Surgical resection of cavernous angioma located within eloquent brain areas: International survey of the practical management among 19 specialized centers.

PURPOSE: The practical management of cavernous angioma located within eloquent brain area before, during and after surgical resection is poorly documented. We assessed the practical pre-operative, intra-operative, and post-operative management of cavernous angioma located within eloquent brain area. METHOD: An online survey composed of 61 items was sent to 26 centers to establish a multicenter international retrospective cohort of adult patients who underwent a surgical resection as the first-line treatment of a supratentorial cavernous angioma located within or close to eloquent brain area. RESULTS: 272 patients from 19 centers (mean 13.6 ± 16.7 per center) from eight countries were included. The pre-operative management varied significantly between centers and countries regarding the pre-operative functional assessment, the pre-operative epileptological assessment, the first given antiepileptic drug, and the time to surgery. The intra-operative environment varied significantly between centers and countries regarding the use of imaging systems, the use of functional mapping with direct electrostimulations, the extent of resection of the hemosiderin rim, the realization of a post-operative functional assessment, and the time to post-operative functional assessment. The present survey found a post-operative improvement, as compared to pre-operative evaluations, of the functional status, the ability to work, and the seizure control. CONCLUSIONS: We observed a variety of practice between centers and countries regarding the management of cavernous angioma located within eloquent regions. Multicentric prospective studies are required to solve relevant questions regarding the management of cavernous angioma-related seizures, the timing of surgery, and the optimal extent of hemosiderin rim resection.

Predictors of Epileptic Seizures and Ability to Work in Supratentorial Cavernous Angioma Located Within Eloquent Brain Areas.

BACKGROUND: The postoperative outcomes and the predictors of seizure control are poorly studied for supratentorial cavernous angiomas (CA) within or close to the eloquent brain area. OBJECTIVE: To assess the predictors of preoperative seizure control, postoperative seizure control, and postoperative ability to work, and the safety of the surgery. METHODS: Multicenter international retrospective cohort analysis of adult patients benefitting from a functional-based surgical resection with intraoperative functional brain mapping for a supratentorial CA within or close to eloquent brain areas. RESULTS: A total of 109 patients (66.1% women; mean age 38.4 ± 12.5 yr), were studied. Age >38 yr (odds ratio [OR], 7.33; 95% confidence interval [CI], 1.53-35.19; P = .013) and time to surgery > 12 mo (OR, 18.21; 95% CI, 1.11-296.55; P = .042) are independent predictors of uncontrolled seizures at the time of surgery. Focal deficit (OR, 10.25; 95% CI, 3.16-33.28; P < .001) is an independent predictor of inability to work at the time of surgery. History of epileptic seizures at the time of surgery (OR, 7.61; 95% CI, 1.67-85.42; P = .003) and partial resection of the CA and/or of the hemosiderin rim (OR, 12.02; 95% CI, 3.01-48.13; P < .001) are independent predictors of uncontrolled seizures postoperatively. Inability to work at the time of surgery (OR, 19.54; 95% CI, 1.90-425.48; P = .050), Karnofsky Performance Status ≤ 70 (OR, 51.20; 95% CI, 1.20-2175.37; P = .039), uncontrolled seizures postoperatively (OR, 105.33; 95% CI, 4.32-2566.27; P = .004), and worsening of cognitive functions postoperatively (OR, 13.71; 95% CI, 1.06-176.66; P = .045) are independent predictors of inability to work postoperatively. CONCLUSION: The functional-based resection using intraoperative functional brain mapping allows safe resection of CA and the peripheral hemosiderin rim located within or close to eloquent brain areas.

Impact of intraoperative stimulation brain mapping on glioma surgery outcome: a meta-analysis.

PURPOSE: Surgery for infiltrative gliomas aims to balance tumor removal with preservation of functional integrity. The usefulness of intraoperative stimulation mapping (ISM) has not been addressed in randomized trials. This study addresses glioma surgery outcome on the basis of a meta-analysis of observational studies. METHODS: A systematic search retrieved 90 reports published between 1990 and 2010 with 8,091 adult patients who had resective surgery for supratentorial infiltrative glioma, with or without ISM. Quality criteria consisted of postoperative neurologic examination details and follow-up timing. New postoperative neurologic deficits were categorized on the basis of timing and severity. Meta-analysis with a Bayesian random effects model determined summary event rates of deficits as well as gross total resection rate and eloquent locations. Meta-regression analysis explored heterogeneity among studies. RESULTS: Late severe neurologic deficits were observed in 3.4% (95% CI, 2.3% to 4.8%) of patients after resections with ISM, and in 8.2% (95% CI, 5.7% to 11.4%) of patients after resections without ISM (adjusted odds ratio, 0.39; 95% CI, 0.23 to 0.64). The percentages of radiologically confirmed gross total resections were 75% (95% CI, 66% to 82%) with ISM and 58% (95% CI, 48% to 69%) without ISM. Eloquent locations were involved in 99.9% (95% CI, 99.9% to 100%) of resections with ISM and in 95.8% (95% CI, 73.1% to 99.8%) of resections without ISM. Relevant sources of heterogeneity among studies were ISM, continent, and academic setting. CONCLUSION: Glioma resections using ISM are associated with fewer late severe neurologic deficits and more extensive resection, and they involve eloquent locations more frequently. This indicates that ISM should be universally implemented as standard of care for glioma surgery.

New insights into the anatomic dissection of the temporal stem with special emphasis on the inferior fronto-occipital fasciculus: implications in surgical approach to left mesiotemporal and temporoinsular structures.

OBJECTIVE: To analyze the 3-dimensional relationships of the inferior fronto-occipital fasciculus (IFOF) within the temporal stem using anatomic dissection and to study the surgical application. METHODS: Ten postmortem human hemispheres (5 right, 5 left) were dissected using the Klingler fiber dissection technique. The 3-dimensional relationships of the IFOF with different landmarks of the temporal stem, insula, and temporal lobe were analyzed and measured. RESULTS: An average distance of 10.9 mm (range, 8-15 mm) was observed between the limen insulae and the anterior edge of the IFOF under the inferior limiting sulcus of the insula. This anterior one-third of the temporal stem is crossed by the uncinate fasciculus. The IFOF crosses the posterior two-thirds of the temporal stem, in the space between the posterior limit of the uncinate fasciculus and the lateral geniculate body. The average superoinferior distance between the IFOF and the inferior limiting sulcus was 3.8 mm. The auditory radiations and the claustro-opercular and insulo-opercular fibers of the external and extreme capsules pass through the temporal stem above the IFOF, whereas the optic radiations pass below. CONCLUSION: In the transsylvian approach to the mesiotemporal structures in the left dominant hemisphere, an incision within the posterior 8 mm from the limen insulae is less likely to damage the IFOF than more posterior incisions along the inferior limiting sulcus. In the temporal transopercular approach to left temporo-insular gliomas, the IFOF constitutes the deep functional limit of the resection within the temporal stem.

Parasagittal transinsular electrodes for stereo-EEG in temporal and insular lobe epilepsies.

OBJECTIVE: Direct invasive EEG recordings of the insula - due to its particular anatomical position, deeply seated between both opercula - can only be performed with intracerebral electrodes. To date, the technique most commonly used for insular stereoelectroencephalography (SEEG) is the orthogonal-transopercular electrode approach with the Talairach methodology. We propose another technique utilizing MRI with transinsular parasagittal electrodes and a posterior entry point. This avoids passing through the opercula and sylvian vessels running over the insular surface. METHODS: Nine patients, whose seizures implicated the insula, underwent brain surgery. Under general anesthesia with a Leksell frame, 3D-T(1) SPGR MRI with gadolinium enhancement was achieved. Surgical planning was performed using the StealthStation with an entry point in the parieto-occipital junction setting the target for the first contact of the lead at the most anterior part of the insula. The trajectory was manipulated in order to have at least 4 contacts per electrode track within the insular cortex. All patients had a postoperative MRI to verify the exact position of each contact. RESULTS: Insular seizures were recorded in all patients. There was neither intracranial bleeding nor infection. In all of the cases, except 1, the seizures recorded were found to be propagations of the primary epileptic zone located in the temporal lobe, either in the mesial structures or the superior temporal gyrus. Eight patients were operated, 7 with an antero-mesial temporal lobectomy and 1 with dysplasia of the superior temporal gyrus. No insular resections were performed. CONCLUSION: We report a novel technique for insular SEEG with parasagittal electrodes, parallel to the insular cortex, with an entry point at the parieto-occipital junction. This technique is based entirely on use of MRI, and avoids passing through the opercula and sylvian vessels.

Long-term brain plasticity allowing a multistage surgical approach to World Health Organization Grade II gliomas in eloquent areas.

Although the goal of surgery for World Health Organization Grade II gliomas is maximal extent of resection, complete tumor removal is not always possible when the glioma involves eloquent areas. The authors propose a multistage surgical approach to highly crucial areas that are classically considered inoperable, enabling optimization of the extent of resection while avoiding permanent cognitive deficits due to induced functional reshaping in the interim between the 2 consecutive operations. To demonstrate such plasticity, the authors used a combination of sequential functional MR imaging and intraoperative electrical stimulation mapping before and during surgeries spaced by several years in 2 patients who each underwent 2 separate resections of Grade II gliomas located in the left dominant premotor area. During several years of follow-up after the first procedure, both patients had unremarkable examination results and normal socioprofessional lives. There was no malignant transformation. Based on their experience with these cases, the authors suggest that in cases of incomplete glioma removal, a second operation before anaplasia should be considered, made possible by brain reorganization after the first operation.

Long-term follow-up of Huntington disease treated by bilateral deep brain stimulation of the internal globus pallidus.

Deep brain stimulation is now accepted as a safe and efficient treatment for movement disorders including selected types of dystonia and dyskinesia. Very little, however, is known about its effect on other movement disorders, particularly for "choreic" movements. Huntington disease is a fatal autosomal-dominant neurodegenerative disorder characterized by movement disorders, progressive cognitive impairment, and psychiatric symptoms. Bilateral chronic stimulation of the internal globus pallidus was performed to control choreic movements in a 60-year-old man with a 10-year history of Huntington disease. Chronic deep brain stimulation resulted in remarkable improvement of choreic movements. Postoperative improvement was sustained after 4 years of follow-up with a marked improvement in daily quality of life.

Antero-ventral internal pallidum stimulation improves behavioral disorders in Lesch-Nyhan disease.

The Lesch-Nyhan syndrome is an X-linked recessive disorder caused by a deficiency in hypoxanthine-guanine phosphoribosyl transferase, a purine salvage enzyme. Affected individuals exhibit a characteristic neurobehavioral disorder with delayed acquisition of motor skills, dystonia, severe self-mutilations, and aggressive behavior. Deep brain stimulation has been previously proposed for controlling isolated involuntary movements and psychiatric disorders. We applied a double bilateral simultaneous stimulation to limbic and motor internal pallidum in one patient for controlling both behavioral and movement disorders, respectively. The injurious compulsions disappeared; dystonia and dyskinesia were decreased at 28 months follow-up.

Intradural extramedullary spinal ependymoma: a benign pathology?

STUDY DESIGN: Spinal ependymoma is a benign central nervous system tumor described as an intramedullary lesion more frequently located at the conus medullaris. It has been described exceptionally in the literature as an intradural extramedullary tumor. OBJECTIVE: Presentation of an extremelly rare location and evolution of extremedullary ependymoma and discussion of its probable origin, differential diagnosis, treatment options, and follow-up. SUMMARY OF BACKGROUND DATA: This case demonstrates an unusual location of a benign ependymal tumor in the extramedullary space with a total resection, which recurred in a lower level with a malignant transformation with the same extramedullary location. METHODS: The authors present the case of a 47-year-old woman with a subacute spinal cord dysfunction and an intradural extramedullary D2-D3 tumor mimicking meningioma or neurinoma. At surgery, an encapsulated intradural extramedullary mass was found, but neither dural attachment nor medullary infiltration was present. RESULTS: After complete resection, anatomic-pathologic studies confirmed that the lesion was a benign classic ependymoma. Good neurologic outcome was achieved, and no residual tumor was present at magnetic resonance imaging (MRI) control performed at 3 and 9 months later. One year after surgery, a new intradural extramedullary tumor was found at the D4 level without recurrence at D2. The patient was operated on again, but at this time the histologic study showed an anaplastic ependymoma with a proliferation index of 25% measured by Ki-67. Whole central nervous system radiotherapy was performed. CONCLUSION: All of the previously reported cases of spinal intradural extramedullary ependymomas carried out a benign course. The case we are reporting is the first one in which malignant transformation occurred. This tumor should be taken into account in the differential diagnosis of intradural extramedullary lesions. Moreover, close follow-up is recommended for this unusual location of ependymomas.