Periodic lateralized epileptiform discharges in multiple sclerosis: a case report.

The occurrence of epileptiform abnormalities on the EEG in patients with multiple sclerosis (MS) is rare. The following case correlates the clinical, EEG, MRI, and single photon emission computed tomographic (SPECT) findings in a patient with a long history of MS and acute onset of focal motor seizures and confusion. Two routine EEGs, brain MRI, and brain SPECT were performed. The patient was a 44-year-old woman with a long history of clinically definite MS of the relapsing-remitting and secondary progressive form with three events of focal motor seizures followed by generalized tonic-clonic seizures and postictal confusion. The first EEG done during admission showed periodic lateralized epileptiform discharges in the right temporal region. Brain MRI done several weeks later showed scattered T2 hyperintensities in several locations, including the periventricular and subcortical white matter bilaterally. Brain SPECT using Tc99-Neurolite demonstrated decreased perfusion on the right parietal and temporal lobes. This case suggests that focal motor seizures and a transient state of altered consciousness can be the result of an exacerbation of MS. The neurophysiologic expression of these clinical manifestations may present as periodic lateralized epileptiform discharges on the EEG and decreased regional perfusion on brain SPECT.     

Focal-onset myoclonic seizures and secondary bilateral synchrony

We present a child with myoclonic seizures, epileptic spasms and generalized epileptiform discharges on scalp EEG. Magnetoencephalography and coherence-phase analysis for scalp EEG suggested focal onset in the left hemisphere. Intracranial video EEG confirmed seizure onset in the left frontal lobe with ictal high-frequency oscillations. Left frontal lobectomy suppressed his seizures. Subsets of myoclonic seizures can be focal in origin despite bilaterally synchronized epileptiform discharges on scalp EEG.     

Psychosis and epileptic seizures in Wilson's disease with predominantly white matter lesions in the frontal lobe

Although psychiatric symptoms are not rare in Wilson's disease (WD), their association with epileptic seizures has not been reported. We describe three patients with such clinical manifestations who had predominantly cerebral white matter lesions in the frontal lobe. There were two men and one woman with ages ranging from 20 to 26 yr. The early presentations were psychiatric symptoms and epileptic seizures with or without secondary generalization. The psychiatric features were usually misinterpreted as schizophrenia-like disease and the diagnosis was delayed. The cerebral white matter lesions on magnetic resonance imaging (MRI) were usually asymmetric and mainly restricted to the frontal lobes. The present observation suggests that early onset of psychiatric manifestations and seizures commonly occur in WD with frontal white matter lesions.     

Effect of corticosteroids in a twin child with idiopathic localization-related epilepsy

Corticosteroids have been used only in the treatment of special epileptic syndromes or epileptic encephalopathies, such as infantile spasms. We report an antiepileptic effect of corticosteroids that were used for treatment of nephropathy in a monozygotic twin child with idiopathic localization-related epilepsy (I-LRE). The patient and her monozygotic twin sister exhibited repeated partial seizures at two years of age and electroencephalogram (EEG) showed focal spikes in the occipital area and, on other occasions, the centro-parietal areas. After oral antiepileptic drugs were started, the twins still exhibited occasional seizures. The patient had IgA nephropathy at four years of age and intravenous methylprednisolone and oral prednisolone were administered. Her seizures and epileptiform discharges on EEG disappeared, while her sister continued to have seizures and EEG abnormalities. When the dose of oral predonisone was reduced, the seizures relapsed and EEG again revealed focal spikes. We conclude that corticosteroids exhibit efficacy towards seizures and epileptiform discharges on EEG in patients with I-LRE without epileptic encephalopathies.     

Contribution of magnetic resonance imaging in 100 cases of refractory partial epilepsy with normal CT scans

One hundred epileptic patients were included in this study according to the following criteria: intractable partial epilepsy, normal CT scan and focal EEG abnormalities. Eighty-nine patients were suffering from complex partial seizures of temporal or frontal origin, 55 and 34 cases respectively. Eleven patients presented with only simple partial seizures. MRI was abnormal in 31 patients. The abnormalities were: focal T2 increased signal intensity (13 cases) most often temporal (10 cases), cryptic arteriovenous malformation (4 cases), focal T1 and T2 signal abnormality (4 cases), focal atrophy (2 cases) and multiple abnormal T2 signals scattered in the white matter (8 cases). The site of MRI abnormalities was consistent with electroclinical data in 22 patients, of whom 20 had a temporal lobe epilepsy. Thus MRI proved to be more often abnormal in temporal than in frontal lobe epilepsy (36 p. 100 and 5.9 p. 100 respectively) when the CT scan is normal. However MRI data, particularly focal T2 hypersignals should be confronted to electroclinical and metabolic findings whenever functional surgery is considered.     

Epileptic seizures in subcortical vascular encephalopathy

Cerebrovascular disease is one of the most common causes of epilepsy in the elderly. Most of the studies published relate to cortical infarction, subarachnoid, and intracranial hemorrhage, whereas the incidence of epilepsy from subcortical ischemia, i.e. deep lacunar infarctions and diffuse white matter lesions, is obscure. Therefore, we prospectively examined 18 patients with the precisely defined diagnosis of subcortical vascular encephalopathy (SVE), who were admitted to our hospital due to epileptic seizures (group A), and compared them to a similarly selected group matched for age, sex, risk factors, and neurological deficits with an equivalent severity of SVE but without seizures (group B). Subcortical lacunar infarctions were significantly more frequent in group A than group B (15/18 versus 4/18, p < 0.001), whereas neither the extension, degree, distribution of periventricular white matter changes, nor the presence of internal hydrocephalus, focal or diffuse cortical atrophy showed any statistical significance. However, a temporal constant theta or delta EEG focus was present in 10/18 patients in group A but only in 1/18 patients from group B (p 0.005). 10/18 patients developed epilepsy with further seizures during follow-up. The association of SVE, multiple subcortical lacunas, and temporal EEG abnormalities are suggestive for an increased risk for epileptic seizures, which is particularly important for the treatment of patients with SVE if uncertain paroxysmal episodes occur, e.g. transient ischemic attacks, seizures, or cardiac syncope.     

Epileptic seizures in neurofibromatosis type 1 are related to intracranial tumors but not to neurofibromatosis bright objects

Objective

To investigate the relationship between intracranial lesions and epileptic seizures in neurofibromatosis type 1 (NF1) patients.

Background

NF1 is one of the most common autosomal dominant neurocutaneous disorders, and epilepsy is more prevalent in NF1 patients than in the general population. Epileptic seizures were found to be related to various types of intracranial lesions in NF1 patients.

Methods

The clinical characteristics of NF1 (1986–2006 in Chung-Gung Memorial Hospital), diagnosed on the basis of the criteria of the National Institutes of Health Consensus Conference (1988), were reviewed by 2 neurologists. We diagnosed epileptic seizures of NF1 patients on the basis of clinical appearances and a history of antiepileptic drugs. Magnetic resonance images were also evaluated by 2 neuroradiologists to confirm the locations of brain tumors or neurofibromatosis bright objects (NBOs). The locations of NBOs were classified into 4 categories: cortex and hippocampus, subcortical white matter, basal ganglia, and infratentorial area. The association between the location of the lesions and the occurrence of seizure in NF1 patients was analyzed statistically.

Results

The medical records of 630 NF1 patients were reviewed. In this cohort, 37 (5.87%) NF1 patients had epileptic seizures. The patients include 22 males (59.5%) and 15 females (40.5%). The mean seizure onset age was 14.8 years (2 months to 72 years). The most common seizure pattern was partial onset seizures, 3 simple partial seizures, and 14 complex partial seizures. Other seizure types found include 15 primary generalized seizures (2 absence seizures and 13 generalized tonic–clonic seizures), 2 infantile spasms, and 3 unclassified.A total of 172 (23 with epilepsy and 149 without epilepsy) NF1 patients underwent MRI examinations. NBOs were identified in 16 (69.6%) epilepsy patients and in 108 (72.5%) patients without epilepsy. The location or the number of these intracranial lesions does not show significant correlation with the occurrence of epilepsy in our cohort. Among 11 NF1 patients with intracranial tumors, 4 patients had seizures (36.36%), vs. 19 out of 161 NF1 patients (11.80%) without tumors.

Conclusion

The occurrence of epileptic seizures in NF1 patients is related to intracranial tumors but not to NBOs.     

结节性硬化合并婴儿痉挛症的外科治疗

目的 探讨结节性硬化所致婴儿痉挛症的手术适应证、方法和预后.方法 回顾性分析采用外科手术治疗17例结节性硬化症伴婴儿痉挛症的经验.结果 术后疗效按Engel分级,Ⅰ级11例,Ⅱ级4例,Ⅲ级2例.术前评估为单致痫灶的2例患者均为Ⅰ级.局限在一侧大脑半球的8例多灶性癫痫患者中,6例Ⅰ级,2例Ⅱ级.双侧大脑半球均有致痫灶但以一侧为主的7例患者中,3例Ⅰ级,2例Ⅱ级,2例Ⅲ级.平均智商(IQ)从术前的52.6分提高到61.8分.结论 结节性硬化所致婴儿痉挛症具有良好的外科预后,对部分药物难治性患者在评估确定责任结节后可以考虑进行外科干预.

Abstract：

Objective To investigate the surgical indications, methods and outcomes of infantile spasms with tuberous sclerosis(TS). Method Surgical treatment of 17 infantile spasms patients with TS was reviewed. Single epileptogenic tuber or lobe was resected when a focal epileptic discharge was indicated according to preoperative evaluation and EcoG, and multiple lobes or tubers resection were chosen to deal with hemisphere limited multiple epileptic foci. Anterior corpus callosotomy was added when contralateral hemisphere showed epileptic discharges. Results Acording to follow - up of 3 years in average after surgery, 11 patients had an Engel Class Ⅰ outcome, 4 patients had rare seizure ( Engel Class Ⅱ ), and 2 patients had a reduction in seizure frequency (Engel Class Ⅲ ). The mean IQ of patients was improved from 52.6 to 61. 8. Conclusions Epilepsy surgery in tuberous sclerosis with infantile spasms showes a favourable outcome,and surgical intervention should be considered in some intractable infantile spasms after corresponding tuber is derermined.     

Acute encephalopathy with biphasic seizures and late reduced diffusion with visual disturbance and higher brain dysfunction

Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a recently described clinicoradiologic syndrome. Clinically, a prolonged febrile seizure is followed by subsequent seizures which occur several days after the initial seizure. On MRI, reduced diffusion appears predominantly in the frontoparietal subcortical white matter at the time of the subsequent seizures. The main symptom between the initial and subsequent seizures is disturbance of consciousness. We report a case with AESD who presented 1) reduced diffusion on MRI which was dominant in the occipital lobe, and 2) reversible visual disturbance followed by higher brain dysfunction such as a cognitive deficit and disturbed speech. A 2-year-old Japanese girl was admitted because of visual disturbance which appeared 4 days after a generalized tonic-clonic seizure associated with fever. Two days later, she had another seizure when MRI revealed reduced diffusion in the subcortical white matter. The MRI finding was not typical of AESD in that reduced diffusion appeared dominantly in the occipital lobe. Normal ophthalmologic findings and abnormal visual evoked potential results suggested that her visual disturbance was due to an impaired visual pathway in the subcortical white matter in the occipital lobe. The present case indicates that there is a subgroup of AESD in which the subcortical lesion seen on MRI is dominant in the occipital lobe.     

An unusual case of cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy with occipital lobe involvement

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an autosomal dominant angiopathy caused by a mutation in the notch 3 gene on chromosome 19. Clinically, patients may be asymptomatic or can present with recurrent ischemic episodes and strokes leading to dementia, depression, pseudobulbar palsy, and hemi- or quadraplegia. Additional manifestations that have been described include migraine (mostly with aura), psychiatric disturbances, and epileptic seizures. Neuroimaging is essential to the diagnosis of CADASIL. On imaging CADASIL is characterized by symmetric involvement by confluent lesions located subcortically in the frontal and temporal lobes as well as in the insula, periventricularly, in the centrum semiovale, in the internal and external capsule, basal ganglia, and brain stem; with relative sparing of the fronto-orbital and the occipital subcortical regions. We describe a 49 year old male with CADASIL with absence of temporal lobe findings on MRI but predominant lesions within the periventricular white matter, occipital lobes with extension into the subcortical frontal lobes, corpus callosum and cerebellar white matter. Although CADASIL characteristically presents with anterior temporal lobe involvement, these findings may be absent and our case addresses the atypical imaging findings in CADASIL.     

Clinical spectrum of epileptic spasms associated with cortical malformation.

The spectrum of clinico-electrical characteristics of epileptic spasms associated with cortical malformation was studied in detail. The subjects were 15 patients suffering from spasms and cortical malformation demonstrated by MRI. The types of cortical malformation causing spasms were various, including hemimegalencephaly, diffuse pachygyria, focal cortical dysplasia, and polymicrogyria. Ohtahara syndrome was diagnosed in 3 patients, and West syndrome in 8. Symptomatic localization-related epilepsy preceded West syndrome in 4 patients, and a transition from Ohtahara syndrome to West syndrome was observed in one. West syndrome was followed by symptomatic generalized epilepsy including Lennox-Gastaut syndrome in 4 patients. Nine patients showed a condition which was labeled "epilepsy with partial seizures and spasms" (EPS) and characterized by the coexistence of partial seizures and spasms, and multifocal epileptic discharges on EEG. Spasms occurred only as EPS in 5 patients. EPS appeared following Ohtahara syndrome or West syndrome in 4 patients, and showed a transition to symptomatic localization-related epilepsy in 4. However, EPS did not evolve into generalized epilepsy, and persisted until the time of last follow-up in 5 patients. Therefore, the clinico-electrical pictures of patients with spasms and cortical malformation were diverse and not always limited within those of typical generalized epilepsy.     

A distinct asymmetrical pattern of cortical malformation: large unilateral malformation of cortical development with contralateral periventricular nodular heterotopia in three pediatric cases

PURPOSE: To describe a distinct asymmetrical pattern of cortical malformation with large focal malformations of cortical development (MCDs) and contralateral periventricular nodular heterotopia (PNH). METHODS: We identified three patients with epilepsy and focal EEG abnormalities. Each patient underwent 1.5-Tesla magnetic resonance imaging (MRI) to obtain sagittal T1-weighted, axial fluid-attenuated inversion recovery (FLAIR), fast spin-echo (FSE) T2-weighted, and coronal fast spin-echo inversion recovery (FSEIR) T2-weighted images; coronal spoiled gradient recalled (SPGR) T1-weighted images were obtained in two cases. RESULTS: Patient 1, an 18-year-old right-handed man, had a 4-year history of intractable seizures. MRI revealed a right frontal subcortical heterotopia (SH) and a single left anterior PNH. Patient 2, a 10-year-old left-handed boy, had a 4-year history of epilepsy. MRI revealed a large region of SH in the left temporal, parietal, and occipital lobes and three right-sided PNH. Patient 3, a 16-month-old girl, had medically refractory infantile spasms. MRI revealed a large MCD in the left parietal lobe with contiguous underlying periventricular heterotopia as well as a small contralateral PNH. CONCLUSIONS: These cases together illustrate a distinct asymmetrical pattern of a large focal MCD with small contralateral PNH. The asymmetrical involvement of the two hemispheres suggests that the stage of maximal disruption of cortical development may differ between the two hemispheres. Further study into the mechanisms underlying such asymmetrical patterns of cortical malformation should enhance our understanding of cortical development as well as hemispheric lateralization.     

Epileptic spasms in older pediatric patients: MEG and ictal high-frequency oscillations suggest focal-onset seizures in a subset of epileptic spasms

OBJECTIVES: To elucidate the pathophysiology of intractable epileptic spasms in older children by describing the interictal magnetoencephalography spike sources (MEGSSs), intracranial EEG ictal-onset zones (IOZs) and their ictal high-frequency oscillations (HFOs) and surgical outcomes. METHODS: We studied five patients (4.5-14 years) who underwent surgery following intracranial video-EEG (VEEG) monitoring. We analyzed clinical profiles, MRIs, scalp and intracranial VEEGs, and MEGSSs. We localized ictal HFOs using a sampling rate of 1000 Hz and multiple band frequency analysis (MBFA). RESULTS: Seizure onset ranged from 0.4 to 8 years. Three patients presented with asymmetrical spasms. Interictal scalp VEEG recorded predominantly unilateral epileptiform discharges in four; generalized and multifocal in one. Ictal scalp VEEG showed generalized high-amplitude slow waves with superimposed fast waves in four patients; hemispheric electrodecremental episodes in one. MRI findings were normal in three, hemispheric polymicrogyria and periventricular heterotopia in one each. All patients had unilateral MEGSS clusters. Ictal HFOs, ranging from 150 to 250 Hz, localized over Rolandic and frontal regions in four, with one also having extensive temporo-occipital HFOs. After cortical resection, three patients were seizure free; one had >90% reduction in seizure frequency. One patient experienced residual seizures after resection of the hemispheric ictal HFO region. CONCLUSION: Unilateral clusters of MEGSSs overlapped regional IOZs in older patients with epileptic spasms. High spatio-temporal MBFA before and during spasms revealed the regional ictal HFOs. Seizure-free outcomes following resection of zone with MEGSS clusters and ictal HFOs suggested that a subset of epileptic spasms was focal-onset seizures.     

Interictal temporal lobe epileptiform discharges and their relationship to secondarily generalized epilepsy

In a previous study of epileptic phenomena in 19 patients with partial complex seizures, it was noted that seizures of left temporal lobe origin had a higher incidence of secondary generalization. To evaluate this observation further, we retrospectively reviewed reports of EEGs for evidence of focal interictal epileptiform discharges (FIED) of temporal lobe origin and correlated this finding with seizure type. Of 3,276 EEG reports reviewed, 195 showed FIED. The medical records of these patients were reviewed and 79 had sufficient information therein to enable seizure classification. Of 79 patients, 61 had secondary generalized seizures, 45 with left temporal FIED, 16 with right FIED. Of 79 patients, 18 had partial seizures, 13 with right temporal FIED, 5 with left FIED (p less than 0.001). These findings suggest that seizures of left temporal lobe origin may have a higher incidence of secondary generalization. The significance of this observation and its relevance to medical and surgical treatment of complex partial seizures is discussed.     

Merosin-negative congenital muscular dystrophy,occipital epilepsy with periodic spasms and focal cortical dysplasia. Report of three Italian cases in two families

We report clinical, EEG and neuroimaging findings of three patients in two Italian families with merosin-negative congenital muscular dystrophy (CMD), drug-resistant occipital epilepsy, diffuse persistent cerebral white matter changes and focal cortical dysplasia. Clinical and epilepsy histories, EEG and neuroimaging findings were very similar in all patients. Seizures started in childhood and mainly consisted of periodic spasms, a particular type of partial seizure characterized by clusters of epileptic spasms. The motor expression of the spasms was very mild so that they had been frequently missed or misinterpreted as non-convulsive generalized absence seizures. Interictal EEG showed occipital spike-waves and bilateral synchronous slow spike-wave discharges. Ictal EEG showed prolonged periodic sequences of slow waves with associated fast rhythm complexes, characteristic of periodic spasms. Two patients had normal intelligence, one patient presented moderate mental retardation. Focal cortical dysplasia in the posterior areas of the brain, in addition to marked diffuse white matter alterations, was detected in the magnetic resonance images of all patients. Findings in these patients indicate that in merosin-negative CMD brain involvement can include cortical dysplasia, in addition to white matter changes. In such cases the brain damage can lead to a childhood-onset localization-related symptomatic occipital epilepsy. Epileptic seizures and cortical dysplasia can be, however, difficult to detect in CMD. The clinical semiology of epileptic seizures may in fact be modified because of muscular weakness. This implies that epilepsy may be misdiagnosed or even missed and EEG-polymyographic recordings may be necessary to identify it. Similarly, cortical dysplasia may be very localized and visible by neuroimaging only if it is carefully investigated on the basis of epileptological and EEG-polymyographic findings.     

Temporal lobe epilepsy caused by domoic acid intoxication: Evidence for glutamate receptor–mediated excitotoxicity in humans

We describe the development of temporal lobe epilepsy in an 84-year-old man who had suffered domoic acid intoxication. Following intoxication he had nausea, vomiting, confusion, and coma. Generalized convulsions and complex partial status epilepticus progressively developed. After 3 weeks he improved and was seizure free with severe residual memory deficit. Electroencephalograms initially showed periodic epileptiform discharges, later evolving to epileptic abnormalities over frontotemporal regions with diffuse slow waves. Eight months after the intoxication the electroencephalogram was normal. One year after the acute episode, complex partial seizures developed. Electroencephalograms showed epileptic discharges independently over both temporal lobes, with left-sided predominance. Magnetic resonance imaging revealed a hyperintense T2-weighted signal and atrophy of both hippocampi; a positron emission tomographic scan showed bitemporal decreased glucose metabolism. Pneumonia developed and the patient died 31/4 years after the intoxication. Autopsy disclosed severe bilateral hippocampal sclerosis. The seizures following acute domoic acid intoxication, the postmortem pathology, and the fact that temprol lobe epilepsy developed 1 year after intoxication indicate that the human hippcampus is also vulnerable to kainate receptor excitotoxicity, and provide strong evidence supporting the role of excitotoxic injury in epileptogenesis. This report provides a unique human parallel to, and validates the animal model of, Kainate-induced epilepsy as an important tool for studying temporal lobe epilepsy.     

Lesion Localization in Periodic Lateralized Epileptiform Discharges: Gray or White Matter

Summary: We analyzed the results of neuroimaging studies in patients with periodic lateralized epileptiform discharges (PLEDs) or bilateral independent periodic lateralized epileptiform discharges (BIPLEDs) for localization of lesions in gray or white matter to determine if “cortical isolation” is a critical mechanism in the pathogenesis of this phenomena. We assessed 32 patients who had undergone computed tomography (CT) exclusively and 8 patients who had undergone magnetic resonance imaging (MRI) with or without CT. The superior resolution necessary for adequate lesion localization allowed use of only the MRI scans from the 8 patients. Six patients had scans with cortical and subcortical gray and white matter lesions, and 1 patient had a cortical gray matter lesion only. One patient had an indeterminate scan. No patient had white matter lesions only. Our findings in patients with PLEDs and BIPLEDs correlate with postmortem data in patients with generalized periodic EEG patterns that show consistent localization of lesions in the gray matter. These findings do not support cortical isolation as the critical or sole mechanism in PLEDs or BIPLEDs.     

Generalized periodic epileptiform discharges in a child with Dravet syndrome

Generalized periodic epileptiform discharges can occur in a variety of epileptic syndromes and herald impending seizures. Such discharges are also extremely rare in children. Cerebral visual impairment can be associated with generalized and partial onset seizures, particularly those involving the occipital lobe. The authors present a case of a 10-month-old boy whose electroencephalogram revealed generalized periodic epileptiform discharges following resolution of status epilepticus. Such discharges warranted further monitoring, during which he experienced 2 additional seizures. He also was discovered to have cerebral visual impairment, which slowly resolved following termination of seizure activity. The child was subsequently found to have a de novo mutation of the sodium channel, voltage-gated, type I, alpha subunit (SCN1A) gene consistent with Dravet syndrome.     

West syndrome in Thailand: a hospital-based survey.

The prevalence of West syndrome in Thailand had not been studied. During January 1997 to December 1999, at the Department of Pediatrics, Ramathibodi Hospital, Bangkok, Thailand, of the total 628 first seen epileptic patients, aged 0-15 years, 31 patients (4.9%) with West syndrome were encountered. Sixteen were males. The mean age at presentation was 9.7 months (range 4-32 months) and at seizure onset was 5.7 months (range 2-11 months). Twenty-two patients had typical spasms, four each had generalized tonic seizures and focal seizures prior to the onset of infantile spasms, and another patient had myoclonic seizures. Nineteen patients were classified as symptomatic. Electroencephalogram revealed hypsarrhythmia in six patients, multifocal independent epileptiform discharges in 24 patients, and focal epileptiform discharges in four patients. Abnormal brain-imaging findings were demonstrated in six of the 11 patients investigated. Vigabatrin was given to 17 patients whereas one patient was given valproate. Others who had been severely retarded were given phenobarbital in combination with benzodiazepine. Six patients were lost to follow-up. Of the remaining patients, the duration of follow-up ranged from 7 to 44 months (mean 28 months). Infantile spasms disappeared in 11 patients, persisted in six patients, and changed to other form in eight patients. The developmental outcome was apparently normal in five, slightly delayed in four, moderately delayed in ten, and severely delayed in eight patients.     

Surgical outcomes for intractable epilepsy in children with epileptic spasms

Epileptic spasms, or seizures marked by flexor, extensor, or flexor-extensor spasms, are not always responsive to medical management. The purpose of our study was to evaluate the outcome of epilepsy surgery in children with medically intractable epileptic spasms. We identified 11 children with epileptic spasms who underwent lesionectomy (36%), lobectomy (27%), multi-lobectomy (9%), hemispherectomy (18%), or corpus callosotomy (9%). At the time of surgery, 6 children had developed other concurrent seizure type(s), including simple partial (9%), complex partial (27%), partial undifferentiated (9%), primary generalized tonic clonic (9%), tonic (9%), atonic (27%), and myoclonic (9%) seizures. Six children (55%) were seizure free at last follow-up from initial surgery. Predictors of favorable outcome included lack of focal slowing and the presence of less than 2 interictal epileptiform abnormalities on postoperative electroencephalogram (P = .035 and .035, respectively). Favorable outcome was significantly associated with parent/caregiver report of improved postoperative developmental outcomes (P = .026).