Sequential MRI, Cerebrospinal Fluid and Peripheral Blood Immunologic Analysis in a Natalizumab-Treated Patient With Progressive Multifocal Leukoencephalopathy

Abstract

We report a natalizumab (NTZ)-treated relapsing-remitting multiple sclerosis (RRMS) patient evaluated for worsening of pyramidal and cerebellar dysfunction. MRI showed Gd⁺ lesions in juxtacortical frontal areas and middle cerebellar peduncles indicative of progressive multifocal leukoencephalopathy (PML). Cerebrospinal fluid (CSF) showed 47 JC virus (JCV) copies/mL, high CD8⁺ and plasmacytoid dendritic cells (pDCs) compared to peripheral blood (PB). MRI after NTZ discontinuation and plasma exchange revealed PML aggravation. CSF JCV increased to 475 as CD4⁺, CD19⁺ and pDCs. CSF 8 months later showed high CD4⁺, CD19⁺, pDCS, not JCV. MRI showed severe focal cortical atrophy and axonal degeneration of pontocerebellar fibers. Specific immune-cell response in the central nervous system (CNS) may be essential for control of PML.




J Med Cases. 2015;6(4):163-166
doi: http://dx.doi.org/10.14740/jmc2079w