[Insular epilepsy: The Montreal experience]. / L'épilepsie insulaire : l'expérience montréalaise.

In this article, we review recently published data on the role of the insula in refractory partial epilepsy and summarize our own experience in the investigation and treatment of this entity. Case studies and evoked responses obtained from insular cortical stimulation reveal a wide array of clinical manifestations which may mimic temporal, frontal or parietal lobe seizures. Clinicians should hence lower their threshold to sample the insula with intracerebral electrodes. Lack of recognition of insular seizures may explain part of epilepsy surgery failures. Advances in microneurosurgery open the way to safer insular resection.

Cerebellar lesions: is there a lateralisation effect on memory deficits?

BACKGROUND: Until recently, neurosurgeons eagerly removed cerebellar lesions without consideration of future cognitive impairment that might be caused by the resection. In children, transient cerebellar mutism after resection has lead to a diminished use of midline approaches and vermis transection, as well as reduced retraction of the cerebellar hemispheres. The role of the cerebellum in higher cognitive functions beyond coordination and motor control has recently attracted significant interest in the scientific community, and might change the neurosurgical approach to these lesions. The aim of this study was to investigate the specific effects of cerebellar lesions on memory, and to assess a possible lateralisation effect. METHODS: We studied 16 patients diagnosed with a cerebellar lesion, from January 1997 to April 2005, in the "Centre Hospitalier Universitaire Vaudois (CHUV)", Lausanne, Switzerland. Different neuropsychological tests assessing short term and anterograde memory, verbal and visuo-spatial modalities were performed pre-operatively. RESULTS: Severe memory deficits in at least one modality were identified in a majority (81%) of patients with cerebellar lesions. Only 1 patient (6%) had no memory deficit. In our series lateralisation of the lesion did not lead to a significant difference in verbal or visuo-spatial memory deficits. FINDINGS: These findings are consistent with findings in the literature concerning memory deficits in isolated cerebellar lesions. These can be explained by anatomical pathways. However, the cross-lateralisation theory cannot be demonstrated in our series. The high percentage of patients with a cerebellar lesion who demonstrate memory deficits should lead us to assess memory in all patients with cerebellar lesions.

Sub-acute delayed failure of subthalamic DBS in Parkinson's disease: the role of micro-lesion effect.

OBJECTIVE: To study delayed failure after subthalamic nucleus (STN) deep brain stimulation in Parkinson's disease (PD) patients. METHODS: Out of 56 consecutive bilaterally STN-implanted PD patients, we selected subjects who, after initial clinical improvement (1 month after surgery), lost benefit (delayed failure, DF). RESULTS: Five patients developed sub-acutely severe gait disorders (DF). In 4/5 DF patients, a micro-lesion effect, defined as improvement without stimulation, was observed; immediate post-operative MRI demonstrated electrode located above or behind to the STN. CONCLUSIONS: Patients presenting micro-lesion effect should be carefully monitored, as this phenomenon can mask electrodes misplacement and evolution in DF.

Progressive unilateral hemispheric atrophy in an infant with neurofibromatosis.

INTRODUCTION: Cerebrovascular diseases are rarely seen in neurofibromatosis type 1. These include vascular occlusive disease, moyamoya vessels, aneurysms, arteriovenous malformations and fistulae. CASE REPORT: We describe the case of an infant with genetically proven neurofibromatosis type 1 and progressive brain hemiatrophy over months, due to primary narrowing of intracranial carotid artery branches, as demonstrated by successive brain imaging. She presented with refractory seizures and a progressive hemiparesis associated with developmental delay. Surgical material from hemispherotomy done at 18 months showed severe abnormalities of the small vessels. CONCLUSION: Cerebrovascular changes seen in neurofibromatosis can be diffuse and progressive, with secondary hemiparesis, epilepsy and developmental delay.

Rationale, mechanisms of efficacy, anatomical targets and future prospects of electrical deep brain stimulation for epilepsy.

Electrical stimulation of deep brain structures is a promising new technology for the treatment of medically intractable seizures. Performed in vitro and on animal models of epilepsy, electrical stimulation has shown to reduce seizure frequency. Preliminary results on humans are encouraging. However, such improvements emerge despite a lack of understanding of the precise mechanisms underlying electrical stimulation either delivered directly on the epileptogenic zone (direct control) or through an anatomical relay of cortico-subcortical networks (remote control). Anatomical targets such as the thalamus (centromedian nucleus, anterior thalamus, mamillary body and mamillothalamic tracts), the subthalamic nucleus, the caudate nucleus and direct stimulation of the hippocampal formation have been successfully investigated. Although randomized controlled studies are still missing, deep brain stimulation is a promising treatment option for a subgroup of carefully selected patients with intractable epilepsy who are not candidates for resective surgery. The effectiveness, the optimal anatomic targets, the ideal stimulation parameters and devices, as well as patient selection criteria are still to be defined.

[Indications and results of stereotactic radiosurgery with LINAC for the treatment of acoustic neuromas: preliminary results]. / Indications et résultats du traitement des neurinomes de l'acoustique par radiothérapie stéréotaxique avec LINAC: résultats préliminaires.

INTRODUCTION: Radiosurgery is presently becoming an alternative to microsurgical resection of acoustic neuromas. The interest of radiosurgery consists in its lower morbidity compared to surgery and likely in similar rates of long-term tumor control. The goal of our study was to assess the clinical outcome (hearing preservation and neurological complications) as well as tumor control after low-dose radiosurgery for unilateral acoustic neuromas. MATERIAL AND METHODS: Since April 2002, 22 patients with untreated acoustic neuromas underwent stereotactic radiosurgery using a linear accelerator (LINAC) and a micromultileaf collimator (mMLC, Brain Lab) at a low-dose of 12 Gy. The average age was 56.4 years (range 29-73 years). The treatment volume was 0.03 to 6.04 cm(3) (median 1.85 cm(3)). The median follow-up period was 18 months (range 6-36 months). RESULTS: No morbidity was observed during the treatment. Preservation of a serviceable hearing (classes I and II according to the Gardner-Robertson scale) was achieved in 10 of 14 patients (71%). Radiological tumor growth control was obtained in all patients (100%). Trigeminal neuropathy was observed in two patients. One of these patients also experienced a slight facial weakness. CONCLUSIONS: Low dose radiosurgery provides a low rate of post-therapeutic morbidity and yields the preservation of a serviceable hearing in 70% of cases. Tumor control is observed in all patients, but a longer follow-up period is needed to confirm the stability of the tumor size.

Third ventriculostomy vs ventriculoperitoneal shunt in pediatric obstructive hydrocephalus: results from a Swiss series and literature review.

INTRODUCTION: Few series compare endoscopic third ventriculostomies (ETV) and ventriculoperitoneal shunts (VPS). To avoid the complications after a shunt insertion, there is an increased tendency to perform a third ventriculostomy. We reviewed all pediatric patients operated in the French-speaking part of Switzerland for a newly diagnosed obstructive hydrocephalus since 1992 and compared the outcome of patients who benefited from ETV to the outcome of patients who benefited from VPS. There were 24 ETV and 31 VPS. DISCUSSION: At 5 years of follow-up, the failure rate of ETV was 26%, as compared to 42% for the VPS group. This trend is also found in the pediatric series published since 1990 (27 peer-reviewed articles analyzed). CONCLUSION: In accordance to this trend, although a statistical difference cannot be assessed, we believe that ETV should be the procedure of choice in pediatric obstructive hydrocephalus.

Speech arrest with stimulation may not reliably predict language deficit after epilepsy surgery.

The authors present a patient in whom electrical cortical stimulation of the posterior temporal cortex induced speech arrest, comprehension deficits, and other language-related impairments. This area was ultimately resected because of persistence of a severe seizure disorder. No postoperative aphasia was observed despite the cortical stimulation results, and the patient is since seizure free. These findings question the well-established principle that corticography directly reflects local cortical functions in all patients.

Early surgery for brainstem cavernomas.

BACKGROUND: The purpose was to review our experience with the surgical management of brainstem cavernomas (BSCs) and especially the impact of the surgical timing on the clinical outcome. METHOD: We retrospectively reviewed 22 patients harboring a BSC, who underwent 23 procedures. FINDINGS: Surgery was carried out during the early stage after the last haemorrhage, with a mean delay of 21.6 days (range 4-90 days). Sixteen procedures were performed after a first bleeding event while seven after multiple bleedings. Complete resection was achieved in 19 patients (86.4%). Early after surgery, 12 patients (52.2%) improved neurologically, 5 (21.7%) were stable and 6 (26.1%) worsened. New postoperative deficits were noted after 9 procedures (39.1%). Statistically significant factors for postoperative aggravation were: late surgery (P = 0.046) and multiple bleedings (P = 0.043). No patient operated on within the first 19 days after bleeding did worsen (n = 11), as opposed to 6 out of 12 who did when operated on later. After a mean follow-up of 44.9 months, 20 patients (90.9%) were improved, 1 patient (4.6%) was worse and 1 patient was lost to follow-up (4.6%), after reoperation for rebleeding of a previously completely resected cavernoma. Late morbidity was reduced to 8.6%. The mean Glasgow Outcome Scale (GOS) at the end of the follow-up period was 4.24, compared to a mean preoperative GOS of 3.22 (P<0.001). Complete neurological recovery of motor deficits, sensory disturbances, cranial nerves (CNs), internuclear ophtalmoplegia and cerebellar dysfunction were respectively 41.7%, 38.5%, 52.6%, 60.0% and 58.3%. Among the most affected CNs: CN 3, CN 5 and CN 7 were more prone to completely recover, respectively in 60.0%, 70.0% and 69.2%. CONCLUSIONS: Surgical removal of BSCs is feasible in experienced hands with acceptable morbidity and good outcome. Early surgery and single bleeding were associated with better surgical results.

[Current treatment of Parkinson's disease: problems and controversies]. / Traitement actuel de la maladie de Parkinson: difficultés et controverses.

Treating patients with Parkinson's disease is not an easy task for the physician who is facing a disease well responsive to symptomatic therapy, yet escaping any curative approaches. In spite of the large therapeutic armamentarium available, many issues remained unsolved, as indications of a particular therapeutic agent are only loosely defined and evolving according to various parameters such as disease progression and severity, the profile of potentially serious adverse effects, the physician's level of expertise and patient's expectations. The growing experience acquired with subthalamic nucleus deep brain stimulation has shown that indications for such a surgery have to be cautiously examined. After initial therapeutic enthusiasm, we are now at a time of problems and controversies.

[Current epilepsy treatment in adults]. / Le traitement actuet des épitepsies chez l'adulte.

The prevalence of epilepsy is about 1%. Only two thirds of these patients respond satisfactorily to an antiepileptic drug (AED) treatment. New AED did not clearly improve this overall efficacy, but often show a better tolerability as compared to old AED. This may allow a more targeted choice, especially in some delicate clinical situations, such as for the treatment of women in childbearing age, or patients receiving other drugs with possible pharmacokinetic interactions. Invasive approaches should be considered early in the course of treatment-resistant epilepsy, and may offer a complete seizure remission in selected cases. On the background of recent acquisitions from the literature, the pros and cons of different treatment options are presented. This is followed by the discussion of some clinical relevant situations.

The role of posterior fossa decompression in acute cerebellitis.

BACKGROUND: We present two cases of children who were diagnosed with cerebellitis with acute cerebellar swelling. This rare pathology is potentially fatal, and no clear treatment guidelines are described in the literature. DISCUSSION: Considering our experience, we discuss the different therapeutic strategies and propose aggressive surgical measures consisting of external ventricular drainage and posterior fossa decompression in case of failure of early response to medical treatment to limit secondary cerebellar and brainstem lesions.

Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement.

Rasmussen encephalitis (RE) is a rare but severe immune-mediated brain disorder leading to unilateral hemispheric atrophy, associated progressive neurological dysfunction and intractable seizures. Recent data on the pathogenesis of the disease, its clinical and paraclinical presentation, and therapeutic approaches are summarized. Based on these data, we propose formal diagnostic criteria and a therapeutic pathway for the management of RE patients.

Electrophysiological characteristics of limbic and motor globus pallidus internus (GPI) neurons in two cases of Lesch-Nyhan syndrome.

OBJECTIVE: Lesch-Nyhan syndrome is a rare and debilitating condition characterized by dystonia and self-mutilating behavior. In order to shed light on the pathophysiology of dystonia, we report the pallidal electrophysiological activity recorded in two patients during deep brain stimulation surgery (DBS). METHODS: Microrecordings were performed on 162 neurons along four tracks aimed at the right and left anterior (limbic) and posterior (motor) globus pallidus internus (GPI). RESULTS: Regardless of the anesthetic agent used (propofol or sevoflurane), both patients showed similar neurons firing rates in the four regions studied, namely the limbic and motor portions of the globus pallidus externus (GPE) or GPI. In both patients, firing rates were similar in the GPE (12.2+/-1.8 Hz, N=38) and GPI (13.2+/-1.0 Hz, N=83) portions of the limbic track, while the motor GPE fired at a higher frequency (23.8+/-2.7 Hz, N=18) than the motor GPI (12.5+/-1.4 Hz, N=23). CONCLUSIONS: These results demonstrate that light propofol or sevoflurane anesthesia influences pallidal activity in a similar way. Electrophysiological recordings suggest that Lesch-Nyhan syndrome might be characterized by analogous firing frequencies in the limbic GPE and GPI while motor GPE would tend to fire at higher rate than the motor GPI. It is therefore tempting to suggest that the symptoms that are observed in Lesch-Nyhan syndrome might result from motor GPI inhibition. SIGNIFICANCE: This observation may confirm the Albin and Delong's model of the basal nuclei in hypokinetic and hyperkinetic disorders.

Suicide after successful deep brain stimulation for movement disorders.

The authors observed a high rate of suicide (6/140 patients, 4.3%) in a large cohort of patients with movement disorders treated with deep brain stimulation (DBS). Apparent risk factors included a previous history of severe depression and multiple successive DBS surgeries, whereas there was no relationship with the underlying condition, DBS target, electrical parameters, or modifications of treatment. Paradoxically, all patients experienced an excellent motor outcome following the procedure. The authors propose that patients at high risk for suicide should be excluded from DBS surgery.

Subthalamic nucleus deep brain stimulation in Parkinson disease patients over age 70 years.

The effects of subthalamic nucleus deep brain stimulation were studied in 52 consecutive patients (13 over age 70, 15 under age 60, 24 age 60 to 70). All groups had improvement of motor fluctuations and dyskinesia. Patients over age 70 had worsening of Unified Parkinson's Disease Rating Scale motor scores on medication, despite less medication reduction. Their activities of daily living and axial subscores worsened, particularly in those with preoperative gait difficulties.

L-dopa-induced dyskinesia improvement after STN-DBS depends upon medication reduction.

The authors studied the long-term evolution of levodopa-induced dyskinesia (LID) after levodopa challenge in two groups of six STN-deep brain stimulation-treated Parkinson disease (PD) patients, one requiring medication after surgery and the other not. A dramatic (96%) reduction of LID severity was obtained in the six postoperatively untreated patients compared to a moderate improvement (47%) in the treated group (p < 0.03). These data support dopaminergic stimulation and striatal desensitization as major determinants of LID in PD.

Early acquisition of typical metabolic features upon differentiation of mouse neural stem cells into astrocytes.

Specific metabolic features, such as glutamate reuptake, have been associated with normal functions of mature astrocytes. In this study, we examined whether these characteristics are acquired together with classical phenotypic markers of differentiated astrocytes. Differentiation of E14 mouse neurospheres into astrocytes was induced by the addition of fetal bovine serum (FBS). Degree of differentiation was assessed by reverse transcription-polymerase chain reaction (RT-PCR) and immunofluorescence for both GFAP and nestin. Neural stem cells expressed nestin but not GFAP, while differentiated astrocytes were immunopositive for GFAP but displayed low levels of nestin expression. A strong increase in the expression of the glutamate transporter GLAST and the monocarboxylate transporter MCT1 accompanied phenotypic changes. In addition, active glutamate transport appeared in differentiated astrocytes, as well as their capacity to increase aerobic glycolysis in response to glutamate. Leukemia inhibitory factor (LIF) and ciliary neurotrophic factor, but not interleukin-6, triggered the expression of phenotypic and morphological characteristics of astrocytes. In addition, exposure to LIF led to the appearance of metabolic features typically associated with astrocytes. Altogether, our results show that acquisition of some specific metabolic features by astrocytes occurs early in their differentiation process and that LIF represents a candidate signal to induce their expression.