Drug-resistant temporal lobe epilepsy is associated with postural control abnormalities.

Epilepsy is responsible for falls that are not systematically associated with seizures and that therefore suggest postural impairment. There are very few studies of postural control in patients with epilepsy and none of them focus on temporal lobe epilepsy (TLE), although part of the vestibular cortex is located in the temporal cortex. The aim of this study was to evaluate the characteristics of postural control in a homogeneous population of patients with complex partial TLE. Twenty-six patients with epilepsy and 26 age-matched healthy controls underwent a sensory organization test combining six conditions, with and without sensory conflicting situations. Patients with epilepsy displayed poorer postural control, especially in situations where vestibular information is necessary to control balance. In addition to potential antiepileptic drug side effects, vestibular dysfunction could be related to the temporal pathology. Our study allows for a better understanding of the mechanism underlying falls in this population of patients.

[Cranial nerve functional neurosurgery: evaluation of surgical practice]. / La neurochirurgie fonctionnelle des nerfs crâniens Evaluation de l'activité

We report the results of an investigation carried out on the activity of functional neurosurgery of the cranial nerves in the French-speaking countries, based on the analysis of a questionnaire addressed to all the members of the SNCLF. Eighteen centers responded to this questionnaire, which showed that activities and indications varied greatly from one unit to another. The results appear homogeneous and comparable with those reported in the literature. The questionnaire sought to provide a global perspective, open to the comments and questions of all responders on the various techniques raised, with the objective of establishing a common decisional tree for these pathologies and providing if possible to a consensus for better dissemination of these therapies.

[Germ-cell tumors of the central nervous system in childhood: retrospective study of 13 patients]. / Les tumeurs germinales du système nerveux central de l'enfant : étude rétrospective de 13 patients.

BACKGROUND AND PURPOSE: Germ cell tumors (GCT) of the central nervous system are rare (2% of all brain tumors in children). Although originating from germ cells, GCT cover a spectrum of different tumors with different management and prognosis, depending on whether they secrete tumor markers or not. The aim of this study is to present a series of children with GCT and comment on overall management practices. METHODS: We retrospectively reviewed 13 children under the age of 18 years (nine boys and four girls), treated in the same institution between 1986 and 2006 for one or more primitive GCT of the central nervous system. RESULTS: Median age at diagnosis is 12.9 years (7-17 years). Tumor markers (alpha foetoprotein [alphaFP], human chorionic gonadotrophin [betaHCG]) were assessed 11 times in blood as well as cerebrospinal fluid (CSF). Tumors were located as follows: pineal region (10 cases), hypothalamus (eight cases), basal ganglia (one case) and corpus callosum (one case). Six were bifocal (pineal region and hypothalamus). Clinical signs were mostly dominated by diabetes insipidus and intracranial hypertension. Seven children required surgery for hydrocephalus. Tumor markers were positive in three cases and these children subsequently received chemotherapy followed by radiotherapy, except one child. Eventually, the three patients with positive markers required surgery because of a residual lesion. The eight other patients had a stereotactic biopsy for diagnosis. At the end of follow-up, treatment morbidity appears to be low and neither death nor recurrence was observed. Mean follow-up is 8.85 years (2-20 years). CONCLUSIONS: The prognosis of cerebral GCTs in children is excellent because of their pronounced chemo- and radiosensitivity. Surgery is crucial for diagnosis in the event of negative markers, or if there is evidence of residual tumor with normalization of tumor markers at the end of chemotherapy. Tumor markers must be monitored to check the diagnosis and for follow-up. The place of tumor biopsy during endoscopic third ventriculostomy (performed if hydrocephalus is present) is still debated.

Predicting the outcome of shunt surgery in normal pressure hydrocephalus.

We studied retrospectively the effectiveness of the repeated lumbar CSF tap test (RTT), lumbar external CSF drainage (LED) and radioisotope cisternography (RIC) in predicting the outcome of shunt surgery, as well as the diagnostic and prognostic value of periventricular hyperintensity (PVH) and of the classic clinical triad in normal pressure hydrocephalus. Two hundred and seventy patients were referred to the Departments of Neurosurgery, in Nancy, France and in Istanbul, Turkey. The decision to perform surgery was based on the clinical presentation (all patients had at least two symptoms of the classic clinical triad), neuroimaging examinations and the results of the RTT (taps were performed on three consecutive days and at each tap a minimum of 30 to 40 cc of CSF was removed), the LED (drainage was performed for 3 days and the volume of CSF drained daily was a minimum of 150 to 250 cc) or the RIC. After all shunt procedures, postoperative assessments verified improvements in 88% of the RTT group, 91% of the LED group and 66% of the RIC group. Gait disturbance had improved in 90% at the end of the second and twelfth month follow-up. Cognitive dysfunction had improved in 79% at the second and in 77% at the twelfth month follow-up. Urinary incontinence had improved in 66% at the second and in 62% at the twelfth month follow-up. From the surgical point of view, the greatest difficulty is not to make the diagnosis, but rather to identify the appropriate patients to operate on. The decision to perform shunt surgery should be based on strict clinical findings associated with CT and MRI criteria and especially with positive RTT or LED test results.

[Natural history of intramedullary cavernomas. Results of the French Multicentric Study]. / Histoire naturelle des cavernomes intramédullaires et traitement chirurgical. Résultats de l'étude française multicentrique.

INTRODUCTION: The prevalence of cerebral cavernomas is about 0.5% in the general population. In contrast, spinal cord cavernomas are considered as rare. The objective of this study was to determine the natural history of spinal cord cavernomas in a multicentric study. METHODS: Clinical and neuroradiological findings were retrospectively collected. Diagnosis was based on pathological criteria or magnetic resonance (MR) findings. RESULTS: Fifty-three patients were included (26 males, 27 females). Mean age at onset of symptoms was 40.2 years (range: 11-80). Initial symptoms were progressive (32) and acute myelopathy (20). One patient was asymptomatic. Clinical symptoms were related to spinal cord compression (24) and hematomyelia (19). Cavernoma location was dorsal (41) and cervical (12.). MR findings consisted of hyperintense signal on T1 and T2 sequences (19 cases), mixed hyperintense and hypointense signal (33 cases), and hypointense signal on T1 and T2 sequences in 1 case. Mean size was 16.3 mm (range: 3-54). Forty patients underwent surgical resection. Improvement was observed in 20 patients and worsening of neurological symptoms in 11. Length of follow up was 7.1 years. At the end of the study, 26 patients were autonomous, 18 handicapped and 1 bedridden. CONCLUSION: This study provided precise data on the clinical and MR patterns of these lesions. The natural history is associated with a higher risk of hemorrhage recurrence, but is favorable in many operated patients. Microsurgery is the treatment of choice for most of these lesions.

[Childhood diencephalic pilocytic astrocytoma. A review of seven observations]. / Les astrocytomes pilocytiques du diencephale de l'enfant. à propos de sept observations.

BACKGROUND AND PURPOSE: Pilocytic astrocytoma (PA) is a WHO grade I tumor of the central nervous system mostly arising in children and young adults. Management of diencephalic PA is a difficult challenge. Surgical treatment has to cope with both the difficulties of deep location and eloquent area tumors. MATERIALS AND METHODS: We retrospectively reviewed seven pediatric cases (female: 4, male: 3) of diencephalic PA. Opto-chiasmatic tumors were excluded from the series. Mean age at diagnosis was 108 months (9 years) (range: 4 month-18 years), median age was 111 months. Median follow-up for the series was 125 months. Tumor locations were as followed: right thalamus: 2, both thalami: 1, hypothalamus: 3, and right basal ganglia: 1. At the onset, the first symptom was mostly raised intracranial pressure. The delay in diagnosis ranged from 48 hours up to 6 years. TREATMENT: a shunting procedure was performed in 3 patients, a direct surgical approach in 5 patients (gross total removal: 2; partial removal: 3) and one patient had only a biopsy. Three children were re-operated. Three patients were treated by radiationtherapy (RT) after surgery. Chemotherapy was delivered for 4 children. RESULTS: The overall survival rate was 71.4 months (almost 6 years) (range: 3-184 months). Median survival rate was 42 months (3.5 years). Three children died, two by tumor progression and one death related to late side-effects of RT. Four patients have a good quality of life with GOS I (n = 3) or II (n = 1). We observed tumor regression in two patients at 1 and 17 years after the beginning of treatment. Correct diagnosis was only made for two cases at the initial pathological examination. CONCLUSION: The course of diencephalic PA is still unpredictable. The tumor can be controlled by a partial surgical removal, and a residual tumor can sometimes decrease in size after surgery. Gross total removal of these tumors, although difficult, may be performed. With cranial navigation systems, the risk is low. Pathological diagnosis is sometimes difficult to assess.

[Subdural empyema in children: therapeutic strategy. Five cases]. / Empyèmes sous-duraux de l'enfant: stratégie thérapeutique. A propos de cinq cas.

We report a series of five subdural empyema (SDE) in children and young adults treated in the same neurosurgical department. These five cases were reviewed retrospectively. There were four boys and one girl, aged from three months to 18 years at time of diagnosis (median age: 7 years). SDE following intracranial surgery were excluded from the study. All patients were treated surgically (burr hole evacuation or craniotomy, repeated in some cases), followed by intravenous antibiotic therapy (mean time: 52 days) adapted to the micro-organism. Only the two patients treated by large craniotomy at first had a single surgical procedure. Involved micro-organisms are as follow: Streptococcus intermedius (n=2), Streptococcus pneumoniae (n=1), Escherichia coli (n=1), absence of any identified micro-organism (n=1). The five patients are alive (median follow-up: 22 month) without any sequelae. We advocate an aggressive surgical treatment of SDE in children with a large bone flap to allow the surgeon to remove pus and membranes as much as possible, even in the interhemispheric fissure, followed by intravenous appropriate antibiotherapy and eradication of the source of infection. Even this "aggressive" treatment may sometimes not avoid re-operation. A careful follow-up is mandatory, because of the high risk of recurrence.

Neurosurgery and the intracranial venous system.

1) Numerous of the so-called "unpredictable" post-operative complications are likely to be related to the lack of prevention or non-recognition of venous problems, especially damages to the dangerous venous structures, namely: the major dural sinuses, the deep cerebral veins and some of the dominant superficial veins like the vein of Labbé. 2) Tumors invading the major dural sinuses (superior sagittal sinus, torcular, transverse sinus)--especially meningiomas--leave the surgeon confronted with a dilemma: leave the fragment invading the sinus and have a higher risk of recurrence, or attempt at total removal with or without venous reconstruction and expose the patient to a potentially greater operative danger. Such situations have been encountered in 106 patients over the last 25 years. For decision-making, meningiomas were classified into six types according to the degree of sinus invasion. Type 1: meningioma attached to outer surface of the sinus wall; Type II: one lateral recess invaded; Type III: one lateral wall invaded; Type IV: one lateral wall and the roof of the sinus both invaded; Types V and VI: sinus totally occluded, one wall being free of tumor in type V. In brief, our surgical policy was the following: Type I: excision of outer layer and coagulation of dural attachment; Type II: removal of intraluminal fragment through the recess, then repair of the dural defect by resuturing recess. Type III: resection of sinus wall and repair with patch (fascia temporalis). Type IV: resection of both invaded walls and reconstruction of the two resected walls with patch. Type V: this type can be recognized from type VI only by direct surgical exploration of the sinus lumen. Opposite wall to the tumor side is free of tumor, it is possible to reconstruct the two resected walls with patch. Type VI: removal of involved portion of sinus and restoration with venous bypass. 3) As 20% of the patients presenting with manifestations of intracranial hypertension due to occlusion of posterior third of the superior sagittal sinus, torcular, predominant lateral sinus or internal jugular vein(s) develop severe intracranial hypertension, venous revascularisation by sino-jugular bypass--implanted proximally to the occlusion and directed to the jugular venous system (external or internal jugular vein)--can be a solution.

[Methylation status of RARbeta gene promoter in low and high grade cerebral glioma. Comparison with normal tissue. Immuno-histochemical study of nuclear RARbeta expression in low and high grade cerebral glioma cells. Comparison with normal cells. 48 tumors]. / Statut de méthylation du promoteur du gène RARB dans les tumeurs cérébrales gliales de bas et de haut grade. Comparaison avec le tissu sain. Etude immuno-histochimique du marquage au RARbeta des noyaux des cellules tumorales dans les gliomes de bas et de haut grade. Comparaison avec les cellules saines, à propos de 48 tumeurs.

Retinoic acid receptor beta (RARbeta) is a nuclear receptor often deregulated in tumors. An immunohistochemical study was conducted to examine the level of expression of this receptor in the nucleus of glial cell tumors (low and high grade glioma) as well as a study of the methylation status of the gene promoter coding this receptor on the same tumor samples. A comparison with normal tissue was done each time. 48 tumors were eligible for the study (15 glioblastomas, 20 grade III oligodendrogliomas and 13 grade II oligodendrogliomas). A constant decrease of RARbeta expression was found by comparison with normal tissue whatever the histological grade of the tumor, suggesting a deregulation of RARbeta gene expression. Methylation of RARbeta promoter gene was a rare event (12.5% of all cases), except for grade III oligodendrogliomas (20%), and is thus not a major event of this gene deregulation. Other reasons of this deregulation of RARbeta should be studied, such as loss of 3p24 heterozygoty, mRNA studies and RARbeta interactions with other retinoid receptors.

Efficacy of external fractionated radiation therapy in the treatment of meningiomas: a 20-year experience.

BACKGROUND: This is a retrospective analysis of a series of meningiomas treated by radiotherapy. MATERIALS AND METHODS: From 1978 to 1997, 45 patients with intracranial meningiomas were referred for external fractionated radiotherapy at Centre Alexis Vautrin. All patients were given 50-70Gy to the tumor bed (median: 56Gy), 1.8-2Gy per fraction. RESULTS: Evaluation was performed in June 1999 using the Kaplan-Meyer actuarial method with a median follow-up of 30 months (range: 1-166), relapse-free survivals (RFSs) were 75% at 5 years and 67% at 8 years; overall survival (OS) was 74% at 5 and 8 years. For the 26 benign histologically documented lesions, RFSs were 95% at 5 years and 81% at 8 years; OS was 85% at 5 and 8 years. One major radiation-induced complication occurred in this series (decline of cognitive function). According to the indication of radiotherapy, we divided the series into four groups: postoperative irradiation after a first subtotal resection (11 patients), 5-year RFS was 90%; after first recurrence (+/-salvage surgery, 14 patients), 73%; after further recurrence (+/-salvage surgery, 11 patients), 67%; as exclusive treatment (nine patients), 80%. Atypical and malignant lesions (n=7) all relapsed before 24 months of follow-up, all patients but one died before 42 months. Age at the time of irradiation (> or =60 vs. <60 years) and radiotherapy dose (> or =60 vs. <60Gy) did not influence local control or OS. Atypical and malignant lesions (WHO grades II and III) meningiomas had a worse outcome than benign lesions (WHO grade I, P<0.01). CONCLUSIONS: These results compare favorably with previously published data. External fractionated radiotherapy is well tolerated and effective. There is still a debate about the place of radiotherapy in the treatment of meningiomas: after subtotal resection, should radiotherapy be given postoperatively or at the time of progression? Should radiotherapy replace surgery when the risk of postoperative sequellae is high? Prospective randomized trials would be required to address these issues.

Stroke related to a dermoid cyst: case report.

OBJECTIVE AND IMPORTANCE: We report the case of a woman presenting with sudden neurological deficit, revealing a parasellar dermoid cyst. To our knowledge, this clinicopathological finding is the first reported in the literature. CLINICAL PRESENTATION: A neurological examination of the patient revealed a left hemiparesis, including central facial palsy, which hampered her speech. The well-documented neuroradiological work-up (including computed tomography, magnetic resonance imaging, and magnetic resonance angiography) demonstrated right frontorolandic ischemia caused by a right supra- and parasellar dermoid cyst leading to middle and anterior cerebral arterial stenoses. INTERVENTION: Surgical intervention, using a right subfrontopterional approach, was successful. Complete dermoid cyst removal was achieved. The mechanism of the arterial stenoses is extensively discussed and is thought to result from an inflammatory reaction of the basal vessels. CONCLUSION: The patient recovered fully. Nevertheless, postoperative magnetic resonance imaging confirmed cerebral infarction.

Aneurysm clipping after endovascular treatment with coils: a report of eight patients.

Between January 1990 and December 1994, patients with subarachnoid hemorrhage related to ruptured aneurysms who were referred to our institution were treated by neurosurgical and neuroradiological teams. In each patient, the respective indications for neurosurgical or endovascular treatment were discussed, taking into consideration patients' age and the morphological and topographical aneurysm features. We report eight cases of patients with subarachnoid hemorrhage who underwent operations after primary endovascular procedures (Hunt and Hess scores III, IV, and V). The indications for surgical treatment were as follows. First, deliberate partial occlusion of the aneurysm (two aneurysms of the internal carotid artery and one aneurysm of the anterior communicating artery) was performed to obtain only partial clotting of the aneurysm sac by free coils. However, this procedure was discontinued in favor of the use of Guglielmi detachable coils. The second indication was partial occlusion after an endovascular procedure (two aneurysms of the middle cerebral artery and one internal carotid artery aneurysm). The third indication was re-expansion of the aneurysm 1 year after the endovascular treatment (one middle cerebral artery aneurysm). The final indication was secondary rupture of the aneurysm sac and false aneurysm around the migrating coil (one aneurysm of the pericallosal artery). During surgery, the aneurysm sac appeared translucent. The coils bulged out and stretched the aneurysm sac. One ruptured the membrane leading to a subarachnoid hemorrhage during the endovascular procedure. No hemorrhage occurred during the surgical clipping. Aneurysm obliteration was easily performed, especially when the packing was partial, but was very difficult when the complete aneurysm closure led to a stenosis of the parent vessel. A giant sylvian aneurysm rest, visible only with angiography, was left untreated. This series illustrates an original experience, which led us to conclude that aneurysm surgery with coils in place is not as difficult as is often thought.

[Thrombosis of deep cerebral veins in form adults: clinical features and diagnostic approach]. / Les thromboses veineuses profondes de l'encéphale de l'adulte: aspects cliniques et approche diagnostique.

Thrombosis of deep cerebral veins is a rare condition, and is associated with a poor prognosis. We report four new cases observed between 1994 and 1997. All four cases were women, aged less than 45 years. Initial symptoms associated alteration of consciousness, change in mental status, progressive headache and vomiting. We observed also uni or bilateral signs of long tract injury. In three cases, diagnosis initially suspected by CT scan was confirmed with encephalic MRI. For the last patient, conventional angiography was needed. Thrombosis affected straight sinus, vein of Galien and internal cerebral veins in all patients. Basilar veins were also affected in one patient, without dural sinuses extension. Lateral sinus was involved in two others cases, and superior sagittal in the last patient. Etiology remains undetermined in one patient, associated with post-partum, use of oral contraceptive pill, and familial protein S deficiency, one case each. Outcome was favorable in all four cases with anticoagulation therapy. Precocity of diagnosis is determinant and MRI is usefull in this issue. These observations show that evolution of deep veins thrombosis can be favorable, without needing fibrinolytic therapy.

[Intracerebral cavernous angioma]. / Les angiomes caverneux intracérébraux.

Thirty four cases (18 operated, 16 non-operated), of cavernous angioma are reported. The presenting symptoms and signs were epilepsy in 22 cases, neurologic deficit in 9 and cerebromeningeal hemorrhage without vigilance disorders in 3. In the operated group 15 angiomas were supratentorial, 3 were subtentorial. In the non-operated group symptoms and signs were epilepsy in 11 cases and a neurologic deficit related to a brain stem lesion in 4/5 cases. Follow-up of 2 to 39 years after the first fit episode and of 1 to 7 years after initial diagnosis suggested that the risk of hemorrhage is low. Indications for neurosurgery are discussed as a function of the site of the angiomas and of the operative hemorrhagic risk.

Mucoceles of the sphenoidal sinus. Report of six cases and review of the literature.

Six personal cases of mucocele of the sphenoidal sinus are reported, and 124 cases from the literature are reviewed. Sphenoidal sinus mucocele is a benign and rare lesion which has long been unrecognized. Clinical features include fronto-orbital pain, oculomotor palsies, loss of visual acuity, exophthalmos and anosmia. Our series is of particular interest since four of our six patients presented with endocrine disorders. Recent advances in neuroradiological methods should rapidly lead to the correct diagnosis. Treatment consists of aspiration and drainage of the mucocele via a trans-sphenoidal approach. The results are good, and ophthalmoplegia usually subsides. In our experience, endocrine disorders do not always respond to treatment. The prognosis of sphenoidal sinus mucocele depends on the preoperative duration of the loss of visual acuity.

Dissection of intracranial vertebral arteries revealed by subarachnoidal haemorrhage. Report of seven cases.

Between April 1986 and April 1991, seven cases of meningeal haemorrhage consecutive to dissection of the 4th segment of a vertebral artery were observed at the Neuroradiology Department of the Nancy University Hospital. Four of the 7 patients were men, and 3 were women; mean age varied from 45 to 61 years. The clinical presentation was not suggestive, except in one case where the haemorrhagic episode was associated with Wallenberg's syndrome, pointing to the aetiological diagnosis. The outcome was variable. Two patients died of early complications of the haemorrhage which occurred before treatment could be given. In 2 others patients treated with antiplatelet agents the outcome was favourable; however, at angiography the lesions regressed in one of these two patients, but they progressed in the other with formation of a false aneurysm. The remaining 3 patients were treated by endovascular occlusion of the lesion-bearing vessel: one died of complications of a haemorrhage-induced vasospasm; the other two are symptomless after a 2 to 6 months follow-up.

[Endovascular treatment of vasospasm]. / Traitement endovasculaire du vasospasme.

Cerebral vasospasm remains a leading cause of heightened morbidity and mortality rates following aneurysmal subarachnoid hemorrhage despite the apparent benefit of recent medical therapeutics. Successful resolution of medically refractory angiographically demonstrated vasospasm with concomitant reversal of delayed neurological deficit has been observed after balloon angioplasty. Subsequent reports confirmed these encouraging results but also emphasized the limitations of the technique and the risks of complications. Intraarterial papaverine infusion has been performed for the treatment of diffuse cerebral vasospasm with controversial results and has also been combined with angioplasty either to facilitate balloon navigation or to treat arteries inaccessible to balloon catheterization. All these different endovascular approaches explain the confusion existing about the indications, timing and efficacy of the endovascular treatments. This article reviews several clinical and experimental studies dealing with these questions.

[Respiratory depression after intrathecal injection of morphine: value of in situ naloxone]. / Dépression respiratoire après injection intrathécale de morphine: intérêt de la naloxone in situ.

A case of delayed respiratory depression following an intrathecal injection of hyperbaric morphine hydrochloride is reported. This injection was made during a lumbar myelography in a 60 year old patient suffering from metastatic epiduritis unrelieved by oral or parenteral drugs. The differences in densities between the CSF, hyperbaric opiate solution and contrast medium explain the migration of the morphine hydrochloride from the lumbar thecal space to the basal cisternae, giving a fall in the responsiveness to CO2 of the brain stem respiratory centres. Parenteral naloxone did not reverse this ventilatory depression. Only the myosis and the analgesia disappeared. After 16 h of various attempts of reversal by parenteral injections, an intrathecal injection of naloxone was tried. This small dose (0.1 mg), given intrathecally, resulted in a prompt return to normal of respiratory function.

[Subarachnoid hemorrhage caused by aneurysm rupture. Surgery or embolization?]. / Hémorragies sous-arachnoïdiennes par rupture anévrismale. Chirurgie ou embolisation?

Traditionally the aneurysms of the circle of Willis have been an indication for neurosurgery. New technologies of endovascular treatment with electrically detachable coils resulted in a different therapeutical concept since four years. A series including 140 patients has been treated in our institution from 1 January 1992 to 31 December 1994, 94 of them presenting with a subarachnoid haemorrhage. Out of these 140 patients, 84 were treated with surgery, 51 with the endovascular technique, five with surgery after incomplete or unsuccessful endovascular treatment. Surgery was indicated in patients presenting early after bleeding, devoid of vasospasm, with a favourable Hunt and Hess grading and in aneurysms located in the anterior part of the circle of Willis. Endovascular treatment was indicated in patients admitted with delay, with severe vasospasm, a poor Hunt and Hess grading and in all aneurysms of the vertebrobasilar arterial network. Age was of less importance in comparison to the status of the vessels for selection of the method of treatment. Giant aneurysms are difficult to treat as surgery is faced with the size of the aneurysmal itself and endovascular technique with the width of the aneurysmal neck.