Childhood-onset nonprogressive chronic encephalitis

Philippe Rizek; Christina Cheung; Richard S McLachlan; Brent Hayman-Abello; Donald H Lee; Robert R Hammond; Seyed M Mirsattari

doi:10.1016/j.yebeh.2013.11.005

Childhood-onset nonprogressive chronic encephalitis

Epilepsy Behav. 2014 Feb:31:85-90. doi: 10.1016/j.yebeh.2013.11.005. Epub 2013 Dec 22.

Authors

Philippe Rizek¹, Christina Cheung¹, Richard S McLachlan¹, Brent Hayman-Abello², Donald H Lee³, Robert R Hammond⁴, Seyed M Mirsattari⁵

Affiliations

¹ Department of Clinical Neurological Sciences, Western University, London, Canada.
² Psychology Services, London Health Sciences Centre, London, Ontario, Canada.
³ Department of Medical Imaging, Western University, London, Canada.
⁴ Department of Clinical Neurological Sciences, Western University, London, Canada; Department of Pathology, Western University, London, Canada.
⁵ Department of Clinical Neurological Sciences, Western University, London, Canada; Department of Medical Imaging, Western University, London, Canada; Department of Medical Biophysics, Western University, London, Canada; Department of Psychology, Western University, London, Canada. Electronic address: smirsat2@uwo.ca.

PMID: 24368410
DOI: 10.1016/j.yebeh.2013.11.005

Abstract

Purpose: The purpose of this study was to describe a series of patients with pathologically proven chronic encephalitis who had a nonprogressive course during a long follow-up, suggestive of a "benign" variant of Rasmussen's encephalitis (RE).

Methods: Four patients who were referred to our Comprehensive Epilepsy Program at London Health Science Centre in London, Ontario, were diagnosed with chronic encephalitis on a pathological basis after epilepsy surgery to treat their partial-onset seizures.

Results: None of our four cases followed the typical course of RE despite their childhood-onset seizures between ages 2 and 12years. One was preceded by a mild head trauma and fever at onset. None had epilepsia partialis continua (EPC). Their long-term follow-up revealed a nonprogressive form of the syndrome with respect to the neurological examination, EEG, MRI, and neuropsychological findings.

Conclusion: These cases extend the spectrum of childhood-onset intractable epilepsy with chronic encephalitis to include nonprogressive variants of RE. The absence of EPC may be a prognostic indicator of a nonprogressive course.

Keywords: CE; Chronic encephalitis; EEG; EPC; Epilepsia partialis continua; Epilepsy surgery; MRI; Natural history; Nonprogressive variant; RE; Rasmussen's; Rasmussen's encephalitis; chronic encephalitis; electroencephalography; epilepsia partialis continua; magnetic resonance imaging.

MeSH terms

Adult
Age of Onset
Electroencephalography
Encephalitis / complications*
Epilepsy / diagnosis*
Epilepsy / etiology*
Female
Follow-Up Studies
Humans
Magnetic Resonance Imaging
Male
Neuropsychological Tests