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Progressive Leg Pain and Weakness

Digvijaya D. Navalkele, MD; Maria-Magdalena Georgescu, MD; Dennis K. Burns, MD; Tasha Greenberg, MD; Steven Vernino, MD, PhD
JAMA Neurol. 2013;70(4):510-514. doi:10.1001/jamaneurol.2013.2158.
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A 54-year-old man presented with progressive asymmetric leg pain and weakness. He had a history of invasive squamous cell carcinoma that was fully treated 2 years earlier. His leg symptoms progressed relentlessly during several months. Imaging studies demonstrated enhancement of the cauda equina and leptomeninges of the lower spinal cord. Initial cerebrospinal fluid examination showed an elevated protein concentration and lymphocytic pleocytosis with no malignant cells on cytological analysis. There was short-term improvement in symptoms and cerebrospinal fluid abnormalities with intravenous steroids. Two additional cerebrospinal fluid studies showed normal cytological findings, elevated IgG synthesis, and elevated antibody titers to varicella-zoster virus. Over time, the patient worsened, developed cranial neuropathies, and ultimately died. The pathological diagnosis and the approach to the clinical data are discussed.

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Figure 1. Neuroimaging findings. A, T1-weighted sagittal postgadolinium magnetic resonance image of the spine shows enhancement of the lumbosacral roots and the superficial aspect of the lower spinal cord. B, T1-weighted axial postgadolinium magnetic resonance image of the lumbosacral spine shows enlargement and enhancement of the cauda equina roots.

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Grahic Jump Location

Figure 2. Neuropathological findings from the spinal cord and nerves show massive infiltration of the spinal nerves and meninges by neoplastic squamous cells. A-C, Cross-section through a thoracic spinal nerve root shows numerous invading neoplastic cells of squamous origin (cytokeratin 5/6, original magnification ×20 [A]; hematoxylin-eosin, original magnification ×500 [B]; and cytokeratin 5/6, original magnification ×200 [C]). D, Meningeal infiltration by neoplastic cells is seen at the same level as in A (hematoxylin-eosin, original magnification ×200). E, Longitudinal section through a cervical spinal nerve shows neoplastic cells with purple-stained nuclei lining the axons as well as severe myelin destruction evidenced by the fragmented blue stain (Luxol fast blue, periodic acid–Schiff, and hematoxylin-eosin, original magnification ×200). F, Cervical spinal cord section shows marked demyelination of the posterior columns (Luxol fast blue, periodic acid–Schiff, and hematoxylin-eosin, original magnification ×20). G, Enlargement of the area from F shows central chromatolysis of a motor neuron from the anterior horn (arrow) (Luxol fast blue, periodic acid–Schiff, and hematoxylin-eosin, original magnification ×200).

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