[Acute pandysautonomia and nicotinic acetylcholine receptor antibodies]

Acute pandysautonomia is an idiopathic, acute or subacute autonomic neuropathy, which diffusely affects pre- and post-synaptic, and sympathetic and parasympathetic nerves. The recent discovery of serum autoantibodies against the nicotinic acethylcholine receptor (nAChR) on autonomic ganglia has led to a better understanding of its pathogenesis as well as the emergence of a new disease entity named autoimmune autonomic ganglionopathy (AAG). Based on the detection of these antibodies in various dysautonomic conditions, AAG is considered a broad-spectrum disease entity that includes acute pandysautonomia as well as secondary autonomic neuropathy, restricted forms of dysautonomia (postural tachycardia syndrome and chronic intestinal pseudoobstruction), and chronic dysautonomia, mimicking pure autonomic failure. Reproduction of experimental AAG animals by active immunization with peptides derived from ganglionic nAChR or passive transfer of ganglionic nAChR antibodies strongly indicates that ganglionic nAChR antibodies are pathogenic in AAG development. There are no controlled treatment trials for AAG, and its optimal therapy remains uncertain. Recent reports suggest that combined immunotherapies using immunosuppressive agents with plasma exchange or intravenous immunoglobulin are effective for some intractable cases. An optimal protocol of combined immunotherapies should be established in controlled clinical trials in the future.