Kazumi Matsuda

Autoantibodies and Cell-mediated Autoimmunity to NMDA-type GluRε2 in Patients with Rasmussen's Encephalitis and Chronic Progressive Epilepsia Partialis Continua

Summary:  Purpose: To evaluate antibody‐mediated and cytotoxic T cell–mediated pathogenicity that has been implicated as the autoimmune pathophysiological mechanism in Rasmussens encephalitis.

A Kv4.2 truncation mutation in a patient with temporal lobe epilepsy.

Temporal lobe epilepsy (TLE) has a multifactorial etiology involving developmental, environmental, and genetic components. Here, we report a voltage-gated potassium channel gene mutation found in a TLE patient, namely a Kv4.2 truncation mutation. Kv4.2 channels, encoded by the KCND2 gene, mediate A currents in the brain. The identified mutation corresponds to an N587fsX1 amino acid change, predicted to produce a truncated Kv4.2 protein lacking the last 44 amino acids in the carboxyl terminal. Electrophysiological analysis indicates attenuated K+ current density in cells expressing this Kv4.2-N587fsX1 mutant channel, which is consistent with a model of aberrant neuronal excitability characteristic of TLE. Our observations, together with other lines of evidence, raise the intriguing possibility of a role for KCND2 in the etiology of TLE.

Postictal mania versus postictal psychosis : Differences in clinical features, epileptogenic zone, and brain functional changes during postictal period

Summary:  Purpose: To clarify the differences between postictal mania (PIM) and postictal psychosis (PIP).

Rapid amygdala gamma oscillations in response to fearful facial expressions

Neuroimaging studies have reported greater activation of the human amygdala in response to emotional facial expressions, especially for fear. However, little is known about how fast this activation occurs. We investigated this issue by recording the intracranial field potentials of the amygdala in subjects undergoing pre-neurosurgical assessment (n=6). The subjects observed fearful, happy, and neutral facial expressions. Time-frequency statistical parametric mapping analyses revealed that the amygdala showed greater gamma-band activity in response to fearful compared with neutral facial expressions at 50-150 ms, with a peak at 135 ms. These results indicate that the human amygdala is able to rapidly process fearful facial expressions.

Neuroradiologic Findings in Focal Cortical Dysplasia: Histologic Correlation with Surgically Resected Specimens

Summary:  Purpose: We investigated the neuroradiologic characteristics of focal findings of surgically resected specimens obtained from 47 patients with focal cortical dysplasia (FCD).

Recommendation of Early Surgery from the Viewpoint of Daily Quality of Life

Summary: We surveyed pre‐ and postoperative levels of satisfaction with a range of the daily quality‐of‐life (QOL) domains in 132 sets of epilepsy surgery patients and their families. All patients underwent resective surgery for temporal lobe epilepsy and were monitored for >2 years. Patient and family assessments showed patients’ overall QOL markedly improves after surgery, depending on freedom from seizures. However, factors such as social contacts, family relations, or financial status improved little. Some families and patients were not satisfied with the postsurgical status, despite freedom from seizures. Patients who had surgery at a later age were not so satisfied with their postsurgical status as were patients who had surgery at a younger age, particularly on the QOL domains of role activities, memory function, leisure activities, or emotional well‐being. This lower satisfaction level in older patients likely results from a variety of problems affecting patients during the long‐lasting epileptic process; social handicaps, psychologic conflicts, and deterioration of cognitive/behavioral functions. Based on each case, we recommend that investigations start at an early stage of the illness, so that surgical intervention may be considered as early as possible.

Dystonic posturing associated with putaminal hyperperfusion depicted on subtraction SPECT.

Summary:  Purpose: Dystonic posturing (DP) is one of the most reliable lateralizing indicators for temporal lobe epilepsy (TLE). We evaluated the ictal hyperperfusional areas in patients with DP by using ictal–interictal subtraction single‐photon emission computed tomography (SPECT).

Quantitative Analysis of Benzodiazepine Receptor in Temporal Lobe Epilepsy: [125I]Iomazenil Autoradiographic Study of Surgically Resected Specimens

Summary:  Purpose: To evaluate the changes of the inhibitory neurotransmitter receptor system related to epileptogenesis by measuring central benzodiazepine receptors (BZDRs) in surgically resected specimens of temporal lobe epilepsy by using [125I]iomazenil autoradiography.

KCC2 was downregulated in small neurons localized in epileptogenic human focal cortical dysplasia

Focal cortical dysplasia (FCD), which is characterized histologically by disorganized cortical lamination and large abnormal cells, is one of the major causes of intractable epilepsies. γ-aminobutyric acid (GABA)(A) receptor-mediated synchronous depolarizing potentials have been observed in FCD tissue. Since alterations in Cl(-) homeostasis might underlie these depolarizing actions of GABA, cation-Cl(-) cotransporters could play critical roles in the generation of these abnormal actions. We examined the expression patterns of NKCC1 and KCC2 by in situ hybridization histochemistry and immunohistochemistry in FCD tissue obtained by surgery from patients with intractable epilepsy. KCC2 mRNA and protein were expressed not only in non-dysplastic neurons in histologically normal portions located in the periphery of the excised cortex, but also in dysplastic cells in FCD tissue. The levels of KCC2 mRNA and protein were significantly decreased in the neurons around large abnormal neurons (giant neurons), but not in giant neurons, compared with non-dysplastic neurons. The neurons localized only around giant neurons significantly smaller than non-dysplastic neurons. However NKCC1 expression did not differ among these cell types. These results suggest that the intracellular Cl(-) concentration ([Cl(-)](i)) of small neurons might increase, so that depolarizing GABA actions could occur in the FCD tissue of epileptic foci.

NEURAL CONNECTION BETWEEN BILATERAL BASAL TEMPORAL REGIONS: CORTICO-CORTICAL EVOKED POTENTIAL ANALYSIS IN PATIENTS WITH TEMPORAL LOBE EPILEPSY

OBJECTIVEIn patients with temporal lobe epilepsy, invasive electroencephalographic study has shown that epileptic activities arising from the unilateral temporal lobe often propagate to the contralateral temporal lobe. Which commissural pathways are responsible for this spreading remains controversial. Some previous studies, however, have suggested that interhemispheric connections between bilateral basal temporal regions (BTR) might have a significant role in propagation of epileptic activities. METHODSWe attempted to elucidate the neural connections between bilateral BTRs using the cortico-cortical evoked potential (CCEP) method. Five consecutive patients with temporal lobe epilepsy who underwent intracranial electroencephalographic monitoring were studied. RESULTSCCEP responses were recorded from a total of 24 electrodes after stimulation of the contralateral BTRs (24 CCEPs/720 recordings; 3.33%). There were 3 types of CCEP waveform: type N-P (16 of 24; 66.7%) consisting of an initial negative peak followed by a positive peak; type N (4 of 24; 16.7%) showing a negative peak only, and type P (4 of 24; 16.7%) showing a positive peak only. The latencies ranged from 48.2 to 102.3 ms (mean, 65.5 ms) for negative peaks and 70.2 to 122.0 ms (mean, 95.2 ms) for positive peaks. In all patients, the basal temporal language area was associated with at least 1 CCEP, either as a stimulated region or a recorded region (11 of 24; 45.8%). CONCLUSIONThese data indicate that there is a neural connection between bilateral BTRs. In consideration of the involvement of the basal temporal language area, we speculate that these responses may reflect some physiological connections between bilateral BTRs.