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Background and importance We report the case of a patient with multiple neuroepithelial tumors of different histological cell types. In this patient, synchronous appearance of tumors of the central nervous system, ie, cervical ependymoma and a fourth ventricle choroid plexus papilloma, was noted. Clinical presentation A 36-year-old man presented with sensory disturbance of the bilateral upper extremities. Magnetic resonance imaging revealed a heterogeneously enhanced mass with syringomyelia in the upper cervical cord and a well-enhanced mass in the fourth ventricle. The patient underwent bilateral suboccipital craniotomy and laminectomy from the level of C1 to C3 for both lesions. The lesions were grossly resected. Histological and immunohistochemical examinations revealed that the cervical tumor was an ependymoma, and the fourth ventricle tumor was a choroid plexus papilloma. Conclusion The appearance of synchronous multiple neuroepithelial tumors of different histological cell types is extremely rare, and this is the first known case of ependymoma occurring synchronously with choroid plexus papilloma, both tumors having different histological cell types. Displacement and maldifferentiation of primitive, multipotent neuroepithelial cells may be a causative factor for this unique presentation.

BACKGROUND: Although the treatment of intracranial aneurysms with detachable coils is now widely accepted, the problem of coil compaction and recanalization remains to be solved. OBJECTIVE: To prevent recanalization by inducing intra-aneurysmal organization through prepared platinum coils coated with a novel cyclic peptide, SEK-1005, which can accelerate wound healing. METHODS: Using a rat aneurysm model, we examined the tissue response to these coils. An SEK-1005-coated coil (SC) or unmodified coil was inserted into the ligated external carotid artery (ECA) sac of rats. The sacs were removed on day 14 or 42 after coil insertion and subjected to conventional and immunohistochemical examination. We evaluated the tissue response in the ECA sacs and compared the percentage of organized areas in the ECA sacs of rats with SCs and unmodified coils. RESULTS: In SC rats, tissue organization was accelerated and the proliferation of α-smooth muscle actin- and vimentin-positive cells was promoted. On days 14 and 42, tissue organization was significantly greater in the ECA sacs of rats with SCs. CONCLUSION: SCs accelerated intra-aneurysmal organization in our rat aneurysm model suggesting that platinum coils coated with the novel cyclic peptide SEK-1005 may prevent recanalization and improve the clinical outcome in patients treated by coil embolization.

OBJECTIVE:To investigate the characteristic clinical behavior and develop guidelines for the clinical management of posterior cerebral artery aneurysms, we reviewed 21 cases treated during a 15-year period.METHODS:Between 1988 and 2002, we treated 21 patients (10 male, 11 female; mean age, 49.8 yr) with posterior cerebral artery aneurysms at Kumamoto University Hospital and its affiliated hospitals. Data relating to the clinical, radiological, and surgical approaches were analyzed.RESULTS:There were 20 saccular aneurysms, 6 of which were giant or large aneurysms, and 1 dissecting aneurysm. Of the 21 patients, 15 presented with subarachnoid hemorrhage; in 3 patients, an unruptured saccular aneurysm was found incidentally during an evaluation for other cerebral pathological conditions. Two patients presented with progressive homonymous hemianopsia because of the mass effect of the aneurysm, and 1 patient experienced the sudden onset of homonymous hemianopsia because of thrombosis of the aneurysm and afferent artery. Open or endovascular surgery was performed in 19 patients: 14 made a good recovery, 2 had a moderate disability because of angiospasm or infarction after aneurysm trapping, 1 had a severe disability because of angiospasm and cerebral contusion, and 2 died because of severe angiospasm. Of 2 conservatively treated patients, 1 made a good recovery but the other died as a result of rebleeding.CONCLUSION:Posterior cerebral artery aneurysms have specific clinical characteristics compared with aneurysms located elsewhere. The aneurysmal site and size and the surgical technique did not significantly influence treatment outcomes.

OBJECTIVE AND IMPORTANCE: Spinal arachnoid cysts are rare, and their cause and pathogenesis remain controversial. We experienced a rare case with a large congenital melanocytic nevus in which a spinal arachnoid cyst contained nevus cells, suggesting the congenital nature of a spinal arachnoid cyst. CLINICAL PRESENTATION: A 37-year-old Japanese man had been born with a large melanocytic nevus on his back. He experienced intermittent pain radiating to both thighs and to the lower back and waist. A magnetic resonance imaging study disclosed the presence of a posterior intradural extramedullary arachnoid cyst extending from T10 through T12. His spinal cord was displaced anteriorly and flattened. INTERVENTION: An osteoplastic laminoplasty was performed, and the arachnoid cyst was totally removed. The cyst membrane exhibited many foci of brown deposits, and histological examination disclosed the presence of melanin-containing cells in the cyst membrane. Morphologically and immunohistochemically, the melanin-containing cells in the cyst membrane were similar to nevus cells in the dermis. CONCLUSION: The histological findings of our case suggest that the patient's spinal arachnoid cyst was formed at the same stage of development as the melanocytic nevus.

Objective We analyzed the community incidence and early prognosis of first-ever aneurysmal subarachnoid hemorrhages (SAHs) to obtain the same prognostic information used by physicians and families in a defined geographic area in Japan. Methods During the 5-year period from January 1, 1996, to December 31, 2000, 2115 patients were registered in the Data Bank for Cerebral Aneurysms of Kumamoto Prefecture, a defined area in Japan with a population of 1.86 million. Registration was based on a comprehensive referral system for SAH patients; computed tomographic scans were available for all patients, including those who were moribund or dead on arrival. We compared the treatment outcomes at 3 months after the first-ever SAH in surgically and conservatively treated patients. Results The age-adjusted annual incidence of SAH for men, women, and both sexes was 15.9, 26.6, and 21.6 per 100,000 person-years, respectively. In men, the incidence reached a plateau after age 40 years. In women, conversely, it rose after age 40 and peaked in the 9th decade of life. Of the 1634 surgically treated patients, 1153 (70.6%) had a favorable outcome; this was true for only 27 of 477 (5.7%) conservatively treated patients. Four patients were lost to follow-up. The clinical outcome did not differ between patients treated by open surgery and those treated endovascularly. Approximately 30% of our patients were older than 70 years at the time they experienced their first SAH. Conclusion The incidence of SAH obtained in this study was similar to earlier Japanese reports and Finnish studies. The importance of managing elderly patients with cerebral aneurysms will continue to increase.

OBJECTIVE AND IMPORTANCE Hyperperfusion has been reported after carotid endarterectomy or stenting for stenosis of the internal carotid artery. Because few reports have examined postoperative hyperperfusion after treatment for dural arteriovenous fistulae (DAVFs), we present a case describing a patient who manifested this clinical entity. CLINICAL PRESENTATION The patient was a 63-year-old man with a DAVF in the left transverse sigmoid sinus with retrograde leptomeningeal venous drainage. He experienced slowly progressive disorientation lasting for several months. Preoperative single-photon emission computed tomography with 123I-labeled N-isopropyl-p-iodoamphetamine revealed an area of hyperintensity on T2-weighted magnetic resonance imaging (MRI) scans that coincided with the hypoperfusion area; it was not increased after acetazolamide challenge. Complete DAVF obliteration was achieved by embolization, then sinus isolation. After treatment, he experienced frequent generalized convulsions that were terminated by 2-day barbiturate therapy. INTERVENTION On T2-weighted MRI scans obtained 3 days after surgery, the hyperintense area not only persisted but had expanded to the left parietal lobe. Moreover, a subcortical hyperintense lesion was recognized on T1-weighted MRI scans; this was considered to reflect cortical laminar necrosis. Single-photon emission computed tomography revealed hyperperfusion in the left parietal lobe; it changed to hypoperfusion a month after treatment. CONCLUSION In patients with DAVFs with preoperative findings of marked low perfusion and a poor perfusion reserve, postoperative study may reveal hyperperfusion on single-photon emission computed tomography or cortical laminar necrosis on MRI. This may be evidence of severe perfusion disturbance as a result of venous infarction. In these patients, careful blood pressure control and early treatment of seizures are important after DAVF treatment.

The prevention of rebleeding is one of the most important issues in the successful treatment of moyamoya disease with hemorrhagic onset. However, the natural course of hemorrhagic moyamoya disease and the characteristics of rebleeding remain unclear.To study the natural course of the disease in conservatively treated patients, we analyzed long-term follow-up reports on 36 patients (12 male and 24 female) treated without bypass surgery at our hospitals before 1994 (mean follow-up, 12.7 +/- 7.1 yr; range, 2.9-27.0 yr).Of our 36 patients, 22 (61.1%) experienced rebleeding; there were 29 rebleeding episodes. The outcomes in patients with rebleeding were worse than in patients who did not rebleed (P0.05); in most cases, unsatisfactory results were attributable to rebleeding. Kaplan-Meier and multivariate analysis of rebleeding-free survival showed that age at onset (36 yr) was a statistically significant risk factor; sex, hypertension, type of intracranial bleeding, and outcome after the first bleeding episode were not. Although the intervals between the first and subsequent episodes varied, both the rebleeding rate and the number of rebleeding episodes were highest in patients 46 to 55 years old.Rebleeding is the most important factor in unsatisfactory outcomes of patients with hemorrhagic moyamoya disease. Rebleeding seems to be age-related; rebleeding occurs at an increased rate when patients reach the age range of 46 to 55 years. Patients with hemorrhagic moyamoya must be followed up throughout their lives, even if their neurological status is excellent.

WARRIORS THROUGHOUT THE AGES have considered the unprotected head to be particularly vulnerable. The traditional Japanese helmet, the Kabuto, was the part of a protective suit of armor that reflected the wearer's character and personality. Over time, it changed from a primarily protective device to a vehicle for the expression of a distinctly Japanese sense of magnificent artistry. Each Kabuto was custom-made, and these helmets remain without peer in the world. They were designed and crafted to answer the demands of their era, and thus they provide historical evidence not only of the state of Japanese warfare, but also of social organization, metallurgical knowledge, artisanship, and aesthetic sensibility. Even now, during the national Golden Week Festival in early May, Kabuto are displayed in the alcoves of Japanese homes as a petition that the boys in the house be granted courage and good health. Thus, even in modern Japan, the Kabuto represents a talisman. We present an overview of the evolution of the Kabuto, with special reference to the Japanese sword and the warrior tradition.

OBJECTIVE AND IMPORTANCE We present two interesting cases involving carotid-cavernous dural fistulae draining only or predominantly into the petrosal vein after previous incomplete, complicated, endovascular treatments. Transvenous embolization with Guglielmi detachable coils, via the petrosal vein, during surgical exposure completely obliterated the fistulae. CLINICAL PRESENTATION A 64-year-old man manifesting left ocular symptoms after incomplete embolization of a left carotid-cavernous dural fistula and a 56-year-old woman manifesting left hemiparesis after complicated embolization of a right carotid-cavernous dural fistula were referred to our hospital. A percutaneous transvenous approach was attempted in both cases, but the catheter could not reach the fistula site. A combined open surgical and endovascular approach was then used. INTERVENTION The hemispheric branch of the petrosal vein was exposed via a retromastoid craniectomy. The catheter was then directly introduced into the hemispheric branch, followed by navigation into the fistula site. The fistula was completely embolized with Guglielmi detachable coils. CONCLUSION The technique of surgical transvenous embolization via a petrosal vein is a valuable alternative for the treatment of carotid-cavernous dural fistulae that drain only or predominantly into the petrosal vein, when the percutaneous transvenous route is not accessible.

Background The natural course of unruptured vertebral artery dissecting aneurysms (VADAs) remains unclear. Objective The purpose of this retrospective study was to develop a strategy for treating unruptured VADAs based on long-term follow-up. Methods Our study population consisted of 100 patients with unruptured VADAs; in 66, the initial symptom was headache only, 30 presented with ischemic symptoms and 4 with mass effect. All underwent magnetic resonance imaging and magnetic resonance angiography at the time of admission and 2 weeks and 1, 3, 6, 12, and 24 months after the onset. If the dissection site was demonstrated to be enlarged on magnetic resonance imaging and magnetic resonance angiography without the manifestation of new symptoms, the patients received additional treatment to prevent bleeding. Results Of the 100 patients, 4 underwent early intervention because of symptom exacerbation. The other 96 were initially treated conservatively; during follow-up, 5 manifested lesion enlargement on magnetic resonance angiography. Nine patients received additional treatment; 1 underwent direct surgery with trapping of the dissection site, and 8 underwent coil embolization. The other 91 patients continued to be treated conservatively; the dissection site remained unchanged in 70, improved or healed in 18, and disappeared in 3 patients. We treated 38 patients with recurrent ischemic attacks with antiplatelet therapy. No patients experienced bleeding or permanent neurological deficits during follow-up. Conclusion The nature of an unruptured VADA is not highly aggressive. However, if the dissection site enlarges without the manifestation of new symptoms, it should be occluded. In patients with recurrent ischemic attacks antiplatelet therapy should be considered.

To evaluate the usefulness of measuring lumbar intraspinal epidural pressure (ISEDP) measurements for the estimation of intracranial pressure, we studied the relationship between ISEDP and intracranial epidural pressure (ICEDP) in 12 patients with high intracranial pressure after neurosurgical procedures. ISEDP was measured with a Gaeltec catheter-tip pressure transducer placed percutaneously in the lumbar epidural space with a Touhy needle. ICEDP was determined by the conventional method. During the measurement, some manipulations were carried out. ISEDP and ICEDP measurements exhibited a linear correlation. In all but one patient with normal cerebrospinal fluid, ISEDP was 84 to 100% of ICEDP. In patients with mild subarachnoid hemorrhage, ISEDP was 82 to 86% of ICEDP. In patients with severe subarachnoid hemorrhage, ISEDP was 45 to 57% of ICEDP and always fluctuated in parallel with ICEDP. ISEDP accurately reflected ICEDP in response to manipulations such as breath holding, neck compression, compression at the cranial defect, mannitol administration, and coughing. These data suggest that ISEDP measurement is useful in monitoring intracranial pressure in patients with intracranial hypertension. In addition, the measurements can be obtained easily and safely.

OBJECTIVE: External manual carotid compression is a noninvasive method to treat cavernous sinus (CS) dural arteriovenous fistulae (DAVF). We studied a group of patients with CS-DAVF to identify what factors made complete resolution of their clinical symptoms and closure of the DAVF on magnetic resonance angiography (MRA) by compression therapy possible. METHODS: We treated 23 patients with CS-DAVF without cortical venous drainage or a recent decline in visual acuity by compression therapy. All were followed up by magnetic resonance angiography at 1, 3, 6, and 12 months after treatment and the characteristics of the imaging findings, their neurological symptoms, and the patterns of symptom improvement were examined. RESULTS: In Group A (n = 8), complete resolution was achieved by manual carotid compression. In the other 15 patients (Group B), complete resolution was not obtained. Group B manifested significantly higher ocular pressure and a significantly longer interval between symptom onset and compression treatment. In Group A, venous drainage was via the superior orbital vein with or without involvement of the inferior petrosal sinus. Closure of the CS-DAVF occurred within 4.1 months after the start of treatment. In three patients, symptom improvement progressed steadily and gradually. The other five patients with complete resolution experienced transient worsening of their symptoms at 2 to 4 months after the start of treatment and symptom resolution occurred within 4 to 7 months. CONCLUSION: We identified lower ocular pressure, a shorter interval between symptom onset and compression treatment, and venous drainage solely via the superior orbital vein without involvement of the inferior petrosal sinus as the factors in our CS-DAVF patients that made complete resolution by manual carotid compression possible.

OBJECTIVE: Endothelial tight junctions form the main barrier of the blood-brain barrier (BBB). In human hemangioblastomas, cyst formation is a common and important clinical manifestation. Although most researchers consider that the cyst formation in hemangioblastomas may be caused by the breakdown of the BBB, the underlying molecular mechanisms for cyst formation remain unknown. At present, there are few reports about the change of tight junctions in microvessel endothelium of human hemangioblastomas. The purpose of this research is to investigate the change of tight junction and its major molecular components in microvessel endothelium of human hemangioblastomas. METHODS: Twenty-four consecutive patients with cerebellar hemangioblastomas were studied. Tight junctions in the microvessels of hemangioblastomas and the control brain were examined by electron microscopy. Immunohistochemistry and double immunofluorescent microscopy were used to analyze the expression of CLN5 and its relationship with astrocytic endfeet in the control brain and hemangioblastomas. Quantitative real-time reverse-transcriptase polymerase chain reaction and Western blots were used to investigate the expression level of CLN5 in hemangioblastomas. Triple immunofluorescent microscopy was used to analyze the coexpression of vascular endothelial growth factor, vascular endothelial growth factor-R1, and placenta growth factor on microvessels of hemangioblastomas. Clinical and experimental data were correlated and analyzed by the one-way analysis of variance, Kruskal-Wallis test, and Spearman rank correlation test. RESULTS: In the control brain, the paracellular cleft between adjacent endothelial cells is sealed by continuous strands of tight junctions. In cystic hemangioblastomas, a significant paracellular cleft could be found between adjacent endothelial cells. Some endothelial cells were connected with adherens junction and no tight junction was found between them. Compared with the control brain, expression of CLN5 was decreased in cystic hemangioblastomas (P < 0.05). Phosphorylated CLN5 was detected in most hemangioblastomas, but not in the control brain. Microvessels in hemangioblastomas showed a significant absence of astrocytic endfeet. Coexpression of vascular endothelial growth factor, vascular endothelial growth factor-R1, and placenta growth factor was detected in the endothelial cells. The Spearman rank correlation test showed a significant correlation between a greater degree of CLN5 expression and less morphological cystic formation in these patients studied (correlation coefficient = −0.520; P = 0.009). CONCLUSION: The continuity of tight junctions of the BBB is interrupted in human cerebellar hemangioblastomas. Significant absence of astrocytic endfeet and tight junctions can be found in microvessels of hemangioblastomas, which may lead to the breakdown of the BBB in these tumors. These findings suggest that the absence of tight junctions might play a role in cyst formation of hemangioblastomas.

OBJECTIVE We performed a retrospective study to assess long-term outcomes in very old patients with ruptured intracranial aneurysms. The aim of the study was to develop guidelines for choosing surgical or conservative treatment with the goal of maintaining patients' quality of life. METHODS Between 1996 and 1999, 76 consecutive patients, aged 80 to 89 years, with ruptured intracranial aneurysms were treated at 18 participating centers. They were divided retrospectively into operated and nonoperated groups, and their preoperative characteristics and treatment outcomes were analyzed. Their Glasgow Outcome Scale and Barthel Index scores at least 2 years after the insult were recorded and analyzed from the perspective of their admission Hunt and Hess grade. RESULTS Our retrospective review encompassed 5.9 years. Of the original 76 patients, 54 (71.1%) died, 13 (17.1%) had a good outcome (Barthel Index ≥60), and 9 (11.8%) had a poor outcome (Barthel Index CONCLUSION Our results suggest that patients in the 9th decade of life with ruptured intracranial aneurysms can be considered for surgical treatment if they have a Hunt and Hess grade of I or II. The highest rate of favorable results was achieved in patients with small internal carotid artery-posterior communicating artery aneurysms.

OBJECTIVE AND IMPORTANCEReports of idiopathic hypertrophic cranial pachymeningitis have increased as a result of advances in magnetic resonance imaging. This is the first documented case of idiopathic hypertrophic cranial pachymeningitis associated with a dural arteriovenous fistula involving the straight sinus. We discuss possible causes of the association and the treatment options.CLINICAL PRESENTATIONA 64-year-old man presented with a headache and visual disturbance. Gadolinium-enhanced T1-weighted magnetic resonance imaging demonstrated homogeneously stained meninges and prominent enhancement of the tentorium and falx. Angiograms demonstrated a dural arteriovenous fistula of the straight sinus.INTERVENTIONAlthough surgical excision of the straight sinus and subsequent corticosteroid therapy failed to relieve the patient's visual symptoms, subsequent surgical decompression of the optic nerve resulted in improvement and stabilization.CONCLUSIONNarrowing or occlusion of the tentorial sinuses and narrowing of the straight sinus by extensive dural fibrosis of the tentorium and falx, attributable in turn to idiopathic hypertrophic cranial pachymeningitis, may have resulted in the development of a dural arteriovenous fistula. We propose surgical decompression of the optic nerve as an alternative treatment during the active phase of the disease in patients who exhibit resistance to corticosteroid therapy.

We report a 14-year-old girl who had been diagnosed incidentally as having a cerebral arteriovenous malformation (AVM) in the left thalamus. The AVM disappeared spontaneously without treatment. Follow-up angiography performed 6 years after the original angiograms confirmed the complete disappearance of the AVM. Some possible mechanisms giving rise to the thrombosis of an AVM are discussed.