MRI findings in the diagnosis and monitoring of rasmussen's encephalitis

Abstract

-Rasmussen's encephalitis is a devastating syndrome of multifocal brain dysfunction and focal seizures. Magnetic resonance (MR) findings, associated with clinical data and electroencephalogram (EEG), may indicate the diagnosis and could be an indicative of prognosis. We studied 5 patients with Rasmussen's encephalitis, assessing clinical history and MR images. All patients had refractory focal seizures with a predominant motor component associated with hemispheric atrophy, that was proportional to severity of disease and neurological deficits in these patients. Gray and white matter abnormal signal on T2 MR images were found in patients who had hemiparesis. It was not related to the duration of the disease but to aggressiveness. MR proton spectroscopy in severe disease showed lactate and choline increase and decreased NAA, reflecting neuronal and axonal loss, gliosis and elevated membrane turnover and recent -crisis (not controlled). MR studies, in addition to help in diagnosis, may be useful for monitoring metabolic changes and progression of disease in Rasmussen's encephalitis.KEy WoRds: Rasmussen's encephalitis, MRI, spectroscopy, epilepsy. Achados de RM no diagnóstico e monitorização da encefalite de RasmussenResumo -A encefalite de Rasmussen é uma devastadora síndrome com disfunção cerebral multifocal e convulsões focais. Achados de ressonância magnética (RM), associados aos dados clínicos e de eletrencefalograma (EEG), podem indicar o diagnóstico e podem ser indicativos de prognóstico. Foram estudados 5 pacientes com encefalite de Rasmussen, avaliando a história clínica e imagens de RM. Todos os pacientes apresentavam crises epilépticas focais refratárias com componente predominantemente motor associadas à atrofia hemisférica, que foi proporcional à gravidade da doença e déficits neurológicos nestes pacientes. Alteração da intensidade de sinal nas substâncias branca e cinzenta, nas sequências ponderadas em T2, foram encontradas nos pacientes com hemiparesia. Ela não estava relacionada com a duração da doença, mas à severidade. A espectroscopia de prótons por RM na doença severa demonstrou aumento dos níveis de colina e lactato e diminuição de N-acetilaspartato, refletindo perda neuronal e axonal, gliose e aumento de turnover de membrana e crise recente (não controlada). Estudos de RM, além de ajudar no diagnóstico, podem ser úteis para acompanhar alterações metabólicas e progressão da doença na encefalite de Rasmussen.PAlAvRAs-chAvE: encefalite de Rasmussen, RM, espectroscopia, epilepsia.

Parry Romberg Syndrome: 7 Cases and Literature Review

“…3,16,17,25,26 PRS also shares features with Rasmussen encephalitis, an autoimmune/inflammatory disorder affecting 1 side of the brain, resulting in refractory epilepsy and progressive hemiplegia and typically affecting children in the first decade of life. 14,27,28 On imaging, Rasmussen encephalitis is characterized by predominately unilateral hemispheric areas of hyperintense T2 signal with eventual development of unilateral ce- rebral atrophy, 27,28 findings that may be indistinguishable from intracranial imaging features of PRS. There have been reported cases of coexisting Rasmussen encephalitis and PRS in pediatric patients, 4,8,14,15,17 supporting a possible pathophysiologic correlation between these 2 entities.…”

“…In younger patients, differentiating these 2 entities may be difficult, but clinical signs and symptoms, such as the presence of epilepsia partialis continua, a hallmark symptom of Rasmussen encephalitis, and involvement of cutaneous structures should guide the clinician and radiologist to the correct diagnosis. 1,4,7,8,27,28 Other diseases in which facial asymmetry is a prominent clinical feature include hemifacial microsomia (first and second brachial arch syndrome) and Goldenhar syndrome, but unlike PRS, these conditions are typically congenital and nonprogressive. 19,21,22 Hemifacial hyperplasia causes asymmetry of the face as well, but rather than atrophy, this entity is characterized by overgrowth and hyperplasia.…”

Complete right cerebral hemispheric diffusion restriction and its follow-up in a case of Rasmussen's encephalitis

“…Clinically, patients have abrupt onset of seizures with progressive neurological decline 1–3. Diagnosis can be performed on the basis of the clinical picture and neuroimaging demonstrating progressive cerebral hemiatrophy 3. Imaging in the acute phase may reveal cerebral oedema.…”

Oriented dialogue in the treatment of Rasmussen's encephalitis

“…A ER é caracterizada por desordem neurológica progressiva devastante, com epilepsia, hemiplegia e declínio cognitivo [1]. As convulsões são focais, envolvem exclusivamente um lado do corpo e são refratárias ao uso de anticonvulsivantes [2] [3]. Achados de ressonância magnética (RM), associados aos dados clínicos e de eletroencefalograma (EEG) podem indicar o diagnóstico [2].…”

“…As convulsões são focais, envolvem exclusivamente um lado do corpo e são refratárias ao uso de anticonvulsivantes [2] [3]. Achados de ressonância magnética (RM), associados aos dados clínicos e de eletroencefalograma (EEG) podem indicar o diagnóstico [2]. O tratamento ainda persiste em discussão, pois o número de casos não é suficiente para estudos detalhados [1].…”