Basal plate dysplasia presenting as a hamartoma of the pontine tegmentum
Katsetos, C.D.; Ambler, M.; Sweeney, K.
Archives of Pathology and Laboratory Medicine 111(9): 854-857
1987
ISSN/ISBN: 0003-9985 PMID: 3632304 Document Number: 301275
A child with an intractable, nonfocal seizure disorder, progressive lethargy, and psychomotor retardation had a malformation involving rhombencephalic structures deriving from the embryonic basal plate. Its principal component was a mature neuroepithelial hamartomatous mass arising from the pontine tegmentum. Diencephalic, metencephalic, and myelencephalic derivatives of the alar plate were remarkably spared. To our knowledge, the morbid anatomy and embryopathology of this central nervous system malformation has not been previously described.