Slowly progressive hemiparesis in childhood as a consequence of Rasmussen encephalitis without or with delayed-onset seizures.
Five young children developed slowly progressive hemiparesis as the initial manifestation of Rasmussen encephalitis (RE). Three have remained seizure free over an observational period of 1.3-1.9 years. In the remaining two patients, seizures occurred after 0.5 and 0.6 years respectively. We suggest that RE might be presently underdiagnosed and should be suspected in cases of new onset hemiparesis. In this series, three out of five patients showed oligoclonal bands on examination of cerebrospinal fluid (CSF) which represented additional diagnostic hints towards an immune-mediated condition. According to recently published formal diagnostic criteria, evidence of progressive cerebral hemiatrophy or bioptic identification of RE-typical inflammation confirms the diagnosis in such cases. Long-term immunotherapy is recommended in order to prevent further tissue loss and functional decline.
Bien
C G
CG
Department of Epileptology, University of Bonn, Bonn, Germany. christian.bien@ukb.uni-bonn.de
Elger
C E
CE
Leitner
Y
Y
Gomori
M
M
Ran
B
B
Urbach
H
H
Wilken
B
B
Korn-Lubetzki
I
I
D001921:Brain / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D004660:Encephalitis / Q000150:complications* / Q000175:diagnosis*; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D008297:Male; D043025:Oligoclonal Bands / Q000134:cerebrospinal fluid; D010291:Paresis / Q000209:etiology*; D012640:Seizures / Q000209:etiology*
D043025:Oligoclonal Bands