title: Adult-onset Rasmussen's encephalitis: anatomical-electrographic-clinical features of 7 Italian cases.
abstract: 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.
authors.affiliation: Division of Clinical Epileptology, Istituto Nazionale Neurologico, C. Besta, Milan, Italy. fvillani@istitutobesta.it
authors.forename: Flavio
authors.initials: F
authors.lastname: Villani
authors.forename: Alessandro
authors.initials: A
authors.lastname: Pincherle
authors.forename: Carlo
authors.initials: C
authors.lastname: Antozzi
authors.forename: Luisa
authors.initials: L
authors.lastname: Chiapparini
authors.forename: Tiziana
authors.initials: T
authors.lastname: Granata
authors.forename: Roberto
authors.initials: R
authors.lastname: Michelucci
authors.forename: Guido
authors.initials: G
authors.lastname: Rubboli
authors.forename: Isa
authors.initials: I
authors.lastname: Simone
authors.forename: Rosella
authors.initials: R
authors.lastname: Bellomo
authors.forename: Roberto
authors.initials: R
authors.lastname: Spreafico
mesh_terms: 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
chemical_list: D019788:Fluorodeoxyglucose F18