POEMS SYNDROME WITH CASTLEMAN DISEASE - AN UNUSUAL PRESENTATION

Chronic Inflammatory Demyelinating Polyneuropathy(CIDP) is an acquired immune mediated disorder presenting assymmetric proximal and distal sensory motor polyneuropathy. It is treatable with immunosuppressant. CIDP may beassociated with HIV-1 infection, SLE, para neoplastic disorders and Paraproteinemias. Paraproteinemias is abnormalmonoclonal antibody or light chain in blood secondary to plasma cell disorder like multiple myeloma, Waldenstrommacroglobulinemia, monoclonal gammopathy of undetermined significance (MUGS), POEMS (Polyneuropathy,Organomegaly, Endocrinopathy, monoclonal plasma cell proliferative disorder and Skin Changes) syndrome andCastleman disease with or without POEMS syndrome. These account for approximately 10% of CIDP. DiagnosingParaproteinemic neuropathy is necessary to prevent delay in diagnosis, start appropriate treatment and estimateprognosis of the condition.POEMS syndrome is due to clonal proliferation of plasma cells. Castleman disease (CD), also known as angiofollicular orgiant lymph node hyperplasia, is a rare non-neoplastic lymphoproliferative disorder .We present a Case report of relatively young male with right parotid swelling with chronic sensorimotor quadriparesiswith wasting, with no predominant neuropathic pain and cranial nerve involvement. On Nerve Conduction Studies it wasfound to have severe sensori-motor neuropathy which was not associated with pain, an unlikely feature of either CIDP orCastleman disease; on further evaluation was found to have POEMS syndrome. Thus high index of suspicion is necessaryto diagnose this condition.This case report emphasizes the fact that patients with Castleman disease-POEMS Syndrome may present at youngerage than usual with short duration of illness, with severe sensorimotor neuropathy which may not be associated withpain. High index of suspicion and appropriate investigations may lead to early diagnosis and prompt treatment.