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Paraneoplastic Subacute Sensory Neuronopathy Secondary to a Malignant Mixed Müllerian Tumor

From the Departments of ¹Neurobiology, ²Pathology, and ³Neurology and Neurosurgery, Yale University School of Medicine, New Haven, Connecticut.

BACKGROUND: Paraneoplastic subacute sensory neuronopathy is a rapidly progressive autoimmune disorder commonly associated with small cell cancers. Relentless destruction of dorsal root ganglion cells by cytotoxic T cells leads to a poor prognosis.

CASE: A 42-year-old woman developed sensory loss in both lower extremities 10 days after debulking of a uterine malignant mixed müllerian tumor. She progressed to sensory loss over the entire body, with initially preserved strength, severe dysmetria, and truncal ataxia. Her serum was positive for antineuronal nuclear antibody–1 (anti-Hu), confirming the diagnosis of paraneoplastic subacute sensory neuronopathy. Despite treatment with intravenous immunoglobulin, methylprednisolone, and plasmapheresis, she remained severely disabled.

CONCLUSION: Because neuronal damage is irreversible, early recognition may be the only means to prevent severe neurologic disability.