Atypical Progressive Multifocal Leukoencephalopathy in a Patient with Antisynthetase Syndrome

Moncef Belhassen-Garcia; Alberto Rábano-Gutiérrez; Virginia Velasco-Tirado; Angela Romero-Alegria; Maria-Luisa Pérez-Garcia; Jose Angel Martin-Oterino

doi:10.2169/internalmedicine.54.2748

CASE REPORTS

Atypical Progressive Multifocal Leukoencephalopathy in a Patient with Antisynthetase Syndrome

Moncef Belhassen-Garcia, Alberto Rábano-Gutiérrez, Virginia Velasco-Tirado, Angela Romero-Alegria, Maria-Luisa Pérez-Garcia, Jose Angel Martin-Oterino

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Keywords: Anti-Jo-1, antisynthetase syndrome, rituximab, progressive multifocal leukoencephalopathy, JC virus

JOURNAL OPEN ACCESS

2015 Volume 54 Issue 5 Pages 519-524

DOI https://doi.org/10.2169/internalmedicine.54.2748

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Abstract

Antisynthetase syndrome is a disorder belonging to the dermatomyositis/polymyositis group, with high rates of morbidity and mortality. We herein present the case of a 71-year-old man who was diagnosed with antisynthetase syndrome and treated with rituximab. Almost three years later, the patient showed right-sided hemiparesis that ultimately progressed to complete hemiplegia and advancing cognitive deterioration with a poor clinical outcome. The neuropathological diagnosis was progressive multifocal leukoencephalopathy. Treatment with rituximab for antisynthetase syndrome itself plays a fundamental role in the development of infectious complications.

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