Ludwig Benes

Cerebral cavernomas in the adult. Review of the literature and analysis of 72 surgically treated patients.

Abstract. The authors review the pertinent literature dealing with all aspects of cerebral cavernous malformations in the adult. Clinical, neuroradiological, pathological, and epidemiological aspects are presented. The clinical significance of bleeding from cavernous malformations and various hemorrhage patterns are discussed in relation to the factors that influence hemorrhage rates. Recent reports describing the genetic mechanisms of inheritance, de novo formation, and angiogenesis of cavernomas are reviewed as well. Brainstem cavernomas have received special attention, since their clinical management is controversial in the literature. Presently, microsurgical removal is favored by the majority of authors and stereotactic radiosurgery appears to be inappropriate for prevention of bleeding from a cavernoma. Our own case material consists of data of 72 patients operated upon during the past 5 years. Twenty-four patients harbored the lesion within the brainstem, 18 within the deep white matter of the hemispheres, 12 in the basal ganglia or thalamus, 11 in superficial areas of the hemisphere, and seven within the cerebellum. The perioperative morbidity rate was 29.2% (21/72) while the rate of long-term morbidity was 5.5% (4/72), with no mortality in this series. It is concluded that cerebral cavernous malformations, including lesions in critical regions of the brain, can be treated microsurgically with excellent results and an acceptable morbidity.

Initial hyperglycemia as an indicator of severity of the ictus in poor-grade patients with spontaneous subarachnoid hemorrhage

An association between hyperglycemia and outcome in spontaneous subarachnoid hemorrhage (SAH) has been sporadically reported. Our hypothesis was that hyperglycemia is a sign of central metabolic disturbance linked with specific appearances on computerized tomography (CT) scans reflecting different degrees of corresponding brain injury. The admission plasma glucose level, initial CT findings, and outcome after 6 months were analysed in a cohort of 99 patients with SAH in Hunt & Hess Grade IV or V. The CT scans were quantitatively assessed for subarachnoid blood, intracerebral hematoma, intraventricular hemorrhage, hydrocephalus, midline shift and compression of the perimesencephalic cisterns. These findings were combined to determine a three-point CT severity score. All patients showed elevated (>5.8 mmol/l) plasma glucose levels on admission. Mortality among 33 patients with glucose concentration below 9.0 mmol/l was 33.3%, 71.1% for the 45 patients with glucose level between 9.0 and 13.0 mmol/l, and 95.2% for the 21 patients with concentration above 13.0 mmol/l (P<0.0001). Glucose level was higher in Grade V than in Grade IV patients (mean+/-SD) (11.8+/-3.2 vs 9.8+/-2.9 mmol/l; P=0.0012). Patients with mild CT findings (n=10) had the lowest glucose level (8.9+/-1.8 mmol/l; P=0.0082), whereas patients with severe findings (n=56) had the highest glucose (11.4+/-3.5 mmol/l; P=0.011). Despite association with clinical grade and extent of CT findings, logistic multiple regression revealed the admission plasma glucose level to be an independent prognosticator of outcome. The prognostic potential of the initial plasma glucose level may be beneficial in management protocols of poor-grade SAH patients.

A novel platform for image-guided ultrasound.

OBJECTIVE:The combination of classic neuronavigation and intraoperative ultrasound is a recent innovation in image guidance technology. However, this technique requires two hardware components (neuronavigation and an ultrasound system). It was the aim of the study to describe a new simplified technology of a so-called one-platform navigation system developed by our institution in collaboration with the industry and to demonstrate its range of various applications. METHODS:An ultrasound device (IGSonic; BrainLAB, Munich, Germany) is integrated into the VectorVision2 navigation system (BrainLAB, Munich, Germany). The IGSonic Probe 10V5 is connected to the VectorVision Navigation station via an IGSonic Device Box. Once the ultrasound probe is calibrated, the navigated ultrasound displays the sonographic image of the intracranial anatomy on the navigation screen in a composed overlay fashion. It might depict vascular structures within the ultrasound plane by a duplex mode. Ultrasound can also be operated independently from navigation. RESULTS:The VectorVision2 system combines intraoperative ultrasound data sets with preoperatively acquired neuronavigation data sets in plug and play fashion. The system provides a cost-effective intraoperative imaging modality that offers a good anatomic orientation by various composite images, including the display of the amount of brain shift. In our institution, the comprehensible interface led to a routine use of the technology by several neurosurgeons who had not been familiar with the ultrasound technology before. CONCLUSION:The integration of an ultrasound device into an existing navigation system has been successfully developed. The system offers a friendly user interface and cost-effective intraoperative imaging feedback. Although brain shift can be visualized by an image overlay technology as demonstrated by the present system, future developments should aim at fusion techniques of both intra- and preoperative image data sets.

Is preoperative high-resolution magnetic resonance imaging accurate in predicting neurovascular compression in patients with trigeminal neuralgia? A single-blind study

High-resolution magnetic resonance imaging (HR-MRI) using three-dimensional fast imaging employing steady-state acquisition (3D-FIESTA) and double-dose contrast-enhanced three-dimensional fast spoiled gradient echo (3D-FSPGR) sequences is considered to be a useful tool in detecting neurovascular compression in patients with trigeminal neuralgia. The purpose of this study was to analyze the accuracy and preoperative diagnostic value of these high-resolution imaging techniques in patients with trigeminal neuralgia, in a single-blind study. The preoperative MRI images of 21 consecutive patients were matched to one neuroradiologist, who was blind as to which side exhibited the symptoms. The images and post-processing multiplanar reconstructions were compared with the video-documented operative observations. HR-MRI using only 3D-FSPGR sequences demonstrated neurovascular compression in accordance with the intraoperative finding in 11 patients (52.4%). In the subgroup where, additionally, 3D-FIESTA sequences were available, neurovascular compression was in accordance with the intraoperative finding in 71.4% (n=7). High-resolution magnetic resonance imaging using double-dose contrast-enhanced 3D-FSPGR and 3D-FIESTA sequences is currently not sufficient enough to make an accurate prediction of neurovascular compression in a single-blind setting. These 3D imaging techniques currently provide only limited information, and one should consider their use carefully when identifying patients with trigeminal neuralgia from operation until image quality is improved by superior image resolution that can accurately discriminate vessels surrounding the trigeminal root entry zone.

Image-guided transsylvian, transinsular approach for insular cavernous angiomas.

OBJECTIVE Surgical treatment of cavernomas arising in the insula is especially challenging because of the proximity to the internal capsule and lenticulostriate arteries. We present our technique of image guidance for operations on insular cavernomas and assess its clinical usefulness. METHODS Between 1997 and 2003, with the guidance of a frameless stereotactic system (BrainLab AG, Munich, Germany), we operated on eight patients who harbored an insular cavernoma. Neuronavigation was used for 1) accurate planning of the craniotomy, 2) identification of the distal sylvian fissure, and, finally, 3) finding the exact site for insular corticotomy. Postoperative clinical and neuroradiological evaluations were performed in each patient. RESULTS The navigation system worked properly in all eight neurosurgical patients. Exact planning of the approach and determination of the ideal trajectory of dissection toward the cavernoma was possible in every patient. All cavernomas were readily identified and completely removed by use of microsurgical techniques. No surgical complications occurred, and the postoperative course was uneventful in all patients. CONCLUSION Image guidance during surgery for insular cavernomas provides high accuracy for lesion targeting and permits excellent anatomic orientation. Accordingly, safe exposure can be obtained because of a tailored dissection of the sylvian fissure and minimal insular corticotomy.

Intraoperative ultrasound assistance in treatment of intradural spinal tumours

OBJECTIVE Currently, the standard practice to treat intradural spinal tumours involves microsurgical resection of the lesions. It is essential to be able to locate the lesion precisely to reduce the risk of neurological morbidity. The purpose of this study was to evaluate intraoperative ultrasonography (IOUS) in visualizing intradural spinal tumours, and assess its potential to improve surgical precision and minimize surgical trauma. METHODS Between January 2006 and July 2007, 30 patients with suspected intradural spinal tumours underwent surgery with the aid of IOUS. There were 13 patients with intramedullary tumours (ependymoma=2, astrocytoma=5, hemangioblastoma=2 and metastasis=4); and 14 patients with extramedullary tumours (meningioma=6, neurinoma=6, filum terminale ependymoma=1 and lipoma=1). In 3 patients histopathology did not reveal any neoplasm despite an MRI suggesting tumour. Their sonographic features are analyzed and the advantages of IOUS are discussed. RESULTS The shape and expansion of intradural tumours could be visualized on IOUS. The sonographic visualization allowed adapting the approach to an appropriate location and size before dura opening. Certain sonographic features can be used for a differential diagnosis of different intradural tumours. In addition, IOUS can inform neurosurgeons about the location of the neoplastic tissue, its relation to the spinal cord and the size of residual tumour following excision. CONCLUSIONS IOUS is a sensitive intraoperative tool. When appropriately applied to assist surgical procedures, it offers additional intraoperative information that helps to improve surgical precision and therefore might reduce the procedure related morbidity.

Transsulcal approach supported by navigation-guided neurophysiological monitoring for resection of paracentral cavernomas.

OBJECTIVES The aim of the study is to evaluate tools that can improve surgical precision and minimize surgical trauma for removal of cavernomas in the paracentral area. Moreover, the surgical strategies for the treatment of symptomatic epilepsy in cavernoma patients are discussed. PATIENTS AND METHODS Between June 2000 and July 2007, 17 patients suffering from paracentral cavernoma underwent surgery via a transsulcal approach with the aid of neuronavigation, functional mapping and neurophysiological intraoperative monitoring. To optimize outcome for procedures in the paracentral area, the hemosiderin-stained tissue was removed entirely except for a small proportion on the side of precentral gyrus. RESULTS All cavernomas and their adjacent sulci could be precisely located with the aid of ultrasonography-assisted neuronavigation. By combining preoperative fMRI and intraoperative neurophysiological monitoring, including SEP, MEP and cortical mapping, the motor cortex could be defined in all cases. Thus damage to the primary motor area could be avoided during resection of cavernomas. All the lesions located in the paracentral area were removed completely via transsulcal microsurgical approach without neurological deficits. No significant seizures were induced during surgery. CONCLUSIONS The successful excision of these lesions was effected by the following four key factors: (1) the precise location of the lesion supported by intraoperative neuronavigation; (2) the preservation of the eloquent area with the aid of functional mapping; (3) a minimally invasive transsulcal microsurgical approach; and (4) the entire removal of cavernoma and hemosiderin-stained tissue.

The role of underlying structural cause for epilepsy classification: Clinical features and prognosis in mesial temporal lobe epilepsy caused by hippocampal sclerosis versus cavernoma

Purpose:  The recent “Report of the ILAE Commission on Classification and Terminology” recommends an epilepsy classification that gives more emphasis to the underlying structural or metabolic cause rather than to the localization of the epileptogenic zone. The aim of the present study was to investigate differences in clinical features, treatment response, and prognosis in patients with mesial temporal lobe epilepsy (MTLE) caused by hippocampal sclerosis (MTLE‐HS) or singular mesiotemporal cavernomas (MTLE‐C) in order to evaluate the impact of underlying pathology on the course of the disease while controlling for localization.

Quality of life after brainstem cavernoma surgery in 71 patients.

BACKGROUND:Symptomatic patients with a brainstem cavernoma are treated surgically with increasing frequency. Generally, the patients benefit from this difficult surgical intervention is quantified by the assessment of neurological symptoms. OBJECTIVE:To document the beneficial effect of surgery in a larger patient population by assessing the postoperative quality of life (QoL). METHODS:In a series of 71 surgically treated patients, a detailed neurological status was assessed by Patzold Rating and Karnofsky Performance Status Scale. Patients rated their QoL with the Short Form 36 Health Survey. To document the effect of surgery on QoL, we devised a supplementary questionnaire. The last 24 patients completed Short Form 36 Health Survey pre- and postoperatively. RESULTS:Karnofsky Performance Status Scale improved in 44 of 71 surgical patients (62%), remained unchanged in 19 (27%), and deteriorated in 8 (11%) individuals. Patzold Rating showed a more detailed picture of the neurological symptoms. It correlated significantly with Karnofsky Performance Status Scale, which underscores its usefulness for patients with brainstem lesions. In the Short Form 36 Health Survey score, the Mental Component Summary improved with surgery (paired test, P = .015). In addition, 58 individuals (82%) declared a clear subjective benefit of surgery. CONCLUSION:The results of this large series support the notion that microsurgical removal of a brainstem cavernoma represents an effective therapy in experienced hands and is generally associated with good clinical outcome, both neurologically and in terms of QoL.

Candidate genes for the progression of malignant gliomas identified by microarray analysis

Malignant astrocytomas of World Health Organization (WHO) grade III or IV have a reduced median survival time, and possible pathways have been described for the progression of anaplastic astrocytomas and glioblastomas, but the molecular basis of malignant astrocytoma progression is still poorly understood. Microarray analysis provides the chance to accelerate studies by comparison of the expression of thousands of genes in these tumours and consequently identify targeting genes. We compared the transcriptional profile of 4,608 genes in tumours of 15 patients including 6 anaplastic astrocytomas (WHO grade III) and 9 glioblastomas (WHO grade IV) using microarray analysis. The microarray data were corroborated by real-time reverse transcription-polymerase chain reaction analysis of two selected genes. We identified 166 gene alterations with a fold change of 2 and higher whose mRNA levels differed (absolute value of the t statistic of 1.96) between the two malignant glioma groups. Further analyses confirmed same transcription directions for Olig2 and IL-13Rα2 in anaplastic astrocytomas as compared to glioblastomas. Microarray analyses with a close binary question reveal numerous interesting candidate genes, which need further histochemical testing after selection for confirmation. IL-13Rα2 and Olig2 have been identified and confirmed to be interesting candidate genes whose differential expression likely plays a role in malignant progression of astrocytomas.