Adult-onset Rasmussen's encephalitis: anatomical-electrographic-clinical features of 7 Italian cases.
OBJECTIVE
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.
Villani
Flavio
F
Division of Clinical Epileptology, Istituto Nazionale Neurologico, C. Besta, Milan, Italy. fvillani@istitutobesta.it
Pincherle
Alessandro
A
Antozzi
Carlo
C
Chiapparini
Luisa
L
Granata
Tiziana
T
Michelucci
Roberto
R
Rubboli
Guido
G
Simone
Isa
I
Bellomo
Rosella
R
Spreafico
Roberto
R
D000328:Adult; D017668:Age of Onset; D001284:Atrophy; D001921:Brain / Q000473:pathology; D003937:Diagnosis, Differential; D004569:Electroencephalography / Q000706:statistics & numerical data; D004576:Electromyography; D004660:Encephalitis / Q000145:classification / Q000175:diagnosis* / Q000235:genetics; D017036:Epilepsia Partialis Continua / Q000145:classification / Q000175:diagnosis / Q000235:genetics; D004828:Epilepsies, Partial / Q000175:diagnosis; D005073:Evoked Potentials, Somatosensory; D005260:Female; D019788:Fluorodeoxyglucose F18; D007839:Functional Laterality / Q000502:physiology; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000706:statistics & numerical data; D008875:Middle Aged; D010641:Phenotype; D049268:Positron-Emission Tomography; D012189:Retrospective Studies
D019788:Fluorodeoxyglucose F18