TY - JOUR
T1 - Adult-onset Rasmussen's encephalitis
T2 - Anatomical-electrographic-clinical features of 7 Italian cases
AU - Villani, Flavio
AU - Pincherle, Alessandro
AU - Antozzi, Carlo
AU - Chiapparini, Luisa
AU - Granata, Tiziana
AU - Michelucci, Roberto
AU - Rubboli, Guido
AU - Simone, Isa
AU - Bellomo, Rosella
AU - Spreafico, Roberto
PY - 2006/12
Y1 - 2006/12
N2 - 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.
AB - 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.
KW - Epilepsia partialis continua
KW - Focal motor epilepsy
KW - Myoclonus
KW - Rasmussen's encephalitis
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U2 - 10.1111/j.1528-1167.2006.00876.x
DO - 10.1111/j.1528-1167.2006.00876.x
M3 - Article
C2 - 17239105
AN - SCOPUS:33845531683
VL - 47
SP - 41
EP - 46
JO - Epilepsia
JF - Epilepsia
SN - 0013-9580
IS - SUPPL. 5
ER -