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Clinical Pathologic Conference |

Rapid Multifocal Neurologic Decline in an Immunocompromised Patient

Julie Anne Kromm, MD, FRCPC1,2; Christopher Power, MD, FRCPC1; Gregg Blevins, MD1; Loree Larratt, MD3; F. K. H. van Landeghem, MD4; Jeremy Rempel, MD5
[+] Author Affiliations
1Division of Neurology, Department of Medicine, University of Alberta, Edmonton, Alberta, Canada
2Department of Critical Care Medicine, University of Calgary, Calgary, Alberta, Canada
3Division of Hematology, Department of Medicine, University of Alberta, Edmonton, Alberta, Canada
4Neuropathology Specialty Group, Department of Laboratory Medicine and Pathology, University of Alberta, Edmonton, Alberta, Canada
5Department of Radiology and Diagnostic Imaging, University of Alberta, Edmonton, Alberta, Canada
JAMA Neurol. 2016;73(2):226-231. doi:10.1001/jamaneurol.2015.2658.
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A man in his early 70s with a diagnosis of chronic lymphocytic leukemia and being treated with prednisone, fludarabine, cyclophosphamide, and rituximab presented with progressive multifocal neurologic decline. The patient died 2 months after the onset of this decline despite extensive clinical and laboratory investigation and a trial of methylprednisolone therapy. The approach to the immunosuppressed patient with progressive neurologic decline, neuropathologic findings, and final diagnosis are discussed.

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Figure 1.
Magnetic Resonance Imaging

Magnetic resonance image at 5 weeks after the onset of neurologic symptoms in the patient described in this report. Hyperintensities (arrowheads) on fluid-attenuated inversion recovery involving the left cerebellar hemisphere (A), bilateral pons (B), bilateral midbrain peduncles (C), and extending up into the thalami bilaterally (D).

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Figure 2.
Pathologic Characteristics

A, Luxol fast blue-periodic acid–Schiff and hematoxylin-eosin staining of the central pons showing bilaterally symmetric areas of demyelination (original magnification ×10). B, Optic nerve tissue immunostained for myelin basic protein, showing reduced immunoreactivity and disruption of myelin structure (arrowheads) (original magnification ×40). C, CD68-immunoreactive macrophages infiltrating the optic nerve (original magnification ×20). D, High-power magnification showing macrophages with phagocytosed myelin (arrowheads). E, Neurofilament immunostaining of the pons showing myelin phagocytosis by macrophages (arrowhead) (original magnification ×40). F, Neurofilament immunostaining of the optic nerve showing occasional axonal retraction bulbs (arrowhead) (original magnification ×40).

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