Mutations of CCM3/PDCD10 cause 10-15% of hereditary cerebral cavernous malformations. The phenotypic characterization of CCM3-mutated patients has been hampered by the limited number of patients harboring a mutation in this gene. This is the first report on molecular and clinical features of a large cohort of CCM3 patients. Molecular screening for point mutations and deletions was used to identify 54 CCM3-mutated index patients. Age at referral and clinical onset, type of inaugural events and presence of extra-axial lesions were investigated in these 54 index patients and 22 of their mutated relatives. Mean age at clinical onset was 23.0 ± 16 years. Clinical onset occurred before 10 years in 26% of the patients, and cerebral hemorrhage was the initial presentation in 72% of these patients. Multiple extra-axial, dural-based lesions were detected in 7 unrelated patients. These lesions proved to be meningiomas in 3 patients who underwent neurosurgery and pathological examination. This ‘multiple meningiomas' phenotype is not associated with a specific CCM3 mutation. Hence, CCM3 mutations are associated with a high risk of early-onset cerebral hemorrhage and with the presence of multiple meningiomas.

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Intramedullary abscesses of the spinal cord are uncommon. Most of them occur in association with heart, pulmonary or urogenital infections. We report two cases of intramedullary spinal cord abscesses secondary to congenital dermal sinus. Only 14 cases of such an association have previously been reported. In our cases, dermal sinus was associated with an epidermoid tumour. The clinical presentation, pathogenesis, magnetic resonance imaging findings, surgical management and outcome are discussed.

Object. The authors sought to assess the functional tolerance and tumor control rate of cavernous sinus meningiomas treated by gamma knife radiosurgery (GKS). Methods. Between July 1992 and October 1998, 92 patients harboring benign cavernous sinus meningiomas underwent GKS. The present study is concerned with the first 80 consecutive patients (63 women and 17 men). Gamma knife radiosurgery was performed as an alternative to surgical removal in 50 cases and as an adjuvant to microsurgery in 30 cases. The mean patient age was 49 years (range 6–71 years). The mean tumor volume was 5.8 cm3 (range 0.9–18.6 cm3). On magnetic resonance (MR) imaging the tumor was confined in 66 cases and extensive in 14 cases. The mean prescription dose was 28 Gy (range 12–50 Gy), delivered with an average of eight isocenters (range two–18). The median peripheral isodose was 50% (range 30–70%). Patients were evaluated at 6 months, and at 1, 2, 3, 5, and 7 years after GKS. The median follow-up period was 30.5 months (range 12–79 months). Tumor stabilization after GKS was noted in 51 patients, tumor shrinkage in 25 patients, and enlargement in four patients requiring surgical removal in two cases. The 5-year actuarial progression-free survival was 92.8%. No new oculomotor deficit was observed. Among the 54 patients with oculomotor nerve deficits, 15 improved, eight recovered, and one worsened. Among the 13 patients with trigeminal neuralgia, one worsened (contemporary of tumor growing), five remained unchanged, four improved, and three recovered. In a patient with a remnant surrounding the optic nerve and preoperative low vision (3/10) the decision was to treat the lesion and deliberately sacrifice the residual visual acuity. Only one transient unexpected optic neuropathy has been observed. One case of delayed intracavernous carotid artery occlusion occurred 3 months after GKS, without permanent deficit. Another patient presented with partial complex seizures 18 months after GKS. All cases of tumor growth and neurological deficits observed after GKS occurred before the use of GammaPlan. Since the initiation of systematic use of stereotactic MR imaging and computer-assisted modern dose planning, no more side effects or cases of tumor growth have occurred. Conclusions. Gamma knife radiosurgery was found to be an effective low morbidity—related tool for the treatment of cavernous sinus meningioma. In a significant number of patients, oculomotor functional restoration was observed. The treatment appears to be an alternative to surgical removal of confined enclosed cavernous sinus meningioma and should be proposed as an adjuvant to surgery in case of extensive meningiomas.

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Object. Because central nervous system white matter exerts a powerful inhibitory effect on axonal growth, implantation of nerve grafts or rootlets into the cervical spinal cord following ventral root avulsion injury should, ideally, be performed directly through the ventral root exit zone (VRExZ), which is located near the anteromedial aspect of the anterior horn; the grafts/rootlets should not be implanted into the white matter of the lateral cord. This is not possible when using a conservative posterior approach. Therefore, the authors have studied the anatomy encountered when using the anterolateral approach and evaluated the technique in the particular case of avulsed ventral nerve roots. They also present a case illustration of the procedure, which is used currently in their department. Methods. Anterior access to the rootlets is obtained using a lateral interscalenic approach; the vertebral artery is exposed and mobilized, and oblique drilling of the vertebral bodies (VBs) is performed. Because the articular processes and half of the VBs are preserved, fusion is not required. The approach allows the surgeon to expose the anterior aspect of the cervical dura and the entire length of the emerging spinal nerves. The anterior aspect of the dura is opened at the desired levels for VRExZ exposure, and the position is ideal for implantation of the graft/rootlets. The interscalenic dissection is mandatory so that the lesions of the supraclavicular plexus can be evaluated and repaired. If necessary, the anterior approach allows for exploration of the infraclavicular plexus during the same procedure. Conclusions. The use of a true anterior approach to the ventral rootlets appears to be a valuable and appropriate approach that avoids extensive laminectomy/facetectomy while reimplantation is performed through the anterolateral sulcus itself. In this approach, however, reimplantation of dorsal roots into the spinal cord remains impossible.