion (MCAO) and then were reperfused for up to 24 h. Immediately after MCAO, recombinant human TFPI (rhTFPI) (50 or 20 microg/kg/min) was administered by means of a continuous intravenous injection for 4.5 h. RESULTS AND CONCLUSIONS: TF immunoreactivity decreased or scattered in the ischemic area after reperfusion, however, an increased TF expression was observed in the microvasculature with the surrounding brain parenchyma and it peaked at 3 to 6 h, which coincided with the start of fibrin formation. On the other hand, total TF protein in ischemic area continued to exist and did not remarkably change until 24 h after reperfusion. At 24 h after reperfusion, the total infarct volume in the group treated with 50 microg/kg/min rhTFPI was significantly smaller than that in the controls (saline). Western blotting and immunohistochemical studies showed that rhTFPI treatment resulted in a decrease of fibrin in the ischemic brains and microvasculature. TF-mediated microvascular thrombosis is thus considered to contribute to focal cerebral ischemia/reperfusion injury. The continuous infusion of rhTFPI until a peak of TF-mediated microvascular thrombosis therefore attenuates the infarct volume by reducing fibrin deposition in the cerebral microcirculation.

C-type natriuretic peptide is specifically augmented by pituitary adenylate cyclase-activating polypeptide in rat astrocytes.

In rat-cultured astrocytes, pituitary adenylate cyclase-activating polypeptide (PACAP) activates gene expression and secretion of C-type natriuretic peptide (CNP) in a dose- and time-dependent manner. These results suggest that PACAP might be involved in the regulation of CNP biosynthesis in astrocytes.

The environment of increased concentration of docosahexaenoic acid in glioblastoma may suppress the anti-tumor effect of macrophages.

Regarding glioblastoma, there has been controversy over whether a large number of infiltrating macrophages act as anti-tumor effectors or not. It has been exhibited that intratumoral lipid environments have a possible influence on anti-tumor immunity. Necrosis of glioblastoma and non-necrotic tissues of astrocytic tumors were analyzed to compare the amount of free docosahexaenoic acid (DHA) content. The apoptosis inducible effects of DHA on macrophages derived from peripheral blood monocytes and cultured glioma cells were evaluated by flow cytometry. The influence of DHA on the anti-tumor effects of macrophages was assessed at 0, 30, 60, and 90 mM of DHA by (51)Cr releasing assay and MTT assay. The mean concentration of free DHA in necrotic tissues (757.6 mmol/kg) was 5 times higher than that in non-necrotic tissues (147.2 mmol/kg). The DHA concentration of 30 mM induced apoptosis in macrophages, however, glioma cells were not affected even at a DHA concentration of 60 mM. Macrophages pre-exposed to DHA for 24 h decreased the cytotoxicity to U251MG cells as shown by (51)Cr releasing assay. Total viability of co-cultured macrophages and U251MG cells showed an increase at high concentrations of DHA (60 and 90 mM) according to 24 h MTT assay, although each separate culture did not. The DHA concentration in necrosis of glioblastoma was sufficient for macrophages to cause apoptosis and suppress their anti-tumor effects. The results suggest that liberated DHA in necrosis can induce apoptosis in macrophages and inhibit their functions.

Clinical implications of associated venous drainage in patients with cavernous malformation.

OBJECT: The authors reviewed angiograms obtained in patients with cavernous malformations to identify and characterize coexisting venous drainage. METHODS: Fifty-seven patients with cavernous malformations treated at the authors' institutions between 1994 and 2002 were classified into three groups according to the venous system adjacent to the malformation on angiography studies. In Group A patients (23 patients) the malformations had no venous drainage; in Group B patients (14 patients) the lesions were associated with typical venous malformations; and in Group C patients (20 patients) the lesions had atypical venous drainage (AVD). The risk of hemorrhage based on the type of associated venous drainage was analyzed, and the usefulness of magnetic resonance (MR) imaging compared with digital subtraction (DS) angiography in demonstrating associated AVD was determined. Fifty-seven patients harbored 67 cavernous malformations: Group A patients had 29 cavernous malformations with no associated venous drainage; Group B patients had 17 lesions associated with venous malformations; and Group C patients harbored 21 lesions, 20 of which manifested AVD. Symptomatic hemorrhage was present in 10 (43.5%) of 23 Group A patients and in 28 (82.4%) of 34 Groups B and C patients. Although high-resolution MR imaging revealed the presence of associated venous malformations in 11 (78.6%) of 14 Group B patients, such studies demonstrated AVD in only two (10%) of 20 Group C patients. CONCLUSIONS: Patients harboring cavernous malformations plus venous malformations or AVD are more likely to present with symptomatic hemorrhage than are patients with cavernous malformation alone. The actual incidence of associated venous drainage may be underestimated when MR imaging alone is used rather than combined with DS angiography.

Intractable hiccups as a presenting symptom of cerebellar hemangioblastoma. Case report.

The authors report on a 52-year-old woman with a cerebellar hemangioblastoma who presented with a 2-year history of intractable hiccups. Computerized tomography scans and magnetic resonance images revealed a cerebellar hemangioblastoma with compression of the brainstem at the level of the medulla oblongata. The patient has been free of hiccups and has been neurologically intact since the day after total removal of the tumor. A review of the literature on medullary lesions presenting with intractable hiccups is provided.

Postoperative regression of desmoplastic infantile gangliogliomas: report of two cases.

OBJECTIVE AND IMPORTANCE: Desmoplastic infantile gangliogliomas (DIGs) are extremely rare tumors that respond well to treatment. However, their biological behavior remains to be clarified. We describe two patients whose DIGs spontaneously regressed after surgery, without adjuvant therapy. CLINICAL PRESENTATION: A 9-month-old girl presented with left hemiparesis, and a 6-month-old boy presented with increasing head circumference. For both patients, neuroimaging demonstrated a huge cystic tumor that included a solid portion and was widely attached to the dura. Gadolinium-diethylenetriamine penta-acetic acid produced strong enhancement. INTERVENTION: One patient underwent partial and the other subtotal tumor removal. Histologically, both tumors were diagnosed as DIGs. Postoperatively, the residual tumors were monitored without adjuvant therapy, and both regressed in several months. CONCLUSION: Our experience suggests that DIGs may include a subgroup of tumors with a tendency for spontaneous regression, possibly attributable to the induction of apoptosis.

Pontine malignant astrocytoma with hemorrhagic onset--case report.

A 7-year-old boy presented with acute onset of left hemiparesis and headache, followed by disturbance of consciousness. Neuroimaging studies showed pontine hemorrhage. Surgery was performed to remove a massive hematoma. Histological examination of the wall revealed anaplastic astrocytoma. Postoperative radiation therapy and several types of chemotherapy were administered. However, the tumor recurred and he died 9 months after onset. Hemorrhagic onset of pontine glioma is rare and carries an extremely poor prognosis.

The expression of tissue factor correlates with proliferative ability in meningioma.

Tissue factor (TF) is a cell-surface glycoprotein responsible for initiating the extrinsic pathway of coagulation; this is inhibited by tissue factor pathway inhibitor (TFPI). As TF reportedly regulates tumor growth and angiogenesis, we investigated the role of TF in meningioma. Using immunohistochemical methods, we studied the expression of TF, TFPI, MIB-1 labeling index in 44 meningiomas to determine whether those factors reflect histological grade and proliferative ability. CD31 and CD68 immunostaining was used to assess vascular density and macrophage infiltration, respectively. Additionally we assessed the influence of TF on meningioma cell proliferation by MTT assay. TF was expressed in 1 of 34 (2.9%) benign, 1 of 5 (20%) atypical, and all of 5 anaplastic meningiomas. TFPI was detected in 2 benign (5.9%), 1 atypical (20%), and 3 (60%) anaplastic meningiomas. Both TF and TFPI expression was significantly correlated with the MIB-1 labeling index (LI). However, neither TF nor TFPI showed a correlation with vascular density. The density of tumor-associated macrophages was not correlated with TF or TFPI immunoreactivity. MTT assay revealed that TF increased the proliferation of meningioma cells. Although some macrophages expressed TF, a great number of the TF immunopositive parenchymal cells in the meningiomas were tumor cells. The present study suggest that the TF system reflects the proliferative ability and malignancy of meningiomas.

Germinoma with syncytiotrophoblastic giant cells recurring 13 years after radiotherapy for a pineal germinoma--case report.

A 28-year-old man presented with progressive mental deterioration. Thirteen years earlier he had undergone radiation therapy for a pineal germinoma. Neuroimaging showed a homogeneously enhanced mass lesion along the ventricular wall. The cerebrospinal fluid (CSF) level of human chorionic gonadotropin (HCG) was high. Endoscopic biopsy revealed that the tumor was a two-cell pattern germinoma. The diagnosis was germinoma with syncytiotrophoblastic giant cells (STGC). He first received three cycles of chemotherapy with carboplatin and etoposide and then two cycles with ifosfamide, cisplatin, and etoposide. Subsequently, the tumor size decreased markedly and the CSF level of HCG returned to normal. However, he died 22 months after the recurrence. Histological verification and measurement of tumor markers are necessary for the optimum treatment of germ cell tumors. Adequate initial chemotherapy and long-term follow up are essential for patients with germinoma with STGC.

Huge arachnoid cyst of the posterior fossa with cerebellar tentorium dysplasia associated with juvenile polyposis.

We report an infant with a huge arachnoid cyst of the posterior fossa with dysplasia of the cerebellar tentorium and meningeal sinus and associated juvenile polyposis. Neuroimaging studies disclosed a huge median cystic lesion extending posterosuperiorly over the cerebellum. The cerebellar tentorium was raised to the parietal area; the vermis was normoplastic. Cystography showed no direct communication with the 4th ventricle or subarachnoid space. We discuss the differential diagnosis of median cysts of the posterior fossa and the association of juvenile polyposis.

Tumefactive myelinoclastic diffuse sclerosis--case report.

A 6-year-old boy presented with mental disturbance and progressive left hemiparesis. Magnetic resonance imaging demonstrated large intracranial mass lesions with ring-like enhancement. His neurological condition deteriorated rapidly. Open biopsy via craniotomy was performed under the suspicion of tumor. Histological examination showed massive demyelination and axon preservation, but no tumor cells. The diagnosis was myelinoclastic diffuse sclerosis (MDS). He was treated with high-dose methylprednisolone and improved dramatically. MDS is a rare demyelinating disorder of the central nervous system that affects mainly children and may mimic a brain tumor. MDS must be included in the differential diagnosis in young patients with a brain tumor with atypical radiological appearance.