We performed detailed DNA sequencing analysis on type III collagen cDNA from 58 patients with either intracranial artery aneurysms or cervical artery dissections. The 58 patients were of seven different nationalities; among the patients were three pairs of relatives, so that 55 were unrelated, and of these, 29 had at least one blood relative with either an intracranial artery aneurysm or a cervical artery dissection. The age of the patients at the time of diagnosis ranged from 15 to 68 years (mean +/- SD = 40.3 +/- 11.0). The study group consisted of 25 males and 33 females. The analysis covered 3,232 nucleotides of significant (nonredundant) sequences per allele; therefore, we analyzed as many as 355,520 nucleotides. Mutations in the coding sequences for the triple-helical domain of type III collagen were excluded in 40 individuals with intracranial aneurysms and 18 individuals with cervical artery dissections. Direct sequencing of polymerase chain reaction products allowed mutations to be excluded with a high degree of confidence. Mutations that markedly decreased expression from one allele were also excluded in 42 of the 58 individuals, since the presence of both bases at one or more polymorphic sites in the 42 patients showed that two alleles were transcribed. The results indicated that mutations in the gene for type III procollagen (COL3A1) are not a common cause of either intracranial artery aneurysms or cervical artery dissections.
We report a rare case of anaplastic oligodendroglioma with extracranial metastasis, showing 1p19q codeletion in both the brain tissue and the metastatic site. A 53-year-old man first presented with a left frontal tumor. The tumor was subtotally removed and irradiation was performed for the residual tumor and tumor bed. Two years after the initial treatment, several tumors appeared on his neck and one was resected. Histological examination revealed anaplastic oligodendroglioma, proved to be the same as the previous brain tumor. The patient refused further treatment, and died 30 months after the initial treatment. Autopsy demonstrated multiple extracranial metastases in the vertebrae, lymph nodes, spinal dura mater, thymus gland, and chest wall. We confirmed 1p19q loss of heterozygosity in both lesions, suggesting that 1p19q co-deletion might important to extracranial metastasis of oligodendroglioma.
A man of 35 years, who had had three attacks of subarachnoid hemorrhage in the previous 3 years, was admitted to hospital with complaints of headache and priapism. There had been intermittent priapism with abnormal acceleration of sexual desire since the first attack, and erection of the penis had persisted with intolerable pain after the last attack of subarachnoid hemorrhage. A carotid angiogram revealed an aneurysm at the junction of the left internal carotid and posterior communicating arteries. Clipping of the aneurysmal neck was successfully performed. However, priapism continued for 22 days after the operation and resulted in sexual impotence. The neurological problems of priapism are discussed with special reference to a hypothalamic lesion caused by the ruptured intracranial aneurysm in this report.
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