Rasmussen Ensefaliti
Özet
Rasmussen ensefaliti, çocukluk çağında görülen kronik ve ilerleyici bir nöroinflamatuvar hastalık olup; dirençli fokal nöbetler, kognitif gerileme ve tek taraflı serebral atrofi ile karakterizedir. Tanı süreci klinik gözlemler, EEG ve MRG bulgularına dayanmakta, tedavi protokollerinde ise immünmodülatör ilaçlar ilerlemeyi yavaşlatmak amacıyla kullanılmaktadır. Farmakolojik tedavilerin yetersiz kaldığı durumlarda, nöbet kontrolünü sağlamak için tek kesin çözüm genellikle cerrahi girişimdir. Erken tanı ve doğru cerrahi planlama, hastaların uzun dönem prognozunu iyileştiren temel faktörlerdir.
Rasmussen encephalitis is a chronic, progressive inflammatory neurological disorder affecting children, characterized by intractable focal seizures, cognitive decline, and unilateral cerebral atrophy. Diagnosis is established through clinical presentation, EEG, and MRI findings, while treatment primarily focuses on immunomodulatory therapies to slow disease progression. When medical management fails to control seizures, surgical intervention remains the definitive treatment option to achieve long-term seizure freedom. Timely diagnosis and effective surgical planning are critical for optimizing patient prognosis and functional outcomes.
Referanslar
Rasmussen T, Olszewski J, Lloyd-Smith D. Focal seizures due to chronic localized encephalitis . Neurology . 1958; 8:435 – 445.
Oguni H, Andermann F , Rasmussen T . The natural history of the syndrome of chronic encephalitis and epilepsy: a study of the MNI series of 48 cases .Adv Neurol 1992;57:419-433.
Varadkar S, Bien CG, Kruse CA, et al. Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances. Lancet Neurol. 2014;13:195–205.
Klaa H, Ben Younes T, Benrhouma H, et al. Rasmussen's Encephalitis: A Report of a Tunisian Pediatric Case and Literature Review. Case Rep Neurol Med. 2020;2020:6810237.
Pradeep K, Sinha S, Mahadevan A, et al. “Clinical, electrophysiological, imaging, pathological and therapeutic observations among 18 patients with Rasmussen’s encephalitis,” Journal of Clinical Neuroscience. 2016;25: 96–104.
Rogers SW, Andrews PI, Gahring LC, et al. Autoantibody to glutamate receptor GluR3 in Rasmussen’s encephalitis . Science . 1994; 265:648 – 651.
Pardo CA, Arroyo S, Ringing EPG, et al. Neuronal injury in Rasmussen’s chronic encephalitis is mediated by cytotoxic t-cells . Epilepsia . 1994;35:89 .
Andrews PI , Dichter MA , Berkovic SF , et al. Plasmapheresis in Rasmussen’s encephalitis . Neurology . 1996; 46: 242 – 246.
Freeman JM. Rasmussen’s syndrome: progressive autoimmune multi-focal encephalopathy. Pediatr Neurol. 2005;32: 295 – 299.
Bien CG, Tiemeier H, Sassen R, et al. Rasmussen encephalitis: incidence and course under randomized therapy with tacrolimus or intravenous immunoglobulins. Epilepsia. 2013;54: 543–550.
Prakash K. 2008. Localization- related epilepsies Pellock JM, Bourgeois BFD, Dodson WE eds. Pellock’s Pediatric Epilepsy Diagnosis and Therapy. (377-386) Third Edition. , LLC: Demos Medical Publishing.
Bien CG, Widman G, Urbach H, et al. The natural history of Rasmussen’s encephalitis . Brain. 2002;125:1751 – 1759.
Chiapparini L. Diagnostic imaging in 13 cases of Rasmussen’s encephalitis: can early MRI suggest the diagnosis? Neuroradiology. 2003;58:250 – 257.
Longaretti F, Dunkley C, Varadkar S, et al. Evolution of the EEG in children with Rasmussen’s syndrome. Epilepsia. 2012;53: 1539 – 1545.
Olson HE, Lechpammer M, Prabhu SP et al. Clinical application and evaluation of the Bien diagnostic criteria for Rasmussen encephalitis. Epilepsia. 2013;54:1753–1760.
Frucht S. Dystonia, athetosis, and epilepsia partialis continua in a patient with late-onset Rasmussen’s encephalitis. Movement Disorders. 2002;17:609–612.
Varghese B, Aneesh M, Singh N, et al. A case of Rasmussen encephalitis: the differential diagnoses and role of diagnostic imaging. Oman Medical Journal. 2014;29:67–70.
Bien CG, Granata T, Antozzi C, et al. Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement. Brain J Neurol. 2005;128:454–71.
Pradeep K, Sinha S, Mahadevan A, et al. Clinical, electrophysiological, imaging, pathological and therapeutic observations among 18 patients with Rasmussen’s encephalitis. Journal of Clinical Neuroscience. 2016;25:96–104.
Yamazaki E, Takahashi Y, Akasaka N, et al. Temporal changes in brain MRI findings in Rasmussen syndrome. Epileptic Disorders. 2011;13:229–239.
Matthews PM, Andermann F, Arnold DL. A proton magnetic resonance spectroscopic study of focal epilepsy in humans . Neurology . 1990;40:985 – 989.
Orsini A, Foiadelli T, Carli N, Costagliola G, Masini B, Bonuccelli A, et al. Rasmussen's encephalitis: From immune pathogenesis towards targeted-therapy. Seizure. 2020;81:76-83
Fiorella DJ, Provenzale JM, Coleman RE, Crain BJ, Al-Sugair AA. F-fluorodeoxyglucose positron emission tomography and MR imaging findings in Rasmussen encephalitis. AJNR Am J Neuroradiol. 2001;22:1291–1299.
Prayson RA, Frater JL. Rasmussen encephalitis: a clinicopathologic and immunohistochemical study of seven patients . Am J Clin Pathol. 2002;117:776 – 782.
Bien CG, Bauer J, Deckwerth TL, et al. Destruction of neurons by cytotoxic T cells: a new pathogenic mechanism in Rasmussen’s encephalitis . Ann Neurol. 2002;51:311 – 318.
Pardo CA, Vining EP, Guo L, et al. The pathology of Rasmussen syndrome: stages of cortical involvement and neuropathological studies in 45 hemispherectomies . Epilepsia . 2004;45:516 – 526.
Granata T, Fusco L, Gobbi G, et al. Experience with immunomodulatory treatments in Rasmussen’s encephalitis. Neurology. 2003;61:1807–1810.
Tang C, Luan G, Li T. Rasmussen's encephalitis: mechanisms update and potential therapy target. Ther Adv Chronic Dis. 2020;29:11:2040622320971413.
Takahashi Y, Yamazaki E, Mine J, Kubota Y, Imai K, Mogamiet Y, et al. Immunomodulatory therapy versus surgery for Rasmussen syndrome in early childhood. Brain Dev 2013;35(8):778–85
El Tawil S, Morris R, Mullatti N, et al. Adult onset Rasmussen’s encephalitis associated with reflex language induced seizures responsive to Rituximab therapy. Seizure. 2016;42: 60–62.
Bien CG, Gleissner U, Sassen R, Widman G, Urbach H, Elger CE. An open study of tacrolimus therapy in Rasmussen encephalitis. Neurology 2004;62(11):2106–9
Schwab N, Schneider-Hohendorf T, Wiendl H. Therapeutic uses of anti-alpha4- integrin (anti-VLA-4) antibodies in multiple sclerosis. Int Immunol 2015;27(1):47–53
Orsini A, Costagliola G, Perna D, et al. Efficacy and tolerability of mycophenolate mofetil in a pediatric Rasmussen syndrome. Epilepsy Behav Rep. 2020;13:100334.
Chen H, Qiu W, Zhang Q, et al. Comparisons of the efficacy and tolerability of mycophenolate mofetil and azathioprine as treatments for neuromyelitis optica and neuromyelitis optica spectrum disorder. Eur J Neurol 2017;24(1):219–26.
Guan Y, Zhou J, Luan G, et al: Surgical Treatment of Patients with Rasmussen Encephalitis. Stereotact Funct Neurosurg 2014;92: 86-93.
Bien CG, Schramm J. Treatment of Rasmussen encephalitis half a century after its initial description: promising prospects and a dilemma. Epilepsy Res. 2009;86: 101–112.
Lewis DV, Shinnar S, Hesdorffer DC, et al. Hippocampal sclerosis after febrile status epilepticus: the FEBSTAT study. Ann. Neurol. 2014;75: 178–185.
