Posterior cortex epilepsy secondary to ulegyria: Is it a surgically remediable syndrome?

Usui, Naotaka; Mihara, Tadahiro; Baba, Koichi; Matsuda, Kazumi; Tottori, Takayasu; Umeoka, Shuichi; Nakamura, Fumihiro; Terada, Kiyohito; Usui, Keiko; Inoue, Yushi

doi:10.1111/j.1528-1167.2008.01697.x

Cited by 24 publications

(25 citation statements)

References 11 publications

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“…That unilateral ULG may be associated with refractory seizures and amenable to surgical treatment was already known (Usui et al., ). Here, we show that the condition we denominate BP‐ULG presents a clinical picture often indistinguishable from bilateral perisylvian PMG yet, in sharp contrast with the latter (Kuzniecky et al., ), refractory seizures can be treated with resective surgery, despite the bilaterality of the lesions.…”

Section: Discussionmentioning

confidence: 91%

Bilateral perisylvian ulegyria: An under‐recognized, surgically remediable epileptic syndrome

et al. 2013

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SUMMARYPurpose: Interest in the association of epilepsy and pseudobulbar palsy was rekindled since the identification through magnetic resonance imaging (MRI) of bilateral perisylvian polymicrogyria (PMG). Seizures are often intractable, but resective epilepsy surgery has not been recommended. However, a similar clinical picture can be encountered in patients with bilateral perisylvian destructive lesions, which fit the description of ulegyria (ULG). We report a series of patients with epilepsy and pseudobulbar palsy due to bilateral perisylvian ULG (BP-ULG), show that hippocampal sclerosis (HS) is often associated and highlight the fact that in this entity, unlike in malformative bilateral perisylvian PMG, seizures may be surgically treated. Methods: The motor, cognitive, epileptologic, and imaging features of 12 patients with perisylvian ULG followed at three institutions are described. For patients with refractory seizures, we detail extracranial and intracranial electrographic recordings, surgical strategies, histopathologic analyses of the resected tissue, and outcome of surgical treatment. Descriptive statistics were used for quantitative and categorical variables. Student's t-test was used to compare means, and a p < 0.05 was considered significant. Key Findings: Pseudobulbar palsy and mental retardation were present in all patients with symmetrical BP-ULG. Five had refractory seizures. There was no relationship between the severity of the pseudobulbar palsy or of the mental retardation and the degree of seizure control with medication. The five patients in whom seizures were refractory to medication had significantly earlier age of onset and longer duration of epilepsy (p < 0.05). Dual pathology with associated unilateral HS was present in four. One patient with dual pathology had a temporolimbic electroclinical picture and had an anterior temporal lobectomy (ATL) based upon noninvasive evaluation. The other four had ictal semiology suggesting involvement of both temporolimbic and perisylvian cortex. Intracranial electroencephalography (EEG) showed concomitant seizure onset in the anterior temporal region and in the ipsilateral ULG in three of the four with dual pathology and in the ulegyric cortex in the one without HS. Resection guided by a combination of semiology, MRI, and extra and intracranial EEG led to complete seizure control in two and almost complete seizure control (Engel class II) in two other patients. The only surgical failure was an isolated ATL in a patient with dual pathology, and concomitant seizure onset in both lesions according to semiology and intracranial EEG. Significance: Our findings suggest that BP-ULG mimics the clinical features of bilateral perisylvian PMG. In patients with refractory seizures, recognition of this entity should lead to consideration of resective surgery despite the bilateral ULG.

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Section: Discussionmentioning

confidence: 91%

Bilateral perisylvian ulegyria: An under‐recognized, surgically remediable epileptic syndrome

et al. 2013

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“…17 Recently, ulegyria, a form of gliosis, has also been reported to be associated with drug-resistant epilepsy. 19,37 Focal gliosis has also…”

Section: Discussionmentioning

confidence: 99%

Predictors of seizure outcome following resective surgery for drug-resistant epilepsy associated with focal gliosis

Dash

Rathore

Jeyaraj

et al. 2019

Journal of Neurosurgery

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OBJECTIVE The authors studied the clinical characteristics and postoperative outcomes of drug-resistant epilepsy associated with focal gliosis. METHODS From their epilepsy surgery database, the authors selected the patients with drug-resistant epilepsy and MRI-defined focal gliosis who underwent focal resective surgery. All patients underwent standard presurgical evaluation. Intracranial electroencephalography (EEG) was performed in patients with discordant presurgical data, ill-defined lesions, and lesions close to eloquent regions. Completeness of resection was defined on the basis of extraoperative and intraoperative electrocorticography studies. Favorable postoperative outcome was defined as Engel class I outcome during the last 2 years of follow-up. RESULTS Sixty-six patients fulfilled inclusion criteria. An initial precipitating injury was present in 38 (57.6%) patients, mainly in the form of perinatal injury (n = 10), trauma (n = 10), and meningoencephalitis (n = 8). Gliosis involved a single lobe in 38 (57.6%) patients and 2 adjacent lobes in 14 (21.2%) patients; the remaining 14 (21.2%) patients had multilobar gliosis. In patients with unilobar or bilobar gliosis, the posterior region of the head was involved in 34 (65%) patients and the frontal lobes in 12 (23%) patients. During a median follow-up of 4 years (range 2-9 years), 41 (62.1%) patients had favorable outcome. On multivariate analysis, the presence of a well-defined aura (p = 0.019), electrocorticographically defined completeness of resection (p = 0.024), and normal postoperative EEG findings at 1 year (p = 0.003) were predictive of favorable postoperative seizure outcome. CONCLUSIONS Focal gliosis is a common etiology for drug-resistant extratemporal epilepsy in developing countries and is most often located in the posterior region of the head. The majority of these patients have perinatal injuries or neurological infections as initial precipitating injuries. Patients with focal gliosis have good postoperative seizure outcomes after well-planned resective surgery.

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“…Therefore, the atrophy and thinning of the subcortical white matter at deep portions of the convolutions lead to the specific “mushroom” shape of ulegyria in the affected brain regions. 3,13,14 Consequently, this unique blood supply results in ulegyria involving primarily the posterior brain regions 2,8 although more anterior lesions may also occur. 8,15 In the point of this fact, primarily the occipital or parietooccipital regions 2,8 as well as the perisylvian area has been reported as common localizations for the occurrence of ulegyric lesions in earlier studies.…”

Section: Discussionmentioning

confidence: 99%

“…3,13,14 Consequently, this unique blood supply results in ulegyria involving primarily the posterior brain regions 2,8 although more anterior lesions may also occur. 8,15 In the point of this fact, primarily the occipital or parietooccipital regions 2,8 as well as the perisylvian area has been reported as common localizations for the occurrence of ulegyric lesions in earlier studies. 4,15 In line with these, ulegyria was located in the posterior or perisylvian brain areas in all patients except the one with frontal localization in the present series.…”

Section: Discussionmentioning

confidence: 99%

“…1 The scarring has a specific configuration with small atrophic circumvolutions at the bottom of a sulcus underlying an intact gyral apex, leading to mushroom-shaped gyri. 2,3 Either unilateral or bilateral lesions affect varying degrees of brain areas that may extend from small regions to extensive lesions involving the whole hemisphere. 3 An “epileptogenic” island of cortex may occur because of such a disconnection which results in seizure generation.…”

Section: Introductionmentioning

confidence: 99%

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A Rare Cause of Epilepsy: Ulegyria Revisited in a Series of 10 Patients

et al. 2021

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Introduction. Ulegyria results from perinatal hypoxic-ischemic brain injury in term infants. The specific mushroom-shaped configuration of ulegyria results from small atrophic circumvolutions at the bottom of a sulcus underlying an intact gyral apex. Clinically, ulegyria is generally associated with epilepsy. Here, we aimed to delineate the characteristics of patients with ulegyria and the epileptic seizures they experience. Material and methods. Medical records including radiology and pathology reports, video-electroencephalographic (EEG) analysis, operative notes, hospital progress and outpatient clinic notes were reviewed retrospectively in a total of 10 ulegyria patients. Results. Patients ages ranged between 24 and 58 years (mean, 32 ± 9.8 years). Past medical history was confirmed for neonatal asphyxia in 2 (20%). Neurological examination was remarkable for spastic hemiparesis in 1 (10%) patient with perisylvian ulegyria and for visual field deficits in 2 patients (20%) with occipital ulegyria. Ulegyria most commonly involved the temporoparietal region (n = 5, 50%) followed by the perisylvian area (n = 2, 20%). Except the one with bilateral perisylvian ulegyria, all patients had unilateral lesions (n = 9, 90%). Hippocampal sclerosis accompanied ulegyria in 2 patients (20%). All patients experienced epileptic seizures. Mean age at seizure onset was 8.8 ± 5.4 years (range, 2-20 years). Interictal scalp EEG and EEG-video monitoring records demonstrated temporoparietal and frontotemporal activities in 5 (50%) and 2 (20%) patients, respectively. The seizures were successfully controlled by antiepileptic medication in 8 patients (n = 8, 80%). The remaining 2 patients (%20) with concomitant hippocampal sclerosis required microsurgical resection of the seizure foci due to medically resistant seizures. Discussion. Ulegyria is easily recognized with its unique magnetic resonance imaging characteristics and clinical presentation in the majority of cases. It is highly associated with either medically resistant or medically controllable epileptic seizures. The treatment strategy depends on the age at onset and extends of the lesion that has a significant impact on the severity of the clinical picture.

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Posterior cortex epilepsy secondary to ulegyria: Is it a surgically remediable syndrome?

Cited by 24 publications

References 11 publications

Bilateral perisylvian ulegyria: An under‐recognized, surgically remediable epileptic syndrome

Bilateral perisylvian ulegyria: An under‐recognized, surgically remediable epileptic syndrome

Predictors of seizure outcome following resective surgery for drug-resistant epilepsy associated with focal gliosis

A Rare Cause of Epilepsy: Ulegyria Revisited in a Series of 10 Patients

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