Repeated Acute Exacerbations of Chronic Inflammatory Demyelinating Polyradiculoneuropathy Accompanied by Pain and Swelling in Distal Extremities

Abstract

An 81-year-old man experienced acute progression of weakness in the extremities accompanied by a fever, tenderness, and swelling in distal parts of the extremities. He had flaccid tetraparesis with fasciculations and general hyporeflexia. Nerve conduction studies indicated demyelinating sensorimotor neuropathy. A cerebrospinal fluid examination revealed elevated proteins without pleocytosis. Immunological treatments were effective, but his symptoms exhibited repeated relapse and remission phases. He was diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with an acute onset. The highlight of this case is pain with inflammatory reaction recognized as red flags of CIDP, with the clinical course and electrophysiological findings compatible with CIDP.

Keywords: chronic inflammatory demyelinating polyradiculoneuropathy; fever; nerve conduction studies; pain; red flags.

Figure 1.

Swelling on the right hand. The patient also experienced tenderness in distal parts of the extremities.

Figure 2.

Left median nerve conduction study findings before (A-C) and after (D-F) treatment. (A) Prolongations of motor distal latency and distal compound motor action potential duration, reduction of motor conduction velocity, and motor conduction block were evident. (B) An F-wave was not evoked. (C) Sensory distal latency prolongation and reductions in sensory nerve action potential and sensory conduction velocity were found. (D) Motor distal latency prolongation and conduction block were improved. (E) F-waves were observed, but the latency was prolonged after treatment. (F) Sensory distal latency prolongation and reduction of sensory nerve action potential were improved.

Figure 3.

Left median nerve somatosensory evoked potential findings before (A) and after (B) treatment. (A) No components were evident before treatment. (B) Components appeared, but their latencies were prolonged in the peripheral nerve after treatment.