Kwang-Ki Kim

Surgical Outcome and Prognostic Factors of Cryptogenic Neocortical Epilepsy

Surgical treatment of cryptogenic neocortical epilepsy is challenging. The aim of this study was to evaluate surgical outcomes and to identify possible prognostic factors including the results of various diagnostic tools. Eighty‐nine patients with neocortical epilepsy with normal magnetic resonance imaging (35 patients with frontal lobe epilepsy, 31 with neocortical temporal lobe epilepsy, 11 with occipital lobe epilepsy, 11 with parietal lobe epilepsy, and 1 with multifocal epilepsy) underwent invasive study and focal surgical resection. Patients were observed for at least 2 years after surgery. The localizing values of interictal electroencephalogram (EEG), ictal scalp EEG, interictal 18F‐fluorodeoxyglucose positron emission tomography (FDG‐PET), and subtraction ictal single‐photon emission computed tomography were evaluated. Seventy‐one patients (80.0%) had a good surgical outcome (Engel class 1–3); 42 patients were seizure free. Diagnostic sensitivities of interictal EEG, ictal scalp EEG, FDG‐PET, and subtraction ictal single‐photon emission computed tomography were 37.1%, 70.8%, 44.3%, and 41.1%, respectively. Localization by FDG‐PET and interictal EEG was correlated with a seizure‐free outcome. The localizing value of FDG‐PET was greatest in neocortical temporal lobe epilepsy. The focalization of ictal onset and also ictal onset frequency in invasive studies were not related to surgical outcome. Concordance with two or more presurgical evaluations was significantly related to a seizure‐free outcome. Ann Neurol 2005

Prognostic factors in anterior temporal lobe resections for mesial temporal lobe epilepsy : Multivariate analysis

Summary: Purpose: Even though there have been several studies on the prognostic factors of temporal lobe epilepsy (TLE) after lobectomy, no studies have been performed for homogeneous mesial TLE. Furthermore, most studies on the predictors of outcome of epileptic surgery were based on univariate analyses and did not consider modern epileptic surgery investigation modalities such as brain magnetic resonance imaging (MRI) and positron emission tomography (PET). We attempted to identify the prognostic factors in anterior temporal lobectomy (ATL) for mesial TLE with multivariate analysis.

Prognostic Factors for the Surgery for Mesial Temporal Lobe Epilepsy: Longitudinal Analysis

Summary:  Purpose: Determining long‐term prognostic factors of surgery for mesial temporal lobe epilepsy (MTLE) is important for identifying ideal candidates and predicting the prognosis for individual patients. We tried to identify the prognostic factors of anterior temporal lobectomy (ATL) for MTLE with longitudinal multivariate analysis.

Parietal Lobe Epilepsy: The Semiology, Yield of Diagnostic Workup, and Surgical Outcome

Summary:  Purpose: To characterize the clinical features, the prognostic value, and diagnostic sensitivities of various presurgical evaluations and the surgical outcomes in parietal lobe epilepsy (PLE), we describe 40 patients who were diagnosed as having PLE, including 27 surgically treated patients.

FDG-PET Images Quantified by Probabilistic Atlas of Brain and Surgical Prognosis of Temporal Lobe Epilepsy

Summary:  Purpose: This study evaluated the relation between hypometabolism, diagnosed by fluorodeoxyglucose positron emission tomography (FDG‐PET), and the surgical outcome of a large and homogeneous series of cases of mesial temporal lobe epilepsy (mTLE), by using a probabilistic atlas of the human brain (statistical probabilistic anatomical maps: SPAM).

Visual working memory revealed by repetitive transcranial magnetic stimulation

We evaluated whether repetitive transcranial magnetic stimulation (rTMS) could be utilized for studying the hemispheric lateralization and anatomical localization of the cortical areas of the visual system that are concerned with object-related visual working memory. In eight normal volunteers, visual working memory was tested during rTMS delivery over nine regions in each hemisphere. Visual working memory was significantly disturbed by rTMS over the right hemisphere compared with the left (P<0.05). The disturbance in visual working memory by rTMS was significant over the right inferior frontal (F8), inferior temporal (T8), and middle parietal (P4) areas compared with the control region (P<0.05). This study suggests that visual working memory is lateralized to the right hemisphere and localized in the right inferior frontal, inferior temporal, and middle-parietal areas. As a non-invasive tool, rTMS may be useful for the functional localization of the working memory system.

Subclinical hypothyroidism during valproic acid therapy in children and adolescents with epilepsy.

The aim of this study was to evaluate the incidence of thyroid dysfunction during valproic acid (VPA) therapy in children and adolescents with epilepsy. The serum levels of thyroid-stimulating hormone (TSH), free thyroxine, and triiodothyronine were evaluated in 61 children with epilepsy who received VPA monotherapy for more than 6 months and in 144 controls. We analyzed the effect of age, seizure type, duration of VPA treatment, dose of VPA, and serum level of VPA on thyroid function. The incidence of subclinical hypothyroidism was significantly higher in patients with VPA therapy than in controls (52.4 vs. 16.7%; p < 0.001). In addition, of the 61 patients, 5 (8.1%) exhibited TSH levels that were >10 μIU/mL. However, none of the patients and controls showed overt hypothyroidism. Serum VPA level and daily dose of VPA were correlated with TSH level. Subclinical hypothyroidism developed frequently in children and adolescents during VPA therapy.

The lateralizing and surgical prognostic value of a single 2-hour EEG in mesial TLE

We investigated 109 patients who received anterior temporal lobectomy for intractable mTLE by post-operative follow-up for at least 11/2 years. We reviewed pre-operative 2-hour interictal EEGs, counted interictal epileptiform discharges (IEDs), and compared the lateralization of IEDs with the side of surgery and surgical outcome. Twenty of 22 patients who had no spikes and 44 of 51 who had unitemporal spikes became seizure free after surgical resection. The correct lateralization of the epileptogenic side was possible in 90. 9% of the patients with unitemporal IEDs. In 12 seizure-free patients of 15 patients with less than 70% predominance of IED in one temporal lobe, the positive predictive value of the lateralization was 41.7%. In 16 seizure-free patients of 21 with more than 70% preponderance of IED in one lobe, the positive predictive value was 81.3%. Surgical outcome of patients with unitemporal and bitemporal IEDs were not significantly different. Interictal scalp EEG can be used as a lateralizing tool in mTLE when the temporal IEDs appear with more than 70% preponderance in one side. Although the presence of bitemporal IEDs often causes confusion in terms of the correct lateralization, it does not affect the surgical outcome.

Application of semiological seizure classification to epileptic seizures in children

To better define seizure characteristics and to examine whether semiological seizure classification (SSC) can be appropriately applied to the seizures of infants and children, we studied 152 videotaped seizures recorded in 133 children. Seizure semiology was described on the basis of a series of behavioural, sensory, and motor phenomena according to SSC. Special emphasis was placed on whether one semiology can be representative of a patients whole semiology sequence. If one semiology was able to represent the whole sequence, the seizure was classified according to SSC. Ninety of 152 seizures (59.2%) could be classified as a single seizure type by SSC. However, only 19 of 53 seizures (35.9%) consisting of two semiologies, three of 24 seizures (12.5%) consisting of three semiologies, and one of eight seizures (12.5%) consisting of four semiologies could be classified according to SSC. Although SSC is very efficient, it is more accurately a descriptive terminology for clinical ictal events than a classification system.

G.P.23

Congenital myopathies and congenital muscular dystrophies are groups of clinically, pathologically, and genetically heterogeneous disorders. Even for the experienced clinicians, an accurate genetic diagnosis has often been challenging due to the heterogeneity and complexity of these groups of disorders. One gene can cause a wide variety of clinical and/or pathological features, while similar clinical features can be caused by mutations in different genes. Since next generation sequencing (NGS) is an effective diagnostic tool for the parallel investigation of a large number of genes, it has been increasingly used in recent clinical practices to diagnose these genetically and phenotypically heterogeneous diseases. Here, we present the result of a targeted NGS panel analysis in early onset myopathies. We selected 703 known pathogenic genes causing congenital myopathies, congenital muscular dystrophies, metabolic and mitochondrial myopathies, distal myopathies, channelopathies, neuromuscular junction disorders, and diseases of peripheral nerve. Total 42 infants or children with early onset ( COL6A1 (5), COL6A3 (1), LMNA (3), ACTA1 (2), MTM1 (1), DOK7 (1), and GARS (1). Our results suggest that targeted NGS has a significant potential to synergy with clinical and pathological analysis for an effective diagnosis of primary myopathies.