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Rapidly Progressive Weakness in MGUS-Associated Chronic Inflammatory Demyelinating Polyneuropathy: A Rare Presentation and Review

Published on: 20th July, 2026

Monoclonal gammopathy of undetermined significance (MGUS) may be associated with peripheral neuropathy; however, IgG MGUS–associated chronic inflammatory demyelinating polyneuropathy (CIDP) is uncommon and generally follows a slowly progressive or relapsing course. We report a man in his early 40s who developed rapidly progressive sensorimotor weakness resulting in quadriparesis and inability to ambulate within eight weeks. Examination demonstrated severe proximal and distal weakness, glove-and-stocking sensory loss, and absent reflexes. Cerebrospinal fluid protein was markedly elevated, and nerve conduction studies/electromyography demonstrated acquired demyelination with secondary axonal loss. Evaluation identified IgG MGUS, supporting the diagnosis of MGUS-associated CIDP after alternative etiologies were excluded. Intravenous immunoglobulin was discontinued on day 3 following the development of a pulmonary embolism. The patient subsequently completed eight plasma-exchange sessions, with improvement in lower-extremity strength to 3/5, upper-extremity strength to 4/5, and sensory symptoms. He was discharged to rehabilitation with planned monthly plasma exchange.recognised clinical spectrum of IgG MGUS–associated CIDP and demonstrates that meaningful neurological improvement may occur with plasma exchange despite rapid progression and severe initial disability.
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