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Familiare und multiple Kavernome beruhen haufig auf autosomal-dominant vererbten Mutationen, die sich mit einer Penetranz von etwa 60% klinisch manifestieren. Wir berichten uber einen 2-jahrigen Patienten mit angeborener vaskularer Hautmalformation des Oberschenkels. Bei der zerebralen Bildgebung wurde ein eingeblutetes Kavernom diagnostiziert und anschliesend mikrochirurgisch reseziert. Die Familienanamnese war positiv, und beim Indexpatienten wurde eine neue CCM1-Mutation (c.1780_1783delinsTACCTGTTACCAAA) gefunden, sodass eine prasymptomatische genetische Diagnostik im Rahmen einer genetischen Beratung in dieser Familie moglich ist.

Background: Gliomas are the most common primary tumours of the central nervous system and exhibit rapid growth that is associated with neovascularisation. Adrenomedullin is an important tumour survival factor in human carcinogenesis. It has growth promoting effects on gliomas, and blockade of its actions has been experimentally shown to reduce the growth of glioma tissues and cell lines. There is some evidence that the calcitonin receptor-like receptor (CRLR) mediates the tumorigenic actions of adrenomedullin. Aim: To determine whether CRLR is expressed in human gliomas and the probable cellular targets of adrenomedullin. Methods: Biopsies from 95 human gliomas of varying grade were processed for immunohistochemical analysis using a previously developed and characterised antibody to CRLR. Results: All tumour specimens were positive for CRLR. As previously found in normal peripheral tissues, CRLR immunostaining was particularly intense in the endothelial cells. This was evident in all the various vascular conformations that were observed, and which are typical of gliomas. In addition, clear immunostaining of tumour cells with astrocyte morphology was observed. These were preferentially localised around vessels. Conclusions: This study has shown for the first time that the CRLR protein is present in human glioma tissue. The expression of the receptor in endothelial cells and in astrocytic tumour cells is consistent with the evidence that its endogenous ligand, adrenomedullin, may influence glioma growth by means of both direct mitogenic and indirect angiogenic effects. CRLR may be a valuable target for effective therapeutic intervention in these malignant tumours.

OBJECTIVE To investigate if the intracisternal distribution of subarachnoid hemorrhage (SAH) following aneurysm rupture allows the correct prediction of the symptomatic aneurysm site. [nl] METHODS Ninety-nine consecutive patients with acute SAH and angiographically proven aneurysm were included into the study. The parent vessel of the diagnosed aneurysms were the anterior communicating artery (ACoA) in 38 patients, the middle cerebral artery (MCA) in 26 patients, the internal carotid artery (ICA) in 25 patients, the pericallosal artery (A2) in 5 patients, the basilar artery (BA) in 4 patients and the vertebral artery (VA) in 1 patient. In 21 patients, an additional asymptomatic aneurysm was diagnosed. The initial computerized tomography (CT) scans of the 99 patients were given to 2 experienced vascular neurosurgeons, who were blinded for the angiography findings. The 2 investigators had to predict the site of the ruptured aneurysm. [nl] RESULTS Investigator 1 correctly predicted the aneurysm site in 56 (57 %), investigator 2 in 59 of the 99 patients (60 %). Investigator 1 correctly identified 81 % of the MCA aneurysms, and investigator 2 74 % of the ACoA aneurysms. However, in only 46 of the 99 patients (47 %), the aneurysm site was correctly predicted by both investigators together. [nl] CONCLUSION The results indicate, that the distribution of the subarachnoid blood as shown on the first CT scan after aneurysm rupture barely allows to predict the symptomatic aneurysm site. Thus, neurosurgical decision making (identification of the ruptured aneurysm in patients with multiple aneurysms; surgical exploration in patients with non-perimesencephal SAH, but negative angiography) should not rely on the first CT scan after SAH.

A unilateral laminotomy for bilateral access to the lumbar spinal canal was investigated in human cadaver spine specimens to test its practicability in the treatment of spinal stenosis. Micro-surgical decompression was performed by partial resection of the ipsilateral facet, the medial portion of the laminar arch, the contralateral facet and by complete removal of the ligamentum flavum. Anatomical, radiological and morphometrical studies on 4 adult cadaver spine specimens have proved the feasibility of this unilateral approach. Complete bilateral flavectomy and partial bilateral facetectomy were the essential surgical steps for an adequate operative decompression.

The authors report about a 3-years experience with helical CT and 3-D surface reconstruction applied in neurosurgical patients. All examinations were performed in addition to preexisting diagnostic CT, MRI, or angiography. The aim of this study was to assess the clinical value of this method with regard to planning of the surgical approach to anterior, middle, and posterior skull base and spinal lesions. 75 examinations of 55 patients were analysed and ranked as follows: A = examination with significant additional information for neurosurgical planning of skull base or spinal procedures or for postoperative evaluation of the neurosurgical approach, B = examination with some useful information for the neurosurgical planning or postoperative control, however, without significant advantage as compared to established diagnostic methods, C = examination without significant additional information. Classification was performed independently by two experienced surgeons. Examinations of anterior, middle, and posterior skull base lesions including cerebral aneurysms were in the majority rated as helpful and significantly informative, (A = 21, B = 24, C = 9, n = 54). Three-dimensional imaging of the spine was of clinical value only in specific cases (A = 6, B = 6, C = 9, n = 21). The authors conclude that three-dimensional imaging is a valuable diagnostic tool for pre- and postoperative imaging of tumorous and vascular lesions adjacent to the skull base, allowing for optimal surgical approaches with minimal invasiveness.

Six patients with a dural arteriovenous fistula (DAVF) of the floor of the anterior cranial fossa underwent microsurgical treatment. Two of them were operated using a conventional frontobasal approach, and four using an interhemispheric approach. The interhemispheric approach offers the advantages of sparing the frontal sinus, minimizing frontal lobe retraction, and providing a visual angle perpendicular to the floor of the anterior fossa and an excellent view of the fistula located on the cribriform plate at the level of the foramen caecum. Using the interhemispheric route, the malformation was occluded in all the cases by dividing the vascular connection between the dura of the cribriform plate and the intradural draining vein. There were no complications related to the surgical procedure. This route avoids some disadvantages of the more frequently reported frontobasal approach. It is therefore a recommendable alternative for the management of frontal DAVFs.

Although primary treatment of medulloblastoma is now successful in a high percentage of patients, its secondary manifestations still bear a poor prognosis. Thorough studies of secondary manifestations are therefore pivotal to plan therapeutic approaches for the long-term management of medulloblastoma. Here we describe the incidence of secondary tumour manifestations in 66 patients of a single centre who underwent surgery for medulloblastoma between 1975 and 1990. No patient was excluded due to a poor postoperative course. Thirty-five patients showed evidence of secondary tumour growth. Of these, 17 suffered from local recurrence, and 27 developed metastastatic disease. The median latencies for secondary manifestations were 25 months for local recurrence (n = 17), 11 months for spinal metastases (n = 10), 15 months for supratentorial metastases (n = 8), 8 months for subleptomeningeal dissemination (n = 6), and 23 months for systemic metastases (n = 8). Two patients developed primary metastatic spread to the posterior fossa. Of 8 patients with supratentorial metastases, 6 developed fronto-basal lesions. In our patients, 89% of secondary lesions occurred within less than 3 years after primary diagnosis. 85% of patients with extra-axial tumour spread had been treated with a permanent shunt. Radical tumour resection and radiotherapy with 30 Gy to the neuraxis and 20 Gy boost to the posterior fossa was an important prognostic factor in this series. Patients with additional chemotherapy did not benefit significantly from this treatment. We conclude that optimal management of the primary lesions should aim at (i) total resection, (ii) avoid permanent shunting, and (iii) completion of the radiotherapy with inclusion of the medial frontobasal cisterns in the radiotherapeutic regimen. Our analysis suggests that adequate postoperative screening programmes should consist of 3-monthly scans of the neuraxis in the first three postoperative years and 6-monthly scans thereafter.

Although recently survival of some medulloblastoma patients increased remarkably, it remains a serious diagnosis in others. In order to predict the postoperative prognosis in patients treated for medulloblastoma, a new staging, scoring and grading system was developed. Sixty-six patients operated on microsurgically between 1975 and 1990 at a single neurosurgical center were fully followed-up. No patient was excluded due to a poor postoperative course. Completion of commonly used radiotherapy protocols was attempted in all patients. Survival of patients was evaluated by the Kaplan-Meier method. The following 5 parameters were selected to define subgroups: patients' age, tumour location and histology, degree of resection and presence or absence of metastases. Patients older than 10 years had a better prognosis than individuals aged 10 or less (p < 0.01), patients with lateral tumours had a better prognosis than patients with midline tumours with brain stem infiltration (p < 0.05), patients with complete tumour resection had a more favourable prognosis than individuals with subtotal (p < 0.01) or partial resection (p < 0.001), patients without metastases at the time of diagnosis had a better prognosis than individuals without such evidence (p < 0.001), patients with the desmoplastic tumour variant had a better prognosis than patients with classical tumour histology (p < 0.01). According to the prognosis of a distinct subgroup, scoring points were distributed which correlated with the degree of inter-subgroup significances. The sum of a single patient's scoring points was called the total score. Based on this score, three groups of prognosis were distinguished.(ABSTRACT TRUNCATED AT 250 WORDS)

We carried out 22 examinations to determine the value of three-dimensional (3D) volumetric CT (spiral CT) for planning neurosurgical procedures. All examinations were carried out on a of the first generation spiral CT. A tube model was used to investigate the influence of different parameter settings. Bolus injection of nonionic contrast medium was used when vessels or strongly enhancing tumours were to be delineated. 3D reconstructions were carried out using the integrated 3D software of the scanner. We found a table feed of 3 mm/s with a slice thickness of 2 mm and an increment of 1 mm to be suitable for most purposes. For larger regions of interest a table feed of 5 mm was the maximum which could be used without blurring of the 3D images. Particular advantages of 3D reconstructed spiral scanning were seen in the planning of approaches to the lower clivus, acquired or congenital bony abnormalities and when the relationship between vessels, tumour and bone was important.

Twelve patients with severe intraventricular haemorrhage (IVH) underwent intraventricular thrombolysis with recombinant tissue plasminogen activator (rtPA). External ventricular drainage was performed in all patients within 24 hours of haemorrhage. Fibrinolytic therapy was started within 24 hours from the onset of symptoms in ten cases, and in two further cases after 48 hours and 5 days, respectively. Two to 5 mg of rtPA were injected via the ventricular catheter into one or both lateral ventricles. The injection was repeated at intervals ranging from 6 to 24 hours until CT scans demonstrated a substantial reduction of intraventricular blood. The total rtPA doses per patient ranged from 3 to 31 mg. CT scans showed a marked reduction of intraventricular blood and normalization of ventricular size within 24 to 48 hours from the beginning of the fibrinolytic therapy. Rapid reduction of elevated intracranial pressure by continuous diversion of cerebrospinal fluid could be achieved in all patients, because the ventricular catheters never became obstructed by clotted blood during the fibrinolytic therapy. During the period of treatment, the level of consciousness, as classified according to the Glasgow Coma Scale, improved from a mean value of 7 to 12. One fatal case of meningitis most probably due to the ventriculostomy was the only complication related to the treatment. This method of treatment might improve the prognosis in patients in whom a large intraventricular haematoma volume, ventricular dilatation, and impaired cerebrospinal fluid circulation are major determinants for the outcome.

Over a 15-year period, 15 patients with craniospinal malformations were operated on using the transoral route. Ten patients had basilar impression and five had atlantoaxial dislocation; nine patients had associated lesions. Patients treated with reposition and dorsal fusion recovered well without ventral decompression. We found that transoral odontoidectomy is suitable as a primary decompressing measure only in ventral compression or nondislocated atlantoaxial conditions.

The authors review 26 patients with deep-seated cavernous angiomas which were removed by microsurgery. Ten of the angiomas were located in the insula and basal ganglia, 2 in the thalamus, 5 in the midbrain, 8 in the pons, and 1 in the brachium pontis. The patients were among 73 consecutive cases operated on between August 1983 and December 1989 for symptomatic cavernous angiomas in various locations. In 11 cases total excision of the cavernoma was achieved without producing additional neurological deficits. Postoperative neurological recovery was delayed in 7 patients. In the remaining 8, the complicated postoperative course was caused by bleeding from residual parts of the malformation or damage to long-tract pathways in two cases, respectively, vascular injury during dissection in three cases, and paradoxical air embolism in one case. In order to achieve a satisfactory surgical result, it is stressed that particular attention has to be paid to the operative approach, to careful dissection and complete removal of the malformation, to perforating arteries, and to anomalous venous drainage.

The present study aims to provide preliminary results of the thermal effects on rat brain tissue after argon plasma coagulation (APC). It also presents and discusses the clinical experiences in the treatment of brain tumor using APC.A controlled study of APC in the rat brain was conducted. Twelve rats were randomly divided into 2 experimental groups. In the first group (n = 6), histopathological evaluation was performed 2 days following the coagulation. In the second group (n = 6), the evaluation was performed 12 days post operation. In a prospective study of APC-treated tumor tissue in 3 patients, the depth of plasma penetration and histological alteration were evaluated.In the animal experiment, extent of tissue defect became significantly smaller after 12 days (p = 0.010). The maximum depth of tissue alteration after APC application was limited to 2.15 mm (range: 1.5-2.15 mm) at day 2. The histological alteration of tissue after the thermal injury can be divided into 3 zones. In addition, the depth of tissue alteration in the APC-treated human brain tumor was measured in vertical and horizontal planes under light microscope. Similar to the animal experiment result, penetration of the plasma energy in human brain tumors was limited to a maximum of 2.13 mm (range: 1.6-2.13 mm).The limited depth of energy penetration may confirm APC as a safe and beneficial tool for coagulation of human brain tissue. However, further clinical studies are required to evaluate the suitability and indications of this method in brain tumor treatment.

The objective of this study was to determine the feasibility, toxicity, and potential therapeutic benefits of an adjuvant active immunotherapy using a tumour specific ganglioside (GD2) conjugate for the adjuvant treatment of recurrent or progressive gliomas. Seven patients with proven GD2 expression in surgical specimens underwent a vaccination course with GD2-KLH/MPL-A conjugate. The follow-up was performed according to WHO guidelines regarding common toxicity criteria. Antibody titres against the ganglioside and the adjuvants were analysed. All patients developed a local type 4 reaction. Anti-GD2-antibody titres could not be detected, despite high titres against the immunoadjuvants. No tumour regression was observed. The disease remained stable for a median of 21.5 weeks (6-34 weeks). The median survival time after the first immunization was 47 weeks. The medial total survival time was 76 weeks. Adverse effects have not been observed. Active GD2-KLH/MPL-A immunization was technically feasible, but did not elicit anti-GD2 antibody generation.

In many cases, the treatment of neuropathic pain by intrathecal opioids fails to meet expectations. In a trial involving 10 patients, the intrathecal administration of clonidine combined with opioids in the treatment of chronic pain was introduced in our department for the first time. Eight patients with neuropathic pain syndromes were subjected to a continuous intrathecal clonidine application in addition to intrathecal morphine. At an average dose of 44 µg clonidine/day, a 70–100% reduction in pain was achieved. Residual non-neuropathic pain in 4 of 8 patients was successfully treated with clonidine and low doses of opioids. On the basis of the results achieved so far, we recommend that clonidine should be routinely tested for intrathecal drug administration, especially in patients with a prominent neuropathic pain component.

Over a 15-year period, 15 patients with craniospinal malformations were operated on using the transoral route. Ten patients had basilar impression and five had atlantoaxial dislocation; nine patients had associated lesions. Patients treated with reposition and dorsal fusion recovered well without ventral decompression. We found that transoral odontoidectomy is suitable as a primary decompressing measure only in ventral compression or nondislocated atlantoaxial conditions.

Continuous intrathecal baclofen infusion (CIBI) is a widely accepted therapy for the treatment of severe spinal spasticity. There is increasing evidence that CIBI has similar effects on patients with supraspinal spasticity resulting from either hypoxic or traumatic brain injury.1 A proportion of patients with supraspinal spasticity present with additional autonomic dysfunction. These dysfunctions are often unresponsive to conventional antiadrenergic medication. Patients may present with severe arterial hypertension, tachycardia, hyperhidrosis, hypersalivation, and bronchial hypersecretion. Mortality is mainly influenced by these symptoms. In addition to antiadrenergic medication sedatives and analgesics are often required. During intensive care treatment such medication often leads to a prolongation of artificial ventilation and delayed rehabilitation. During rehabilitation autonomic instability additionally interferes with physiotherapeutic activity. In the follow up of patients with severe supraspinal spasticity we noted the positive influence of intrathecal baclofen infusion on autonomic instability.1 Study of the literature did not disclose similar findings. So far, it had not yet been pointed out that CIBI has a positive influence on symptoms originating from severe hypoxic or traumatic brain damage. Eighteen patients with severe tetraspasticity from either hypoxic or traumatic brain injury were …

The complications of anterior discectomy without fusion were analyzed on the basis of 450 consecutive cases treated surgically for degenerative disc disease. There was no death related to the procedure. The most common complication was a worsening of the pre-existing myelopathy. This occurred in 3.3%, including one case with severe medullary contusion. Wound infection developed in 1.6%. Additional radicular symptoms and wound haematoma, respectively, occurred in 1.3%; recurrent nerve palsy, Horner's syndrome, and respiratory insufficiency, respectively, in 1.1% of the cases. Epidural haematoma and instability of the cervical spine, respectively, occurred in 0.9%, nerve root lesion and aseptic spondylodiscitis, respectively, in 0.4%. There was one case each (0.2%) with a pharyngeal lesion, meningitis due to dural perforation, transient additional myelopathy, and epidural abscess. The results and the management of complications are discussed in relation to numerous previously published reports, including posterior procedures and anterior fusion techniques. Precise knowledge of all potential accidents and pitfalls related to the surgical procedure and of their aetiology may contribute to preventing failures. The rate of complications in this series has been reduced in the past years by better patient selection, by paying more attention to correct positioning of the patient during the operation, and by meticulous removal of all offending structures. Discectomy without interbody fusion is now considered to be a reasonably safe procedure with an acceptable operative morbidity and lack of mortality.