Abstract
A 68-year-old man presented with a two-week history of ascending, symmetric, sensory neuropathy concerning an acute inflammatory demyelinating polyneuropathy that briefly responded to intravenous immunoglobulin (IVIg) therapy. The initial workup was negative for acquired causes. After three months of poor response to standard therapies, he was hospitalized for severe disability, unintentional weight loss, and additional, unexplained neurologic symptoms including cerebellar ataxia, dysarthria, and muscle twitching. Positron emission tomography revealed hypermetabolism isolated to the bone marrow. Bone marrow biopsy confirmed the diagnosis of diffuse large B-cell lymphoma (DLBCL). Due to rapidly worsening performance status, plasmapheresis was initiated prior to treatment with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) chemotherapy. His symptoms initially improved following plasmapheresis and resolved with chemotherapy. One year following treatment, he remains in complete remission. This case describes a unique paraneoplastic neurologic syndrome involving the central and peripheral nervous system that responded well to plasmapheresis and systemic chemotherapy.
Highlights
Paraneoplastic neurologic syndromes (PNS) are a heterogeneous group of immune-mediated, neurologic disorders associated with malignancy
A 68-year-old man presented with a two-week history of ascending, symmetric, sensory neuropathy concerning an acute inflammatory demyelinating polyneuropathy that briefly responded to intravenous immunoglobulin (IVIg) therapy
Bone marrow biopsy confirmed the diagnosis of diffuse large B-cell lymphoma (DLBCL)
Summary
Paraneoplastic neurologic syndromes (PNS) are a heterogeneous group of immune-mediated, neurologic disorders associated with malignancy. He first sought medical attention for progressive, ascending numbness and paresthesia of his lower extremities and hands At that time, he met diagnostic criteria for acute inflammatory demyelinating polyneuropathy including characteristic electrodiagnostic and cerebrospinal fluid (CSF) analysis findings. Due to the disease being primarily detected in the bone marrow, a biopsy was repeated at three and 12 months with low-normocellular marrow, and without evidence of lymphoma, dyspoiesis, or other clonal expansion He has not been hospitalized since completing chemotherapy. He has worked hard to regain strength, initially with physical therapy, followed by a daily weight-lifting and bicycling regimen at home While he believes he has not fully regained his level of physical fitness from prior to therapy, he is highly active, and his only residual symptom is low-grade peripheral neuropathy of his bilateral feet. We plan to follow the patient with repeat PET/CT imaging and bone marrow biopsy at two years, after which point he will be followed by labs and exams in conjunction with his primary care physician
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