Contralateral MRI abnormalities in candidates for hemispherectomy for refractory epilepsy

Purpose: To assess the impact of contralateral magnetic resonance imaging (MRI) findings on seizure outcome after hemispherectomy for refractory epilepsy. Methods: We retrospectively reviewed 110 children, 0.4–18 (median 5.9) years of age, who underwent hemispherectomy for severe refractory epilepsy at Cleveland Clinic Children’s Hospital. In children with contralateral (as well as ipsilateral) MRI findings appreciated preoperatively, the decision to proceed to surgery was based on other features concordant with the side with the most severe MRI abnormality, including ipsilateral epileptiform discharges, lateralizing seizure semiology, and side of hemiparesis. Results: We retrospectively observed contralateral MRI abnormalities (predominantly small hemisphere, white matter loss or abnormal signal, or sulcation abnormalities) in 81 patients (74%), including 31 of 43 (72%) with malformations of cortical development (MCD), 31 of 42 (73%) with perinatal injury from infarction or hypoxia, and 15 of 25 (60%) with Rasmussen’s encephalitis, Sturge‐Weber syndrome, or posttraumatic encephalomalacia. Among 84 children (76%) with lesions that were congenital or acquired pre‐ or perinatally, 67 (83%) had contralateral MRI abnormalities (p = 0.02). Contralateral findings were subjectively judged to be mild or moderate in 70 (86%). At follow‐up 12–84 (median 24) months after surgery, 79% of patients with contralateral MRI abnormalities were seizure‐free compared to 83% of patients without contralateral MRI findings, with no differences based on etiology group or type or severity of contralateral MRI abnormality. Discussion: MRI abnormalities, usually mild to moderate in severity, were seen in the contralateral hemisphere in the majority of children who underwent hemispherectomy for refractory epilepsy due to various etiologies, especially those that were congenital or early acquired. The contralateral MRI findings, always much less prominent than those in the ipsilateral hemisphere, did not correlate with seizure outcome and may not contraindicate hemispherectomy in otherwise favorable candidates.     

Aphasia after hemispherectomy in an adult with early onset epilepsy and hemiplegia

A 55 year old left handed man with left hemisphere subcortical encephalomalacia, seizures, language impairment, and right hemiparesis from a motor vehicle accident at age five was evaluated for epilepsy surgery. The patient continued to speak and followed commands during a left intracarotid amobarbital test (IAT). Left functional hemispherectomy resulted in expressive aphasia. Based on postoperative outcome, language was bilateral. The injury after primary development of language function, the predominantly subcortical lesion, and the late timing of surgical intervention well past development and plasticity may have been factors in the emergence of postoperative aphasia.     

改良解剖性大脑半球切除术治疗婴儿偏瘫伴顽固性癫痫的疗效观察

目的探讨改良解剖性大脑半球切除术治疗婴儿偏瘫伴顽固性癫痫的效果。方法采用改良的解剖性大脑半球切除术治疗婴儿偏瘫伴顽固性癫痫患者27例,随访其临床效果并分析原因。结果术后25例患者癫痫发作停止,2例基本控制。术后患者行为异常改善,神经功能障碍无加重,康复期缩短,无远期合并症出现。结论改良的解剖性大脑半球切除术在理论和实践上更加合理,是治疗婴儿偏瘫伴顽固性癫痫的有效方法。     

Outcome of hemispheric surgeries for refractory epilepsy in pediatric patients

BACKGROUND: Hemispheric brain lesions are commonly associated with early onset of catastrophic epilepsies and multiple seizure types. Hemispheric surgery is indicated for patients with unilateral intractable epilepsy. Although described more than 50 years ago, several new techniques for hemispherectomy have only recently been proposed aiming to reduce operatory risks and morbidity. MATERIALS AND METHODS: We present the clinical characteristics, presurgical workup, and postoperative outcome of a series of pediatric patients who underwent hemispherectomy for medically intractable epileptic seizures. Thirty-nine patients with medically intractable epilepsy underwent surgery from 1996 to 2005. RESULTS AND DISCUSSION: We analyzed demographic data, interictal and ictal EEG findings, age at surgery, surgical technique and complications, and postsurgical seizure outcome. There were 74.4% males. Tonic and focal motor seizures occurred in 30.8 and 20.5% of the patients. Most frequent etiologies were Rasmussen encephalitis (30.8%) and malformation of cortical development (23.1%). Postsurgical outcomes were Engel classes I and II for 61.5% of the patients. In general, 89.5% of the patients exhibited at least a 90% reduction in seizure frequency. All patients had acute worsening of hemiparesis after surgery. Basically, two surgical techniques have been employed, both with similar results, although a trend has been noted toward one of the procedures which produced consistently complete disconnection. Patients with hemispheric brain lesions usually have abnormal neurological development and intractable epilepsy. When video-EEG monitoring and magnetic resonance imaging show unilateral disease, the patient may evolve with a good surgical outcome. We showed that a marked reduction in seizure frequency may be achieved, with acceptable neurological impairments.     

Developmental plasticity after right hemispherectomy in an epileptic adolescent with early brain injury

Objectives The authors present the case of an adolescent affected with refractory epilepsy due to a neonatal ischemic infarction of the right medial cerebral artery. Hemiplegic since the first months of life, she began presenting motor partial seizures associated with drop attacks at 4.5 years; these were initially well controlled by antiepileptic drugs, but at 10 years seizures appeared again and became refractory. Thus, at 14 years and 10 months, she was submitted to a right hemispherectomy that made her rapidly seizure free. In the post-surgical follow-up lasting 5 years, neuropsychological serial assessments showed an impressive progressive improvement of cognitive skills, namely, visuospatial abilities. This case seems to challenge the widely spread feeling that functional catch-up in brain-injured children could only occur early in life. In effect, the astonishing recovery especially of visuospatial skills in our case occurred in adolescence after a late surgical intervention of right hemispherectomy.Methods Different neuropsychological aspects are discussed. The reorganisation process recovered the spatial and linguistic abilities as well as the verbal and visuospatial memory; however, there was a persistent impairment of complex spatial and perceptual skills as well as recall abilities. Despite the deficit of complex visual stimuli processing, the patient showed a good performance in the recognition of unknown faces.Conclusions Probably, the absence of seizures in the first 4 years of life could have allowed a generally adequate compensatory reorganisation, successively masked by the persistent and diffuse epileptic disorder. The seizure control produced by surgery eventually made evident the effectiveness of the brain reorganisation.     

A pilot trial with modified Atkins' diet in adult patients with refractory epilepsy

OBJECTIVES: At Ghent University Hospital, the feasibility and efficacy of the modified Atkins' diet was evaluated in adult patients with refractory epilepsy. The Atkins' diet restricts carbohydrate intake and was originally designed for weight loss. PATIENTS AND METHODS: During a 6-month trial period, a carbohydrate restriction of 20g/day was in place. During a 36h hospital admission, patients were instructed about the diet. Patients underwent clinical neurological testing, EEG, ECG, blood and urine analyses and mood evaluation before and during the trial. Seizure frequency and side effects were recorded in seizure diaries and followed up at monthly clinic visits. RESULTS: Eight patients were included in the study. Three out of eight patients followed the diet for 6 months. One out of three patients showed a >50% seizure reduction, 1/3>30%, and 1/3<30%. Side effects such as constipation and diarrhoea were mild and occurred mainly during the initial week of the diet. Patients reported improved concentration and well being. This was confirmed by improved scores on the Beck Depression Inventory Scale. CONCLUSION: This pilot study shows that the modified Atkins' diet is feasible in an adult population, and that seizure frequency reduction is possible. The results need to be confirmed in larger prospective, controlled studies with comparison groups.     

Outcome after corticoamygdalohippocampectomy in patients with refractory temporal lobe epilepsy and mesial temporal sclerosis without preoperative ictal recording

Purpose:   We report on the surgical outcome obtained in patients with refractory temporal lobe epilepsy with mesial temporal sclerosis (MTS) who were evaluated preoperatively without ictal recording and were submitted to corticoamygdalohippocampectomy.
Methods:   Two hundred twelve patients with refractory temporal lobe epilepsy were evaluated by means of clinical history, neurological examination, interictal electroencephalography (EEG), magnetic resonance imaging (MRI), and neuropsychological testing. MRI disclosed unilateral MTS in all patients. All patients were submitted to corticoamygdalohippocampectomy at the side determined by MRI.
Results:   Interictal EEG showed unilateral temporal lobe spiking in 176 patients; in 36 patients, bilateral discharges were found. Mean follow-up time was 2.7  years. One hundred ninety-four patients (92%) were classified as Engel's class I. Eighteen patients (8%) were rated as Engel's class II. Thirty-two out of 36  patients, in whom bilateral discharges were found, were in Engel's class I. Sixty percent of the patients had an improvement in memory function related to the nonoperated temporal lobe. Fifty-nine percent of the patients had a 10-point increase in general IQ postoperatively. Verbal memory decline was noted in three patients. Pathological examination showed MTS in all patients.
Conclusions:   It is possible to adequately select good surgical candidates for temporal lobe resection using MRI and interictal EEG alone. In patients with MRI-defined MTS, we should expect a 90% postoperative remission rate. Cognitive decline was very rarely seen in this patient population. The finding of MTS on MRI is the single most important prognostic factor for good outcome after temporal lobe surgery.     

Outcome after extended callosal section in patients with primary idiopathic generalized epilepsy

Purpose:   We report the outcome of patients with refractory idiopathic generalized epilepsy (IGE) who were submitted to extended one-stage callosal section.
Methods:   Eleven patients with IGE who were submitted to extended one-stage callosal section were studied. Preoperative workup included history and neurologic examination, interictal, and ictal electroencephalography (EEG) recording, high resolution 1.5T magnetic resonance imaging (MRI) and intelligence quotient (IQ) testing. All patients were submitted to extended one-stage microsurgical callosal section, leaving only the splenium intact.
Results:   Preoperative ictal patterns included repetitive spike and wave or polyspike and wave discharges or fast epileptic recruiting rhythm. MRI showed no focal lesions. Preoperatively, mean general IQ was 85. Postoperatively, at least a 75% reduction in the frequency of generalized tonic–clonic seizures was noted in all patients. In three patients absences disappeared completely, and the others had at least 90% reduction in seizure frequency. Only one patient had myoclonic seizures preoperatively, and these seizures disappeared after callosal section. After surgery, mean general IQ was 89. A very clear increase in attention level was noted in all patients. Postoperative interictal EEG recordings showed rupture of bilateral synchrony in all patients.
Discussion:   This article reports on a large and homogeneous series of patients with refractory IGE submitted for callosal section. There was a marked decrease in generalized seizure frequency and increase in the attention level in this patient population. Our results suggest that corticocortical interaction might have a role in IGE pathogenesis. Callosotomy is a safe, effective, and underused palliative procedure in these well-selected patients with refractory IGE.     

Outcome after decompressive craniectomy in patients with dominant middle cerebral artery infarction: A preliminary report

Introduction:

Life-threatening, space occupying, infarction develops in 10-15% of patients after middle cerebral artery infarction (MCAI). Though decompressive craniectomy (DC) is now standard of care in patients with non-dominant stroke, its role in dominant MCAI (DMCAI) is largely undefined. This may reflect the ethical dilemma of saving life of a patient who may then remain hemiplegic and dysphasic. This study specifically addresses this issue.

Materials and Methods:

This retrospective analysis studied patients with DMCAI undergoing DC. Patient records, operation notes, radiology, and out-patient files were scrutinized to collate data. Glasgow outcome scale (GOS), Barthel index (BI) and improvement in language and motor function were evaluated to determine functional outcome.

Results:

Eighteen patients between 22 years and 72 years of age were included. 6 week, 3 month, 6 month and overall survival rates were 66.6% (12/18), 64% (11/17), 62.5% (10/16) and 62.5% (10/16) respectively. Amongst ten surviving patients with long-term follow-up, 60% showed improvement in GOS, 70% achieved BI score >60 while 30% achieved full functional independence. In this group, motor power and language function improved in 9 and 8 patients respectively. At last follow-up, 8 of 10 surviving patients were ambulatory with (3/8) or without (5/8) support. Age <50 years corresponded with better functional outcome amongst survivors (P value –0.0068).

Conclusion:

Language and motor outcomes after DC in patients with DMCAI are not as dismal as commonly perceived. Perhaps young patients (<50 years) with DMCAI should be treated with the same aggressiveness that non-DMCAI is currently dealt with.Key Words: Craniectomy, dominant, middle cerebral artery, outcome, stroke     

Functional hemispherectomy is safe and effective in adult patients with epilepsy

IntroductionFunctional hemispherectomy (FH) is a well-established therapeutic option for children with epilepsy with parenchymal damage confined to one hemisphere, yet its application in adults remains rare. The intention of our study was to investigate postoperative clinical and epileptological outcome in adults who received FH for intractable epilepsy.Materials and methodsWe retrospectively analyzed 12 adult patients (18–56 years) with intractable epilepsy due to unihemispheric pathology. All patients underwent FH. Postoperative neurological and cognitive outcome as well as seizure status were evaluated with a mean follow-up period of 4.9 years.ResultsTen patients (83%) were seizure-free (Engel I), and two (17%) had recurrent seizures at last follow-up. Apart from one patient requiring operative revision for bone flap infection, no perioperative morbidity or mortality occurred. Postoperative functional assessment revealed deterioration of motor function in 7 patients, whereas 5 remained unchanged. Language was unchanged in 8 patients. The absence of background slowing in preoperative electroencephalogram (EEG) as well as ictal and interictal EEG patterns located ipsilateral to the side of surgery was associated with favorable seizure outcome.ConclusionFavorable seizure control and acceptable functional outcome can be achieved by FH in adults with intractable epilepsy. The risk of postoperative deficits is moderate and even older patients are able to manage postoperative motor impairment. Therefore, FH should be considered in case of unihemispheric lesions also in adults.     

Reorganization of Pericruciate cortical projections to the spinal cord and dorsal column nuclei after neonatal or adult cerebral hemispherectomy in cats

This is a quantitative study of changes in distribution and density of terminals of the corticospinal tract in the cervical spinal cord and dorsal column nuclei (DCN) in cats with left cerebral hemispherectomy performed neonatally or in adulthood. Kittens received hemispherectomy at a mean of 12.1 postnatal days and were compared, as adults, to adult-lesioned cats of similar survival time. All animals, including controls, received injections of [3H]leucine-proline and were sacrificed 5 days later. Injection sites and terminal fields were reconstructed from autoradiography-processed tissue. The label filled comparable extents of areas 4 gamma and 3a of the right cerebral cortex. Coronal sections from upper and lower cervical cord levels, and from the brainstem (cuneate and gracile nuclei) were studied. Computer-image processing procedures were used to count labeled particles from multiple sites of the dorsal horn and DCN, bilaterally. In the spinal cord of intact and adult-hemispherectomized cats, most terminals were found in lamina VI, and adjacent laminae V and VII contralateral to the injection side. The major finding was that neonatal-lesioned cats showed a significant increase in axon terminals in areas ipsilateral to the injection. The topography of distribution of the novel terminals was similar to that in the contralateral side and the originating fibers appeared to have crossed the midline from that side. A similar reorganization occurred in the gracile nucleus where, in intact and adult-lesioned cats, the cortical terminals also predominated in the side contralateral to the injection. In contrast, neonatal-lesioned animals showed a significant increase in terminal density ipsilateral to the cortical injection. These findings are discussed as an alternative mechanism for postlesion remodeling of the corticospinal tract in animals with the pyramidal crossing completed at the time of birth.     

Two-year outcome audit in an adult learning disability population with refractory epilepsy

Current evidence suggests that epilepsy outcome for people with a learning disability is poor, with the majority remaining refractory to treatment. There is very little evidence from randomised controlled trials in this population and despite the many uncontrolled variables it is suggested that an outcome audit may be one method of adding to the evidence base. This audit reports on the outcome for 37 patients with learning disability and refractory epilepsy. All patients were seen for the first time before March 2001 and the mean number of seizures for the sample was 10.4 per month. The exit audit included all patients at a date 2 years after their initial visit following a programme of medication changes. The mean seizure frequency had reduced to 5.9 per month. Ten patients had become seizure-free and 76% had experienced an improvement in seizure frequency overall. A simple questionnaire was administered to carers and relatives at the exit audit in an attempt to establish an impression of global changes in alertness, assertiveness and challenging behaviour following interventions. Sixty-five percent of patients were regarded as being more aware and interactive with their surroundings following medication changes and 49% were reported to be more assertive. Thirty percent presented with an increase in behaviours regarded as challenging and 22% were reported to present with less challenging behaviour. The results of this audit suggest that the outcome for the majority of patients with learning disability and refractory epilepsy may be better than that has been previously reported.     

Outcome after surgery in patients with refractory temporal lobe epilepsy and normal MRI.

Our purpose is to determine predictors of outcome in patients with refractory temporal lobe epilepsy and normal high resolution magnetic resonance imaging (MRI) who undergo surgical therapy. We identified 23 patients who underwent temporal lobectomy and had normal pre-operative MRI, including surface coil phased array temporal lobe imaging. All were followed at least 2 years after surgery. We graded outcome as seizure-free, > 75% reduction in seizures, or < 75% reduction in seizures. We examined pre-operative interictal and ictal electroencephalographic (EEG) findings, age of onset, gender, duration of epilepsy, risk factors, family history, physical findings, age at operation, side of operation, and pathology of resected tissue in order to determine if any of these factors were associated with outcome. Overall, 48% (11/23) of patients were seizure-free, 39% (9/23) had > 75% reduction in seizures, while 13% (3/23) had < 75% reduction in seizures. Only the EEG findings were useful in predicting outcome. When ictal onsets arose from basal-temporal regions, 61% (11/18) of patients were seizure-free, while none (0/5) were seizure-free when seizures arose from mid-posterior temporal regions (P = 0.04). Interictally, if all epileptiform patterns were localized exclusively to one basal-temporal region, a finding that invariably correlated with ictal onsets, 78% (7/9) of patients were seizure-free, while only 29% (4/14) were seizure-free if discharges were bilateral or multifocal (P = 0.04). We conclude that surgery may be a reasonable treatment for some patients with intractable temporal lobe seizures and normal MRI. The best outcomes occur when seizure onsets and interictal epileptiform patterns are exclusive to one basal-temporal region. Unfavorable outcomes are most likely to occur when ictal origins are from mid-posterior temporal regions and when interictal discharges are bitemporal or multifocal in distribution.