SHORT REPORT

Favourable outcome of progressive multifocal leucoencephalopathy in two patients with dermatomyositis

S Vulliemoz¹, F Lurati-Ruiz², F-X Borruat³, J Delavelle⁴, I J Koralnik⁵, T Kuntzer⁶, J Bogousslavsky⁶, F Picard¹, T Landis¹, R A Du Pasquier⁶

¹ Service de Neurologie, Hôpitaux Universitaires de Genève, Geneva, Switzerland
² Service d’ Immunologie et allergologie, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland
³ Unité de Neuro-ophtalmologie, Centre Hospitalier Universitaire Vaudois, Lausanne
⁴ Unité de Neuro-radiologie, Hôpitaux Universitaires de Genève
⁵ HIV/Neurology Center, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA
⁶ Service de Neurologie, Centre Hospitalier Universitaire Vaudois

Correspondence to:
Correspondence to:
R A Du Pasquier
Service de Neurologie and Service d’Immunologie, Centre Hospitalier Universitaire Vaudois, BT-02 1011 Lausanne, Switzerland; renaud.Du-Pasquier{at}chuv.ch

Progressive multifocal leucoencephalopathy (PML), a demyelinating disease caused by the JC virus (JCV), occurs in immunosuppressed patients and carries a poor prognosis. A favourable outcome is reported in two patients with PML and dermatomyositis. Immunosuppressive drugs were stopped in patient 1 but could only be partially tapered in patient 2. The JCV-specific CD8+ T cell response was strong in patient 1 and weak in patient 2. Both were treated with cytosine-arabinoside, and patient 2 was also treated with mirtazapine, a 5HT_2A receptor antagonist. Combination of these drugs might be helpful to treat HIV-negative patients with PML.

Abbreviations: Ara-C, cytosine-arabinoside; JCV, JC virus; MRI, magnetic resonance imaging; PCR, polymerase chain reaction; PML, progressive multifocal leucoencephalopathy

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