Rasmussen’s Encephalitis

Introduction: Rasmussen’s encephalitis (RE) is an inflammatory encephalopathy characterized by progressive refractory focal seizures, cognitive deterioration and focal neurological deficit that occur with gradual atrophy of one brain hemisphere. Case presentation: We report a case of an 18-year-old male with a history of abnormal body movements involving the right half of the body without loss of consciousness for the last 15 years. Noncontrast computed tomography (NCCT) head and magnetic resonance imaging (MRI) revealed hemiatrophy of the left cerebral hemisphere. Conclusion: RE is a rare disease; hence, diagnosing and managing such patients may be challenging. Our aim is to draw attention of the treating physicians towards this disease with the help of this case report.

Application of 18F-flurodeoxyglucose positron emission tomography in patients with status epilepticus / 中华神经科杂志

Status epilepticus is a neurological emergency with unknown pathogenesis, controversial treatment options, and poor prognosis. In recent years, with the development of 18F-flurodeoxyglucose positron emission tomography ( 18F-FDG PET) imaging technology, further studies on status epilepticus have been carried out from the perspective of molecular metabolism. This article reviews the current role of 18F-FDG PET in patients with status epilepticus on etiology, disease activity, electric activity, location of epileptogenic foci, and prognosis evaluation.

A young child of anti-NMDA receptor encephalitis presenting with epilepsia partialis continua: the first pediatric case in Korea / 소아과

Anti-N-methyl D-aspartate receptor (anti-NMDAR) encephalitis, recently recognized as a form of paraneoplastic encephalitis, is characterized by a prodromal phase of unspecific illness with fever that resembles a viral disease. The prodromal phase is followed by seizures, disturbed consciousness, psychiatric features, prominent abnormal movements, and autonomic imbalance. Here, we report a case of anti-NMDAR encephalitis with initial symptoms of epilepsia partialis continua in the absence of tumor. Briefly, a 3-year-old girl was admitted to the hospital due to right-sided, complex partial seizures without preceding febrile illness. The seizures evolved into epilepsia partialis continua and were accompanied by epileptiform discharges from the left frontal area. Three weeks after admission, the patient's seizures were reduced with antiepileptic drugs; however, she developed sleep disturbances, cognitive decline, noticeable oro-lingual-facial dyskinesia, and choreoathetoid movements. Anti-NMDAR encephalitis was confirmed by positive detection of NMDAR antibodies in the patient's serum and cerebrospinal fluid, and her condition slowly improved with immunoglobulin, methylprednisolone, and rituximab. At present, the patient is no longer taking multiple antiepileptic or antihypertensive drugs. Moreover, the patient showed gradual improvement of motor and cognitive function. This case serves as an example that a diagnosis of anti-NMDAR encephalitis should be considered when children with uncontrolled seizures develop dyskinesias without evidence of malignant tumor. In these cases, aggressive immunotherapies are needed to improve the outcome of anti-NMDAR encephalitis.

Prevalência dos fatores instrínsecos e extrínsecos do processo de aprendizagem em crianças com epilepsia / Prevalence of intrinsic and extrinsic factors of the learning process in children with epilepsy

Objetivo levantar a prevalência dos fatores intrínsecos e extrínsecos que podem interferir no processo de aprendizagem em crianças com epilepsia. Métodos este estudo descritivo foi realizado no Ambulatório de Neurologia Infantil do Hospital de Pediatria Professor Heriberto Bezerra (HOSPED) da UFRN. A obtenção dos dados ocorreu durante setembro/2009 a março/2010 por meio da aplicação de um questionário com pais e cuidadores de crianças com epilepsia. A amostra foi constituída por 41 crianças, seguindo os seguintes critérios de inclusão: a) pais ou cuidadores de crianças com diagnóstico inequívoco de epilepsia atendidas no ambulatório do HOSPED; b) crianças com idades entre 3 e 12 anos; e c) pais ou responsáveis assinarem o termo de consentimento livre e esclarecido. Resultados 61% das crianças apresentaram diagnóstico de epilepsia pura. 59% tiveram sua primeira crise antes dos 03 anos de idade. 34% apresentavam crises do tipo generalizada. 51% apresentavam crises no período da pesquisa. 98% estavam em tratamento medicamentoso para controle das crises, sendo 55% monoterapia e 45% politerapia. 76% estavam inseridas na escola, sendo 50% em escolas públicas. 66% nunca repetiram o ano. 49% das crianças tiveram assiduidade escolar prejudicada em virtude das crises. 64% nunca foram excluídas da escola pelos professores devido a epilepsia e 85% dos pais afirmaram superproteger os filhos. Conclusão o estudo concluiu que, além da epilepsia, as crianças com essa patologia são também expostas a outros fatores, decorrentes da doença, que podem influenciar negativamente no processo de aprendizagem dessas crianças. .

Purpose to raise the prevalence of intrinsic and extrinsic factors of the learning process in children with epilepsy. Methods this descriptive study was conducted at the Clinic of Neurology Children’s Hospital of Pediatrics Professor Heriberto Bezerra, HOSPED – UFRN. Data collection occurred during the September/2009 to March/2010 through a questionnaire with parents and carers of children with epilepsy. The sample comprised 41 children, according to the following inclusion criteria: a) parents or caregivers of children with an unequivocal diagnosis of epilepsy seen at the outpatient clinic of HOSPED; b) children aged between 3 and 12 years; and c) parents or guardian sign the consent form free and clear. Results 61% of children were diagnosed with pure epilepsy. 59% had their first crisis before the age of 03. 34% presented generalized crisis type. 51% presented crisis during the survey period. 98% were on medications to control crisis, and from these children, 55% monotherapy and 45% polytherapy. 76% were at school, 50% inserted in public school. 66% never repeated the school year. 49% of children had school attendance affected because of the crisis. 64% have never been excluded from school by teachers because of epilepsy and 85% of parents affirmed to overprotect their children. Conclusion the study concluded that, in addition epilepsy, children with that pathology are also exposed to other factors, resulting from the disease, which may negatively affect these children learning process. .

Clinical characteristics, etiology and long-term outcome of childhood epilepsia partialis continua / 临床儿科杂志

Objectives To investigate etiology, clinical characteristics and outcome in children with epilepsia partialis continua (EPC). Methods Sixty-three pediatric patients with EPC were retrospectively analysed. The patients aged (5.53±3.65) years old, with brain CT scans or MRIs after diagnosis, basic laboratory tests, cerebrospinal lfuid analysis and electroencephalog-raphy. The average follow-up time was (22.19±21.19) months (6-72 months). Results The median duration of EPC was 11 days (1-180 days). The causes of EPC were inlfammatory and immune-mediation (36 cases, 57.14%, Rasmussen’s encephalitis included), metabolic disorders (8 cases, 12.70%), brain structure abnormalities (5 cases, 7.94%), vascular malformation (5 cases, 7.94%), dual causes (3 cases, 4.76%), post brain surgery (2 cases, 3.17%) and cryptogenic pathogenesis (4 cases, 6.35%). Neurological dysfunc-tions were observed in 44 cases (69.84%). Age, routine cerebrospinal lfuid abnormalities, the presence of inlfammation and im-mune mediated, EPC long duration, involving the right upper extremity were the risk factors of poor prognosis. Conclusions The most common causes of childhood EPC are inlfammation and immune-mediated central nervous system diseases. Patients with early age of onset, a great tendency of longer duration of EPC and cerebrospinal lfuid abnormalities, involving the right upper ex-tremity have a poor prognosis.

Propofol Infusion Syndrome in a Patient with Epilepsia Partialis Continua

Propofol has been used for the treatment of refractory status epilepticus, but propofol can cause propofol infusion syndrome (PRIS). PRIS is rarely developed and often fatal. The syndrome is characterized by metabolic acidosis, rhadomyolysis, and cardiac failure. Most reports were described in critically ill patients undergoing long-term propofol infusion at high doses. But many systemic confounding factors are mixed with the fatality of PRIS and there are no report on epilepsia partialis continua which has stable systemic conditions. We report PRIS in a patient with epilepsia partialis continua.

A Case of Transient Hemolysis Caused by Midazolam

No abstract available.

Propofol Infusion Syndrome in a Patient with Epilepsia Partialis Continua / 대한간질학회지

Propofol has been used for the treatment of refractory status epilepticus, but propofol can cause propofol infusion syndrome (PRIS). PRIS is rarely developed and often fatal. The syndrome is characterized by metabolic acidosis, rhadomyolysis, and cardiac failure. Most reports were described in critically ill patients undergoing long-term propofol infusion at high doses. But many systemic confounding factors are mixed with the fatality of PRIS and there are no report on epilepsia partialis continua which has stable systemic conditions. We report PRIS in a patient with epilepsia partialis continua.

A Case of Cerebral Hemiatrophy Developed after Partial Status Epilepticus

A 20-years old man with a history of complex partial seizure, was admitted due to continuous focal clonic movements in the left upper and lower extremities lasting for about 24 hours. In emergency room, focal clonic movements were stopped with an emergent intravenous lorazepam injection. Two months later, left-sided hemiparesis remained and cere-bral hemiatrophy was noted. We report a case of a 20-years old man who showed rapid development of cerebral hemiat-rophy after epilepsia partialis continua.

Kojewnikow syndrome 12 cases report / 中华神经科杂志

Objective To investigate causes for Kojewnikow syndrome,by observing its clinical characteristics,electroencephalography and findings in imaging scanning. Methods Twelve patients with Kojewnikow syndrome were accessed with clinical observation, electroencephalography and imaging scanning. Results Kojewnikow syndrome is clinically charaterized by secouse twitching and simple partialis motor continua.Viral encephalitis is by far the most of common cause for Kojewnikow syndrome, followed by meningo-encephalitis,cerebral glioma,cerebral cysticercosis, cerebral infarction, diabetes, and cryptogenic epilepsyKojewnikow syndrome tends to occur in watershed area. Conclusions Kojewnikow syndrome falls into two main groups.It is important to improve the diagnosis and find the causes for Kojewnikow and to access its clinical manifestations, electroencephalography and findings in the imaging scanning.The seizures cannot be readily controlled by the anticonvulsants,so the prognosis is poor.

Idiopathic hypertrophic cranial pachymeningitis: Report of 2 cases & Review of literatures

Idiopathic hypertrophic cranial pachymeningitis(IHCP) is a rare chronic pro gressive fibrosing inflammation of pachymeninges of unknown origin. Since the spreading of CT and MRI, there has been a few cases of IHCP have been reported. We describe two patients of IHCP with brain parenchymal involvement presented as epilepsia partialis continua which has not been described as a symptom of IHCP and review the previous reported literatures. IHCP commonly presents headache, multiple cranial nerve palsy, ataxia, and sometimes seizure. In many cases, the CSF finding is noninfectious inflammation, and the erythrocyte sedimentation rate is elevated. The brain MRI is the best noninvasive tool for diagnosis of IHCP and shows diffuse thickening and enhancing dura, especially posterior fossa. IHCP responds steroid initially but recurs frequently and progresses chronically.