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Emma C. Tallantyre, BMBS; Simon M. L. Paine, BMBS; Colin P. Sharp, PhD; James S. Lowe, DM; Bruno Gran, MD, PhD

Arch Neurol. 2009;66(8):1021-1024.

Objective  To report the clinical and radiologic features in a patient with myelofibrosis who developed atypical progressive multifocal leukoencephalopathy.

Design  Case report.

Setting  Tertiary referral center.

Patient  A 72-year-old man with myelofibrosis and mild leukopenia experienced progressive limb weakness and dysarthria.

Results  Imaging revealed almost complete sparing of the white matter with isolated involvement of the brainstem and deep gray matter. Postmortem examination led to definitive diagnosis of progressive multifocal leukoencephalopathy and demonstrated an unusual miliary pattern of disease rather than the typical confluent involvement. Genetic analysis revealed a mutation in the transcription control region of the JC polyomavirus, prompting speculation about the pathogenesis of progressive multifocal leukoencephalopathy.

Conclusions  Leukopenia may render patients effectively immunosuppressed. The differential diagnosis should include progressive multifocal leukoencephalopathy even in patients with atypical clinical and radiologic features.

Author Affiliations: Division of Clinical Neurology (Drs Tallantyre and Gran) and Department of Neuropathology (Drs Paine and Lowe), University of Nottingham, Nottingham, England; and Centre for Infectious Diseases, University of Edinburgh, Edinburgh, Scotland (Dr Sharp).

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