Rasmussen encephalitis

Definition:

Rasmussen encephalitis is a rare chronic neurological disorder, characterised by unilateral inflammation of the cerebral cortex, drug-resistant epilepsy, and progressive neurological and cognitive deterioration. It predominantly affects children, with the onset in adults having a milder course. Infection occurring around two weeks prior to onset is observed in 38% of the patients. There is evidence of autoimmune involvement in the pathogenesis. The immunopathology of this disease is attributed mainly to activated cytotoxic T cells. It has been reported that genetic variations of immunoregulatory genes associated with Rasmussen syndrome.

References:

[1]. C M Quesada, et al. Rasmussen encephalitis with ipsilateral brain stem involvement in an adult patient. BMJ Case Rep. 2009;2009:bcr08.2008.0648. [Content Brief]

[2]. Christian G Bien, et al. Treatment of Rasmussen encephalitis half a century after its initial description: promising prospects and a dilemma. Epilepsy Res. 2009 Oct;86(2-3):101-12. [Content Brief]

[3]. Renato Mantegazza, et al. 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. J Neuroimmunol. 2002 Oct;131(1-2):179-85. [Content Brief]

[4]. Sophia Varadkar, et al. Rasmussen's encephalitis: clinical features, pathobiology, and treatment advances. Lancet Neurol. 2014 Feb;13(2):195-205. [Content Brief]

[5]. Yukitoshi Takahashi, et al. Genetic variations of immunoregulatory genes associated with Rasmussen syndrome. Epilepsy Res. 2013 Dec;107(3):238-43. [Content Brief]

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