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1.
Patients with Sturge-Weber syndrome often present with seizures during the first year of life. Currently, only patients with clinically significant seizures who do not respond to medical treatment are candidates for early epileptic surgery. However, a delay of surgical treatment may result in cognitive deterioration. We studied the correlation between parameters and outcome of seizures to re-examine the criteria for early epilepsy surgery. We performed a retrospective chart review combined with telephone interviews of parents of all Israeli infants with unilateral Sturge-Weber syndrome and early onset seizures, and we examined whether age of seizure onset and seizure intensity were correlated with cognitive level and the degree of hemiparesis at follow-up. We recruited a total of 15 patients with unilateral Sturge-Weber syndrome and early onset seizures, five of whom underwent epilepsy surgery. The mean follow-up period of all the patients was 15 years: six patients had normal intelligence, four had borderline cognitive level, three had mild mental retardation and two had moderate mental retardation. Eight of the ten non-operated patients still experience seizures at follow-up. Cognitive delay was significantly correlated with seizure intensity in the early period, but not with the age of seizures onset, the degree of hemiparesis, or the presence of ongoing seizures. We conclude that high seizure intensity in young patients with Sturge-Weber syndrome is a prognostic marker for mental deterioration.  相似文献   

2.
Outcomes of 32 hemispherectomies for Sturge-Weber syndrome worldwide   总被引:7,自引:0,他引:7  
Kossoff EH  Buck C  Freeman JM 《Neurology》2002,59(11):1735-1738
BACKGROUND: Epilepsy affects 80% of patients with Sturge-Weber syndrome; the majority of seizures begin before the age of 1. When seizures are intractable to medications and unihemispheric, hemispherectomy is often advised. OBJECTIVE: To examine the natural history of patients who underwent hemispherectomy and identify the outcomes in terms of seizure reduction, cognition, and motor deficits. METHODS: A questionnaire was mailed to the parents of patients identified by the Sturge-Weber Foundation as having had a hemispherectomy between 1979 and 2001. Forty-six percent (32/70) of the parents responded. RESULTS: The mean age at onset of seizures was 4 months, and the median age at surgery was 1.2 years. Children had failed to respond to 3.7 anticonvulsants prior to surgery and averaged 387 seizures/month. Forty-seven percent had complications (e.g., hemorrhage and hypertension) in the perioperative period; however, 81% are currently seizure-free, with 53% off anticonvulsants. Hemispherectomy type (anatomic versus functional versus hemidecortication) did not influence outcome. Age at onset of seizures did not predict seizure freedom; however, an older age at hemispherectomy was positively correlated. Postoperative hemiparesis was not more severe than before surgery. Cognitive outcome was not related to the age at operation, side of operation, or seizure freedom. CONCLUSIONS: Children undergoing hemispherectomy presented at a young age and had frequent seizures for approximately 1 year but are now mostly seizure-free. Age at surgery did not have an adverse effect on either seizure or cognitive outcomes.  相似文献   

3.
目的 探讨多脑叶切除治疗Sturge-weber综合征.方法 结合国内外文献,分析1例采用右额颞顶枕开颅选择性多脑叶切除联合致痫皮层热灼术治疗Sturge-weber综合征.结果 术后随访6个月有一次癫痫小发作,无功能障碍.结论 Sturge-weber综合征药物治疗无效,手术治疗是唯一有效的方法.大脑半球切除术最为有效,但并非所有类型的都适合,有的病例导致严重的功能障碍.我科首例行选择性多脑叶切除 致痫皮层热灼术,效果满意.  相似文献   

4.
The Sturge-Weber syndrome includes unilateral cerebral cortical angiomatosis, which often leads to progressive cerebral dysfunction and epileptic seizures that are medically difficult to control. Cerebral resections and hemispherectomy have been successfully performed in the past in intractable epileptic cases. Two children with medically unresponsive generalized seizure activity secondary to the Sturge-Weber syndrome have been surgically treated by dividing their corpus callosum. Cessation of generalized epilepsy was achieved in both cases. Corpus callosotomy is presented as a less destructive and safer procedure in dealing with intractable seizures in the Sturge-Weber syndrome.  相似文献   

5.
Outcome of Epilepsy Surgery in the First Three Years of Life   总被引:18,自引:5,他引:13  
PURPOSE: We analyzed our experience over a 6-year period with early-childhood patients who had undergone epilepsy surgery, and investigated the surgical outcomes. METHOD: We reviewed the medical records of 23 children, ages 0-3 years, who underwent epilepsy surgery between 1991 and 1996. RESULTS: Twenty children had partial seizures; two had infantile spasms; and one had generalized tonic-clonic seizures at onset. The mean age at onset of seizures was 4.7 months, and the mean age at time of surgery was 15.3 months. A total of 32 operations (21 focal cortical resections and 11 hemispherectomies) was performed. Five of 12 children with seizures secondary to a neuronal migration disorder had reoperations, including three who ultimately underwent complete hemispherectomy. The pathology consisted of hemimegalencephaly in three patients, focal cortical dysplasia (FCD) in eight, tuberous sclerosis in one, Sturge-Weber syndrome (SWS) in five, infarction in two, low-grade glioma (LGG) in three, and post-herpes simplex virus encephalitis (HSE) in one. The follow-up period ranged from 1 to 6.5 years (mean, 3.2 years) from patients' last operation. The seizure outcome according to Engel's criteria was class I in 12 patients, class II in three, class III in six and class IV in two. CONCLUSIONS: Seizure outcomes after surgery were less favorable in infants with FCD than in those with SWS and LGG. Seizure outcome for the patients with hemispherectomies was excellent, compared with those who had focal cortical resections.  相似文献   

6.
Sturge-Weber syndrome is a neurocutaneous syndrome with a facial port-wine nevus and neurologic features, typically including seizures and hemiparesis. Glaucoma may also occur. MRI features include leptomeningeal angiomatosis, cortical and pial calcifications, and angiomatous change of the choroid plexus. We reviewed a subset of patients with Sturge-Weber syndrome with the rare finding of deep venous occlusion, and present such a case, unusual by comparison to previously reported cases of Sturge-Weber syndrome with deep venous occlusion. Six previously reported cases were reviewed. All cases presented with seizures; five of six had evidence of leptomeningeal angiomatosis; half had cerebral hemiatrophy. This report presents a unique case lacking clinical seizures, but with a port-wine stain and congenital glaucoma. This patient lacked the radiologic findings of leptomeningeal angiomatosis and hemicerebral atrophy, but demonstrated deep venous occlusion with frontal venous collaterals. There is a wide spectrum of findings in Sturge-Weber syndrome. The lack of seizures and angiomatosis in this case are likely "true-true" and related. The case illustrates the unusual finding of deep venous occlusion in Sturge-Weber syndrome occurring without leptomeningeal angiomatosis. Additionally, it demonstrates that although the initial evaluation is normal, patients may later manifest clinical characteristics of Sturge-Weber syndrome.  相似文献   

7.
BackgroundWe describe the brain magnetic resonance imaging (MRI) abnormalities and neuropathologic findings of patients with Sturge-Weber syndrome and medically refractory epilepsy.MethodsWe reviewed the clinical features, preoperative MRI studies, and pathologic findings of all patients with Sturge-Weber syndrome who underwent excisional surgery for intractable epilepsy at Boston Children's Hospital between 1993 and 2011.ResultsEleven patients (male/female = 4/7) with Sturge-Weber syndrome were identified who underwent surgery for intractable epilepsy (mean age 13 ± 6.2 months), including hemispherectomy (n = 10) and focal cortical resection (n = 1). Mean age at seizure onset was 15 ± 11 weeks. Fifty-five percent (n = 6) of patients exhibited two different types of seizures, and 18% (n = 2) had three types of seizures. Focal clonic seizures were the most common type, occurring in nine patients; apnea was the second most common, occurring in four patients. Brain MRIs were reviewed in five patients. Histopathologic examination revealed varied degrees of cortical morphologic anomaly in seven of 11 patients. Overall, there were no abnormalities in the MRIs that corresponded directly with the pathologic findings except in one patient with polymicrogyria.ConclusionsIn spite of pathologic findings of cortical anomalies in varied degrees, these findings could not be readily detected on brain MRIs. The failure to detect focal cortical dysplasia on MRIs may be attributable to the subtle microscopic nature of the abnormalities; in some of the older individuals, the imaging studies available for review were done during an advanced stage of the disease.  相似文献   

8.
Approximately 15% of patients with Sturge-Weber syndrome demonstrate bilateral intracranial involvement, and the prognosis of these patients is considered particularly unfavorable. We reviewed the clinical and neuroimaging features of patients with Sturge-Weber syndrome and bilateral intracranial involvement. Seizure variables, the presence of hemiparesis, and the degree of developmental impairment at most recent follow-up were compared with imaging abnormalities. Of 110 Sturge-Weber syndrome patients, 14 demonstrated bilateral brain involvement, with an asymmetric pattern on glucose metabolism positron emission tomography. Although most patients manifested frequent seizures initially, associated with frontal hypometabolism on positron emission tomography, six (43%) had achieved good seizure control during follow-up. Bilateral frontal hypometabolism was associated with severe developmental impairment. Two children with bitemporal hypometabolism exhibited autistic features. Hemiparesis was associated with superior frontal (motor cortex) hypometabolism. Three patients underwent resective surgery, resulting in improved seizure control and developmental outcomes. The severity of neurologic complications and clinical course depend on the extent of cortical dysfunction in bilateral Sturge-Weber syndrome. Bilateral frontal and temporal hypometabolism is associated with poor developmental outcomes. Good seizure control and only mild/moderate developmental impairment can be achieved in about 50% of patients with bilateral Sturge-Weber syndrome, with or without resective surgery.  相似文献   

9.
OBJECTIVE: The objective of this paper is to evaluate factors of surgical morbidity from different techniques of hemispherectomy with emphasis on causative pathology. PATIENTS AND METHODS: Thirty patients underwent hemispherectomy in our institution from 1987 to 2003, two presented with Sturge-Weber Syndrome (SWS), sixteen with Rasmussen's Syndrome (RS), eight with established hemispheric lesions (EHL), and four with cortical development malformations (CDM). Six surgeons operated on three patients using anatomical hemispherectomies (AH), 11 patients using functional hemispherectomy (FH), and 16 patients employing hemispherotomy (HT). Surgical technique and causative pathology were studied independently as factors of morbidity in hemispherectomy. RESULTS: Overall mean surgical time was 11:50+/-3:20 h and increased proportionately in pathologies with larger hemispheres. Blood transfusion was particularly influenced by the approach adopted by our team of anesthesiologists, independently of technique or pathology. Pathology was the most important factor related to hydrocephalus as two out of four patients with CDM needed ventriculoperitoneal shunt whilst none with EHL or SWS. Four patients undergoing HT and one FH presented residual bridges connecting the hemispheres, three were reoperated and are seizure free. Two patients with CDM did not improve their seizures worthwhile with surgery and other two (one with RS and other with CDM) were waiting a second procedure due to incomplete inter-hemispheric disconnection. Five patients presented infection and one died after developing meningoencephalitis. CONCLUSION: Hemispherectomies are procedures where pathology and surgical technique interact narrowly. Therefore, in order to study surgical morbidity or outcome, both pathology and technique have to be analyzed independently.  相似文献   

10.
Sturge-Weber syndrome: indications and results of surgery in 20 patients   总被引:6,自引:0,他引:6  
OBJECTIVE: To discuss the indications and timing for resective surgery in patients with Sturge-Weber syndrome (SWS) and medication-resistant epilepsy. BACKGROUND: SWS that causes epilepsy severe enough to merit surgery is rare. Because of the variable natural history of the disease, it is difficult to establish clear-cut indications for surgery and prospective studies are not feasible. Attitudes of clinicians and surgeons remain variable. METHODS: The authors assessed the presurgical epilepsy profile, criteria for surgery, monitoring techniques, and the postoperative outcome of epilepsy in all patients with SWS consecutively admitted between 1972 and 1990 to two referral centers (Paris and Montreal) and underwent surgery for intractable seizures. RESULTS: All 20 patients had a minimal postoperative follow-up of 4 years and all but one are still followed by one of the authors. One patient had a callosotomy, five underwent hemispherectomy, and 14 had cortical resection. Despite variability in the age at onset of seizures (range: 2 months to 12 years), age at operation (range: 8 months to 34 years) and surgical methods, almost all patients benefited from surgery. Visually guided complete resection of the pial angioma and underlying cortex, whenever possible, seemed sufficient; results were no better with intraoperative corticography. In children with previous hemiparesis, hemispherectomy proved particularly effective: all five became seizure free. None of the patients showed any aggravation of cognitive impairment following surgery; none of those who were operated on early presented with severe mental retardation, and 13 of 20 became seizure free. CONCLUSION: Although the natural history of SWS is imperfectly known, increasing duration of seizures and of postictal deficits, increase in atrophy or of calcified lesions or both, are indicative of its progressive nature. Despite the expected heterogeneity that renders formal comparison of the various approaches difficult, the current study provides new evidence to support early surgery in patients with SWS and drug-resistant epilepsy. The authors' results suggest that lesionectomy is a good approach, provided that the pial angioma is unilateral and the resection can be complete.  相似文献   

11.
INTRODUCTION: Sturge-Weber syndrome is a neurocutaneous disease associating facial and pial angioma. Focal epilepsy is a common sign. In a few cases, generalized seizures have been reported. CASE REPORT: We report on a four-year-old girl with Sturge-Weber syndrome. The first focal seizures occurred at three years of age. She developed refractory status epilepticus. At discharge from the PICU, she was on a ketogenic diet and received three antiepileptic drugs. No seizures were observed for four months. The patient then developed several types of seizures: myoclonic seizures, focal clonic seizures, and sudden falls. We were unable to determine the etiology of the falls. Typical myoclonic astatic seizures were identified on video-electroencephalographic recordings. CONCLUSION: Seizures in Sturge-Weber syndrome are usually focal. Four patients with Sturge-Weber syndrome and myoclonoastatic seizures are reported in the literature. We discuss the pathophysiological mechanisms leading from a focal lesion to generalized myoclonoastatic seizures.  相似文献   

12.
Patients with refractory seizures, including those with Sturge-Weber syndrome, undergo functional studies in preparation for surgery. Perfusion studies in Sturge-Weber syndrome by single photon emission computed tomography and positron emission tomography generally demonstrate hypoperfusion in the diseased tissue. We report perfusion-weighted magnetic resonance imaging results in 2 cases of Sturge-Weber syndrome with recent seizures. The affected cerebral tissue showed increased relative cerebral blood flow and volume with prolonged mean transit time and time to peak. Elevated relative cerebral blood flow could be attributed to seizures, whereas increased relative cerebral blood volume might have resulted from vasodilation due to seizure activity or chronic ischemia. These findings point to the variable results of functional studies in Sturge-Weber syndrome that might lead to miscalculations of the lesion area before surgery.  相似文献   

13.
Cerebral functional imaging methods provide information on the location of the epileptic focus in partial epilepsy of adults. We report our experience of one of these methods, single photon emission computed tomography (SPECT), in epilepsy of children. SPECT enables the regional cerebral blood flow (rCBF) to be measured, after inhalation or injection of 133-Xenon, on 5 contiguous, 20 mm thick axial sections, with a 14 mm resolution and negligible brain irradiation. In Sturge-Weber syndrome (13 patients aged 9 months to 18 years) the rCBF was reduced in the same territory as CT abnormalities suggesting ischaemia of the brain tissue lying below the pial angioma; the SPECT image facilitated the diagnosis in 3 patients with atypical CT. In hemimegalencephaly (6 patients aged 1 month to 10 years) the rCBF was extremely low in the hypertrophic hemisphere and in 1 case the SPECT image was determinant in the decision to perform hemispherectomy. In partial epilepsy with normal CT and/or MRI (42 children aged 1 to 15 years) the rCBF was abnormal in 83% of the patients, and its abnormality was located in the same area as the EEG focus in three quarters of the cases. Between seizures, the rCBF was low in 3 out of 4 cases and abnormality decreased after the seizures had ceased (6 patients explored twice); it was high in 1 out of 4 cases. Thus, in children as in adults, cerebral functional imaging provides new data which contribute to the localization and follow-up of epileptic foci.  相似文献   

14.
We report a patient with Sturge-Weber syndrome without facial angioma, who presented with seizures and normal initial imaging results. The patient experienced several years without seizures before a sudden increase in seizure frequency, followed by an atypical evolution of imaging findings prompting biopsy to establish the diagnosis. This case highlights not only the rare presentation of isolated leptomeningeal angiomatosis, but also the potential for atypical evolution of imaging findings through the course of the disease. We detail the imaging findings of our case and review the potential pathophysiological basis for this appearance. Our experience suggests that repeat imaging is warranted in patients with suspected Sturge-Weber syndrome or those with intractable cryptogenic epilepsy, because some imaging features of Sturge-Weber syndrome may manifest over time.  相似文献   

15.
In a child with left hemimegalencephaly and seizures, a reversible operculum syndrome developed when continuous epileptic discharges spread from the left hemisphere to the contralateral central regions. The operculum syndrome lasted for three months until left hemispherectomy was performed. Soon after surgery the seizures and the operculum syndrome resolved. The operculum syndrome is a facio-pharyngo-glosso-masticatory diplegia usually due to structural lesions in both opercular regions. The reversibility of the syndrome in the reported case demonstrates that the operculum syndrome is sometimes functional rather than lesional.  相似文献   

16.
《Journal of epilepsy》1994,7(4):285-289
To define the contribution of the EEG evaluation in children with seizures and Sturge-Weber syndrome, we reviewed EEG data in 14 radiologically confirmed cases. Thirteen exhibited marked voltage attenuation that was localized to the region of the cerebral angiomatosis. Polymorphic slowing occurred ipsilateral to the lesion in six patients and was bilateral in six. By contrast, interictal spikes were evident in only two patients and did not always correlate with the angiomatosis. Seizures were captured in four patients and consistently revealed ictal discharges only at the periphery of the lesion. These findings indicate that abnormalities of EEG background rather than interictal spikes are consistently present early in the course of Sturge-Weber syndrome and correlate with the region of angiomatosis.  相似文献   

17.
Epilepsy surgery in bilateral Sturge-Weber syndrome   总被引:2,自引:0,他引:2  
Two infants with severe drug refractory focal epilepsy caused by Sturge-Weber syndrome and extensive cerebral leptomeningeal angiomatosis were referred for preoperative video-electroencephalographic evaluation. Brain imaging with computed tomography and gadolinium-enhanced magnetic resonance imaging demonstrated bilateral disease in both children with a predominance of involvement of one hemisphere. Clinical examination and neurophysiology with ictal video recording demonstrated epileptogenesis from one hemisphere. Successful surgical treatment with functional hemispherectomy was followed by good long-term seizure control in both patients. The dramatic seizure control was accompanied by markedly improved quality of life for the family and children. These cases indicate that the spectrum of children that may benefit from epilepsy surgery should not be viewed too restrictively, and subsets of children with localization related epilepsy caused by extensive lesions may be resective surgical candidates with a good seizure outcome prognosis.  相似文献   

18.
Sturge-Weber syndrome is associated with leptomeningeal angioma, trigeminal port-wine stain, epilepsy, and glaucoma. Clinically, many patients with Sturge-Weber syndrome are observed to have both seizures and headaches, but this has never been described in the literature. A questionnaire was mailed to 190 patients with reported comorbid epilepsy and headache as identified by the Sturge-Weber Foundation. Sixty-eight surveys were returned anonymously; 55 reported both seizures and headaches. The median age at headache onset was 8 years, with a median of three headaches per month. Fifty-eight percent felt that headaches were an equal or greater problem. Ibuprofen and acetaminophen were the most frequently tried abortive medications; 22% had tried sumatriptan. Only 22% reported a neurologist suggesting the use of an anticonvulsant as a preventive agent. Subjects with a family history of headaches had an earlier age at headache onset (7.5 vs 11 years; P = .02), and those with a family history of seizures were more likely to report behavior problems (69% vs 33%; P = .02). Subjects reporting learning problems or hemiparesis had an earlier age at seizure onset. Migraine-like headaches can be as significant a problem as epilepsy for patients with Sturge-Weber syndrome. Despite this, triptans and prophylactic medications (including anticonvulsants) were used in less than half of the patients. Correlations of family history with both age at symptom onset and behavior problems suggest that genetic substrate could be one factor determining the variable neurologic manifestations seen in Sturge-Weber syndrome.  相似文献   

19.
A case of Sturge-Weber disease treated with left hemispherectomy presented, 11 years later, with complications related to delayed intracranial haemorrhage. A loculation syndrome of the right lateral ventricle was detected and it was corrected by a ventriculoatrial shunt operation. The side of the hemispherectomy was evacuated of all the chronic products of haemorrhage, including the subdural membrane. The patient was relieved of her symptoms. It is considered that complications related to delayed haemorrhage after hemispherectomy are remediable.  相似文献   

20.
Background Perinatal cerebral artery occlusion is responsible for ischemic cerebral infarction leading to brain cavitation and gliosis; the territory of the middle cerebral artery is most frequently involved. The resulting poroencephalic cysts are frequently associated with hemiplegia and epilepsy; that can be managed medically in most cases, only 6–7% of them being refractory to medical treatment. This particular subset of congenitally hemiplegic children will be possible candidates for electrophysiological investigation and eventually for resective surgery. Whatever the kind of surgical treatment, surgery should be performed as soon as possible to optimize functional brain reorganization.Clinical material Twelve children with poroencephalic cysts and refractory epilepsy were studied and operated on at the Divisions of Child Neurology and Pediatric Neurosurgery, the Catholic University Medical School, Rome. The hemiparesis ranged from mild to moderate; the developmental delay was of mild degree in three cases, moderate in four cases and severe in the remaining five. Behavioral disorders were observed in patients with mental retardation; two of them also manifested autistic features. All the children presented with a severe epileptic syndrome (starting almost invariably during the first year of life); six patients presented with a West syndrome followed by symptomatic partial epilepsy; the other six presented with partial epilepsy, followed in two cases by continuous spike-waves during sleep. The electroencephalograph (EEG) recordings disclosed focal unilateral interictal epileptiform abnormalities that usually corresponded to the side of the cystic lesion; however, paroxysmal activity often spread synchronously over the contralateral hemisphere. The selection of candidates for surgical treatment was based on neuroimaging and video-EEG monitoring; in particular, we did not use invasive intraoperative neurophysiologic techniques. The convergence of neuroimaging and neurophysiologic findings guided us in performing a limited cortical excision corresponding to the malacic cortex (cyst “membrane”).Results All the patients underwent excision of the cyst wall. Careful attention was paid not to enter the body of the lateral ventricle to avoid ventriculo–subarachnoid fistulas, eventually responsible for subdural hygroma or cerebrospinal fluid leak. There was one surgery-related death secondary to disseminated intravascular coagulation, following an otherwise uneventful surgical procedure. An elevated systemic blood pressure, secondary to repeated adrenocorticotropic hormone therapy, can represent a possible concurrent factor for this event. No major complications were recorded among the remaining 11 children. Seizure control was excellent in all the 11 survivors in the early postoperative period. Two children presented a relapse of seizures, after an initial improvement, respectively 3 and 4 years after the operation. These two children underwent subsequently a functional hemispherectomy. Overall, seizure outcome was excellent in all the cases. Seven patients (including the two who underwent functional hemispherectomy) are seizure-free (Engel’s class Ia), and in one of them antiepileptic therapy has been weaned. In the remaining five children, seizures are sporadic and definitely improved (Engel’s class II). An improvement of developmental delay, in particular of cognitive competence, was registered in 8 out of the 11 patients. Two of the four severely retarded children, who also presented behavioral abnormalities, did not show any cognitive improvement, whereas some mild improvement of their basal abilities was demonstrated in the other two. All the remaining children, even though maintaining a moderate retardation, definitely improved their abilities; in particular, one of them reached an almost borderline level. The three patients with unchanged neurodevelopmental delay presented also persistent seizures. On the other hand, two children with persistent seizures presented neurodevelopmental improvement.Conclusions Simple surgical excision of the cyst “membrane” of epileptogenic poroencephalic cysts can represent an excellent means to control epilepsy in affected children. However, postoperative seizure persistence and late recurrences, although rare, do not allow to exclude that hemispherectomy or partial resections (based on electrocorticography findings) might represent the good answer at least in some cases.  相似文献   

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