CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY (CIDP) ASSOCIATED WITH MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS

Up to now four patients with acute, purely motor, demyelinating neuropathy and conduction blocks have been described. Three cases had Campylobacter Jejuni (CJ) enteritis. In two ab anti-GM1 were detected, along with ab anti-GalNAc-GD1a in one. Two men (41 and 20 years old) developed weakness respectively ten days after enteritis and rubella. Examination showed in both proximal and distal weakness in all limbs with no sensory loss. Tendon reflexes were normal in the first patient, and brisk in the second. CSF proteins were increased. Serological tests did not support a recent CJ infection. High titres of IgG anti-GD1a and IgM anti-GM1 were found in the first patient. Electrophysiological examination showed, since the first days after onset, partial motor conduction block in ulnar nerves of the first patient and in eight motor nerves of the second patient. Sensory conductions were normal even across the sites of conduction block. Four plasmaphereses were performed. In the first patient conduction blocks gradually improved to disappear in 3 weeks without excessive temporal dispersion of proximal motor responses. In the second patient conduction blocks disappeared in 8–16 weeks with development of excessive temporal dispersion in 4  /  8 nerves.
We deem that acute motor neuropathy with conduction block is a GBS variant in which only motor fibers are involved with early conduction block in intermediate nerve segments. Our electrophysiological findings indicate that conduction block may be of the "anaesthetic type" followed by fast recovery or may progress to de-remyelination with a slower course.