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Sandeep Mittal, Jean-Pierre Farmer, Bernard Rosenblatt, Frederick Andermann, José L. Montes, and Jean-Guy Villemure

✓ Residual seizures after functional hemispherectomy occur in approximately 20% of patients with catastrophic epilepsy. These episodes are traditionally attributed to incomplete disconnection, persistent epileptogenic activity in the ipsilateral insular cortex, or bilateral independent epileptogenic activity. The authors report on the case of an 8-year-old boy with an intractable seizure disorder who had classic frontal adversive seizures related to extensive unilateral left hemispheric cortical dysplasia. The initial intervention consisted of extensive removal of the epileptic frontal and precentral dysplastic tissue and multiple subpial transections of the dysplastic motor strip, guided by intraoperative electrocorticography. Subsequently, functional hemispherectomy including insular cortex resection was performed for persistent attacks. After a seizure-free period of 6 months, a new pattern ensued, consisting of an aura of fear, dystonic posturing of the right arm, and unusual postictal hyperphagia coupled with an interictal diencephalic-like syndrome. Electroencephalography and ictal/interictal single-photon emission computerized tomography were used to localize the residual epileptic discharges to deep ipsilateral structures. Results of magnetic resonance imaging indicated a complete disconnection except for a strip of residual frontobasal tissue. Therefore, a volumetric resection of the epileptogenic frontal basal tissue up to the anterior commissure was completed. The child has remained free of seizures during 21 months of follow-up review.

Standard hemispherectomy methods provide extensive disconnection, despite the presence of residual frontal basal cortex. However, rarely, and especially if it is dysplastic, this tissue can represent a focus for refractory seizures. This is an important consideration in determining the source of ongoing seizures posthemispherectomy in patients with extensive cortical dysplasia. It remains important to assess them fully before considering their disease refractory to surgical treatment.

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Warren Boling, Frederick Andermann, David Reutens, François Dubeau, Laetitia Caporicci, and André Olivier

Object. The goal of this study was to evaluate the efficacy of surgery for temporal lobe epilepsy (TLE) in older (≥ 50 years of age) patients.

Methods. The authors conducted a review of all patients 50 years of age or older with TLE surgically treated at the Montreal Neurological Institute and Hospital since 1981 by one surgeon (A.O.). Only patients without a mass lesion were included. Outcome parameters were compared with those of younger individuals with TLE, who were stratified by age at operation.

In patients aged 50 years and older, the onset of complex partial seizures occurred 5 to 53 years (mean 35 years) prior to the time of surgery. Postoperatively, over a mean follow-up period of 64 months, 15 patients (83%) obtained a meaningful improvement, becoming either free from seizures or only experiencing a rare seizure. Most surgery outcomes were similar in both older and younger individuals, except for a trend to more freedom from seizures and increased likelihood of returning to work or usual activities in the younger patients. Note that a patient's long-standing seizure disorder did not negatively affect their ability to achieve freedom from seizures following surgery.

Conclusions. Surgery for TLE appears to be effective for older individuals, comparing favorably with results in younger age groups, and carries a small risk of postoperative complications.

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Sandeep Mittal, Monika Mittal, José Luis Montes, Jean-Pierre Farmer, and Frederick Andermann

Hypothalamic hamartomas are uncommon but well-recognized developmental malformations that are classically associated with gelastic seizures and other refractory seizure types. The clinical course is often progressive and, in addition to the catastrophic epileptic syndrome, patients commonly exhibit debilitating cognitive, behavioral, and psychiatric disturbances. Over the past decade, investigators have gained considerable knowledge into the pathobiological and neurophysiological properties of these rare lesions. In this review, the authors examine the causes and molecular biology of hypothalamic hamartomas as well as the principal clinical features, neuroimaging findings, and electrophysiological characteristics. The diverse surgical modalities and strategies used to manage these difficult lesions are outlined in the second article of this 2-part review.

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Taner Tanriverdi, André Olivier, Nicole Poulin, Frederick Andermann, and François Dubeau

Object

The authors report long-term follow-up seizure outcome in patients who underwent corpus callosotomy during the period 1981–2001 at the Montreal Neurological Institute.

Methods

The records of 95 patients with a minimum follow-up of 5 years (mean 17.2 years) were retrospectively evaluated with respect to seizure, medication outcomes, and prognostic factors on seizure outcome.

Results

All patients had more than one type of seizure, most frequently drop attacks and generalized tonicclonic seizures. The most disabling seizure type was drop attacks, followed by generalized tonic-clonic seizures. Improvement was noted in several seizure types and was most likely for generalized tonic-clonic seizures (77.3%) and drop attacks (77.2%). Simple partial, generalized tonic, and myoclonic seizures also benefited from anterior callosotomy. The extent of the callosal section was correlated with favorable seizure outcome. The complications were mild and transient and no death was seen.

Conclusions

This study confirms that anterior callosotomy is an effective treatment in intractable generalized seizures that are not amenable to focal resection. When considering this procedure, the treating physician must thoroughly assess the expected benefits, limitations, likelihood of residual seizures, and the risks, and explain them to the patient, his or her family, and other caregivers.

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Antonio Nogueira de Almeida, André Olivier, Felipe Quesney, François Dubeau, Ghislaine Savard, and Frederick Andermann

Object

The purpose of this paper was to define the general efficacy of and morbidity associated with stereoelectroencephalography using modern methods of imaging and to particularize the risks related to specific lobes of the brain.

Methods

All patients admitted to the Montreal Neurological Institute who had undergone either computerized tomography – or magnetic resonance imaging–guided electrode implantation by one surgeon (A.O.) were reviewed. The procedure was considered efficient if the obtained information was sufficient to make a decision either in support of or against surgery.

Two hundred seventeen patients underwent 224 implantations with 3022 electrodes. Complications related to each lobe were as follows: temporal lobe, two abscesses (0.54%); frontal lobe, one abscess and three hematomas (1.4%); and occipital lobe, one hypointense lesion found 1 week after electrode explantation (2.6%). Significant risk factors associated with hematomas were implantation in the frontal lobe (p < 0.05) and the use of four or more implanted electrodes (p < 0.025).

General complications included the following: 26 patients, psychiatric symptoms during monitoring; one patient, meningitis; four patients, scalp cellulitis; and two patients, hemiparesis during angiography in the early 1980s. One of these latter patients maintained a mild hemiparesis and represents the only case of permanent neurological sequela in the entire series. Data obtained during recordings supported an indication for surgery in 178 patients (79.5%), excluded a surgical option in 37 patients (16.5%), and were unsatisfactory in nine patients (4%). Thus, the overall efficacy as defined previously was 96%.

Conclusions

Stereoelectroencephalography is an efficient procedure with low associated morbidity. Bilateral exploration of the temporal lobes has a morbidity rate of approximately 1%. A higher risk of hematomas occurs with the implantation of four or more electrodes in the frontal lobes.

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Sandeep Mittal, Monika Mittal, José Luis Montes, Jean-Pierre Farmer, and Frederick Andermann

Hypothalamic hamartomas are uncommon developmental heterotopic masses composed of an intermixed array of neurons, glia, and myelinated fibers closely associated with the mammillary bodies. Gelastic seizures, the hallmark feature of hypothalamic hamartomas, commonly present in early childhood. However, patients usually also display a disabling clinical syndrome, which may include various other types of refractory seizures with secondary generalization together with progressive cognitive, behavioral, and psychiatric dysfunction. The hamartoma itself has been unequivocally shown to be intrinsically epileptogenic. Over the past 2 decades there has been considerable effort to develop neurosurgical techniques to treat the epileptic syndrome effectively as well as to improve the neurocognitive and behavioral outcome.

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Taner Tanriverdi, Andre Olivier, Nicole Poulin, Frederick Andermann, and François Dubeau

Object

Resection strategies for the treatment of temporal lobe epilepsy (TLE) are a matter of discussion, and little information is available. The aim of this study was to compare seizure outcomes at the 5-year follow-up in patients with medically refractory unilateral mesial TLE (MTLE) due to hippocampal sclerosis (HS) who were treated using a cortical amygdalohippocampectomy (CorAH) or a selective AH (SelAH).

Methods

The authors obtained data from 100 adult patients who underwent surgery for MTLE. Fifty patients underwent a CorAH and 50 underwent an SelAH. Seizure control achieved with each technique was compared using the Engel classification scheme.

Results

Overall, at the 5-year follow-up, favorable (Engel Classes I and II) seizure outcomes were noted in 82 and 90% of patients who had undergone CorAH and SelAH, respectively. Furthermore, 40% of the patients who had undergone a CorAH and 58% of those who had undergone an SelAH were seizure free (Engel Class Ia). There was no statistically significant difference between the 2 surgical approaches in terms of seizure outcome at the 5-year follow-up (p = 0.38).

Conclusions

Both CorAH and SelAH can lead to similar favorable seizure control in patients with MTLE/HS. However, the authors suggest that the transcortical selective approach has the great advantage of minimizing or completely abolishing the impact of dividing several venous and arterial adhesions which are tedious, time consuming, and, at times, associated with some degree of cerebral swelling.

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Sandeep Mittal, José L. Montes, Jean-Pierre Farmer, Bernard Rosenblatt, François Dubeau, Frederick Andermann, Nicole Poulin, and André Olivier

Object

Surgery is an accepted treatment for carefully selected patients with focal epilepsy. In the present study, the authors assessed clinical and surgery-related data obtained in a large series of children suffering from intractable temporal lobe epilepsy (TLE).

Methods

Etiological, pathological, and clinical features of possible prognostic significance were studied in 109 children who underwent surgery for TLE at the Montreal Neurological Institute and Hospital and the Montreal Children's Hospital between 1985 and 2000.

The mean age of patients at seizure onset was 5.5 years and the duration of epilepsy ranged from 0.1 to 17.6 years. Preoperative magnetic resonance imaging revealed mesial sclerosis in 51 patients, a mass lesion in 45, and no visible abnormalities in 12. In six patients invasive monitoring was required. Cortical amygdalohippocampectomy was performed in 72% of patients, whereas 20% underwent transcortical selective amygdalohippocampectomy. In 23 patients a second surgical intervention was necessary. Low-grade tumors were found in 35% and mesial sclerosis was confirmed on pathological evaluation in 45%. Outcome was excellent (seizure free or > 90% reduction) in 94 patients (86%). The patients were followed prospectively for a median of 10.9 years (range 5–20.2 years). There were no permanent neurological complications and no deaths.

Conclusions

Successful postsurgical outcomes, especially in patients treated for mesial temporal lobe sclerosis and lesion-related epilepsies, can be obtained in pediatric patients suffering minimal complications. Unfavorable outcomes are most likely to occur when epileptiform discharges are bitemporal or multifocal in distribution and in cases involving incomplete resection of mesiotemporal structures.

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Richard G. Bittar, André Olivier, Abbas F. Sadikot, Frederick Andermann, Roch M. Comeau, Martin Cyr, Terrence M. Peters, and David C. Reutens

Object. To investigate the utility of [15O]H2O positron emission tomography (PET) activation studies in the presurgical mapping of primary somatosensory cortex, the authors compared the magnitude and location of activation foci obtained using PET scanning with the results of intraoperative cortical stimulation (ICS).

Methods. The authors used PET scanning and vibrotactile stimulation (of the face, hand, or foot) to localize the primary somatosensory cortex before surgical resection of mass lesions or epileptogenic foci affecting the central area in 20 patients. With the aid of image-guided surgical systems, the locations of significant activation foci on PET scanning were compared with those of positive ICS performed at craniotomy after the patient had received a local anesthetic agent. In addition, the relationship between the magnitude and statistical significance of blood flow changes and the presence of positive ICS was examined.

In 22 (95.6%) of 23 statistically significant (p < 0.05) PET activation foci, spatially concordant sites on ICS were also observed. Intraoperative cortical stimulation was positive in 40% of the PET activation studies that did not result in statistically significant activation. In the patients showing these results, there was a clearly identifiable t-statistic peak that was spatially concordant with the site of positive ICS in the sensorimotor area. All PET activation foci with a t statistic greater than 4.75 were associated with spatially concordant sites of positive ICS. All PET activation foci with a t statistic less than 3.2 were associated with negative ICS.

Conclusions. Positron emission tomography is an accurate method for mapping the primary somatosensory cortex before surgery. The need for ICS, which requires local anesthesia, may be eliminated when PET foci with high (> 4.75) or low (< 3.20) t-statistic peaks are elicited by vibrotactile stimulation.

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Richard Leblanc, Donatella Tampieri, Yves Robitaille, André Olivier, Frederick Andermann, and Alan Sherwin

✓ The authors describe the association between an anterobasal temporal lobe encephalocele and medically intractable temporal lobe epilepsy in three patients treated successfully by surgery. Two men and one woman, aged 26 to 37 years (mean 31 years), had onset of complex automatism and generalized seizures in their second and fourth decades (mean age 22.7 years). They had been epileptic for 6 to 14 years (mean 8.3 years) before surgery. Preoperative electroencephalograms localized ictal epileptic activity to the left mesial temporal lobe in all cases, and neuropsychological testing revealed dominant temporal lobe dysfunction. Magnetic resonance (MR) imaging demonstrated an anteromedial basal temporal encephalocele extending into the pterygopalatine fossa through a bone defect at the base of the greater sphenoid wing in the region of the foramen rotundum and pterygoid process, a discrete center of embryonal chondrification. At surgery, the encephaloceles were found in front of the uncus, and an area of gliosis extended from the encephalocele to the amygdalohippocampal region. All patients have been seizure-free following anterior temporal resection and amygdalohippocampectomy including the encephalocele. These three cases delineate a condition of disordered embryogenesis wherein a developmental anterobasal temporal encephalocele acts as the substrate for temporal lobe epilepsy. This lesion may be diagnosed preoperatively with MR imaging and should be considered in the differential diagnosis of late-onset temporal lobe epilepsy.