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Pediatric emergency care · Dec 2020
Case ReportsA Case Report of Pediatric Paraneoplastic Dysautonomia.
- Katherine Harer, Jennifer Mann, Nicole Muhlbauer, Jennifer Welch, and Brian Alverson.
- From the Department of Pediatrics, Hasbro Children's Hospital, Providence, RI.
- Pediatr Emerg Care. 2020 Dec 1; 36 (12): e742-e744.
AbstractWe present the case of a 16-year-old girl who presented with severe refractory orthostatic hypotension secondary to pandysautonomia. Initially, she was treated for Guillain-Barré syndrome given clinical symptoms and increased protein on cerebrospinal fluid, but the severity of symptoms and lack of response to intravenous immunoglobulin prompted further evaluation for an autoimmune etiology. She was ultimately diagnosed with paraneoplastic neuropathy secondary to Hodgkin lymphoma. Paraneoplastic neurologic phenomena are rare, occurring in just 0.01% of cancers, and prompt recognition is crucial for initiating appropriate therapy. Rapid progression of severe disabling symptoms should raise suspicion for an underlying malignancy. The patient had limited response to splanchnic vasoconstrictors in addition to α-agonists, anticholinergics, and mineralocorticoids until initiation of modified Hodgkin lymphoma directed chemotherapy plus rituximab.
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