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2014, Number 1

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Rev Med MD 2014; 5.6 (1)

Rasmussen Syndrome: autoimmune encephalitis of difficult control where surgical treatment is the last alternative

Ornelas-Casillas MA, López-Buenrostro SA
Full text How to cite this article

Language: Spanish
References: 6
Page: 84-87
PDF size: 659.54 Kb.


Key words:

Autoimmune encephalitis, hemispherectomy, Rasmussen syndrome.

ABSTRACT

Rasmussen syndrome is an autoimmune encephalitis characterized by continuous partial seizures of difficult control and aphasia. Evidence shows that the autoimmune component of the disease relies on the existence of auto antibodies against subunit 3 of the glutamate receptor (Glu3). There is also a causal association with viral infections; most of them are part of the herpes virus family, like citomegalovirus (CMV) and Epstein-Barr Virus (EBV).
Due to resistance to antiepileptic drugs, management with immunoglobulin shows slower progression of the disease and clinical improvement in most patients. Despite the benefits shown with immunoglobulin, the only therapeutic strategy that has shown better results is hemispherectomy.
We present the case of a 2-year-old girl with seizures after suffering traumatic brain injury. She experienced tonicclonic seizures on the right side of the body with poor response to anti-seizure medications. Magnetic resonance imaging and electroencephalography revealed characteristics that suggested this disease. The patient underwent right frontotemporal lobectomy and biopsies confirmed the Rasmussen Syndrome diagnosis.


REFERENCES

  1. Pryson R & Frater J. Rasmussen encephalitis, a clinicopathologic and immunohistochemical study of seven patients. Am J Clin Pathol 2002; 117: 776- 782.

  2. Bian CG & Schramm J. Treatment of Rasmussen encephalitis half a century after its initial description: Promising prospects and a dilemma. Epilepsy Res. 2009; 86(2-3): 101-12.

  3. Bian CG, Widman G, Urbach H, et al. The natural history of Rasmussen's encephalitis. Guarantors of Brain 2002; 125: 1751-1759.

  4. Bian CG, Granata T, Antozzi T, et al. Pathogenesis, diagnosis and treatment of Rasmussen encephalitis. A European consensus statement. Guarantors of Brain 2005; 128: 454-471.

  5. Aguilar F, Ruvalcaba C, Rayo D, y Rojas JC. Síndrome de Rasmussen. Seguimiento de siete años. Aspectos relacionados con plasticidad cerebral en epilepsia. Revista de Investigación Clínica 2002; 54(3): 209-217.

  6. Caraballo R, Tenembaum S, Cersósimo R, et al. Síndrome de Rasmussen. Revista de Neurología 1998; 26(154): 978-983.




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Rev Med MD. 2014;5.6