1. Ideomotor Apraxia and “Milky Way” Sign in Progressive Multifocal Leukoencephalopathy
- Author
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Eisinger, Robert S., Jones, Felipe J., Pai, Emily Ling-Lin, Xu, Denise J., Berger, Joseph R., and Prasad, Sashank
- Abstract
A 70-year-old woman presented with progressive right arm clumsiness for 1 month (e.g., trouble baking and playing guitar) without weakness or numbness. She had hepatitis C cirrhosis, localized hepatocellular carcinoma treated with radioembolization, and thrombotic thrombocytopenic purpura treated with rituximab 1 year earlier. Neurologic examination revealed ideomotor apraxia (Video 1),1mild right astereognosis, and agraphesthesia. MRI brain showed a left frontoparietal T2/FLAIR-hyperintense lesion with punctate T2 hyperintensities known as the “Milky Way” sign (Figure, A–D),2a finding described in progressive multifocal leukoencephalopathy (PML). There was faint T1-contrast enhancement indicating immune reconstitution (IRIS). The CD4 count was 241. HIV was negative. A comprehensive investigation of secondary causes of lymphopenia was unrevealing. CSF studies were normal, including negative JC virus PCR. Brain biopsy confirmed the diagnosis of PML (Figure, E and F). The patient continued to decline clinically and radiographically because of PML-IRIS and declined immunotherapy. This vignette highlights the clinical presentation of ideomotor apraxia, the “Milky Way” radiographic sign, the possibility of JC virus PCR being negative in early PML and the hallmark pathologic findings of PML.
- Published
- 2024
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