A case of severe chronic inflammatory demyelinating polyradiculoneuropathy with monoclonal gammopathy of undetermined significance with alternating immunoglobulin class to IgM from IgA

Abstract

MP, melphalan 5 mg + prednisolone 32 mg; mPSL, methylprednisolone pulse therapy (1 g/day × 3 days); PSL, prednisolone; DEX, dexamethasone;TLS, totally locked-in syndrome; MV, mechanical ventilation; BPf, blood pressure fluctuations; MMT, manual muscle testing; UE, upper extremities; LE, lower extremities. MMT (UE) indicates average bilateral MMT scores of the biceps brachii, triceps brachii, brachioradialis, wrist extensor, and wrist flexor muscles. MMT (LE) indicates average bilateral MMT scores of the quadriceps, iliopsoas, hamstrings, tibialis anterior, and gastrocnemius muscles. Nov: November. *: The unit of the serum IgG is mg/dl.

Chronic Inflammatory Demyelinating Polyradiculoneuropathy in Association With Concomitant Diseases: Identification and Management

“…Patients with CIDP-MGUS were found to have a more dangerous onset and less severe but longer course of disease and relapse more frequently. Hayashi et al (77) reported a 71-year-old female patient with CIDP and IgA-l MGUS in 2016 who developed quadriplegia, respiratory failure, and rapid fluctuations in blood pressure. After a large dose of corticosteroids and IVIG treatment, the patient's symptoms improved, but quickly relapsed, and showed immunoglobulin IgA-l and IgM-l double positive in the serum.…”