Characteristics of epilepsy in focal cortical dysplasia in infancy

To describe the poorly known characteristics of epilepsy during infancy in focal cortical dysplasia (FCD), one of the most frequent cause of infantile epilepsy. All 28 patients with FCD referred to two specialized centres were retrospectively studied regarding seizure characteristics, psychomotor evaluation, and response to medical and surgical treatment. All patients presented with early partial seizures. Semiology, but not the age of onset, depended on the topography of the dysplasia, with abnormal eye movements in all cases of posterior FCD. Eleven patients also developed infantile spasms (IS), mainly asymmetrical. IS were easily controlled with Vigabatrin or ACTH, but no partial seizures could be medically controlled except in one patient. All patients except one had abnormal neuropsychological findings. Fifteen patients had surgery, eight became seizure free, and seven were significantly improved regarding psychomotor development. Very early and refractory partial seizures, but easily controlled IS are the main characteristics of FCD in infancy. Only the focal ictal semiology may help differentiate the localization of FCD. Its intrinsic epileptogenicity could sustain this clinical pattern. Since the chances for medical control and normal neurodevelopment are poor, surgical treatment should be considered early in infants with FCD.     

Epilepsy surgery in children with focal cortical dysplasia (FCD): results of long-term seizure outcome

The purpose of this study was to assess the effect of epilepsy surgery on seizure outcome in children and adolescents under 18 years with intractable epilepsy due to focal cortical dysplasia. We analysed clinical data, such as age at seizure onset, epilepsy course, localisation of focus from presurgical evaluation, MRI, tissue pathology and seizure outcome in 68 patients 6 months to 9 years after epilepsy surgery. Seizure outcome was classified according to the Engel classification. Mean age at seizure onset was 7 months, ranging from the first days of life to 7 years. All patients had medically intractable epilepsy. Localisation of the lesion was predominantly extratemporal: posterior (uni- or multilobar) 43 %, frontal without central region 26 %, multilobar involving central area 19 % and temporal in 12 %. MRI signs typically seen in cortical dysplasia (FCD) such as localised blurring of gray-white matter junction was found in 68 %, dysgyria in 62 %, thickening of the cortical ribbon in 46 % and T2 signal elongation of the subcortical white matter in 40 % of the patients' MRI. Age at surgery ranged from 5 months to 16 years; 14 patients were under 2 years when operated on. In 34 patients (6 patients under 3 years) subdural grid electrode evaluation was performed prior to surgery. Pathology revealed focal cortical dysplasia without balloon cells (type I) in 60 %, FCD of the balloon cell subtype (type II) in 40 % of the specimens. Postoperative complications were subdural hygroma in 5 and an increased motor deficit in 2 patients. Up to two years after epilepsy surgery 50 % of the children were seizure free (Engel class I), 10 % Engel class II, 33 % Engel class III and 7 % unchanged (Engel class IV). Long-term seizure outcome (> 3 years post surgery) in 32 patients showed similar results (class I 50 %, class II 19 %, class III 28 %, class IV 3 %). Complete resection of the dysplastic lesion was significantly correlated with favorable seizure outcome, whereas seizure outcome was not significantly different in patients with mild (type I) or balloon cell (type II) FCD. Children operated after 6 years of age had no better outcome than children operated in infancy or at preschool age. Epilepsy surgery resulted in good (class I and II) seizure control in 60 % of children with intractable epilepsy due to focal cortical dysplasia.     

A comprehensive clinico‐pathological and genetic evaluation of bottom‐of‐sulcus focal cortical dysplasia in patients with difficult‐to‐localize focal epilepsy

Aims. We comprehensively studied the clinical presentation, stereo‐EEG and MRI findings, histopathological diagnosis, and brain somatic mutations in a retrospective series of drug‐resistant patients with difficult‐to‐localize epilepsy due to focal cortical dysplasia at the bottom of a sulcus (BOS‐FCD). Methods. We identified 10 patients with BOS‐FCD from the Cleveland Clinic epilepsy surgery database submitted for intracranial video‐EEG monitoring. Brain MRI, including voxel‐based morphometric analysis and surgical tissue submitted for histopathology, was reviewed. Paraffin tissue samples from five patients were made available for targeted next‐generation sequencing. Postsurgical follow‐up was available in nine patients. Results. BOS‐FCD was identified in the superior frontal sulcus in six patients, inferior frontal sulcus in one patient, central sulcus in one patient, and intraparietal sulcus in two patients. All patients had stereotyped seizures. Intracranial EEG recordings identified ictal onset at the BOS‐FCD in all 10 patients, whereas ictal scalp EEG had a localizing value in only six patients. Complete resection was achieved by lesionectomy or focal corticectomy in nine patients. Histopathologically, six patients had FCD type IIb and three had FCD type IIa. Next‐generation sequencing analysis of DNA extracted from lesion‐enriched (micro‐dissected) tissue from five patients with FCD type II led to the identification of a germline frameshift insertion in DEPDC5, introducing a premature stop in one patient. Eight out of nine patients with available follow‐up were completely seizure‐free (Engel Class IA) after a mean follow‐up period of six years. Conclusion. Our results confirm previous studies classifying difficult‐to‐localize BOS‐FCD into the emerging spectrum of FCD ILAE type II mTORopathies. Further studies with large patient numbers and ultra‐deep genetic testing may help to bridge the current knowledge gap in genetic aetiologies of FCD.     

Factors influencing surgical outcome in patients with focal cortical dysplasia

A total of 120 patients with histologically proven focal cortical dysplasias (FCD) were retrospectively analysed for prognostic factors for successful epilepsy surgery. Multivariate data analyses showed that older age at epilepsy surgery, occurrence of secondarily generalised seizures and a multilobar extent of the dysplasia were significant negative predictors. In univariate analyses, longer duration of epilepsy, need for intracranial EEG recordings and incomplete resection of the FCD were factors which significantly reduced the chance of becoming seizure free. Histological subtype of the FCD and age at epilepsy onset had no significant predictive value. These findings strongly suggest early consideration of epilepsy surgery in FCD patients.     

Epilepsy surgery in children with gliomatosis cerebri

PURPOSE: Gliomatosis cerebri (GC) is a rare neoplastic disorder that may present as intractable epilepsy during early life. We report our experience regarding the evaluation and the surgical treatment of epilepsy in this population. METHODS: All children evaluated between 1990 and 2006 for surgery of epilepsy (n = 741) with pathologically proven GC were selected. RESULTS: We identified four male children with age at seizure onset ranging from 4 months to 11 years. Two had hemiparesis and one child with infantile spasms was developmentally delayed. Seizures occurred daily (n = 3) or monthly (n = 1). Ictal semiology was consistent with psychomotor seizures (n = 1), partial motor seizures (n = 2), and asymmetric epileptic spasms (n = 1). Surgery was symptomatic and aimed at debulking and controlling the epilepsy. Procedure was individually tailored based on the presurgical evaluation. Brain MRI revealed widespread hemispheric involvement (n = 3) or infiltration of the temporal lobe and basal ganglia (n = 1). Two patients were initially misdiagnosed as hemispheric cortical dysplasia and hemimegalencephaly. Scalp EEG was nonlocalizing in two cases, showed a right temporal focus in one case, and was not performed in one case. Interictal SPECT in one patient revealed widespread hemispheric hypoperfusion. Three cases were resected under ECoG guidance after a mean delay of 11 months after seizure onset. Following functional hemispherectomy (n = 1) or focal cortical resection (n = 2), all children were alive and seizure free with a mean follow-up of 48 months (2-5 years). No unexpected complication was reported. One nonoperated case was alive but still seizing after 15 months follow-up. Chemotherapy was associated in three cases. CONCLUSIONS: GC is a rare cause of medically resistant epilepsy that may present in early life. The lack of a discrete lesion may lead to diagnostic uncertainty, especially in infancy. Epilepsy surgery is an effective therapy that can improve quality of life.     

Clinical characteristics,pathological features and surgical outcomes of focal cortical dysplasia (FCD) type II: correlation with pathological subtypes

Focal cortical dysplasia (FCD) type II is a major cause of drug-resistant epilepsy. In order to gain insight into the possible correlations between FCD II pathological pattern and different clinical characteristics (including clinical information, imaging characteristics and surgical outcomes), different clinicopathological characteristics in two types of FCD II were analyzed (especially in FCD IIb). The mean age of seizure onset and disease duration of 78 patients was 11.0 and 11.2 years, respectively. Patients with FCD type IIb had earlier seizure onset compared with those with FCD type IIa. Pathological subtype IIb was predominantly in frontal lobe and subtype IIa was predominantly seen in temporal. Type IIb demonstrated significantly more signal abnormalities in fluid attenuated inversion recovery (FLAIR) images and T2 images than Type IIa. The rate of satisfactory seizure outcome was 67.64 % in the FCD IIa group, while relative higher, 88.63 %, in the FCD IIb group. All these characteristics may assist in their earlier diagnosis and improve the predictability of surgical management.     

A novel technique of detecting MRI‐negative lesion in focal symptomatic epilepsy: Intraoperative ShearWave Elastography

Focal symptomatic epilepsy is the most common form of epilepsy that can often be cured with surgery. A small proportion of patients with focal symptomatic epilepsy do not have identifiable lesions on magnetic resonance imaging (MRI). The most common pathology in this group is type II focal cortical dysplasia (FCD), which is a subtype of malformative brain lesion associated with medication‐resistant epilepsy. We present a patient with MRI‐negative focal symptomatic epilepsy who underwent invasive electrode recordings. At the time of surgery, a novel ultrasound‐based technique called ShearWave Elastography (SWE) was performed. A 0.5 cc lesion was demonstrated on SWE but was absent on B‐mode ultrasound and 3‐T MRI. Electroencephalography (EEG), positron emission tomography (PET), and magnetoencephalography (MEG) scans demonstrated an abnormality in the right frontal region. On the basis of this finding, a depth electrode was implanted into the lesion. Surgical resection and histology confirmed the lesion to be type IIb FCD. A PowerPoint slide summarizing this article is available for download in the Supporting Information section here .     

Multiple focal cortical dysplasias presenting with intractable epilepsy: case report]

A 14-year-old female, who had intractable epilepsy associated with multiple focal cortical dysplasias (FCD), was reported. She developed intractable epilepsy at the age of 7 and was diagnosed as having frontal lobe epilepsy based on the seizure semiology and interictal EEG. MRI revealed multiple lesions in the right frontal, bilateral occipital and left parietal lobes. EEG demonstrated that ictal discharge was preceded by spike on the right frontal region and FDG-PET showed hypometabolic area in the right frontal lobe. Chronic subdural electrode recordings from the right frontal lobe indicated that ictal onset zone was located around the right frontal lesion, especially frontal tip and base, and these areas including the lesion were resected. Postoperatively, residual seizure was noted although seizure frequency was decreased. It is well known that, postoperatively, satisfactory seizure outcome can be obtained in patients with FCD. However, further investigation in terms of surgical indication and strategies for multiple FCD should be needed.     

Epilepsy surgery in a patient with Lennox-Gastaut syndrome and cortical dysplasia

Lennox-Gastaut syndrome (LGS) is classified as a generalized epilepsy, and is often intractable to antiepileptic drugs. Although corpus callosotomy may sometimes control drop attacks, curative epilepsy surgery is rarely possible in LGS. We report, here, a patient with LGS and focal cortical dysplasia, who became seizure-free after epilepsy surgery. The patient was a 24-month-old boy without perinatal insult in whom seizures began 7 days after birth and who experienced development delay. Brain magnetic resonance imaging (MRI) showed focal cortical lesion with calcification in the right frontal area. At age 13 months, his seizure type changed from tonic seizures to head drops (atonic types) and atypical absence seizures. His interictal electroencephalogram (EEG) showed generalized slow spike and wave discharges, and he was diagnosed with LGS. His seizures were intractable to medical treatment, and a ketogenic diet was not effective. He was evaluated prior to surgery by long-term video-EEG monitoring, which detected many seizures consisted of a sudden onset of falling attacks (atonic type) intermittently followed by atypical absence seizures with diffuse slow wave bursts followed by slow spike and wave discharges in ictal EEG, brain positron emission tomography (PET) and MRI. A right frontal lesionectomy with intra-operative electrocorticography (EcoG) was performed. From the time of lesionectomy to the present, the patient has been seizure free and has been developing normally. Our results suggest that focal resective surgery should be considered in patients with LGS and focal epileptic lesions.     

Disconnective surgery in posterior quadrantic epilepsy: a series of 12 paediatric patients

Aim. To assess the surgical outcomes of temporo‐parieto‐occipital (TPO) and parieto‐occipital (PO) disconnection surgery for children with intractable posterior quadrantic epilepsy and a unilateral posterior quadrant lesion based on MRI and functional imaging abnormality in the TPO region on one side. Methods. A retrospective review of data of 12 children who underwent TPO or PO disconnective surgery was carried out from September 2009 to September 2012. Three‐dimensional surface reconstructions of MRI scans and intraoperative electrophysiological monitoring were used during surgery. Drugs were not discontinued after surgery in any patient. Results. The affected hemisphere was the left in seven patients and the right in five patients. The mean ages at seizure onset and at surgery were four years and 12.3 years, respectively. At the time of surgery, 3 children had atonic seizures, 4 had symptomatic epilepsy with focal seizures and alteration of conscioussness, 4 had secondarily generalised seizures, and 1 child had spasms and tonic seizures. All patients had developmental delay. A pure TPO disconnection was performed in 11 patients and a PO disconnection was performed in the remaining patient. On pathological examination, 3 patients were shown to have focal cortical dysplasia (FCD) Ib, 2 with FCD IIa, 5 with FCD IIb, 1 with gliosis, and 1 with gliosis plus FCD IIa. Following surgery, 2 patients had oedema; 1 required another operation to resect the occipital lobe. At a mean follow‐up of 34.5 months, 9 patients (75%) were classified as Engel class I, 2 as Engel Class II, and 1 as Engel class III. All 12 children had contralateral hemianopia postoperatively and improvement in median IQ (p=0.04) was reported three months postoperatively. Conclusions. With respect to the limits of a retrospective and relatively small sample size series TPO and PO disconnection are safe and effective motor‐sparing epilepsy surgical procedures in selected patients with the epileptiform zone located in the posterior quadrant on one side.     

Clinical Science

Riki Matsumoto , Masako Kinoshita , Junya Taki , Takefumi Hitomi , Nobuhiro Mikuni , Hiroshi Shibasaki , Hidenao Fukuyama , Nobuo Hashimoto , and Akio Ikeda
Focal cortical dysplasia (FCD) is a developmental abnormality of cerebral cortical cell architecture characterized by disorganized cortical layers and by an excess of large, aberrant neurons. FCD increasingly has been recognized as a major cause for intractable partial epilepsy. Epileptic seizures arise based on imbalance between cortical inhibitory and excitatory mechanisms in the epileptic focus. Apart from studies using animal models or surgical specimens, little is known about imbalance between these two mechanisms within FCD to cause epilepsy in patients. A patient with FCD at the left foot primary sensorimotor area was studied for possible brain surgery to treat the epilepsy. A grid (sheet) of recording and stimulating electrodes was placed on the brain as part of standard procedure to "map" the brain prior to removing the parts thought to be responsible for the seizures. Using these implanted electrodes placed on the surface of brain, we investigated the cortical inhibitory and excitatory mechanisms by applying paired electrical pulses directly to the cortex and recording cortical evoked responses from the surrounding cortex. Between seizures, cortical inhibition was abnormally enhanced in the focus compared with normal control cortex. While the patient was having the somatosensory aura (seizure warning), which later evolved to the left leg clonic seizure, the increased cortical inhibition was replaced by increased cortical excitability and decreased inhibition. It was demonstrated that the dynamic alternation of cortical inhibitory and excitatory mechanisms occurred toward increased cortical excitability during seizure generation in a patient with FCD.     

Differential Expression Hallmarks of Interneurons in Different Types of Focal Cortical Dysplasia

Focal cortical dysplasia (FCD) is the main cause of medically intractable pediatric epilepsy. Previous studies have suggested that alteration of cortical interneurons and abnormal cytoarchitecture have been linked to initiation and development for seizure. However, whether each individual subpopulation of cortical interneurons is linked to distinct FCD subtypes remains largely unknown. Here, we retrospectively analyzed both control samples and epileptic specimens pathologically diagnosed with FCD types Ia, IIa, or IIb. We quantified three major interneuron (IN) subpopulations, including parvalbumin (PV)-, somatostatin (Sst)-, and vasoactive intestinal peptide (Vip)-positive INs across all the subgroups. Additionally, we calculated the ratio of the subpopulations of INs to the major INs (mINs) by defining the total number of the PV-, Sst-, and Vip-INs as mINs. Compared with the control, the density of the PV-INs in FCD type IIb was significantly lower, and the ratio of PV/mINs was lower in the superficial part of the cortex of the FCD type Ia and IIb groups. Interestingly, we found a significant increase in the ratio of Vip/mINs only in FCD type IIb. Overall, these results suggest that in addition to a reduction in PV-INs, the increase in Vip/mINs may be related to the initiation of epilepsy in FCD type IIb. Furthermore, the increase in Vip/mINs in FCD type IIb may, from the IN development perspective, indicate that FCD type IIb forms during earlier stages of pregnancy than FCD type Ia.

     

Imag(in)ing seizure propagation: MEG‐guided interpretation of epileptic activity from a deep source

Identification and accurate localization of seizure foci is vital in patients with medically‐intractable focal epilepsy, who may be candidates for potentially curative resective epilepsy surgery. We present a patient with difficult‐to‐control seizures associated with an occult focal cortical dysplasia residing within the deeper left parietal operculum and underlying posterior insula, which was not detected by conventional MRI analysis. Propagated activities from this deeper generator produced misleading EEG patterns both on surface and subdural electrode recordings suggesting initial activation of the perirolandic and mesial frontal regions. However, careful spatio‐temporal analysis of stereotyped interictal activities recorded during MEG, using sequential dipole modeling, revealed a consistent pattern of epileptic propagation originating from the deeper source and propagating within few milliseconds to the dorsal convexity. In this instance, careful dissection of noninvasive investigations (interictal MEG along with ictal SPECT findings) allowed clinicians to dismiss the inaccurate and misleading findings of the traditional “gold‐standard” intracranial EEG. In fact, this multimodal noninvasive approach uncovered a subtle dysplastic lesion, resection of which rendered the patient seizure‐free. This case highlights the potential benefits of dynamic analysis of interictal MEG in the appropriate clinical context. Pathways of interictal spike propagation may help elucidate essential neural networks underlying focal epilepsy. Hum Brain Mapp, 2012. © 2012 Wiley Periodicals, Inc     

Appearance of focal cortical dysplasia on serial MRI after maturation of myelination

Case report It is well known that magnetic resonance imaging (MRI) findings of focal cortical dysplasia (FCD) can change with maturation of myelination. In this paper, we report a patient with intractable epilepsy and negative MRI at the age of 2.5 years, after completion of myelination. Follow-up MRI at the age of 6 years revealed typical FCD findings in the right frontal lobe. During these 3.5 years, electroencephalogram (EEG) consistently depicted an area of irritation in accordance with de novo MRI findings. Intraoperative electrocorticogram showed frequent paroxysmal activity in the right frontal lobe; excision of the epileptogenic cortex resulted in a reduction in seizures. Conclusion It is possible that FCD becomes apparent on MRI even after maturation of myelination; thus, repeated MRI is recommended while EEG continues to demonstrate focal findings.     

Outcomes of resective surgery in children and adolescents with focal lesional epilepsy: The experience of a tertiary epilepsy center

ObjectiveThis study aimed to investigate the efficacy of resective surgery in children with focal lesional epilepsy by evaluating the predictive value of pre- and postsurgical factors in terms of seizure freedom.MethodsThis study included 61 children aged between 2 and 18 years who were admitted to the pediatric video-EEG unit for presurgical workup. Each patient was evaluated with a detailed history, video-EEG, neuroimaging, and postsurgical outcomes according to Engel classification to predict postsurgical seizure freedom. All the possible factors including history, etiology, presurgical evaluation, surgical procedures, and postsurgical results were analyzed for their predictive value for postoperative seizure freedom.ResultsOf the 61 patients, 75% were diagnosed as having temporal lobe epilepsy (TLE), and 25% were diagnosed with extra-TLE. Two years after the surgery, 78.6% were seizure-free, of which 89% had TLE, and 50% had extra-TLE (p < 0.05). Patients were more likely to have a favorable outcome for seizure freedom if they had rare seizure frequency, focal EEG findings, and focal seizures; had a temporal epileptogenic zone; or had TLE and hippocampal sclerosis. On the other hand, patients were more likely to have unfavorable results for seizure freedom if they had younger age of seizure onset, frequent seizures before the surgery, a frontal or multilobar epileptogenic zone, secondarily generalized seizures, extra-TLE with frontal lobe surgery, or focal cortical dysplasia.SignificanceResective surgery is one of the most effective treatment methods in children with intractable epilepsy. A history of young age of seizure onset, frequent seizures before surgery, secondarily generalized seizures, a multilobar epileptogenic zone, frontal lobe surgery, and focal cortical dysplasia (FCD) are the most important predictive factors indicating that a patient would continue having seizures after surgery. On the other hand, focal seizure semiologies, temporal lobe localization, and hippocampal sclerosis indicate that a patient would have better results in terms of seizure freedom.     

Epilepsy Surgery in Infants

Purpose: We report 12 infants who had frontal (n = 3), temporal (n = 2), or temporoparieto-occipital (n = 2) resection or functional hemispherectomies (n = 5) at age 2.5–29 (mean 15.3) months for catastrophic epilepsy due to focal cortical dysplasia (n = 5), Sturge-Weber syndrome (n = 3), ganglioglioma (n = 3), or hemimegalencephaly (n = 1). Seizures began at 1 day to 14 months (mean, 4.0 months) after birth, occurred frequently (often many times per day, and were refractory to antiepileptic drugs. Patients were evaluated for surgery at 2.5–24 (mean 12.4) months of age. Seven patients had hemiparesis and eight had slowed cognitive development. Seizures were characterized by arrest or marked reduction of behavioral motor activity with unclear level of consciousness (n = 4, with temporal or temporoparietal EEG seizures), focal clonic activity (n = 3, with perirolandic EEG seizures), generalized tonic stiffening (n = 3, with temporoparieto-occipital, parietal, or frontal EEG seizures), or infantile spasms and hypsarrhythmia (n = 2, with a frontal tumor or temporoparieto-occipital cortical dysplasia). Methods: Magnetic resonance imaging (MRI) revealed the epileptogenic lesion in all but two patients, both with cortical dysplasia localized by interictal positron-emission tomography (PET) and other clinical or EEG features and confirmed on histopathologic examination of resected tissue. Results: At follow-up 4-86 (mean 32) months after surgery, six patients were seizure free (Engel outcome class I), three had rare seizures with none in at least the previous 6 months (Engel class II), and two had worthwhile improvement (Engel class III). Except for the severely developmentally delayed infant with hemimegalencephaly, several patients had marked “catch-up” developmental progress after class I, 11, or III outcome. Postoperative complications included subdural hematoma over the contralateral hemisphere (one patient) and entrapment and enlargement of the ipsilateral temporal horn (one patient) after hemispherectomy, both corrected uneventfully with a second surgical procedure. One patient died of unexplained causes several hours after frontal lobectomy. No patients had new neurologic deficits after surgery, and one patient had resolution of progressive fluctuating hemiparesis after resection of temporoparieto-occipital cortical dysplasia. Conclusions: Our results agree with previous reports that epilepsy surgery can provide relief from catastrophic epilepsy in carefully selected infants.     

Childhood obsessive-compulsive disorder and cingulate epilepsy.

There are no reports of an association between obsessive-compulsive disorder and cingulate epilepsy in childhood. We report the behavioral, cognitive, and EEG findings in a young girl with medically resistant seizures and severe obsessive-compulsive symptomatology. Her scalp EEG and neuropsychological test scores suggested right frontal lobe dysfunction. The intractability of her seizures and progressive intellectual and psychosocial deterioration prompted evaluation for excisional surgery. Intracranial EEG recording demonstrated a focal seizure origin in the right anterior cingulate gyrus. Cingulotomy resulted in freedom from seizures and significant improvement in her obsessive-compulsive symptoms.     

Ictal kissing in a patient with right frontal lobe epilepsy

When performing pre‐surgical evaluation of patients with refractory epilepsy, the analysis of seizure semiology is one of the key elements used to generate a hypothesis about the location of the epileptogenic zone. Ictal kissing is a very rarely observed ictal automatism described in patients with temporal lobe epilepsy. We present a 62‐year‐old man who was referred to our epilepsy centre for comprehensive evaluation. During prolonged video‐EEG monitoring, six focal‐onset hyperkinetic seizures were registered. In five seizures, the patient repeatedly produced sonorous kisses “into the air”. Initial ictal EEG pattern consisted of rhythmic theta or alpha activity at the right fronto‐polar and fronto‐medial electrodes. MRI depicted focal cortical dysplasia located in the right prefrontal medial cortex. This case suggests that ictal kissing can also occur in the setting of right frontal lobe epilepsy; we therefore believe that this observation expands the anatomo‐clinical correlation for this rare ictal automatism. [Published with video sequences].     

Surgical treatment of epilepsy due to cortical dysplasia: clinical and EEG findings.

Seventeen patients with cortical dysplasia who had surgical resection for medically intractable partial epilepsy were studied. Compared with two groups of surgically treated patients with intractable epilepsy due to tumour (n = 20) and mesial temporal sclerosis (n = 40), patients with cortical dysplasia showed significantly more frequent extratemporal lesions, more frequent non-epileptiform EEG abnormalities and less favourable surgical outcome for seizure control. Patients with cortical dysplasia were younger at onset of seizures and had a lower detection rate of CT abnormalities compared with the tumour group, and lower IQ compared with the mesial temporal sclerosis group. MRI was abnormal in five of seven patients. Six patients became seizure-free or almost seizure-free but eight did not experience relief of seizures. Surgical outcome related to the extent of pathology but not to the histological abnormality. Lesions outside the temporal and frontal lobes were correlated with poor surgical outcome, as were generalised interictal EEG abnormalities, which may reflect extensive or multiple lesions. Ictal intracranial recordings were not useful for presurgical evaluation of cortical dysplasia.     

Uncommon, exceptional and atypical: a case of frontal lobe epilepsy due to cortical dysplasia.

We report a case of frontal lobe epilepsy due to focal cortical dysplasia that included three independent unusual features. The patient, a 45-year-old, right-handed woman, had her first seizure at age 29, well into adulthood. Seizures had been easily controlled with medication for 15 years, then without provocation they became medically intractable during a single, identifiable day. Resection of the dysplastic tissue in the posterior dorsolateral right frontal cortex rendered the patient seizure-free, but produced a significant, non-fluent, Broca type, crossed aphasia. In addition, the seizure semiology was striking and remarkable, a common finding in frontal lobe epilepsy. [Published with video sequences].