The prognosis for control of seizures with medications in patients with MRI evidence for mesial temporal sclerosis

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Neurologists' Awareness and Preparedness on Prion Diseases in Korea

Background Creutzfeldt-Jakob disease (CJD) is very rare human prion disease. But, neurologists take a key role in diagnosis, surveillance and management of the cases because of its complexity and difficulty in diagnosis of the disease. The aim of this study is to investigate the level of awareness and preparedness of Korean neurologists on this rare disease. Methods Survey sheets of self-administered questionnaire were given to Korean neurologists who participated in the 31st Annual Meeting of the Koran Neurological Association. Data from 133 respondents were conducted by descriptive analysis. Results Their answers were as follows: About 62% of neurologists have experienced patients of CJD. Forty-four percent of the patients were confirmed by brain biopsy. Most of neurologists (44%) were not confident to diagnose CJD and the reason why they felt hard to diagnose was due to the variable initial clinical manifestations (45.1%) and the lack of clinical experience (51.9%). Heidenheim variant CJD, proteinase sensitive prionopathy, molecular subtypes of sporadic CJD, diagnostic criteria was not familiar term to Korean neurologists (76.7%, 53.4%, 58.6%, and 62.4% respectively). Opinion for the most useful diagnostic tool was brain MRI (45.1%), CSF 14-3-3 protein (30.1%), typical EEG finding (36.8%) and gene (PRNP) test (42.9%). And they consider none of them are specific for the diagnosis of CJD (89.5%, 73.7%, 83.5%, 91.7%, respectively). Most of the neurologist in this survey answered that the opportunity for education of CJD should be increased (67.7%). Conclusions Most of neurologists have encountered CJD patients although it is very rare disease. Some of the important and fundamental concepts of CJD were not correctly recognized to Korean neurologists, necessitating a persistent support for updating knowledge and information.ope

Two Cases of Generalized Nonconvulsive Status Epilepticus with Eyelid Myoclonia with Absence

Eyelid myoclonia with absence is a distinct syndrome of idiopathic generalized epilepsy, characterized by the triad of eyelid myoclonia associated with brief absences, generalized discharges of 3-6 Hz polyspike and slow waves, which are mainly precipitated by eye closure or photosensitivity. We experienced two women with eyelid myoclonia with absence, who typically showed fixation off sensitivity and catatmenial exacerbation. Carabamazepine monotherapy aggravated the seizure frequency and resulted in nonconvulsive status epilepticus. These cases have not previously been reported in Korea.ope

Fundamental requirements for performing electroencephalography

The performance of electroencephalogram (EEG) recordings is affected by electrode type, electronic parameters such as filtering, amplification, signal conversion, data storage; and environmental conditions. However, no single method has been identified for optimal EEG recording quality in all situations. Therefore, we aimed to provide general principles for EEG electrode selection as well as electronic noise reduction, and to present comprehensive information regarding the acquisition of satisfactory EEG signals. The standards provided in this document may be regarded as Korean guidelines for the clinical recording of EEG data. The equipment, types and nomenclature of electrodes, and the details for EEG recording are discussed.ope

Developmental Aspects of Epileptogenesis

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Thrombosis of the Superior Sagittal Sinus in Behcet’s Disease With Vascular and Enteric Involvements

We report superior sagittal sinus thrombosis as a central nervous system presentation of Behcet’s disease in a patient with peripheral vascular and enteric involvement. The main symptoms were headache and horizontal diplopia due to intracranial hypertension. T1-weighted MRI with gadolinium enhancement revealed a similar pattern of the “empty delta sign” that is usually seen on brain CT. A magnetic resonance venogram revealed filling defects resulting from the thrombosis. These features are helpful for the noninvasive diagnosis of sagittal sinus thrombosisope

A Case of Orgasmic Aura Associated with Temporal Lobe Epilepsy from the Nondominant Hemisphere

We experienced a patient with an orgasmic aura originating from the right mesial temporal structure. A 36-year-old right-handed woman suffered from a specific sensation of sexual arousal and orgasm-like euphoria lasting 1~2 minutes for several years. Video EEG monitoring ascertained those sensations as epileptic in nature arising from the right mesial temporal area through a foramen ovale electrode. The findings of 99mTc-ECD-SPECT and 18F-FDG PET were concordant with those of the brain MRI which was pathologically consistent with hippocampal sclerosis.ope

Electroencephalography for the diagnosis of brain death

Electroencephalography (EEG) is frequently used to assist the diagnosis of brain death. However, to date there have been no guidelines in terms of EEG criteria for determining brain death in Korea, despite EEG being mandatory. The purpose of this review is to provide an update on the evidence and controversies with regarding to the utilization of EEG for determining brain death and to serve as a cornerstone for the development of future guidelines. To determine brain death, electrocerebral inactivity (ECI) should be demonstrated on EEG at a sensitivity of 2 μV/mm using double-distance electrodes spaced 10 centimeters or more apart from each other for at least 30 minutes, with intense somatosensory or audiovisual stimuli. ECI should be also verified by checking the integrity of the system. Additional monitoring is needed if extracerebral potentials cannot be eliminated. Interpreting EEG at high sensitivities, which is required for the diagnosis of brain death, can pose a diagnostic challenge. Furthermore, EEG is affected by physiologic variables and drugs. However, no consensus exists as to the minimal requirements for blood pressure, oxygen saturation, and body temperature during the EEG recording itself, the minimal time for observation after the brain injury or rewarming from hypothermia, and how to determine brain death when the findings of ECI is equivocal. Therefore, there is a strong need to establish detailed guidelines for performing EEG to determine brain death.ope

Prognostic Implications of Epilepsy Onset Age According to Relapse Pattern in Patients with Four-Year Remission

A total of 472 epilepsy patients with a 4-year remission period were divided into 10-year age groups according to age of onset. The relapse patterns during at least 3 years of follow-up were classified as early relapse (ER), late relapse (LR), and seizure-free (SF). The remission probability and multiplicity of prognostic factors were evaluated using univariate and multivariate multinomial logistic analyses. The weighted risk score based on odd ratios (ORs) was used for comparisons of the relative risk of relapse between groups. The group with onset in their 20s had the lowest remission probability among the groups. The risks of relapse in the LR patients and the relative weighted risk score of ER patients in the group with onset in their 20s were 3.11 and 19.44, respectively, which was the highest risk among the age groups. Patients without remission within 1 year had the highest relapse risk, with an OR of 7.18 in ER patients. The OR of relapse in patients with >10 generalized tonic-clonic (GTC) seizures was the second most important prognostic factor in LR patients. The distinct risk and corresponding prognostic factors in LR and ER patients reflected inherent differences between these relapse patterns.ope

A Case of Eosinophilic Meningitis Associated with Idiopathic Hypereosinophilic Syndrome

Idiopathic hypereosinophilic syndrome (IHES) is a rare disease characterized by peripheral blood eosinophilia and eosinophilic infiltration of multiple organs, including the nervous system. The neurologic manifestations in hypereosinophilic syndrome are variable, but eosinophilic meningitis as a manifestation has not been well documented in IHES. We report a case of eosinophilic meningitis associated with IHES, which showed a good response to steroid treatment.ope