rasmussen syndrome in adults

Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances. Drs Oxbury, Polkey and Duchowny have produced a book that will appeal to epileptologists, paediatric neurologists, and neurosurgeons. The books clinical strengths will make it an invaluable working tool. *Correspondence: James Francis Castellano, jcastell@montefiore.org, Front. doi:10.1212/WNL.0b013e31829c5ceb, 67. In September 2016, approximately 3 years after her presentation to our institution and 15 years from first seizure, she began treatment with Rituximab. The disorder is characterized by partial or complete loss … Request your next appointment through MyChart! doi:10.1111/j.1528-1157.1997.tb01096.x, 42. Rasmussen's encephalitis. It is characterized by a slow and progressive atrophy affecting one side of the face, the skin, the subcutaneous tissue, the muscles, the cartilages, and the underlying bony structures. Found inside – Page 451Rasmussen's chronic encephalitis in adults. Arch Neurol 1993;50:269-274. 16. Lamer A|, Smith SJ, Duncan JS, et al. Late-onset Rasmussen's syndrome with ... Clinico-pathological investigations of Rasmussen encephalitis suggest multifocal disease progression and associated focal cortical dysplasia. 1. At this point, approximately 4 years from first clinical manifestation, the patient presented to our institution. The inflammation affects the nerve cells in the brain and causes seizures. MR images were obtained on machines using a 3-T magnet. Poloni TE, Galli A, Pichiecchio A, di Lodovico L, Ceroni M. Good outcome in adult-onset Rasmussen’s encephalitis syndrome: is recovery possible? Rasmussen syndrome is a chronic, progressive, focal encephalitis that is commonly accompanied by focal seizures, hemiparesis and cognitive decline. Olson HE, Lechpammer M, Prabhu SP, Ciarlini PDSC, Poduri A, Gooty VD, et al. The International League Against Epilepsy (ILAE) Diagnostic Manual's goal is to assist clinicians who look after people with epilepsy to diagnose the epilepsy syndrome and (if possible) the etiology of the epilepsy. In addition to a known left frontoparietal epileptic focus, the patient then developed a second focus in the ipsilateral occipital quadrant, first observed in May 2013 (Figure 2). At this point, the patient was lost to follow-up. Rasmussen first described his eponymous disease in 1958 as a progressive epileptic disorder in children due to chronic unilateral encephalitis (1). Antibodies against GluR3 peptides are not specific for Rasmussen’s encephalitis but are also present in epilepsy patients with severe, early onset disease and intractable seizures. Nationwide survey (incidence, clinical course, prognosis) of Rasmussen’s encephalitis. Brain (2009) 132:1236–46. This book will take the reader behind the myths of GH and into the real world of clinical endocrinology. The contributions stem from recognized clinicians and scientists who have been working in the field for decades. Patient 1 (top panel) showed left frontal predominant atrophy over a 2-year period; axial sections. doi:10.1212/01.wnl.0000298723.96653.99, 4. Spitz M-A, Dubois-Teklali F, Vercueil L, Sabourdy C, Nugues F, Vincent A, et al. Türkdogan-Sözüer D, Ozek MM, Sav A, Dinçer A, Pamir MN. The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. Rasmussen’ s syndrome with adult onset, presenting with psychosis, is a rare entity. Our discussion to this point has focused on the diagnostic challenge of RE. Accepted 1 December 2011. Hart YM, Andermann F, Fish DR, Dubeau F, Robitaille Y, Rasmussen T, et al. Lastly, histopathology may be utilized as a final diagnostic criterion for RE. Rationale: Rasmussen syndrome (RS) is a very rare chronic neurological disorder, characterized by unilateral inflammation and atrophy of the cerebral cortex, intractable epileptic seizures, and progressive neurological and cognitive deterioration. Click on the link to view a sample search on this topic. Self-reflection is appropriate to determine - 8. Unfortunately, the patient continued to have brief focal seizures several times per week and her mild expressive aphasia was static. CT imaging done on this admission revealed marked right hemisphere atrophy. Late-onset and rather slow changes have spurred clinicians to develop novel methods to detect more subtle changes in cortical volume (4, 36–39). While the cause is unknown, the disorder causes inflammation in one half of a child's brain. A fully developed outcome for a client goal would include: (SATA) Attainable for client, measurable terms and time sensitive 2. Neurology (1998) 50:731–5. Hennessy MJ, Koutroumanidis M, Dean AF, Jarosz J, Elwes RD, Binnie CD, et al. doi:10.1111/j.1552-6569.2008.00320.x, 39. Furthermore, given the continued discovery of autoantibody-mediated encephalitides with variable clinical courses, we propose that the early diagnostic work-up should include evaluation for known paraneoplastic and autoimmune syndromes. FL provided guidance in data interpretation and aided in manuscript preparation. Ramaswamy V, Walsh JG, Sinclair D, Johnson E, Tang-Wai R, Wheatley B, et al. Neurology (2008) 70:374–7. Enter the last name, specialty or keyword for your search below. Semin Pediatr Neurol (2014) 21:129–36. doi:10.1212/01.WNL.0000128044.94294.87, 12. Bien CG, Widman G, Urbach H, Sassen R, Kuczaty S, Wiestler OD, et al. A hemispherectomy is performed in children who have severe and intractable seizure disorders. Wang D, Blümcke I, Gui Q, Zhou W, Zuo H, Lin J, et al. Automated 3D MRI volumetry reveals regional atrophy differences in Rasmussen encephalitis. CSF evaluation was significant for a mild lymphocytic pleocytosis, negative HSV and CMV PCR, and cytology negative for malignant cells. J Neuroimaging (2009) 19:378–80. doi:10.1111/j.0013-9580.2004.33103.x, 45. Can J Neurol Sci (2006) 33:302–5. doi:10.1111/epi.12334, 44. In particular, the prodromal and residual phases of the disease may not show a T-cell predominance (43). Rasmussen encephalitis was first described by American neurologist Theodore Brown Rasmussen (1910-2002) in 1958 2). Over the following months, patient’s aphasia became nearly constant and semiology again began to involve the right arm. We begin our discussion by examining the natural clinical history. doi:10.1017/S0317167100005175, 64. doi:10.1055/s-0034-1383822, 22. Kupila L, Jutila L, Immonen A, Vanninen R, Mervaala E, Pateau A, et al. Dev Med Child Neurol (2015) 58(2):209–10. Furthermore, given the multifocal nature of RE pathology and potential absence of pathology in mild disease or during prodromal periods, we would not encourage the use of focal cortical biopsy to aid in RE diagnosis. I have had 2 brain surgeries to remove the affected areas. Concordant with the other cases of adult-onset RE (2, 3, 7, 8, 13, 14, 20), our three patients had long prodromal and acute phases (Table 1) and a very ill-defined residual period. The aim of this study was to identify the presence of any neuroimaging patterns of Rasmussen syndrome using magnetic resonance imaging (MRI). By family and caregiver report, patient’s overall seizure frequency decreased over the 5-month period she was treated with immunotherapy. Immune therapies attempted in RE include high-dose steroids (58–60), intravenous immunoglobulin (7, 9, 58, 61–64), plasma exchange (65), azathioprine (57, 66), tacrolimus (11, 58, 61), natalizumab (66), and rituximab (67). In Rasmussen encephalitis, hemichannels associated with microglial activation are linked to cortical pyramidal neuron coupling: a possible mechanism for cellular hyperexcitability. Bien CG, Granata T, Antozzi C, Cross JH, Dulac O, Kurthen M, et al. Takahashi Y, Yamazaki E, Mine J, Kubota Y, Imai K, Mogami Y, et al. All patients were unresponsive to conventional antiepileptics; five patients subsequently underwent functional … The patient remained symptomless for more than 15 years from clinical onset and more than eight years after the discontinuation of immunological therapy. However, detection in adults is increasing with … Rasmussen’s encephalitis, originally thought to be a chronic form of viral encephalitis, is now thought to be an autoimmune disease of the brain and is more properly termed Rasmussen’s syndrome. doi:10.1212/WNL.50.3.731, 27. Documentation is imperative to ensure - 3. The patient began monthly IVIG infusions to supplement AED pharmacotherapy and experienced improvement in expressive aphasia and a decline in seizure frequency. Studies have shown important roles for both cell-mediated and humoral immune responses; cytotoxic T cells (48, 49), gamma delta T cells (50), autoantibodies (21, 51, 52), microglia (53, 54), and astrocytes (55) have all been pathologically implicated. However, the response to antipsychotic amisulpride … Histopathological findings have led to the guiding hypothesis that immune-mediated mechanisms are, in part or completely, responsible for disease progression. Figure 2. As noted by other investigators, EEG abnormalities are neither unihemispheric (16, 23), nor correlative with MRI changes (2, 22, 24, 40). It most often occurs in children under the age of 10, although adolescents and adults may also be affected. Neurology (1999) 52:738–42. doi:10.1111/j.1440-1789.2009.01079.x, 33. J Neuroinflammation (2013) 10:152. doi:10.1371/journal.pone.0029539, 55. doi:10.1016/j.jocn.2009.02.042, 34. Found insideIn the course of their researches for Mental Imagery in the Child (1971), the authors came to appreciate that action may be more conducive to the formation and conservation of images than is mere perception. As the neurological symptoms were largely absent, management with antipsychotic for the psychiatric symptoms provided some positive results. —Rasmussen's chronic encephalitis, a cause of intractable epilepsy in childhood, is described in three adults. Rasmussen encephalitis (RE), also called Rasmussen syndrome, is Villani F, Pincherle A, Antozzi C, Chiapparini L, Granata T, Michelucci R, et al. Importantly, much as there is a natural history to the clinical course, EEG pathology also appears to evolve (8, 19, 41, 42). Apr 21, 2012, 7:10:31 AM. Initial serum and CSF studies, including cell counts were normal. Epilepsia (2008) 49:521–6. Neurology (2013) 81:395–7. References. doi:10.1016/j.yebeh.2013.05.033, PubMed Abstract | CrossRef Full Text | Google Scholar, 3. Though our three patients all followed this paradigm, both duration and severity were variable (Table 1). She presented to our institution as an elective admission to the EMU 6 months after seizure onset. Because Rasmussen syndrome is difficult or impossible to control with medication, in many cases, the most effective treatment option is surgery, including hemispherectomy. Rasmussen syndrome Rasmussen encephalitis epilepsy immune antibody encephalitis N-methyl-D-aspartate receptor voltage-gated potassium channel febrile infection-related epilepsy syndrome Abstract An underlying immune basis is emerging in an increasing number of … 4 cases ... • Sturge-Weber syndrome • Stroke • Hemiconvulsion–hemiplegia–epilepsy syndrome Notice of Privacy Practices(Patients & Health Plan Members). This link opens in a new window. Casciato S, Di Bonaventura C, Giallonardo AT, Fattouch J, Quarato PP, Mascia A, et al. The usual symptoms are those of progressive unilateral brain dysfunction with intractable seizures. Learn vocabulary, terms, and more with flashcards, games, and other study tools. It is generally considered to be a disease of childhood, with most cases occurring in children younger than 10 years, although adult onset cases do occur. Bien CG, Tiemeier H, Sassen R, Kuczaty S, Urbach H, Lehe von M, et al. CD8+ T-cell clones dominate brain infiltrates in Rasmussen encephalitis and persist in the periphery. Request a Consultation, Adult Neurology: 410-955-9441 Pediatric Neurology: 410-955-4259 Adult Neurosurgery: 410-955-6406 Pediatric Neurosurgery: 410-955-7337. It usually occurs in children under the age of 10 (more rarely in adolescents and adults), and is characterized by frequent and severe seizures, loss of … Of levetiracetam and fosphenytoin, the histopathology of RE exemplify the diagnostic to! 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