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Clinical Pathologic Conference |

A Young Woman With Blurred Vision and Distal Paresthesias

Nathan H. Kung, MD1; Nancy L. Bartlett, MD2; Nabeel R. Yaseen, MD3; Gregory P. Van Stavern, MD1,4; Robert C. Bucelli, MD, PhD1
[+] Author Affiliations
1Department of Neurology, Washington University in St Louis, St Louis, Missouri
2Division of Oncology, Department of Medicine, Washington University in St Louis, St Louis, Missouri
3Department of Pathology and Immunology, Washington University in St Louis, St Louis, Missouri
4Department of Ophthalmology, Washington University in St Louis, St Louis, Missouri
JAMA Neurol. 2015;72(12):1519-1523. doi:10.1001/jamaneurol.2015.2390.
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A 29-year-old woman presented with blurred vision and distal paresthesias. Her initial evaluation revealed severe bilateral optic disc edema with distal lower-extremity sensory and motor deficits and electrodiagnostic evidence of a length-dependent mixed demyelinating and axonal polyneuropathy. The results of routine diagnostic testing, including laboratory tests, magnetic resonance imaging, and lumbar puncture, were nondiagnostic. A targeted biopsy was ultimately required for diagnosis. In this article, we discuss the differential diagnosis and outline the clinical evaluation indicated for a patient presenting with demyelinating polyneuropathy and concurrent papilledema.

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Figure 1.
Clinical Images

Top, Magnetic resonance images of the brain showing a partially empty sella, stenosis without thrombosis of the transverse venous sinuses, flattening of the posterior globes, and no structural lesions. Bottom, Humphrey SITA Standard 24-2 visual fields of the left eye and right eye. Fundus photographs showing moderate to severe papilledema in the right eye and left eye. Peripapillary hemorrhages can be seen in inferotemporal portions of both optic discs.

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Figure 2.
Pathologic Specimens

A, Coronal computed tomographic (CT) image of the spine revealing sclerotic lesions at T5 and T9. B, Axial CT image highlighting the sclerotic lesion at T5. C, Bone marrow specimen from T5 vertebral body revealing an abnormally increased number of plasma cells (arrowheads) (original magnification ×600). D-E, In situ hybridization of κ and λ light chains revealing an abnormal 1:2 ratio of positivity (original magnification ×100). F, Axillary lymph node specimen with Castleman disease–like changes with an involuted germinal center (lower left of image), increased interfollicular distance, and increased vascular proliferation (original magnification ×200).

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The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
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