Abstract
Rasmussen’s encephalitis is a rare neurological disease first described in 1958 that is characterized by medico-refractory seizures, focal unilateral cerebral inflammation, and deficits such as hemiparesis. While we still do not have a full understanding of this disease, proposed theories behind its etiology include auto-immune manifestations, immune attack by T cells, and malfunctional alterations in genetic expression. It is classically considered a rare childhood malady with a median age of onset of six years, and cases in adolescents and adults are even rarer, representing up to 10% of all cases to date. In this report, we would like to share a rare case of Rasmussen's encephalitis that occurred in an adolescent. Our 17-year-old male patient presented with signs and symptoms beginning at age 14 and was initially diagnosed with cerebral palsy only to later present with additional symptoms and characteristic EEG and MRI findings that ultimately led to a diagnosis of Rasmussen’s encephalitis. Thus, with this case report, our intent is twofold: to shed light on an atypical presentation of an already rare disease, even rarer in adolescents and adults, and to underscore the importance of keeping a broad differential when it comes to evaluating a patient with seizures.
Highlights
In their pioneering paper from 1958, Rasmussen et al described cases of a neurological malady consisting of focal seizures, unilateral cerebral hemispheric inflammation, and subsequent impairment including hemiparesis
While the pathobiology of this disease process is still incompletely understood, some proposed hypotheses to explain its etiology include T-cell mediated cytotoxicity, antibody-mediated central nervous system (CNS) inflammation, and cellular anomalies related to aberrant gene expression [1,2,3,4,5,6,7,8,9,10,11]
Considered a childhood disease with a median age of onset of six years, it consists of three stages in the presentation: a prodromal phase that could last a few years and includes isolated seizures and mild hemiplegia, an acute phase that follows with simple partial seizures and often epilepsa partialis continua (EPC) mainly arising from a single hemisphere, and lastly a residual phase with often pharmaco-refractory epilepsy as well as permanent motor and cognitive deficits [4]
Summary
In their pioneering paper from 1958, Rasmussen et al described cases of a neurological malady consisting of focal seizures, unilateral cerebral hemispheric inflammation, and subsequent impairment including hemiparesis. They presented the first case of what we understand as Rasmussen’s encephalitis. MRI often shows marked atrophy as well as increased signal intensity from the affected unilateral hemisphere [5]. Treatment of this neurological malady often involves a combination of immunotherapy, antiepileptic medications, and hemispherectomy in cases of treatment-resistant epilepsy [2,7,11]
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
