Encefalitis de Rasmussen: diagnóstico y enfoque terapéutico / Rasmussen encephalitis: diagnostic and therapeutic approaches

Objetivo: describir el caso de una paciente escolar con diagnóstico de encefalitis de Rasmussen de presentación aguda y el enfoque terapéutico. Presentación: paciente femenina de 5 años y nueve meses que cursa con cuadro de evolución progresiva caracterizado por posturas distónicas que se inicia en pierna izquierda seguida de miembro superior ipsilateral asociadas con crisis focales con semiología de inicio opercular que la conducen a estatus epiléptico. La resonancia magnética cerebral evidencia cambios estructurales progresivos con atrofia hemisférica y del núcleo basal derechos, se inicia terapia antiepiléptica e inmunomoduladora con buena respuesta. Discusión: se realizó una revisión de la literatura sobre la presentación clínica, diagnóstico y mejores opciones terapéuticas. Conclusiones: se trata de una escolar con síndrome de Rasmussen en etapa aguda, con adecuada evolución clínica luego de inicio de inmunoterapia. En Colombia existen pocos reportes, nuestro propósito es realizar la presentación de un caso y revisar el enfoque diagnóstico y terapéutico.

Objective: to describe the case of a school-age patient aged 5 years 9 months diagnosed with acute Rasmussen encephalitis (RE), and its therapeutic approach. Case presentation: a girl aged 5 years 9 months presenting with progressive dystonic postures starting in the left lower limb with spread to the ipsilateral upper limb associated with focal seizures characterized by opercular epilepsy semiology which lead to status epilepticus. Cerebral magnetic resonance imaging revealed progressive structural changes with atrophy of the right cerebral hemisphere and basal nuclei. Anti-epileptic and immunmodulator therapy was initiated obtaining good response. Discussion: a review of the literature on RE clinical presentation, diagnosis and best treatment options was conducted. Conclusions: this is the case of a young girl with Rasmussen syndrome in the acute stage evidencing proper clinical progression after receiving immunotherapy. Few reports exist on this topic in Colombia. Our purpose was to present a case and review RE diagnostic and therapeutic approaches.

Etiology and clinical features of epilepsia partialis continua: an analysis of six cases / 中国当代儿科杂志

OBJECTIVE@#To investigate the etiology and clinical features of epilepsia partialis continua (EPC) in children.@*METHODS@#A retrospective analysis was performed for the clinical features, diagnosis and treatment of six children with EPC, and the clinical and laboratory features and prognosis were compared between the children with different etiologies.@*RESULTS@#There were five girls and one boy, with an onset age ranging from one year and seven months to nine years. Two were diagnosed with Rasmussen encephalitis, one was diagnosed with focal cortical dysplasia, one was diagnosed with Alpers syndrome caused by POLG gene mutation, one was diagnosed with Angelman syndrome, and one was diagnosed with tuberculous meningitis. The latter two children had the predisposing factors for acute encephalopathy induced by status epilepticus and craniocerebral operation during the onset of EPC, while the other four children had natural progression of EPC. All the children had focal seizures except EPC, and symptoms included automatism, bilateral asymmetric tonic seizure, deflection, complex motor, and autonomic symptoms, with disturbance of consciousness in some children. EPC often lasted for several days or even several months. All children had abnormalities on head MRI, including local abnormal signal, cortex swelling, diffusive brain atrophy or brain atrophy at one side, local cortex thickening, and cortical necrosis. Head PET/CT scan was performed for three children and found local hypermetabolism or co-existence of hypermetabolism and hypometabolism. All the children had abnormalities on electroencephalography (EEG), with cerebral, hemispheric, or diffusive distribution of abnormal electrical activities, and during the onset of EPC, some EEG changes were recognizable and some were difficult to identify. All the children with EPC were not sensitive to antiepileptic drugs. EPC was relatively self-limiting in the child with Angelman syndrome. The child with focal cortical dysplasia underwent resection of epileptic foci and had good postoperative control, without neurological dysfunction. The child with Rasmussen encephalitis underwent functional hemispherectomy and had no attack after surgery, with neurological dysfunction. The child with Alpers syndrome had the worst prognosis.@*CONCLUSIONS@#EPC is a special type of epileptic seizures. Immune inflammation and metabolic etiologies are the main causes of EPC in children, and the selection of treatment regimens, treatment outcome, and prognosis depend on etiology.

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.

Lemierre's Syndrome Presenting with Atypical Features

Lemierre's syndrome is characterized by anaerobic bacterial infection in the head and neck, causing thrombophlebitis of the jugular vein. This disease is usually associated with a history of pharyngitis. The most common pathogens are Fusobacterium species, particularly Fusobacterium necrophorum. Lemierre's syndrome is seen most commonly in teenagers and young adults. We present a case report of a 67-year-old man with an atypical clinical manifestation of an uncommon pathogen in Lemierre's syndrome with epilepsia partialis continua.

case of rasmussen encephalitis: the differential diagnoses and role of diagnostic imaging

Rasmussen encephalitis is an extremely rare chronic inflammatory neurodegenerative disease affecting a single cerebral hemisphere, causing progressive neurological deterioration and intractable seizures. Imaging plays an important role in diagnosis by demonstrating focal or unihemispheric involvement and excluding other possible causes. Here, we report a case of Rasmussen encephalitis with an update on recent diagnostic criteria and emphasis on differential diagnoses which can be excluded on imaging

Estatus parcial continuo, causa de hiperintensidad de la corteza cerebral en la resonancia nuclear magnética / Continous partial status cause of hyperintensity in cerebral cortex in magnetic resonance imaging

Magnetic Resonance Imaging has demonstrated functional changes of the cerebral cortex in relation to status epilepticus, which can eventually localize the origin of the crisis. The purpose of this presentation is relevant to this condition and pretends to highlight the action of incidental situations that can modify it. We present a 29 year old woman with a neurosurgical intervention for a neuroblastoma irradiated fifteen years ago, which incidentally starts a continuous partial status epilepticus, expressed by clonies of the face and left limbs associated with functional impotence, resistant to oral therapy. Faced with the suspicion of recurrence of the tumor, a brain MRI is performed, showing hyperintensity of all neural areas the right hemisphere, with no evidence of tumor recurrence. Once submitted the status epilepticus, the hyperintensity disappeared in the hemisphere. This extensive reaction of the neural structures might be related to a permanent effect of radiation, which may have caused a mismatch functional glia, of the blood-brain barrier and interneural network.

La Resonancia Nuclear Magnética (RM) ha puesto en evidencia cambios funcionales de la corteza cerebral en relación a estados epilépticos que eventualmente permiten localizar el origen de las crisis. El propósito de esta presentación es hacer relevante dicha condición y destacar la acción de situaciones incidentales que pueden modificarla. Se presenta a una mujer de 29 años portadora de una intervención neuroquirúrgica por un neuroblastoma irradiado hace quince años, que incidentalmente inicia un estatus epiléptico parcial continuo, expresado por clonías de la cara y extremidades izquierdas asociadas a impotencia funcional resistente a la terapia oral. Frente a la sospecha de recidiva de la neoplasia se realiza RM cerebral que muestra una hiperintensidad de todas las áreas neuronales del hemisferio derecho, sin evidencias de recidiva tumoral. Una vez remitido el estado epiléptico se observó una desaparición de estas alteraciones. La interpretación para esta extensa reacción de las estructuras neurales podría estar relacionado con un efecto permanente de la irradiación, que pudo haber provocado un desajuste funcional de la glía, de la barrera hematoencefálica y de la red interneural.

A Case of Transient Hemolysis Caused by Midazolam

No abstract available.

A Case of Nonketotic Hyperglycemia as a Manifestation of Generalized Convulsive Status Epilepticus / 대한내과학회지

Seizure is one of the manifestations of nonketotic hyperglycemia (NKH). Partial motor seizures are observed in most cases and, occasionally, with epilepsia partialis continua. Generalized convulsive status epilepticus caused by NKH is rare. In this report, we present a case of a 68-year-old man who developed generalized convulsive status epilepticus as an initial manifestation of NKH.

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.

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.

Alterações cognitivas associadas à síndrome de Kojewnikoff / Cognitive alterations associated with the Kojewnikoff syndrome

Introdução: A síndrome de Kojewnikoff, associada ao estado de mal epiléptico (EME), também denominada de Epilepsia Parcial Contínua (EPC), é caracterizada por crises somatomotoras,com marcha jacksoniana e mioclonias que afetam a extremidade contralateral sendo o giro pré-central a principalregião cerebral afetada. São raros os estudos na literatura que descrevem as alterações cognitivas destes pacientes. Objetivo: descrever o funcionamento cognitivo de uma pacientecom EPC através de um protocolo de testes neuropsicológicos. Método: Foram realizadas duas sessões com duas horas de duração cada, utilizando-se para tanto, uma bateria de testes neuropsicológicos. Resultados: Os resultados da avaliação indicaram comprometimento de velocidadede processamento de informações, atenção, fluência verbal e memória episódica. Conclusão: Os resultados da avaliação neuropsicológica são compatíveis com os déficits cognitivos esperados em lesões corticais na área motora primária e com os resultados da avaliação clínica pré-existente.

A Case of Long-term Follow-up Rasmussen's Encephalitis / 대한간질학회지

Rasmussen encephalitis (RE) is a rare, severe, and progressive disorder characterized by focal motor seizures, epilepsia partialis continua, hemiparesis, and intellectual decline. Although clinical features of RE are heterogenous, the progression of the disease is generally divided into three stages which are prodromal, acute, residual stages. We report a 29-year-old woman who had shown typical progression of the disease but preserved cognitive function during a longterm follow-up.

Type I diabetes mellitus in a child presenting with epilepsy partialis continua.

Epilepsy partialis continua is an uncommon, localisation-related, seizure characterised by simple partial motor seizures with repetitive clonic jerks. A case of type I diabetes mellitus in a child who had presented with epilepsy partialis continua is presented. Antiglutamic acid decarboxylase 65 antibodies were found in his serum. Seizures were eventually controlled by anti-epileptic drugs, insulin and IV gamma.

Uncommon Neurological Presentation due to Non-Ketotic Hyperglycemia (NKH) –Report to two cases.

Two patients of uncommon neurological presentation due to non-ketotic hyperglycemia (NKH) are being described in this report. Generalized choreo-athetosis and epilepsia partialis continua as presenting symptoms in non-ketotic hyperglycemia is rare. The abnormal movements responded completely to insulin therapy. In acute onset of abnormal movements, besides other causes, metabolic disorders such as non-ketotic hyperglycemia should also be ruled out as they are treatable.

Clinical characteristics and treatment of Rasmussen syndrome in 16 children / 中华儿科杂志

<p><b>OBJECTIVE</b>Rasmussen syndrome (RS) is a chronic inflammatory disease of unknown origin, usually affecting one brain hemisphere. The present study aimed to analyze the electroclinical characteristics and treatment of RS.</p><p><b>METHODS</b>The medical records of 16 children with RS were retrospectively reviewed.</p><p><b>RESULTS</b>Of the 16 children, 8 were males and 8 were females. The age of onset was from 1 year and 11 months to 11 years and 6 months. The first symptom was seizure in all patients. The main seizure type was partial motor seizures. In all the patients, seizures gradually became frequent and in the form of epilepsia partialis continua (EPC). Thirteen cases developed hemiparesis. Fixed hemiparesis occurred from 2 months to 3 years after the onset of seizures. The cognitive deterioration was present in 14. The EEG background activity was abnormal in all the cases, asymmetric slow wave disturbances were bilateral but with unilateral predominance in 11, unilateral delta or theta wave in 8. The presence of interictal epileptiform discharges were found in all cases, unilateral in 11 and bilateral in 5. Seizures were recorded in all patients, no electroclinical correlation was found in 5. Serial magnetic resonance imaging (MRI) showed progressive unihemispheric or focal cortical atrophy in all cases. Six cases transiently showed focal cortical swelling or T2/FLAIR hyperintense signal on early scans. Antiepileptic drugs were not effective in any of the patients. Three of 10 patients receiving immunoglobulin, and 4 of 8 receiving corticosteroids, had some reduction of seizure frequency for a short period. Six patients accepted functional hemispherectomy, in 4 of them seizure no longer occurred and cognitive function was improved. The results of multiple subpial transection in 2 cases and focal resection in one patient were disappointing.</p><p><b>CONCLUSION</b>The clinical features of RS were refractory partial epilepsy, progressive hemiplegia and cognitive deterioration. The EEG background was asymmetric with slow wave activity, interictal epileptiform discharges were unilateral or bilateral, no electroclinical correlation occurred. Serial MRI showed progressive unihemispheric focal cortical atrophy. Antiepileptic drugs were not effective for RS. In some patients, immunoglobulin or corticosteroids could reduce seizure frequency in the short term. Functional hemispherectomy could lead to seizure control and prevent further development of neurological impairment and cognitive deterioration.</p>

Epilepsia parcial continua y un probable Síndrome de Rasmussen: Epilepsia parcial continua y síndrome de Rasmussen / Continua parcial epilepsy and a possible Rasmussen's Syndrome

El Síndrome de Rasmussen es una enfermedad neurológica poco frecuente que inicia en la infancia pero puede progresar hasta la edad adulta. Se caracteriza por la presencia de epilepsia parcial continua y el desarrollo de epilepsia refractoria, datos relevantes en la explotación clínica, alteraciones en los estudios de neuroimágenes especialmente en la resonancia magnética y hallazgos histopatológicos característicos en la biopsia cerebral. Se ha relacionado esta entidad con un episodio previo de encefalitis que predispone a ua reacción inmunológica contra algunos componentes del encéfalo, lo cual lleva a un atrofia de un sólo hemisterio cerebral. Actualmente el tratamiento más efectivo en controlar las crisis convulsivas es la hemisferectomía, aunque algunos pacientes pueden tener mejoría transitoria con el uso de inmunoglobulina intravenosa .Se reporta el caso de una paciente quien reúne los criterios clínicos y radiológicos que hacen suponer sufre de esta enfermedad. Al momento de terminar de redactar este artículo está recibiendo dosis semanales de inmunoglobulina intravenosa para valorar respuesta clínica a la misma. Ante eventual falla terapéutica será sometida a una hemisferectomía de la cual una vez realizado el análisis histopatológico será reportado el caso. Palabras Claves : Síndrome de Rasmussen - Epilepsia Parcial Continua -Epilepsia Refractoria -Hemisferectomía-Inmunoglobulina intravenosa-Resonancia Magnética.

Epilepsia partialis continua as a manifestation of hyperglycemia.

A review of 22 patients who presented with the syndrome of epilepsia partialis continua as symptoms of hyperglycemia and occurred during the phase of hyponatremia and mild hyperosmolality. Epilepsia partialis continua persisted for an average of 9 days,and its duration correlated with the degree of hyponatremia, low blood urea nitrogen(BUN) and measured serum osmolality. In all patients, epilepsia partialis continua was the first symptom leading to the diagnosis of diabetes mellitus. The epilepsia partialis continua ceased by normalization of blood glucose level. All patients survived and did not have any complications. The majority of the patients had evidence of a localized structural brain lesions and low serum sodium. Metabolic abnormalities including hyperglycemia,mild hyperosmolality and hyponatremia contribute to the development of epilepsiapartialis continua in an area of focal brain damage. It is important to determine blood glucose levels in all cases of epilepsia partialis continua.

Vasogenic Edema in a Patient with Partial Status Epilepticus / 대한간질학회지

We report a patient who showed vasogenic edema on MRI in association with partial status epilepticus. The patient, for a month, experienced clonic movements of the right arm and leg. As the amplitude and frequency of the clonic movements increased, resulting in epilepsia partialis continua, MRI showed characteristic vasogenic edema features;normal or decreased signal on diffusion-weighted imaging, and increased apparent diffusion coefficient value in the left parasagittal frontal region with strong leptomeningeal enhancement. This region was corresponded to the leg motor area, which was correlated with the degree of hypemetabolism of ictal FDG-PET findings. Considering limited progression of the seizure activity and focal slow waves on EEG, time-locked by clonic jerks, which might result from synaptic inhibition, this case suggests that vasogenic edema may be associated with a lower intensity of the seizure activity compared with cytotoxic edema noted in most of the reported patients.

A Case of Epilepsia Partialis Continua after Gamma Knife Radiosurgery / 대한간질학회지

The gamma knife radiosurgery (GKS) is a safe and effective neurosurgical approach in the treatment of arteriovenous malformation (AVM). Seizures after GKS are rarely reported, but epilepsia partialis continua (EPC) has not been reported yet. We report a patient with EPC accompanied with cerebral edema after GKS in AVM. A 50-year-old man was admitted for EPC, dysarthria and monoparesis 20 days after GKS due to AVM. Brain MRI showed increased cerebral edema around AVM in the left premotor area. Symptoms were relieved by IV dexamethasone and antiepileptic drugs (AEDs). 15 weeks later, EPC recurred and aphasia and right hemiplegia were rapidly aggravated. The follow up brain MRI showed markedly increased cerebral edema compared with previous MRI. EPC was well controlled by AEDs, but aphasia and hemiplegia were not responsive to IV dexamethasone until trying oral prednisolon. We think that cerebral edema induced by EPC, as a complication of GKR, might aggravate hemiparesis.