title: Electrophysiological findings in Rasmussen's syndrome.
abstract: Rasmussen syndrome is a rare, inflammatory and probably autoimmune disease presenting with epilepsia partialis continua which is generally in the form of myoclonic jerks and involves the upper extremities with or without head involvement. We sought to demonstrate the electrophysiological features in patients with Rasmussen syndrome. We performed continuous electrophysiological recordings of involuntary movement, as well as recordings of startle responses and long latency reflex in three patients with a diagnosis of Rasmussen syndrome. Positive and negative myoclonus were recorded. Startle responses were found to be suppressed. However, long latency reflexes were high in amplitude and one patient even had a C reflex. Stimulus-sensitive positive and negative cortical myoclonus are typical in epilepsia partialis continua of Rasmussen syndrome and degeneration of brainstem and reticulospinal pathways may develop in Rasmussen syndrome.
authors.affiliation: Department of Neurology, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey.
authors.forename: Ayşegül
authors.initials: A
authors.lastname: Gündüz
authors.affiliation: Department of Neurology, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey.
authors.forename: Meral E
authors.initials: ME
authors.lastname: Kızıltan
authors.affiliation: Department of Neurology, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey.
authors.forename: Tülin
authors.initials: T
authors.lastname: Coşkun
authors.affiliation: Department of Neurology, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey.
authors.forename: Şakir
authors.initials: Ş
authors.lastname: Delil
authors.affiliation: Department of Neurology, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey.
authors.forename: Naz
authors.initials: N
authors.lastname: Yeni
authors.affiliation: Department of Neurology, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey.
authors.forename: Çiğdem
authors.initials: Ç
authors.lastname: Özkara
mesh_terms: D000293:Adolescent; D000328:Adult; D001921:Brain / Q000503:physiopathology*; D004569:Electroencephalography / Q000379:methods; D004660:Encephalitis / Q000175:diagnosis / Q000503:physiopathology*; D017036:Epilepsia Partialis Continua / Q000175:diagnosis / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male; D009207:Myoclonus / Q000503:physiopathology*; D055815:Young Adult; D000015:Abnormalities, Multiple; D004424:Ear Canal / Q000002:abnormalities; D013628:Talus / Q000002:abnormalities
keywords: Rasmussen syndrome; epilepsia partialis continua; long latency reflex; myoclonus; startle response