Young Min Lim

Factors predicting seizure outcome of anterior temporal lobectomy for patients with mesial temporal sclerosis

PURPOSEnTo investigate the factors, including those associated with ictal scalp EEG results, related to surgical outcome in patients with pathologically proven mesial temporal sclerosis.nnnMETHODSnWe studied 51 consecutive patients who underwent anterior temporal lobectomy and had at least 4 years of follow-up. Surgical outcome was classified as being seizure-free or not seizure-free during the first two and the subsequent two postoperative years. Clinical variables and scalp EEG parameters were subjected to statistical analysis.nnnRESULTSnOf the 51 patients, 36 (70.6%) were seizure-free during postoperative years 3 and 4. Logistic regression analysis revealed that seizure remission for the first 2 years (p = 0.002) and contralateral propagated ictal discharges (p = 0.015) were independently related to seizure outcome at 4 years. Patients who were seizure-free at 2 years had an 86.5% chance of remaining seizure-free at 4 years. Of the patients without bitemporal asynchrony or switch of lateralization, 88.9% were seizure free at 4 years, compared with 54.5% of patients with asynchrony or switch of lateralization (p = 0.007). These two factors, however, were not predictive of seizure outcome at 2 years.nnnCONCLUSIONSnContralateral propagated ictal discharges, including bitemporal asynchrony and switch of lateralization, unfavorably influence long-term seizure outcome. Long-term seizure control is best when the patient has no such propagation patterns of ictal discharges and is seizure-free during the first 2 years after temporal lobectomy.

Factors Contributing to Clinical Seizure Lateralization in Patients with Mesial Temporal Lobe Epilepsy

Clinical seizure semiology can provide important information on the lateralization of the epileptogenic zone. We investigated factors associated with clinical seizure lateralization in patients with pathologically proven mesial temporal sclerosis. We reviewed 243 seizures of 58 patients. Clinical lateralization was possible in 155 (63.8%) of 243 seizures. Lateralization was correct in 144 (92.9%) of 155 lateralized seizures. Logistic regression analysis showed that age at onset (p = 0.001; odds ra tio = 1.089, 95% confidence interval = 1.035–1.145) and the contralateral propagation pattern of ictal discharges (p = 0.001; odds ratio = 3.544, 95% confidence interval = 1.723–7.289) correlated with clinical seizure lateralization. The patient group with clinically lateralized seizures had a younger age at onset of habitual seizures compared to the clinically nonlateralized group (11.1 ± 6.3 vs. 15.6 ± 8.4 years; p < 0.001). Of seizures without bitemporal asynchrony or switch of lateralization, 70.7% were clinically lateralized compared with only 46.4% of seizures with asynchrony or lateralization switch. The present results suggest that the age of epilepsy onset and the ictal scalp EEG propagation pattern affect clinical seizure lateralization in patients with mesial temporal sclerosis.

A Case of Hodgkin's Lymphoma Associated with Sensory Neuropathy

Peripheral neuropathies occur in lymphoma patients. Causes of neuropathy include chemotherapy, opportunistic infections, and the lymphoma itself. We report a patient with lymphoma whose chief complaint was a sensory loss in the hands and feet. Electrophysiologic studies and sural nerve biopsy showed sensory polyneuropathies. We hypothesize that this neuropathy is associated with lymphoma-related ganglionopathy, and among the possible causes, we suspect that a systemic cause such as a paraneoplastic syndrome is the most likely pathogenic etiology. However, further follow-up will be necessary to see whether sensory symptoms change with lymphoma treatment.

Electrophysiologic features of POEMS syndrome compared with MGUS-related neuropathy

Polyneuropathy, organomegaly, endocrinopathy, M‐protein, and skin changes (POEMS) syndrome and monoclonal gammopathy of undetermined significance (MGUS) are paraproteinemic disorders that can cause demyelinating polyneuropathy. Herein we assessed the findings of nerve conduction studies (NCS) in patients with POEMS syndrome and MGUS‐related neuropathy to determine whether the NCS characteristics can help differentiate between these conditions.

Myasthenia gravis seronegative for acetylcholine receptor antibodies in South Korea: Autoantibody profiles and clinical features

Acquired myasthenia gravis (MG) is a prototype autoimmune disease of the neuromuscular junction, caused in most patients by autoantibodies to the muscle nicotinic acetylcholine receptor (AChR). There seem to be ethnic and regional differences in the frequency and clinical features of MG seronegative for the AChR antibody. This study aimed to describe the autoantibody profiles and clinical features of Korean patients with generalized MG seronegative for the AChR antibody. A total of 62 patients with a high index of clinical suspicion of seronegative generalized MG were identified from 18 centers, and we examined their sera for antibodies to clustered AChR, muscle-specific tyrosine kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (LRP4) by cell-based assays (CBA) and to MuSK by radioimmunoprecipitation assay (RIPA). We also included 8 patients with ocular MG, 3 with Lambert-Eaton myasthenic syndrome, 5 with motor neuron disease, and 9 with other diagnoses as comparators for the serological testing. Antibodies were identified in 25/62 (40.3%) patients: 7 had antibodies to clustered AChR, 17 to MuSK, and 2 to LRP4. Three patients were double seropositive: 1 for MuSK and LRP4, and 2 for MuSK and clustered AChR. The patients with MuSK antibodies were mostly female (88.2%) and characterized by predominantly bulbar involvement (70%) and frequent myasthenic crises (58.3%). The patients with antibodies to clustered AChR, including 2 with ocular MG, tended to have a mild phenotype and good prognosis.

Association of the X-linked Androgen Receptor Leu57Gln Polymorphism with Monomelic Amyotrophy

Abstract Monomelic amyotrophy (MA), also known as Hirayama disease, occurs mainly in young men and manifests as weakness and wasting of the muscles of the distal up-per limbs. Here, we sought to identify a genetic basis for MA. Given the predominance of MA in males, we fo-cused on candidate neurological disease genes located on the X chromosome, selecting two X-linked candidate genes, androgen receptor (AR) and ubiquitin-like modi-fier activating enzyme 1 (UBA1). Screening for genetic variants using patients’ genomic DNA revealed three known genetic variants in the coding region of the AR gene: one nonsynonymous single-nucleotide polymor-phism (SNP; rs78686797) encoding Leu57Gln, and two variants of polymorphic trinucleotide repeat segments that encode polyglutamine (CAG repeat; rs5902610) and polyglycine (GGC repeat; rs3138869) tracts. Notably, the Leu57Gln polymorphism was found in two patients with MA from 24 MA patients, whereas no variants were found in 142 healthy male controls. However, the num-bers of CAG and GGC repeats in the AR gene were within the normal range. These data suggest that the Leu57Gln polymorphism encoded by the X-linked AR gene may contribute to the development of MA. Keywords: X-linked gene, androgen receptor (AR) gene, monomelic amyotrophy (MA), case-control study