Abstract
IntroductionPolyneuropathy is a key feature of polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome, which is a paraneoplastic manifestation of an underlying lymphoproliferative neoplasm. We report the first case of polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome presenting with a pseudosensory level.Case presentationA 59-year-old Tamil woman with long-standing diabetes mellitus and hypertension developed painless, progressive inguinal lymphadenopathy. A contrast-enhanced computed tomography scan showed mild hepatomegaly and intra-abdominal lymphadenopathy. A histological examination of an enlarged inguinal lymph node showed features of a plasma cell-type Castleman disease. She was treated with rituximab. Six months later, she developed gradually ascending numbness and weakness of both lower limbs. On examination, she had flaccid paraparesis (power 3/5) with a sensory level to pinprick at thoracic level 9. Joint position sense was preserved. Her cranial nerves and upper limbs were neurologically normal. Nerve conduction studies confirmed peripheral neuropathy with conduction slowing and a magnetic resonance imaging of her spine did not show cord or root compression. Serum protein electrophoresis showed a monoclonal band. A bone marrow biopsy showed a hypercellular marrow with 30% plasma cells. A repeat contrast-enhanced computed tomography scan showed sclerotic bony lesions involving multiple vertebrae in addition to mild hepatomegaly and intra-abdominal lymphadenopathy. Polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome was diagnosed and she was treated with intravenously administered pulse therapy of dexamethasone and cyclophosphamide. After three cycles of treatment, she regained normal muscle power and sensation.ConclusionsPolyneuropathy in polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome can present as a pseudosensory level.
Highlights
Polyneuropathy is a key feature of polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome, which is a paraneoplastic manifestation of an underlying lymphoproliferative neoplasm
Polyneuropathy in polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome can present as a pseudosensory level
Castleman disease occurs in 11–30% of patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes (POEMS) syndrome which is considered to be a paraneoplastic manifestation of a lymphoproliferative disorder [2, 3]
Summary
Castleman disease is a lymphoproliferative disorder driven by proinflammatory cytokines [1]. A contrast-enhanced computed tomography (CT) scan of her chest, abdomen, and pelvis showed mild hepatomegaly and intra-abdominal lymphadenopathy in addition to inguinal lymphadenopathy. An excisional biopsy of the enlarged lymph node showed evidence of Castleman disease of the plasma cell type with CD3 and Bcl-2 stained reactive pattern of follicles. She was treated with intravenously administered rituximab 375 mg/m2 weekly for 4 weeks. There was reduction in the size of her inguinal lymph nodes Six months later, she developed gradually ascending numbness and weakness of her lower limbs. Following three cycles of treatment, she demonstrated a remarkable improvement in her neurological deficits with recovery of muscle power and sensation to near normal
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