Acute Motor Axonal Neuropathy - History

History

AMAN, also known as Chinese Paralytic Syndrome, was first described by a group of Johns Hopkins University and Pennsylvania State University neurologists in collaboration with neurologists from the Second Teaching Hospital of Hebei Medical School and Beijing Children's Hospital. In 1991, Doctors Guy Mckhann, Jack Griffin, Dave Cornblath and Tony Ho from Johns Hopkins University and Dr. Arthur Asbury from University of Pennsylvania visited China to study a mysterious epidemic of paralytic syndrome occurring in northern China. Every summer, hundreds of children from rural China developed acute paralysis and respiratory failure. Hospitals were overwhelmed with number of cases and often ran out of ventilators and hospital beds. Examination of these children showed that many of them had acute flaccid paralysis and areflexia but with little or no sensory loss. Electrophysiological testing of these children showed motor axonal loss with occasional conduction block with a lack of demyelinating features and normal sensory potentials. In contrast, the common form of Guillain-Barré syndrome in the West often presents with sensory loss and demyelination on electrophysiology testing and is more common in adults. Later, several autopsies confirmed the focus of the immune attack was at the motor axolemma especially around the nodes of Ranvier. These cases showed deposition of antibody and complement along the motor axolemma and associated macrophage infiltration.

A link to Campylobacter jejuni was suspected when a young girl was admitted to Second Teaching Hospital. She had become ill after feeding the family chickens. She developed acute paralysis and respiratory failure. Investigators discovered that several of the chickens in the home displayed similar symptoms and C. jejuni was found in their droppings. Several of the paralysis patients were found to have antibodies to C. jejuni and anti-GD1a antibodies, suggesting a link between the pathogen and the disease.