Adult‐Onset Rasmussen's Encephalitis: Anatomical‐Electrographic‐Clinical Features of 7 Italian Cases

Abstract

Summary:Purpose: A limited number of cases of adult-onset Rasmussen's encephalitis (A-RE) have been reported, but the features of the syndrome are still unclear. The aim of this study was to verify the clinical features of A-RE, and outline a noninvasive approach that may allow its early diagnosis and treatment.Methods: Retrospective evaluation of extensive noninvasive work-up of seven patients with A-RE, including repeat clinical, neurophysiological, and neuroimaging investigations.Results: The study identified two distinct patterns of disease presentation, one characterized by focal motor epilepsy (the "epileptic" phenotype), and the other by focal cortical myoclonus (the "myoclonic" phenotype). Unilateral neurological deficits and brain atrophy were progressive in both phenotypes, but they were more prominent and were detected earlier in the "epileptic" phenotype.Conclusions: The anatomo-electroclinical features of these patients allowed a noninvasive diagnosis of A-RE and identification of two distinct disease phenotypes. Early noninvasive diagnosis can allow faster initiation of treatment.

Neuroimaging follow‐up in a case of Rasmussen's encephalitis with dyskinesias

“…12,14,15 A recent review of seven cases of adult onset RE highlights the role of FDG-PET in revealing early impairment of metabolism when only mild MRI atrophy is evident. 4 In our patient, the finding of a diffuse hypermetabolism of the anterior cortex may be explained by the very early time of the study. The contralateral hypermetabolism of the cerebellar cortex has already been reported.…”

“…Moreover, residual atrophy and neurological deficits are less severe in these patients. 3,4 The adult-onset patient here described showed an atypical course for the very short prodromal phase, about 3 months, a dramatic but short acute stage (about 2 months) and a 1-year disease stabilization followed by a contralateral relapse.…”

“…[5][6][7] Such psychogenic gait disorders can have various more or less characteristic features (Table 1), but none of them is truly conclusive. 3,4,8,9 Our patient presented with a most unusual gait disorder, which could almost be described as a "recumbent gait." This appeared solely upon eye closure, while walking with eyes open was completely normal.…”

Rasmussen encephalitis with dual pathology in a patient without seizures: case report and literature review

“…Although most patients present in childhood, 10 % of patients present in adolescence or adulthood [25]. In these cases, the clinical progression may be slower and the neurological deficits relatively mild [4,15,17,38]. Often, the seizure semiology is characteristic of temporal lobe epilepsy [17,19].…”

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…Rasmussen's encephalitis (RE) is a rare inflammatory disorder that affects primarily children, but can also affect adults [267][268][269][270][271]. The clinical presentation is that of refractory seizures with cognitive decline, hemispheric atrophy, and progressive hemiparesis; young children tend to progress much more rapidly than young adults.…”