Pathology of "landry-guillain-barré syndrome" in children, prevailing in the regions surrounding the Inland Sea of Japan

Histopathological investigations were carried out on five fatal cases of a type of polyneuritis of unknown etiology diagnosed as Landry-Guillain-Barre syndrome, which endemically occurred in children in the regions surrounding the Inland Sea of Japan. The most characteristic pathologic feature in the nervous system was pronounced patchy degenerative changes with slight or moderate degree of inflammatory cell response of focal type in the peripheral and cranial nerves, predominantly in the nerve fibers of the spinal and cranial roots. In the spinal cord, medulla, pons, and in some portions of the cerebrum and cerebellum, engorgement of the small blood vessels as well as edema and the less predominant scattered degenerative changes of ganglioncells and nerve fibers with extremely slight degree of glial response and sparse perivascular cell collections were encountered. The cerebrospinal meninges displayed edema and congestion of the pial blood vessels with focal collections of a small number of lymphocytes and/or monocytes. No advanced involvement of the anterior horn of the spinal cord in a strict sense of anterior poliomyelitis was, however, recognized. These changes may lead the histopathologic diagnosis of the present disease to infectious encephalomyelo-polyradiculoneuritis or a type of infectious polyneuritis. The main histopathologic changes in the visceral organs were a moderate degree of engorgement of the small blood vessels, degeneration of parencymatous organs such as the liver and kidney, hyperplasia or follicular atrophy of the lymphatic tissues, interalveolar pneumonia, focal myositis, and slight degree of round cell infiltrations in the interstitial tissues of the other viscera, such as the liver, heart, and gastrointestinal canal. Based upon the observations on the histopathological changes as well as clinical manifestations, discussions were made on the pathogenesis and etiologic factor of the present endemic disease with critique on the literatures.