Unusual features of acute anti-myelin-associated glycoprotein polyneuropathy

Excerpt

Patients with anti-myelin-associated glycoprotein (MAG) peripheral neuropathy exhibits mild to moderate symmetric extremity weakness, tremor, impaired proprioception, ataxia that typically progress slowly [1, 2]. Anti-MAG antibodies in patients with neuropathy and IgM paraproteinemias are thought to cause axonal degeneration and demyelination by reacting with epitopes present in central and peripheral nervous system, namely with an antigenic glycolipid, identified as sulfoglucuronyl paragloboside (SGPG) [1, 3]. SGPG is found both in axons and myelin of peripheral nerves [1]; therefore, it is reasonable to find both axonal degeneration and demyelination in patients with positive anti-SGPG antibodies [1, 3]. The discovery of SGPG to which all anti-MAG-positive patients immunoreact, has strengthened the view that glycolipids might be the primary antigenic targets [1‐3]. Autonomic symptoms like orthostasis, non-reactive pupils, bradycardia, urinary retention occasionally occuring in patients with monoclonal gammopathy of undetermined significance (MGUS) are thought to be related with primary amyloidosis [1, 3]. …