Acute motor axonal neuropathy: A frequent cause of acute flaccid paralysis in China

TLDR

In northern China, annual epidemics of acute‑onset flaccid paralysis clinically diagnosed as Guillain‑Barré syndrome have been noted for at least two decades. Analysis of more than 3,200 patients shows that most cases occur in summer among rural children and young adults. The syndrome manifests as rapidly progressive ascending tetraparesis with respiratory failure, acellular CSF with delayed protein elevation, normal sensory studies, reduced CMAP amplitudes, Wallerian‑like motor fiber degeneration, yet patients usually recover well and it is distinct from poliomyelitis and demyelinating Guillain‑Barré syndrome.

Abstract

In northern China, annual epidemics of acute-onset flaccid paralysis diagnosed clinically as Guillain-Barré syndrome have been recognized for at least 20 years. On the basis of an historical analysis of more than 3,200 patients, distinctive features include most cases occurring during the summer months among children and young adults, most of whom reside in rural areas. Of 90 patients with acute flaccid paralysis, 88 had a distinctive pattern that shares clinical and cerebrospinal fluid findings with demyelinating Guillain-Barré syndrome, but that differs from Guillain-Barré syndrome physiologically and pathologically. The clinical course is marked by rapidly progressive ascending tetraparesis, often with respiratory failure, but without fever, systemic illness, or sensory involvement. Cerebrospinal fluid is acellular, and elevations of protein content occur in the second or third week of illness. Electrodiagnostic studies show normal motor distal latencies and limb conduction velocities, but reduced compound muscle action potential amplitudes. Sensory nerve action potentials and, when elicitable, F waves are within the range of normal. Recovery is usually good. Autopsy studies have shown Wallerian-like degeneration of motor fibers. These studies establish that this is a distinctive syndrome, distinguishable from poliomyelitis and demyelinating Guillain-Barré syndrome.