Angiotensin-converting enzyme insertion/deletion gene polymorphism in patients with familial multiple cerebral cavernous malformations.

Cavernous malformations can occur in both sporadic and autosomal dominant forms. The aim of this study was to investigate the potential role of insertion/deletion (I/D) polymorphisms of the angiotensin-converting enzyme (ACE) gene in the development of cerebral cavernous malformations (CCM). Forty-one members of two families affected by familial CCM were included in this study. DNA was isolated from peripheral venous blood, and polymerase chain reaction analysis was used to detect I/D polymorphisms of the ACE gene, using HACE3s and HACE3as as primers. Only 10 participants had MRI-confirmed CCM. Of these 10 subjects, seven had the I/D, two had the D/D, and one had the I/I genotype. Of the remaining 31 subjects, 14 had the I/I, 13 had the I/D, and four had the D/D genotype. There was a greater proportion of subjects with the D allele among those with MRI-confirmed CCM than among those without (p<0.05). These results suggest that the D polymorphism of the ACE gene may be involved in the pathogenesis of familial CCM.

Cerebral alveolar echinococcosis. A case report with MRI and review of the literature.

Alveolar echinococcosis is an important zoonotic infection caused by the larval stage of the Echinococcus multilocularis. It is endemic to North America, Central Europa, Russia, China and Turkey. The liver and the lung are the organs most commonly involved. Cerebral alveolar echinococcosis is rare accounting for only 1% of cases. We present a 55-year-old patient with a right frontal mass. T2-weighted MRI series revealed a grape like multilobular, heterogeneous mass with low density. A diagnosis of glial tumor was made. The mass was totally removed. The histopathological examination showed a diffuse growth composed of compartments that are filled with a gelatinous matrix and many brood capsules and protoscolices filled with necrotic tissue. Histopathological findings were consistent with the diagnosis of alveolar echinococcosis. No postoperative complications were observed. There were no lesions in the liver and lungs. The patient was started on albendazole (ABZ) at a daily dosage of 800 mg for 3 months. The patient has remained free of any mass lesion for 5 years. Hypointense grape-like mass with calcification and surrounding white matter edema in T2-weighted MRI should suggest cerebral alveolar echinococcosis. Radical surgery and an adjuvant therapy with ABZ provides useful prolongation of life.

Spinal meningiomas: evaluation of 41 patients.

AIM: Spinal meningiomas are relatively frequent intraspinal tumors. They constitute 25-46% of all primary spinal neoplasms. METHODS: Forty-one patients with spinal meningiomas surgically treated between 1986 and 2001 are reviewed in this report. There were 32 females and 9 males, aged 16 to 73 years old. Tumor location was cervical in 7 cases and thoracic in 34 cases. All tumors were intradural. All of the patients were operated by laminectomy in prone position. RESULTS: Total excision was achieved in 40 (98%) patients and subtotal excision in 1(2%). The majority of the tumors were meningothelial (42%) or psammomatous (25%). There was no surgical mortality. Four patients (10%) suffered from morbidity: One patient with CSF fistula, 1 deep venous thrombosis, 1 case with paraparesis and 1 wound infection was seen. Neither multiple meningiomas nor malign meningiomas were seen in our series. CONCLUSIONS: Spinal meningiomas are benign tumors and should be excised totally. If the preoperative neurological status is not complicated, fairly good outcome can be achieved.

Tentorial meningiomas.

We report our experience with and long-term results of 37 patients with tentorial meningiomas who underwent surgery between 1972 and 1993. The average age was 43 years, and the mean duration of symptoms was 36 months. Headache (83.8%) and extremity or gait ataxia (35.1%) were the most common complaints. On neurological examination, signs of elevated intracranial pressure and cerebellar deficits (51.4%) were the most common findings, followed by third nerve involvement (35.1%). Computed tomography, angiography, and, in recent years, magnetic resonance imaging were used as diagnostic tools and for planning the surgical procedure. According to the primary site of attachment, the tentorial meningiomas were divided into three subgroups: medial, lateral, and falcotentorial. The lateral and medial tumors, with mainly supratentorial development, were approached from above by using a temporal, temporooccipital, or parietooccipital craniotomy. For tumors developing mainly in the posterior cranial fossa, suboccipital craniectomy was performed. In six patients who showed medial tentorial and petrous apex attachment, a combined subtemporal transpetrosal and retromastoid approach was performed. In 31 patients, the tumors were totally removed, and, in 6 patients, only subtotal excision could be done. Seven patients had postoperative complications, but only one of them died of severe brain edema. Our mortality rate was 2.7%. In this article, appropriate preoperative studies, surgical techniques, and surgical results are discussed.