Rethinking the Magnetic Resonance Imaging Findings in Early Rasmussen Encephalitis: A Case Report and Review of the Literature

OBJECTIVE: We present a child with Rasmussen encephalitis and highlight the pitfalls of diagnosis when magnetic resonance imaging (MRI) is negative for atrophy. We review the literature regarding this issue, introduce the FreeSurfer software as a potential means of noninvasive diagnosis, and discuss methods for prompt and definitive treatment. METHODS: In addition to the patient description, we review the English language literature regarding pathologic diagnosis of Rasmussen encephalitis using the key words Rasmussen encephalitis, focal lesions, MRI, atrophy, epilepsia partialis continua and hemiparesis in PubMed. We conducted a retrospective, "volumetric analysis of our patient's MRIs using FreeSurfer. RESULTS: Unlike the majority of patients in the literature with Rasmussen encephalitis, our patient's initial MRI was normal and later showed only a small area of T2 and fluid attenuated inversion recovery high signal despite the presence of epilepsia partialis continua and a rapidly deteriorating clinical course. She did not meet the Rasmussen encephalitis diagnostic criteria until biopsy was obtained but is now seizure-free after functional hemispherotomy performed six months after her initial seizure. FreeSurfer analysis did not show cortical atrophy. CONCLUSION: The Bien criteria have poor sensitivity for the diagnosis of Rasmussen encephalitis when the MRI is negative for atrophy. Tissue diagnosis is essential in such instances. We suggest a high clinical index of suspicion and multidisciplinary collaboration between radiology, pathology, and neurosurgery to facilitate a greater emphasis on biopsy followed by hemispherotomy as definitive therapy for individuals with early Rasmussen encephalitis.