Hearing preservation in medial vestibular schwannomas.

OBJECT: Vestibular schwannomas (VSs) with no or little extension into the internal auditory canal have been addressed as a clinical subentity carrying a poor prognosis regarding hearing preservation, which is attributed to the initially asymptomatic intracisternal growth pattern. The goal in this study was to assess hearing preservation in patients who underwent surgery for medial VSs. METHODS: A consecutive series of 31 cases in 30 patients with medial VSs (mean size 31 mm) who underwent surgery between 1997 and 2005 via a suboccipitolateral route was evaluated with respect to pre- and postoperative cochlear nerve function, extent of tumor removal, and radiological findings. Intraoperative monitoring of brainstem auditory evoked potentials was performed in all patients with hearing. Patients were reevaluated at a mean of 30 months following surgery. RESULTS: Preoperative hearing function revealed American Academy of Otolaryngology-Head and Neck Surgery Foundation Classes A and B in 7 patients each, Class C in 4, and D in 9. Four patients presented with deafness. Hearing preservation was achieved in 10 patients (Classes A-C in 2 patients each, and Class D in 4 patients). Tumor removal was complete in all patients with hearing preservation, except for 2 patients with neurofibromatosis. In 4 patients a planned subtotal excision was performed due to the individual's age or underlying disease. In 1 patient a recurrent tumor was completely removed 3 years after the initial procedure. CONCLUSIONS: The cochlear nerve in medial VSs requires special attention due to the atypical intracisternal growth pattern. Even in large tumors, hearing could be preserved in 37% of cases, since the cochlear nerve in medial schwannomas may not exhibit the adherence to the tumor capsule seen in tumors with comparable size involving the internal auditory canal.

Train time as a quantitative electromyographic parameter for facial nerve function in patients undergoing surgery for vestibular schwannoma.

OBJECT: The authors describe a quantitative electromyographic (EMG) parameter for intraoperative monitoring of facial nerve function during vestibular schwannoma removal. This parameter is based on the automated detection of A trains, an EMG pattern that is known to be associated with postoperative facial nerve paresis. METHODS: For this study, 40 patients were examined. During the entire operative procedure, free-running EMG signals were recorded in muscles targeted by the facial nerve. A software program specifically designed for this purpose was used to analyze these continuous recordings offline. By automatically adding up time intervals during which A trains occurred, a quantitative parameter was calculated, which was named "train time." A strong correlation between the length of train time (measured in seconds) and deterioration of postoperative facial nerve function was demonstrated. Certain consecutive safety thresholds at 0.5 and 10 seconds were defined. Their transgression reliably indicated postoperative facial nerve paresis. At less than a 10-second train time, discrete worsening, and at more than 10 seconds, profound deterioration of facial nerve function can be anticipated. CONCLUSIONS: Train time as a quantitative parameter was shown to be a reliable indicator of facial nerve paresis after surgery for vestibular schwannoma.

Baseline correction of intraoperative electromyography using discrete wavelet transform.

OBJECTIVE: In intraoperative analysis of electromygraphic signals (EMG) for monitoring purposes, baseline artefacts frequently pose considerable problems. Since artefact sources in the operating room can only be reduced to a limited degree, signal-processing methods are needed to correct the registered data online without major changes to the relevant data itself. We describe a method for baseline correction based on "discrete wavelet transform" (DWT) and evaluate its performance compared to commonly used digital filters. METHODS: EMG data from 10 patients who underwent removal of acoustic neuromas were processed. Effectiveness, preservation of relevant EMG patterns and processing speed of a DWT based correction method was assessed and compared to a range of commonly used Butterworth, Resistor-Capacitor and Gaussian filters. RESULTS: Butterworth and DWT filters showed better performance regarding artefact correction and pattern preservation compared to Resistor-Capacitor and Gaussian filters. Assuming equal weighting of both characteristics, DWT outperformed the other methods: While Butterworth, Resistor-Capacitor and Gaussian provided good pattern preservation, the effectiveness was low and vice versa, while DWT baseline correction at level 6 performed well in both characteristics. CONCLUSIONS: The DWT method allows reliable and efficient intraoperative baseline correction in real-time. It is superior to commonly used methods and may be crucial for intraoperative analysis of EMG data, for example for intraoperative assessment of facial nerve function.

A new clinico-pathological classification system for mesial temporal sclerosis.

We propose a histopathological classification system for hippocampal cell loss in patients suffering from mesial temporal lobe epilepsies (MTLE). One hundred and seventy-eight surgically resected specimens were microscopically examined with respect to neuronal cell loss in hippocampal subfields CA1-CA4 and dentate gyrus. Five distinct patterns were recognized within a consecutive cohort of anatomically well-preserved surgical specimens. The first group comprised hippocampi with neuronal cell densities not significantly different from age matched autopsy controls [no mesial temporal sclerosis (no MTS); n = 34, 19%]. A classical pattern with severe cell loss in CA1 and moderate neuronal loss in all other subfields excluding CA2 was observed in 33 cases (19%), whereas the vast majority of cases showed extensive neuronal cell loss in all hippocampal subfields (n = 94, 53%). Due to considerable similarities of neuronal cell loss patterns and clinical histories, we designated these two groups as MTS type 1a and 1b, respectively. We further distinguished two atypical variants characterized either by severe neuronal loss restricted to sector CA1 (MTS type 2; n = 10, 6%) or to the hilar region (MTS type 3, n = 7, 4%). Correlation with clinical data pointed to an early age of initial precipitating injury (IPI < 3 years) as important predictor of hippocampal pathology, i.e. MTS type 1a and 1b. In MTS type 2, IPIs were documented at a later age (mean 6 years), whereas in MTS type 3 and normal appearing hippocampus (no MTS) the first event appeared beyond the age of 13 and 16 years, respectively. In addition, postsurgical outcome was significantly worse in atypical MTS, especially MTS type 3 with only 28% of patients having seizure relief after 1-year follow-up period, compared to successful seizure control in MTS types 1a and 1b (72 and 73%). Our classification system appears suitable for stratifying the clinically heterogeneous group of MTLE patients also with respect to postsurgical outcome studies.

Split facial nerve course in vestibular schwannomas.

OBJECT: The facial nerve in vestibular schwannomas (VSs) is located on the ventral tumor surface in more than 90% of cases; other courses are rare. A split facial nerve course with two distinct bundles has thus far been described exclusively for medial extrameatal tumors. METHODS: Between 1996 and 2005, 16 consecutive cases of 241 surgically treated VSs were observed to have distinct splitting of the facial nerve. The mean tumor size measured 27 mm. In one third of the cases, intrameatal tumor extension with obliteration of the fundus was documented. All patients underwent extensive intraoperative neurophysiological monitoring using multichannel electromyography recordings. Patients were reevaluated 12 months after surgery. In all 16 patients, distinct splitting of the facial nerve was demonstrated. The major portion of the facial nerve followed a typical course on the ventral tumor surface. The smaller nerve portion in all cases ran parallel to the brainstem up to the level of the trigeminal root exit zone and crossed on the cranial tumor pole to the internal auditory canal. The two nerve portions rejoined at the level of the porus acusticus. The smaller portion carried fibers exclusively to the orbicularis oris muscle, whereas the major portion supplied all three branches of the facial nerve. CONCLUSIONS: In VSs, an aberrant course with distinct splitting of the facial nerve adds considerably to the surgical challenge. Long-term facial nerve results are excellent with extensive neurophysiological monitoring, which allows the differentiation and identification of aberrant facial nerve fibers and avoids additional risks to facial nerve preservation.

Preservation of facial nerve function after postoperative vasoactive treatment in vestibular schwannoma surgery.

OBJECTIVE: Facial nerve paresis and hearing loss are common complications after vestibular schwannoma surgery. Experimental and clinical studies point to a beneficial effect of nimodipine and hydroxyethyl starch for preservation of cochlear nerve function. A retrospective analysis was undertaken to evaluate the effect of vasoactive treatment on facial nerve outcome. PATIENTS AND METHODS: Forty-five patients with vestibular schwannoma removal, intraoperative electromyographic monitoring, and postoperative deterioration of facial nerve function were evaluated. Twenty-five patients underwent vasoactive treatment consisting of nimodipine and hydroxyethyl starch for improvement of hearing outcome. Twenty patients did not receive such treatment. Facial nerve function was evaluated before and after surgery, as well as 1 year after the surgical procedure. Patients were comparable regarding age, tumor size, and preoperative facial nerve function. RESULTS: Long-term results of facial nerve function were significantly improved in those patients who experienced severe postoperative deterioration of facial nerve function and received vasoactive treatment as compared with patients who did not receive nimodipine and hydroxyethyl starch after surgery. Treated patients showed a significantly higher rate of complete recovery compared with patients without treatment. CONCLUSION: The study points to a potential effect of vasoactive treatment for facial nerve function after vestibular schwannoma surgery. In particular, patients with postoperative disfiguring facial nerve palsy clearly benefit from intravenous hydroxyethyl starch and nimodipine with respect to a long-term socially acceptable facial nerve function.

In vitro and ex vivo evaluation of second-generation histone deacetylase inhibitors for the treatment of spinal muscular atrophy.

Among a panel of histone deacetylase (HDAC) inhibitors investigated, suberoylanilide hydroxamic acid (SAHA) evolved as a potent and non-toxic candidate drug for the treatment of spinal muscular atrophy (SMA), an alpha-motoneurone disorder caused by insufficient survival motor neuron (SMN) protein levels. SAHA increased SMN levels at low micromolar concentrations in several neuroectodermal tissues, including rat hippocampal brain slices and motoneurone-rich cell fractions, and its therapeutic capacity was confirmed using a novel human brain slice culture assay. SAHA activated survival motor neuron gene 2 (SMN2), the target gene for SMA therapy, and inhibited HDACs at submicromolar doses, providing evidence that SAHA is more efficient than the HDAC inhibitor valproic acid, which is under clinical investigation for SMA treatment. In contrast to SAHA, the compounds m-Carboxycinnamic acid bis-Hydroxamide, suberoyl bishydroxamic acid and M344 displayed unfavourable toxicity profiles, whereas MS-275 failed to increase SMN levels. Clinical trials have revealed that SAHA, which is under investigation for cancer treatment, has a good oral bioavailability and is well tolerated, allowing in vivo concentrations shown to increase SMN levels to be achieved. Because SAHA crosses the blood-brain barrier, oral administration may allow deceleration of progressive alpha-motoneurone degeneration by epigenetic SMN2 gene activation.

Intraoperative high-field-strength MR imaging: implementation and experience in 200 patients.

PURPOSE: To review the initial clinical experience with intraoperative high-field-strength magnetic resonance (MR) imaging of brain lesions in 200 patients. MATERIALS AND METHODS: Two hundred patients (mean age, 46.1 years; range, 7-84 years), most of whom had glioma or pituitary adenoma, were examined with a 1.5-T MR imager equipped with a rotating operating table and located in a radiofrequency-shielded operating theater. A navigation microscope placed inside the 0.5-mT zone and used in combination with a ceiling-mounted navigation system enabled integrated microscope-based neuronavigation. The extent of resection depicted at intraoperative imaging, the surgical consequences of intraoperative imaging, and the clinical practicability of the operating room setup were analyzed. RESULTS: Seventy-seven resections with a transsphenoidal approach, 100 craniotomies, and 23 burr-hole procedures were performed. In 55 (27.5%) of 200 patients, intraoperative MR imaging had immediate surgical consequences (eg, extension of resection in 39% of patients with pituitary adenoma or glioma). In 108 patients the navigation system was used, and for 37 of those patients, functional imaging data were integrated into the navigation system. There was nearly no difference in quality between pre- and intraoperative images. Intraoperative workflow with intraoperative patient transport for imaging was straightforward, and imaging in most cases began less than 2 minutes after sterile covering of the surgical site. No complications resulted from high-field-strength MR imaging. CONCLUSION: The high-field-strength MR imager was successfully adapted for intraoperative use with the integrated neuronavigation system. Intraoperative MR imaging provided valuable information that allowed intraoperative modification of the surgical strategy.

Ictal pleasant sensations: cerebral localization and lateralization.

Ictal pleasant feelings are a rare sign of focal epilepsies. The most popular description was performed by Dostojevskij, who reported an aura by Myshken in one of his books. No convincing evidence has been published concerning the cerebral localization of ictal happiness. In this study, the findings of 11 patients with ictal pleasant feelings are described. In eight patients, the origin of the focal epileptic activity was found in the temporal lobe (most often temporal inferior basal); in three patients, frontal or parietal lobe in addition to temporal lobe involvement was found. According to our findings ictal happiness is a localizing sign pointing to the ictal involvement of temporal mesiobasal areas. Lateralization to the right temporal lobe was found in seven and to the left temporal lobe in four patients.

Standardized evaluation of CT angiography with remote generation of 3D video sequences for the detection of intracranial aneurysms.

Computed tomography (CT) angiography is a well-known imaging technique commonly applied to both the detection and therapy planning of intracranial aneurysms. For this purpose, current studies predominantly focus on three-dimensional (3D) representations of CT angiographic volumes obtained with varying visualization approaches on different computers. Interactive manipulation performed by users individually is an important prerequisite for data analysis. However, this leads to inconsistent and barely reproducible 3D visualization results. Furthermore, the quality of any 3D representation depends on the applied visualization strategy (eg, maximum-intensity projection, shaded-surface display, direct volume rendering). To overcome these limitations, the authors present a novel method for standardized visualization of CT angiographic volumes, consisting of three steps: (a) transfer of the image data to a remote high-end graphics workstation, (b) automatic 3D visualization with high-resolution direct volume rendering, and (c) consecutive video generation performed according to a standardized protocol. The recorded video sequences are transferred for evaluation to a local desktop computer. In the experimental setup, high-quality videos based on 3D visualizations were produced in less than 60 minutes per patient. Although aneurysms above the skull base are usually visualized with excellent quality, the analysis of aneurysms at the skull base is still difficult.