Ruptured vertebral artery-posterior inferior cerebellar artery aneurysm associated with pulseless disease--case report.

A 48-year-old woman with a 29-year history of pulseless disease presented with subarachnoid hemorrhage caused by a rare ruptured intracranial aneurysm of the right vertebral-posterior inferior cerebellar artery. The aneurysm was successfully clipped. Pulseless disease is a chronic and nonspecific vasculitis involving mainly the aorta and its main branches such as the carotid, subclavian, vertebral, and coronary arteries. The cervical vessels are mostly affected in Japanese patients. Almost all patients have ischemic disorders of the involved vessels, manifesting as syncope, visual disturbance, or a faint or absent pulse. The mechanisms responsible for pulseless disease and intracranial aneurysms are likely to be different.

Long-term outcome of unruptured cerebral aneurysms.

BACKGROUND: The frequency at which unruptured cerebral aneurysms are detected has increased due to advances in low- or non-invasive diagnostic techniques. Despite the recent improvements in surgical and medical management of aneurysmal subarachnoid hemorrhages, however, the overall case-fatality rate of this disease is still high. To reduce it, the natural history of unruptured cerebral aneurysms should be better understood. METHODS: The subjects consisted of 156 patients with unruptured cerebral aneurysms who had been admitted to the Department of Surgical Neurology, Jichi Medical School Hospital or Jichi Medical School Ohmiya Medical Center, Japan, between January 1989 and December 1998. All of the patients were classified according to the process by which aneurysms had been detected. The expected number of deaths was calculated by using age- and sex-specific mortality rates obtained from the vital statistics. A standardized mortality ratio and 95% confidence interval were calculated by using the expected and observed numbers of deaths. RESULTS: The standardized mortality ratios differed among the groups according to the process of detecting aneurysms. The incidence rate of rupture of unruptured cerebral aneurysms was 1.3 per 100 person-years. The annual rupture rate of unruptured cerebral aneurysms was higher than previously reported. The survival rate of the group that underwent surgical treatment for unruptured cerebral aneurysms was higher than that of the untreated group. CONCLUSIONS: The annual rupture rate of unruptured cerebral aneurysms may be higher than previously reported.

Recurrent meningitis associated with complete Currarino triad in an adult--case report.

A 58-year-old woman presented with Currarino triad manifesting as recurrent meningitis. Currarino triad is a combination of a presacral mass, a congenital sacral bony abnormality, and an anorectal malformation, which is caused by dorsal-ventral patterning defects during embryonic development. She had a history of treatment for anal stenosis in her childhood. Radiographic examinations demonstrated the characteristic findings of Currarino triad and a complicated mass lesion. The diagnosis was recurrent meningitis related to the anterior sacral meningocele. Neck ligation of the meningocele was performed via a posterior transsacral approach after treatment with antibiotics. At surgery, an epidermoid cyst was observed inside the meningocele. The cyst content was aspirated. She suffered no further episodes of meningitis. The meningitis was probably part of the clinical course of Currarino triad. Radiography of the sacrum and magnetic resonance imaging are recommended for patients with meningitis of unknown origin. The early diagnosis and treatment of this condition are important.

The effect of ST2 gene product on anchorage-independent growth of a glioblastoma cell line, T98G.

The ST2 gene, which is specifically induced by growth stimulation in fibroblasts, encodes interleukin-1 receptor-related proteins and is widely expressed in hematopoietic, helper T, and various cancer cells. However, the physiological as well as pathological functions of the ST2 gene products are not yet fully understood. In this study, we analyzed the expression of the ST2 gene in human glioma cell lines and human brain tumor samples with real-time polymerase chain reaction method, the results of which revealed that the expression level of the ST2 gene in glioma cell lines and glioblastoma samples is significantly lower than that in a fibroblastic cell line, TM12, and benign brain tumors, suggesting the reverse relationship between malignancy and ST2 expression. As we could not detect the soluble ST2 protein in the culture fluid of the T98G glioblastic cell line by ELISA, we established stable transformants of T98G that continuously produce and secrete the ST2 protein, in order to study the effect of the ST2 protein on malignancy. Although we could not detect a remarkable difference in proliferation between transformants and control cells in conventional tissue culture dishes, the efficiency of colony formation in soft agar was significantly decreased in the case of cells that continuously produce the ST2 protein. Furthermore, inhibition of colony formation in soft agar was observed in wild-type T98G cells when purified soluble ST2 protein was added to the culture, in a dose-dependent manner. Taken together, the results suggest that the expression of ST2 suppressed the anchorage-independent growth and malignancy.

Intracranial hemangiopericytoma with extracranial metastasis occurring after 22 years.

A 65-year-old man developed pancreatic and pubic tumors 22 years after craniotomy for a left sphenoid ridge tumor. The histological finding of the primary sphenoid ridge tumor was meningotheliomatous meningioma. The histological appearance of the biopsy specimen of the pubic tumor was hemangiopericytoma. The recurrent intracranial tumor was treated by radiosurgery, and the metastatic tumors were treated by conventional irradiation. Intracranial tumors rarely metastasize outside the central nervous system, except for meningeal hemangiopericytomas. This case indicates that meningeal hemangiopericytoma may metastasize many years after the initial onset and requires long-term follow up.

[Observation of statistics of screening for unruptured cerebral aneurysms in Tochigi prefecture].

Screening for unruptured cerebral aneurysms (UCAs) using magnetic resonance imaging (MRI) and angiography (MRA) is prevalent in Japan. To reveal the prevalence of UCAs found during screening, we collected data of the results in 1999, in Tochigi prefecture. In the prefecture, of which the population was about 2 million, 26 institutions had been established in 1999, and 5,222 persons had been screened. These corresponded to 0.26% of all inhabitants of Tochigi prefecture. Of the 26 institutions, 24 cooperated in this study, and data was collected for 4,961 persons. We investigated the prevalence of UCAs, and compared it with that of subarachnoid hemorrhage (SAH) in Japan using the existing statistics. The UCAs were found in 143 (2.9%) of the 4,961 cases, 69 men and 74 women, with a mean age of 59.2 years. The prevalence of UCAs at screening and the prevalence of SAH in Japan co-relate in that this prevalence increases with age in both UCAs and SAH. However, after the age of 75, the provalence of SAH decreases. People found with UCAs at screening were mainly in their 50's, but the member of those found with SAH increased gradually after that age. The rate of screening of women was lower than that of men, although both the prevalence of UCAs at screening and SAH of women is higher than that of men. We recommend that middle-aged persons, in their 40's and older, should request screening for UCAs.

Differential effects of novel wasp toxin on rat hippocampal interneurons.

We studied the effects of a wasp toxin beta-pompilidotoxin (beta-PMTX) on rat hippocampal CA1 interneurons by the current-clamp technique. The firing patterns of pyramidal neurons and pyramidale interneurons were not affected by beta-PMTX, but in oriens and radiatum interneurons, beta-PMTX converted the action potentials to prolonged depolarizing potentials by slowing the inactivation of Na(+) channels. In lacunosum moleculare interneurons, beta-PMTX induced initial bursting spikes followed by block of succeeding spikes. Comparison of beta-PMTX with a sea anemone toxin, ATX II, revealed that ATX II altered the firing properties of pyramidal neurons and pyramidale interneurons that were unchanged by beta-PMTX. Our results suggest that beta-PMTX modulates Na(+) currents in CA1 interneurons differently in various CA1 neurons and the toxin is useful to classify Na(+) channel subtypes.

Disturbance of hippocampal long-term potentiation after transient ischemia in GFAP deficient mice.

GFAP (glial fibrillary acidic protein) is an intermediate filament protein found exclusively in the astrocytes of the central nervous system. We studied the role of GFAP in the neuronal degeneration in the hippocampus after transient ischemia using knockout mice. Wild-type C57 Black/6 (GFAP(+/+)) mice and mutant (GFAP(-/-)) mice were subjected to occlusion of both carotid arteries for 5-15 min. Hippocampal slices were prepared 3 days after reperfusion and the field excitatory postsynaptic potentials (fEPSP) in the CA1 were recorded. High frequency stimulation induced robust long-term potentiation (LTP) in GFAP(-/-), as in GFAP(+/+) mice. After ischemia, however, the LTP in GFAP(-/-) was significantly depressed. Similarly, paired pulse facilitation (PPF) displayed little difference between GFAP(+/+) and GFAP(-/-), but after ischemia, the PPF in GFAP(-/-) showed a depression. Histological study revealed that loss of CA1 and CA3 pyramidal neurons after ischemia was marked in GFAP(-/-). MAP2 (dendritic) immunostaining in the post-ischemic hippocampus showed little difference but NF200 (axonal) immunoreactivity was reduced in GFAP(-/-). S100beta (glial) immunoreactivity was similar in the post-ischemic hippocampus of the GFAP(+/+) and GFAP(-/-), indicating that reactive astrocytosis did not require GFAP. Our results suggest that GFAP has an important role in astrocyte-neural interactions and that ischemic insult impairs LTP and accelerates neuronal death.

Leptomeningeal dissemination of pilocytic astrocytoma via hematoma in a child. Case report.

A case of recurrent pilocytic astrocytoma with leptomeningeal dissemination (LMD) is described. A cerebellar tumor was diagnosed in a 3-year-old boy, in whom resection was performed. When the boy was 6 years of age, recurrence was treated with surgery and local radiotherapy. At age 13 years, scoliosis was present, but the patient was asymptomatic. Twelve years after initial surgery LMD was demonstrated in the lumbar spinal region without recurrence of the original tumor. This tumor also was subtotally removed. During the procedure, a hematoma was observed adjacent to the tumor, but the border was clear. Histological examination of the spinal cord tumor showed features similar to those of the original tumor. There were no tumor cells in the hematoma. The MIB-1 labeling index indicated no malignant change compared with the previous samples. Radiotherapy was performed after the surgery. The importance of early diagnosis and management of scoliosis is emphasized, and the peculiar pattern of dissemination of the pilocytic astrocytoma and its treatment are reviewed.