Kissing mirror image anterior communicating artery aneurysms--case report.

A 45-year-old male presented with an extremely rare case of symmetrical kissing aneurysms located at the both ends of the anterior communicating artery. Angiography and three-dimensional computed tomography (3D-CT) angiography clearly showed the relationship of the kissing aneurysms. The aneurysms were clipped through the right pterional approach. Temporary clipping of the anterior cerebral arteries for 5 minutes was required to gain enough operative space. The patient was discharged without neurological deficits. 3D-CT angiography, magnetic resonance angiography, or rotatory digital subtraction angiography may be useful for detecting kissing aneurysms, but the most important issue is awareness of this unusual condition.

Isolation and functional characterization of the 5'-upstream region of mouse P/Q-type Ca2+ channel alpha1A subunit gene.

The omega-agatoxin-IVA-sensitive P/Q-type Ca2+ channel is predominantly expressed in the nervous system. To dissect the molecular mechanisms underlying the neuron-specific expression of the P/Q-type channel, we have isolated and characterized the 5'-upstream region of the mouse alpha1A subunit gene. A transcription start site appeared to exist at -269 bp upstream from the start codon as found by 5' RACE analysis. The proximal promoter of the alpha1A subunit gene lacks a typical TATA box, but contains several transcription factor binding sequences, including two Sp1 sites. When linked to a placental alkaline phosphatase (PLAP) reporter gene to examine the promoter activity, the 6.3-kb (-6,273 to +269) 5'-upstream region, but not a smaller 3.0-kb construct (-3, 021 to +269), was able to drive the reporter gene in neuron-like PC12 cells. In contrast, neither of these constructs enhanced the PLAP expression in fibroblast NIH3T3 cells. The sequence between 6.3 and 3.0 kb of the 5'-upstream region did not show promoter activity in either of the cell lines, but enhanced TK promoter activity in PC12 cells, though not in NIH3T3 cells. These results suggest that neuron-specific elements of the alpha1A subunit gene are likely to be located in the distal upstream regions (-6,273 to -3,021) of the 5'-upstream sequence.

Differential distribution of TRP Ca2+ channel isoforms in mouse brain.

Mammalian homologues of the Drosophila TRP proteins, which are essential for light-activated, phosphatidyl-inositide (PI)-dependent Ca2+ conductance in Drosophila photoreceptors, were molecularly identified, to investigate receptor-activated Ca2+ influx in the mammalian nervous system. Two cloned mouse TRP homologues, TRP3 and TRP4, structurally related to the voltage-dependent Na+ channel, were expressed predominantly in the brain, where a sharp contrast in the distribution of the RNA transcripts for TRP isoforms was demonstrated by in situ hybridization analysis. TRP3 mRNA was concentrated in cerebellar Purkinje cells and sparsely localized in the cerebellar granule layer, pontine nuclei, and thalamus, whereas TRP4 mRNA was abundantly expressed in hippocampal CA1 pyramidal neurons, dentate gyrus granule cells, and cerebral cortical neurons, and in the septal nuclei and the mitral layer of olfactory bulb. The distinct spatial patterns of TRP isoforms implicate that neurons are highly heterogeneous in receptor-activated Ca2+ influx responsible for the second phase of PI-mediated rise in intracellular Ca2+.

Adult moyamoya disease progressing from unilateral to bilateral involvement .

A 30-year-old female and a 38-year-old male developed "definite" moyamoya disease after presenting with "probable" moyamoya disease manifesting as ischemic stroke. Angiography at first presentation demonstrated unilateral involvement with normal contralateral findings. The cases were classified as adult type "probable" moyamoya disease according to the diagnostic criteria. Follow-up angiography demonstrated the occlusive lesions had become bilateral, satisfying the criteria for "definite" cases. Serial angiography is required in adults presenting with "probable" moyamoya disease manifesting as ischemic stroke.

Management of adult moyamoya disease.

The surgical management of adult moyamoya disease was evaluated retrospectively. Eight of 10 patients with hemorrhagic stroke had no bleeding episodes and eight of 11 patients with ischemic stroke were asymptomatic postoperatively. Angiography demonstrated neovascularization via the external carotid artery (ECA) in 22 sites (88%) and reduced basal moyamoya vessels in 11 sites (44%) postoperatively. These symptomatic improvements and angiographic findings suggest that reconstructive surgery for adult moyamoya disease patients with hemorrhagic or ischemic stroke is effective in decreasing hemodynamic stress in the basal moyamoya vessels and increasing the cortical blood supply via the ECA.

Aspergillotic aneurysm formation of cerebral artery following neurosurgical operation.

A rare case of intracranial aspergillotic aneurysm following neurosurgical operation for facial spasm is presented. Severe meningitis had persisted several days prior to the subarachnoid hemorrhage which occurred on 70th postoperative day. Angiography demonstrated a fusiform aneurysm at the peduncular segment of the superior cerebellar artery on the other side of operation. As the repeated cultures of CSF were negative, the pathogenetic factor causing aneurysm formation could not be identified before second operation of aneurysmal resection. Pathological study of the specimen revealed the arterial wall being deeply invaded by aspergillotic hyphae.

[An adult case of moyamoya disease associated with marked advance of occlusive lesion in the bilateral carotid system].

We reported an adult case with moyamoya disease in which unilateral obstructive process proceeded to the bilateral lesion during a period of 45 months. A 30-year-old female was admitted to our institute due to frequent ischemic episodes since she was 25 years old. At the time of admission, she was asymptomatic with slightly disturbed psychogenic reactions. At the time of the initial cerebral angiography when she was 25 years old, the right cerebral angiography demonstrated stenosis of the right ICA terminal portion with moyamoya vessels in the basal area and leptomeningeal anastomosis at the right parietal area. The left cerebral angiography revealed normal ICA system with a lightly narrowing Al portion, without Moyamoya vessels. The second cerebral angiography, taken 45 months after the initial angiography, demonstrated progression stenosis of the right ICA terminal portion, with decreasing moyamoya vessels at the basal area, obstruction of the left ICA terminal portion, stenosis at the Al portion with newly developed moyamoya vessels in the left basal area. We concluded at this time that this case was a definite case of moyamoya disease. The third cerebral angiography after right SDP (synangio-dural plasty), 49 months after the initial angiography, revealed, in the right angiography, newly formed anastomotic vessels perfusing the middle cerebral artery region via the extracerebral arteries and in the left cerebral angiography, and an increased obstruction of the ICA terminal portion, transdural anastomosis via the extracerebral arterial system, and a decrease of moyamoya vessels in the basal area. This rare case provided us a good picture of the pathogenic mechanism of moyamoya disease.

Ischemic stroke in infancy, childhood, and adolescence.

The authors studied 34 patients with juvenile ischemic cerebrovascular disease over a 15-year period. Of the 34 patients, 23 had intracranial occlusions attributed to cerebral thrombosis or embolism and 11 had occlusions resulting from moyamoya disease. Clinicopathological features were evaluated in the 23 cases with ischemic stroke, but not those with moyamoya disease. The cause of the arterial occlusion remained undetermined in 11 patients and was found to be an embolism based on congenital heart disease in 8, on trauma in 3, and on infection in 1. Cerebral angiography was performed in 21 patients. Of these, 17 had stenoses or occlusions corresponding to their symptoms. CT scans were performed in 10 patients; the lesion in question showed no stenosis or occlusion with cerebral angiography. With regard to prognosis, patients with unknown etiology had good outcomes compared with those with congenital heart disease. With respect to "acute infantile hemiplegia", 10 patients had convulsive seizures and 4 had a history of an earlier infection. Angiography and CT scans in patients with congenital heart disease demonstrated arterial occlusive sites in the middle cerebral artery region. Three patients had abscesses after their ischemic lesions.

[Autoimmune antibody in moyamoya disease].

The association of autoimmune antibody and HLA antibody has been documented concerning the pathogenesis of moyamoya disease. In this paper, we study the autoimmune antibody in moyamoya disease and discuss its relationship with the pathogenesis. Autoimmune antibody was evaluated in 13 cases of moyamoya disease. The age distribution of the patients ranged from 13 to 55, with an average of 37 years. Eight were females, 5 males. Autoantibody was studied with native DNA (double strand DNA) and ANA (antinuclear antibody). Antibody was noted in 3 cases (23%) in native DNA study, and 2 cases (15%) in ANA study. Significantly high incidence was recognized compared with normal control (4%). In relation to the clinical features, such as initial symptoms, time of onset, neurological findings, no significant correlation was recognized. This study suggests that immunological factors might play a partial role in the pathogenesis of moyamoya disease.