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Academia Mexicana de Neurología, A.C.

2014, Number 2

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Rev Mex Neuroci 2014; 15 (2)

Rasmussen encephalitis: the complexity of management of a potentially drug-resistant epilepsy illustrated by two cases

Carmona-Vázquez CR, Peña-Landín DM, Venzor-Castellanos JP, Pasquel GVPM, Dávila-Gutiérrez G

Language: Spanish
References: 30
Page: 119-124
PDF size: 234.57 Kb.


ABSTRACT

Introduction: Rasmussen Encephalitis (RE) is a chronic focal and progressive disease in childhood associated to mental impairment and hemiparesis. Hemispherectomy has played a major role in seizure treatment since 1950, however, the pathophysiology with immune-mediated basis, has allowed to try different treatment options including intravenous immunoglobulin, steroids and immunosuppressive agents.
Case Reports: We describe two patients with history of continuous partial epilepsy and hemiparesia with seizures refractory to multiple antiepileptic drugs. The brain magnetic resonance image showed hemiatrophy in both cases. We compare the clinical and imaging features as reported so far in literature.
Conclusion: Our cases illustrate that the ER is a chronic progressive disease with poor prognosis. Nowadays the treatment is aggressive, which may involve significant sequelae with limited clinical efficacy.


REFERENCES

  1. Varadkar S, Bien CG, Kruse CA, Jensen FE, Bauer J, Pardo CA. Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances. Lancet Neurol 2014; 13: 195-205.

  2. Stahnisch FW, Nakashima AS. Theodore Brown Rasmussen (1910- 2002). J Neurol 2013; 260: 2694-6.

  3. Ramaswamy V, Walsh J, Sinclair D, Johnson E, Tang-Wai R, Wheatley B. Inflamma some induction in Rasmussen’s encephalitis: cortical and associated white matter pathogenesis. J Neuroinflammation 2013; 109: 152-60.

  4. Mirones I. Role for the CXCR3/CXCL10 Axis in Rasmussen Encephalitis. Pediatr Neurol 2013; 49: 451-7.

  5. Papetti. Immunotherapy in Rasmussen’s encephalitis: when should it be taken into account? Case Reports of Rasmussen’s Syndrome and Literature Review. J Neuropsychiatry Clin Neurosci 2012; 24: 367-71.

  6. Owens G, Huynh M, Chang J, McArthur D, Hickey M, Vinters H. Differential expression of interferon- and chemokine genes distinguishes Rasmussen encephalitis from cortical dysplasia and provides evidence for an early Th1 immune response. J Neuroinflammation 2013; 10: 56-66.

  7. Takahashi Y, Yamazaki E, Mine J, Kubota Y, Imai K, Mogami Y. Immunomodulatory therapy versus surgery for Rasmussen syndrome in early childhood. Brain Dev 2013; 35: 778-85.

  8. Fejerman N, Medina CS, Caraballo RN. Epilepsia. Neurología Pediátrica. Buenos Aires: Médica Panamericana SA; 2010, p. 591-642.

  9. Swaiman KF, Ashwal S, Ferreiro DM. Focal and Multifocal Seizures. Pediatric Neurology; Principles and Practice. Philadelphia: Elsevier; 2012, p. 751-66.

  10. Panayiotopoulos C. Severe neocortical epileptic syndromes in infancy and childhood. A Clinical Guide to Epileptic Syndromes and their Treatment. British: Springer Healthcare Ltd; 2010, p. 327-31.

  11. Pellock J, Bourgeois B, Dodson W, Arthur L. Prensky and Amir Pshytycky, Prakash Kotagal. Localization-Related Epilepsies: Simple Partial Seizures, Complex Partial Seizures, and Rasmussen Syndrome In: Pediatric Epilepsy Diagnosis and Therapy. New York: Demos; 2008, p. 377-86.

  12. Bittner S, Simon O J, Göbel K, Bien CG, Meuth SG, Wiendl H. Rasmussen encephalitis treated with natalizumab. Neurology 2013; 81: 395-7.

  13. Bien CG, Tiemeier H, Sassen R, Kuczaty S, Urbach H, Von Lehe M. Rasmussen encephalitis: Incidence and course under randomized therapy with tacrolimus or intravenous immunoglobulins. Epilepsia 2013; 54: 543-50.

  14. Rassner MP, Van Velthoven-Wurster V, Ramantani G, Feuerstein TJ. Altered transporter-mediated neocortical GABA release in Rasmussen encephalitis. Epilepsia 2013; 54: e41-4.

  15. Papetti L, Nicita F, Granata T, Guerrini R, Ursitti F, Properzi E. Early add-on immunoglobulin administration in Rasmussen encephalitis: The hypothesis of neuroimmunomodulation. Can J Neurol Sci 2011; 77: 917-20.

  16. Feasby T, Banwell B, Benstead T, Bril V, Brouwers M, Freedman M. Guidelines on the use of intravenous immune globulin for neurologic conditions. Transfus Med Rev 2007; 21: S57-S107.

  17. Wirenfeldt M, Clare R, Tung S, Bottini A, Mathern GW, Vinters HV. Increased activation of Iba1(+) microglia in pediatric epilepsy patients with Rasmussen’s encephalitis compared with cortical dysplasia and tuberous sclerosis complex. Neurobiol Dis 2009; 34: 432-40.

  18. Granata T, Fusco L, Gobbi G, Freri E, Ragona F, Broggi G. Experience with immunomodulatory treatments in Rasmussen’s encephalitis. Neurology 2003; 61: 1807-10.

  19. Andrews PI, Dichter MA, Berkovic SF, Newton MR, McNamara JO. Plasmapheresis in Rasmussen’s encephalitis. Neurology 2001; 57: S37-S41.

  20. Bien CG, Granata T, Antozzi C, Cross JH, Dulac O, Kurthen M. Pathogenesis, diagnosis and treatment of Rasmussen encephalitis - A European consensus statement. Brain 2005; 128: 454-71.

  21. Olson H, Lechphammer M, Prabhu S, Ciarlini P, Poduri A, Gooty V. et al. Clinical application and evaluation of the Bien diagnostic criteria for Rasmussen encephalitis. Epilepsia 2013; 54: 1753-60.

  22. Kravljanac R, Djuric M, Jovic N, Djordjevic M, Zamurovic D, Pekmezovic T. Etiology, clinical features and outcome of epilepsia partialis continua in cohort of 51 children. Epilepsy Res 2012; 104: 112-7.

  23. Bien CG, Schramm J. Treatment of Rasmussen encephalitis half a century after its initial description: Promising prospects and a dilemma. Epilepsy Res 2009; 86: 101-12.

  24. Thilo B, Stingele R, Knudsen K, Boor R, Bien CG, Deuschl G. A case of Rasmussen encephalitis treated with rituximab. Nat Rev Neurol. 2009; 5: 458-62.

  25. Alvarez BE, Bien CG, Schramm J, Elger CE, Becker AJ, Schoch S. Autoantibodies to Munc18, cerebral plasma cells and B-lymphocytes in Rasmussen encephalitis. Epilepsy Res 2008; 80: 93-7.

  26. Osorio I, Shnyra A, SantaCruz K, Brewington R, Morrison D. Rasmussen’s encephalitis: Interleukin-10-dependent Tc2 cell polarization may explain its pathophysiology and clinical course. Epilepsy Behav 2007; 1: 206-11.

  27. Freeman JM. Rasmussen’s syndrome: Progressive autoimmune multifocal encephalopathy Pediatr Neurol 2005; 32: 295-9.

  28. Bahi BN, Nabbout R, Plouin P, Bulteau C, Delalande O, Pannier LH. Recent advances in pathogenic concepts and therapeutic strategies in Rasmussen’s encephalitis. Rev Neurol (Paris) 2005; 161: 395-405.

  29. Granata T, Fusco L, Gobbi G, Freri E, Ragona F, Broggi G. Experience with immunomodulatory treatments in Rasmussen’s encephalitis. Neurology 2003; 61: 1807-10.

  30. Cavazzuti GB. Infantile encephalopathies. Neurol Sci 2003; 24: S244-S5.




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