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Endovascular treatment of ruptured vertebrobasilar artery aneurysms with Gugliemi detachable coils (GDC) has become an alternative to surgery. Mid-term angiographic follow-up can now be reported. Of 111 vertebrobasilar aneurysms in 110 patients we treated with GDC since 1992, 53 underwent angiography within 1 year and 59 after more than 18 months. We did not achieve complete occlusion on initial treatment of 23 aneurysms (21%). Complications were observed in 19 patients (17%), leading to permanent clinical disability in eight. Enlargement of the neck or reopening was seen in 12 (23%) of 53 aneurysms followed by angiography within 12 months. Documented recanalisation was treated in four (8%). Angiography was performed after 18-78 months in 59 patients, of whom nine, including three with initially incomplete occlusions, were retreated with GDC. Within the entire second observation period, three (5%) of the 59 patients had a further haemorrhage and were retreated with GDC. Rebleeding proved to be the only factor influencing the clinical outcome of retreated patients.

We present a juvenile case of Alexander's disease with atypical focal magnetic resonance imaging-detected lesions and elevated levels of lactate in cerebrospinal fluid. The diagnosis was based on the neuropathological finding of a diffuse accumulation of Rosenthal fibers within the brain and the spinal cord. The diagnosis was confirmed by detection of a mutation in exon 1 at nucleotide position 249 of glial fibrillary acidic protein cDNA, a finding previously reported in cases of infantile Alexander's disease.

Background and Purpose: Preoperative embolization expands the spectrum of meningioma that can be operated on safely. Our goal was to achieve the distalmost loading of the vascular bed and confluent tumor necrosis with a fibrin glue preparation in the preoperative embolization of meningiomas., Methods: Between 1992 and 1997, 80 patients with a meningioma had diagnostic angiography with a standard transfemoral Seldinger technique, performed with a 6F guiding catheter and digital subtraction angiography. Preoperative embolization was carried out in the same session with an additional microcatheter system. Fibrin glue was the only component used. In all cases, CT was performed immediately after embolization; in nine patients, MR imaging was also performed., Results: Angiography verified the elimination of tumor blush in all patients. The high-density areas seen on postembolization CT scans, caused by the fibrin glue dispersed in the embolized supply area, were found to be necrotic at surgery and were easily removed by suction. Two (2.5%) of the 80 patients had complications associated with embolization that resulted in neurologic deficits., Conclusion: The most effective preoperative embolization of tumors requires a distalmost loading of the vascular bed. Fibrin glue, which is easy to use and safe to handle, causes confluent tumor necrosis within the injected vascular territory.

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease of infancy and young childhood. The clinical presentation includes recurrent unexplained fever with hepatosplenomegaly. Cytopenia, hypofibrinogenemia and/or hypertriglyceridemia and hemophagocytosis in bone marrow, spleen and lymphnode confirm the diagnosis. Hemophagocytosis may not be present at the beginning. In these cases, diagnosis is facilitated by a positive family history, a relapsing course of the disease, the frequent involvement of the central nervous system and positive findings on immunological work-up. Treatment by chemotherapy and immunosuppressants can achieve sustained remissions in most patients and reinduction of remission after relapse is possible. Most children however, eventually die from progressive disease. At present, allogeneic bone marrow transplantation is the only curative therapeutic option. Between August 1992 and May 1997 eleven consecutive patients with HLH received bone marrow from unrelated (n = 7) or matched sibling donors (n = 4). The conditioning regimen consisted of busulfan, VP-16 and cyclophosphamide. Patients engrafted after a median time of 16 days (13-43). Only one patient developed grade III acute GVHD, another patient, grade II acute GVHD. Although regimen-related toxicity was extensive, all patients have survived without signs of HLH after a median follow up of 20 months (8-63). One patient suffers from chronic GVHD, three patients reveal psychomotoric retardation and one patient has severe impairment with spastic tetraparesis, amaurosis and seizures. Our experience shows that HLH can be successfully treated by allogeneic BMT from unrelated donors.

Intracranial aneurysms bear a high risk of morbidity and mortality especially when they have become symptomatic due to rupture or represent a space occupying lesion. Therefore operative treatment is aimed at the obliteration of the aneurysm to eliminate the risk of hemorrhage. Planning of treatment requires a diagnostic resolution capable of showing aneurysms and their anatomic relationship to the adjacent structures. Due to technical advances computed tomography has reached a diagnostic accuracy sufficient to plan aneurysm treatment. This report describes the present state of three-dimensional spiral-CT angiography (3D-spiral-CTA) and our protocol to examine the arteries of the Circle of Willis for the presence and microanatomy of aneurysms. The experience of 46 patients with 64 aneurysms is reported and the diagnostic value of the method in comparison to conventional angiography is evaluated.

The authors report a case of a congenital clivus chordoma that caused cranial nerve palsy and hydrocephalus within a few days after birth. The tumor was well demonstrated by sonography, CT, and MR; the preoperative diagnosis was histologically confirmed after subtotal resection.

We report the first case of a congenital intracranial chordoma. Hydrocephalus, sixth and seventh cranial nerve palsy, and torticollis were observed shortly after birth. The tumour was delineated by sonography, CT scans and MRI and the diagnosis confirmed after subtotal resection at the end of the newborn period.