[Chronic inflammatory demyelinating polyradiculoneuropathy of acute onset: relapse after temporary improvement by plasma exchange and longstanding remission by corticosteroid therapy].

A 59-year-old woman became tetraparetic and unable to stand or walk acutely in several days after she had first developed weakness of her legs and dysesthesia of both hands. Neurological examination revealed facial weakness, proximal dominant tetraparesis, areflexia and acrodysesthesia. The cerebrospinal fluid protein was elevated without pleocytosis, and the nerve conduction velocity was markedly slowed. The sural nerve biopsy indicated demyelination. The tentative diagnosis was Guillain-Barré syndrome (GBS). The initial and second courses of plasma exchanges temporarily improved the weakness, but relapses occurred within a few weeks. The third course of plasma exchange was, therefore, immediately followed by corticosteroid therapy, which successfully improved the tetraparesis. However, weakness again worsened during the rapid reduction of corticosteroid. A high dose of corticosteroid was again started and maintained for two months. The patient recovered gradually and relapses had not occurred so far after gradual reduction of the dose of corticosteroid. Her disease was finally diagnosed as corticosteroid-dependent chronic inflammatory demyelinating polyradiculoneuropathy, although the onset had been as acute as that of GBS. The present case suggests that corticosteroid-dependent chronic inflammatory demyelinating polyradiculoneuropathy may present as a case of acute onset polyradiculoneuropathy resembling GBS, and that corticosteroid therapy must be tried in such a case when plasma exchanges are not effective or relapses occur.