Results of a long-term follow-up after neuroendoscopic biopsy procedure and third ventriculostomy in patients with intracranial germinomas.

OBJECT: The authors report the results of long-term follow-ups in 12 patients with intracranial germinomas who underwent neuroendoscopic procedures before chemotherapy and radiotherapy, and discuss the usefulness and safety of these procedures. METHODS: Between January 1996 and December 2005 at Kyushu University Hospital, 12 patients with intracranial germinomas underwent neuroendoscopic biopsy procedures involving a flexible fiberscope. Eight patients simultaneously underwent endoscopic third ventriculostomy (ETV) for existing obstructive hydrocephalus. All patients received chemotherapy and radiotherapy postoperatively, according to the regimen promulgated by the Japanese Pediatric Brain Tumor Study Group. The patients were followed for an average of 78.6 months (range 15-134 months), and a retrospective study was conducted. RESULTS: Germinomas were histologically verified in all patients. No postoperative deaths or permanent morbidity was related to the neuroendoscopic procedures. No other cerebrospinal fluid diversion, such as that achieved with a ventriculoperitoneal shunt, was needed for the management of hydrocephalus. A complete response to postoperative chemotherapy and radiotherapy was achieved in all cases. Only one patient had a recurrent lesion in the spinal cord 6 years after the initial treatment; however, this patient had undergone only the neuroendoscopic biopsy procedure without ETV. CONCLUSIONS: Neuroendoscopic procedures can permit a precise histological diagnosis of intracranial germinomas and are safe and effective in the management of hydrocephalus associated with these tumors. The risk of tumor dissemination due to the neuroendoscopic procedures appears to be minimal when the appropriate chemotherapy and radiotherapy are provided postoperatively.

[Clinical features and outcomes in patients with asymptomatic moyamoya disease--from the results of nation-wide questionnaire survey].

After induction of noninvasive diagnostic tools, asymptomatic moyamoya disease is occasionally being diagnosed. However, there is no epidemiological or clinical data concerning this condition because of its rarity. To elucidate clinical features and outcomes in patients with asymptomatic moyamoya disease we conducted a nation-wide questionnaire survey. Questionnaires were sent to 88 neurosurgical institutes in 1994 and we received answers from 66 institutions (75% recovery). Diagnosis of moyamoya disease is based on the guidelines established by the research committee on moyamoya disease of the Ministry of Health and Welfare, Japan. Thirty three asymptomatic moyamoya disease patients were collected (11 male, 22 female) and divided into 2 groups; group 1 defined as patients without any symptoms, and group 2 as patients who did not show any symptoms except headache. 80% of group 1 patients were adults, whereas in group 2, children and adults were equally distributed. Follow-up periods were 3 years and 8 months on average. Among conservatively treated 28 patients, 2 patients died from suspected bleeding (mortality 7%) and 4 patients with TIAs resulted in good outcome. Among surgically treated 5 patients, 1 patient showed poor outcome due to post-operative infarction. Other 26 patients showed excellent outcome. Natural course of asymptomatic moyamoya disease seemed benign, but mortality from bleeding is not negligible. A prospective study of asymptomatic moyamoya disease is necessary to clarify its natural course and appropriate management strategy.

CD9 expression in solid non-neuroepithelial tumors and infiltrative astrocytic tumors.

The tetraspan membrane protein CD9 is normally expressed in the mature myelin sheath and is believed to suppress the metastatic potential of certain human tumors. In this study we identified CD9 in a variety of brain tumors by immunohistochemical (IHC) and immunoblotting analyses. We examined 96 tumor samples and three glioma cell lines in addition to a murine brain tumor model of transplanted glioma cells in CD9-deficient mice and control mice. CD9 was expressed not only in solid non-neuroepithelial tumors but also in infiltrative malignant neuroepithelial tumors. Among the neuroepithelial tumors, high-grade astrocytic tumors, including glioblastomas and anaplastic astrocytomas, showed higher immunoreactivity than low-grade cerebral astrocytomas. Thus, CD9 expression in astrocytic tumors correlated with their malignancy. In the murine brain tumor model, transplanted glioma cells were shown to grow and spread through myelinated areas irrespective of the presence or absence of CD9 expression in the recipient's brain. These results indicate that the CD9 expression of astrocytic tumors plays a significant role in the malignancy independent of CD9 expression in the surrounding tissue. This might be explained by the observation that the CD9 molecule is associated with a mitogenic factor, membrane-anchored heparin-binding epidermal growth factor, which is known to be upregulated in malignant gliomas.

Temporal lobe epilepsy and corpora amylacea in the hippocampus: clinicopathologic correlation.

Corpora amylacea (CoA) have been found in about 60% of neurosurgical specimens showing hippocampal sclerosis (HS). To determine clinical and neuroimaging differences between HS with and without CoA, we studied 29 patients (21 male, 8 female; age at surgery, 12 to 49 years) who underwent anterior temporal lobectomy for intractable medial temporal lobe epilepsy. No CoA were noted in the hippocampus of 11 cases, and deposition of CoA was mild and limited to the subependymal and vestigial hippocampal sulcus regions in nine cases; in nine cases, moderate to marked deposition was noted in the pyramidal cell layer, accompanying severe neuronal loss. No significant differences were evident between these three groups for age at onset, frequency and duration of epileptic seizures, the average age at surgery, or surgical results. Hippocampal hyperintensity in fluid-attenuated inversion recovery magnetic resonance images tended to increase with increasing hippocampal deposition of CoA. Formation of CoA appears to be a response to neuronal loss in the pyramidal cell layer, being related to the epileptogenic process as a consequence rather than a cause.

Classification of medullary venous malformations in the temporal lobe: according to location and drainage pathway.

Medullary venous malformation (MVM) is rare in the temporal lobe, and the radiologic characteristics of temporal MVM have not yet been clarified. In 12 previously reported cases with satisfactory angiographic or magnetic resonance information as well as two newly reported here, we analyzed the specific location and hemodynamics of temporal lobe MVMs, particularly with respect to venous drainage. Temporal lobe MVM typically were seen in the superior lateral portion of the temporal lobe near either the atrium or the inferior horn of the lateral ventricle. Venous drainage was classified into two main patterns: deep (three cases) and superficial (11 cases). Superficial drainage could be divided into two subtypes: lateral and anterior. Dilated deep medullary veins converged toward either the lateral wall of the atrium or the inferior horn of the lateral ventricle. In the deep-drainage type, medullary veins drained into subependymal veins such as the inferior ventricular vein and the lateral atrial vein, and then emptied into the basal vein of Rosenthal. The anastomotic lateral mesencephalic vein was involved in one case as a variant of the basal vein. When the subependymal veins and/or the basal vein of Rosenthal or transverse sinus were hypoplasic, the medullary veins drained into either the Sylvian veins (anterior superficial type) or the vein of Labbé (lateral superficial type) through a characteristic large transcerebral vein. Drainage of temporal lobe MVM can be classified as deep, lateral superficial, or anterior superficial.

Type V phosphodiesterase expression in cerebral arteries with vasospasm after subarachnoid hemorrhage in a canine model.

Cyclic GMP (cGMP) mediates smooth muscle relaxation in the central nervous system. In subarachnoid hemorrhage (SAH), decreases in intrinsic nitric oxide (NO) cause cerebral vasospasms due to the regulation of cGMP formation by NO-mediated pathways. As phosphodiesterase type V (PDE V) selectively hydrolyzes cGMP, we hypothesized that PDE V may function in the initiation of vasospasm. This study sought to identify the altered PDE V expression and activity in the vasospastic artery in a canine SAH model. We also used this system to examine possible therapeutic strategies to prevent vasospasm. Using a canine model of SAH, we induced cerebral vasospasm in the basilar artery (BA). Following angiographic confirmation of vasospasm on day 7, PDE V expression was immunohistochemically identified in smooth muscle cells of the vasospastic BA but not in cells of a control artery. The isolation of PDE enzymes using a sepharose column confirmed increased PDE V activity in the vasospastic artery only through both inhibition studies, using the highly selective PDE V inhibitor, sildenafil citrate, and Western blotting. Preliminary in vivo experiment using an oral PDE V inhibitor at 0.83 mg kg(-1) demonstrated partial relaxation of the spastic BA. PDE V activity was increased from control levels within the BA seven days after SAH. PDE V expression was most prominent in smooth muscle cells following SAH. These results suggest that clinical administration of a PDE V inhibitor may be a useful therapeutic tool in the prevention of vasospasm following SAH.

Occurrence of subdural hematoma and resolution of gait disturbance in a patient treated with shunting for normal pressure hydrocephalus.

A 66-year-old man with gait disturbance was diagnosed with normal pressure hydrocephalus (NPH) and treated with ventriculoperitoneal shunting using a programmable valve. The valve ultimately set at a pressure of 40 mm H(2)O after higher settings no longer relieved symptoms. However, this pressure setting was excessively low and was associated with occurrence of bilateral subdural hematomas. Paradoxically, this event was associated with stable improvement of gait. Our patient's gait disturbance was unassociated with muscle weakness, spasticity, cerebellar ataxia, or Romberg's sign, and, therefore, was consistent with a frontal gait disorder. Cerebral cortical blood flow as measured after shunting by single photon emission computed tomography (SPECT) was slightly increased from the value before shunting, possibly because of intracranial hypotension related to the valve setting. Lasting improvement of gait in our case may be a result of increased blood flow in the supplementary motor area (SMA).

Novel visible-light-induced photocurable tissue adhesive composed of multiply styrene-derivatized gelatin and poly(ethylene glycol) diacrylate.

A novel photocurable tissue adhesive glue, which is composed of styrene-derivatized (styrenated) gelatin, poly(ethylene glycol) diacrylate (PEGDA), and carboxylated camphorquinone in phosphate-buffered saline (PBS), was prepared. The prototype formulation suitable for arterial repair was determined based on the gel yield, degree of swelling, tissue adhesive strength, and breaking (or burst) strength in vitro. The formulated photocurable tissue adhesive glue with an appropriate viscosity was converted to a water-swollen gel within 1 min of visible light irradiation. The tissue adhesive glue, which was coated on a rat abdominal aorta incised with a pair of scissors, was immediately converted to a swollen gel upon subsequent irradiation with visible light, and concomitantly hemostasis was completed. Histological examination showed that the produced gel was tightly adhered to the artery shortly after photoirradiation. The gel gradually degraded with time and was completely absorbed within 4 weeks after treatment. These results indicate that the photocurable glue developed here may serve as a tissue adhesive glue applicable to vascular surgery.

Tumours around the foramen of Monro: clinical and neuroimaging features and their differential diagnosis.

The clinical and neuroimaging features of 20 patients with lateral ventricular tumours located around the foramen of Monro were reviewed retrospectively with special emphasis on the differential diagnoses. Histologic types were: eight neurocytomas, four subependymal giant cell astrocytomas (SGCAs), three subependymomas, two fibrillary astrocytomas, and one each of pilocytic astrocytoma, malignant astrocytoma and malignant teratoma. The mean age of the patients with neurocytoma was 29.6 years, with SGCA 13.3 years and with subependymoma 55.3 years. All tumours appeared nodular in shape, and on computed tomography (CT) neurocytomas were either isodense or highdense with the brain, while all subependymomas and SGCAs were lowdense. Calcification was observed in two SGCAs, and one neurocytoma. Five neurocytomas and all four SGCAs showed mild to moderate contrast enhancement, while all three subependymomas showed either no, or scarce, enhancement. Magnetic resonance imaging (MRI) studies were available in 10 patients, with the signal characteristics of four neurocytomas and three SGCAs being nonspecific, while two subependymomas were both hypointense on T1-weighted images and hyperintense on T2-weighted images. Thus important features for differential diagnosis included age of the patient and density on precontrast CT. In this series, either an extensive excision of the tumour or a partial removal, thus relieving the obstruction of the foramina of Monro, usually provided long term survival, with 18 patients surviving a mean of 10.8 years.

Magnetic resonance imaging in cases with encephalopathy secondary to immunosuppressive agents.

The neurotoxic effects of immunosuppressive agents used after transplantation are well known. In most cases a decrease in drug dosage results in resolution of the neurotoxicity. At early stages in the post-transplantation clinical course, neurotoxicity and other complications such as infectious disease, encephalopathy and seizures are sometimes difficult to diagnose with neuroimaging. Recently, diffusion weighted imaging (DWI) has been used in patients with ischemic disease, mitochondrial myopathy, encephalopathy and demyelinating disease. We examined the magnetic resonance images (MRI), including DWI and fluid attenuated inversion recovery image (FLAIR), in three cases of post-transplantation neurological complication: two cases of neurotoxicity and a case of acute disseminated encephalomyelitis (ADEM). Hyper-intense lesions representing neurotoxicity were seen on FLAIR but not on DWI in two cases with neurotoxicity induced by an immunosuppressive agent. In ADEM, hyper-intense lesions were seen on both FLAIR and DWI. Neurotoxicity due to the immunosuppressive agent showed a favorable outcome, although the hyper-intense lesions temporally presented on FLAIR. In the state after transplantation, hyper-intense lesions on FLAIR and DWI represented in the brain from the initial stage, we might be care of other severe complications but for neurotoxicity.

Gait disturbance in patients with low pressure hydrocephalus.

Using criteria of the classification recently described by Nutt et al., we examined gait disorder in five patients with normal pressure hydrocephalus (NPH). Their cerebrospinal fluid (CSF) pressures were in the normal range, and trials of CSF removal produced temporary improvement of symptoms. Surgical procedures to relieve hydrocephalus improved gait disorders in all patients. No patient showed spasticity, sensory ataxia, cerebellar ataxia, extrapyramidal signs, or limb apraxia. All walked slowly with a wide base and a short stride. The arm swing normally associated with walking was preserved. In standing, patients were unsteady and fell easily when pushed. Four patients showed hesitation in initiating walking and in turning. These clinical features fit Nutt's criteria for frontal gait disorder and frontal disequilibrium. Unlike findings in Parkinson's disease, where similar gait disorders may occur, other extrapyramidal signs, Myerson's sign, and upper limb dysfunction were absent in NPH, and arm swing while walking was preserved. We suspect that ventricular dilatation disturbs neuronal connections between the supplementary motor area and the globus pallidus in NPH patients.

Intradural, extramedullary spinal Ewing's sarcoma in childhood.

We describe an 11 year old girl with progressive paraparesis from a spinal tumour. Magnetic resonance imaging showed an intradural, extramedullary mass extending from the C7 level to T1. Neither osteolytic nor osteosclerotic changes were seen in the vertebral bodies. Extraskeletal Ewing's sarcoma was diagnosed histopathologically.

An infant with an intradural lipoma of the cervical spine extending into the posterior fossa.

BACKGROUND: Intradural lipomas of the cervical spine are very rare. These tumours show no association with spinal dysraphism. We describe an infant with cervical spinal cord lipoma. Surgical decompression of the cord resolved all neurologic deficits. CASE DESCRIPTION: An 8 month old female infant presented with retarded development of motor function in the limbs, in addition to dysphagia. Computed tomography identified a tumour of fat density that extended from the medulla to C7. Magnetic resonance imaging showed hyperintensity on both T1- and T2-weighted images. A fat-suppression sequence demonstrated an area of signal enhancement in the dorsal portion of the tumour following administration of gadolinium. The adjacent spinal cord was normal. Partial removal of the tumour was performed together with decompressive laminoplasty. The enhancing region proved to be fibrous tissue. Motor development resumed in the week following operation. CONCLUSION: Retarded motor development was the main manifestation of this infant's rare spinal tumour. Neuroimaging was of considerable diagnostic value; in particular, fat-suppression magnetic resonance imaging demonstrated details of the tumour and surrounding structures. Decompressive laminoplasty and laminectomy with partial removal of the tumour was effective in reversing clinical deficits.

Decreased thalamic metabolism without thalamic magnetic resonance imaging abnormalities following shearing injury to the substantia nigra.

A 36-year-old man had fallen about 8 metres. Radiographs showed a mandibular fracture, indicating rotatory force applied to the head. Fluid-attenuated inversion recovery (FLAIR) imaging showed hyperintensity in both medial temporal lobes, left medial midbrain, right midbrain including cerebral peduncle, left pulvinar, left external capsule, fornix, splenium of corpus callosum, and deep white matter of both frontal lobes. Quantitative [(18)F]fluorodeoxyglucose (FDG)-positron emission tomography (PET) indicated markedly suppressed glucose metabolism in the left thalamus but not markedly in the striatum. At that time the neurologic examination demonstrated complete left hemiparesis, severe rigidity of the right upper extremity, and inability to move the right hand and fingers. Levodopa at 300-600 mg/day improved movement of the fingers, decreased the rigidity in the extremity, and lessened the metabolic abnormality. Diminished metabolism in the left thalamus may have contributed to symptoms. The case illustrates the usefulness of PET in disclosing symptom causing abnormalities not detected by magnetic resonance imaging.

[A case of traumatic sinus pericranii].

Sinus pericranii is a rare vascular anomaly involving an abnormal communication between the extracranial and intracranial circulations. A 33-year-old woman presented with a soft tissue mass at the left frontal region. It was associated with head trauma when she was 12-year-old. The mass had gradually grown and become painful for 20 years. She underwent surgical resection of the mass successfully. This report discusses traumatic sinus pericranii.

[A case of pineoblastoma primary presenting a pineal hemorrhage causing obstructive hydrocephalus].

A 9-year-old female suddenly developed headache and nausea. Computed tomography (CT) revealed pineal mass lesion and obstructive hydrocephalus. One week after the onset, hydrocephalus spontaneously resolved. Magnetic resonance imaging (MRI) revealed that the mass was a subacute hematoma and that hydrocephalus had improved. Sequential MRI 4 months later revealed an enhanced lesion in the pineal region. Total removal of the tumor by the occipital transtentorial approach established the histopathological diagnosis of pineoblastoma.

[A lesion diagnosed by MRI in a case of transient global amnesia].

Transient global amnesia(TGA) had been recognized as a disease without abnormal findings on neuroimaging before magnetic resonance(MRI) imaging was practicable. Recently, abnormal findings on MRI reported in cases of TGA. We here reported a case of TGA showing a transient abnormal intensity in left hippocampus on MRI diffusion-weighted image. A 52-year-old man suddenly became to unable to keep his recent memory without histories of trauma or epilepsy. He showed no abnormal neurologic findings excepting for the recent memory. MRI performed within an hour after onset demonstrated no abnormality on T1- and T2-weighted images. Diffusion-weighted image (b = 1,000) showed hyperintensity in the left medial temporal lobe, and the apparent diffusion coefficient(ADC) in this region was lower(72.8 cm2/s) than that in the contralateral region(94.4 cm2/s). TGA was completely resolved 17 hours after onset.

[Severe hyponatremia in a case of severe spinal injury: sequential examination of factors affecting water-electrolyte balance].

Although hyponatremia has been known to occur in patients with severe spinal cord injury with highly incidence, its mechanism has not been understood well. We examined a 64-year-old patient with severe hyponatremia after spinal cord injury by sequential measuring of the factors affecting water-electrolyte balance, such as antidiuretic hormone, renin, angiotensin II, atrial natriuretic peptide, and brain natriuretic peptide. The patient showed severe hypotension due to dysfunction of the sympathetic nerve. The hyponatremia gradually resolved with the improvement of sympathetic nerve function. According to those results, the sympathetic nerve dysfunction was thought to correlate with the hyponatremia, and it was suggested that the unknown sympathetic regulation of water-electrolyte balance existed.

Unilaterally symptomatic moyamoya disease in children: long-term follow-up of 20 patients.

OBJECTIVE: In unilaterally symptomatic moyamoya disease in children, it remains controversial whether bypass surgery should be performed on the asymptomatic side along with on the symptomatic side. We aimed to verify the validity of our strategy of only performing bypass surgery on the symptomatic side. METHODS: Among 91 pediatric patients with moyamoya disease who underwent bypass surgery in our department between 1980 and 2004, 20 with unilateral ischemic symptoms who were followed for more than 60 months were analyzed in the present study. Initially, we only performed bypass surgery on the symptomatic side for all 20 patients. Among these 20 patients, five developed frequent transient ischemic attacks in the initially asymptomatic side and underwent a second bypass surgery on that side (Group A), eight developed sporadic transient ischemic attacks and were followed up without surgery (Group B), and seven did not experience any ischemic symptoms on the asymptomatic side (Group C). RESULTS: In total, 18 patients progressed well without cerebral infarctions after their last surgery, although some showed deterioration of angiographic stenosis and a transient decrease in the regional cerebral blood flow or cerebral perfusion reserve. One patient in Group A had an intraventricular hemorrhage 5 years after the second operation, and one in Group B had a minor stroke on the initially asymptomatic side. CONCLUSION: In unilaterally symptomatic moyamoya disease, bypass surgery for the asymptomatic side can be delayed until the development of ischemic symptoms, such as frequent transient ischemic attacks.

Ectopic bone formation facilitated by human mesenchymal stem cells and osteogenic cytokines via nutrient vessel injection in a nude rat model.

In vivo studies using bone marrow-derived mesenchymal stem cells are still uncommon. Applications for bone defect replacement in undesirable clinical circumstances such as large defects, bacterial or other pathogen-contaminated fields, and irradiated surgical wound bed necessitate vascularized bone regeneration. Use of a fascial flap including regenerated bone would be a very powerful tool for treatment. It would be especially beneficial in cases where normal bone regeneration is not expected due to a lack of sufficient blood supply, extensive surgical scarring, or bacterial contamination. In this study, we used nude rats in which the superficial epigastric flap of the experimental group was used to wrap around a mixture of human mesenchymal stem cells, bone morphogenetic protein-2, and basic fibroblast growth factor cytokines in a gelatin carrier. These rats showed significantly higher bone mineral density at 4 weeks compared to the other experimental groups containing phosphate buffered saline, human mesenchymal stem cells alone, or the two cytokines alone (p < 0.01). There were no remarkable histologic differences up to 7 days. At 2 weeks, more progressive vascularity and perivascular tissue deposits were seen in the experimental group. Basophilic mineral structure surrounded the fibroblast-like mesenchymal stem cells at 4 weeks, presumably osteoblastic or osteoclastic cell lining. Bone marker immunohistochemistry against alkaline phosphatase and osteocalcin revealed diffuse and distinct immunoreactivity in osteoblastic cells in the experimental group at 4 weeks. Further transcriptional expression of polyomavirus enhancer binding protein 2alphaA suggested that the human transplanted cells proceeded to osteogenic lineage in 4 weeks. These results may be useful as a new approach for bone regeneration.