Jansky-Bielschowsky form of neuronal ceroid-lipofuscinosis. Ocular pathology of the Batten-Vogt syndrome
S. S. Schochet Jr, R. L. Font and H. H. Morris 3rd
The neuronal ceroid-lipofuscinoses (Batten-Vogt syndrome) are a group of at
least four closely related entities that are characterized by the
accumulation of autofluorescent lipopigments in neural, visceral, and
somatic tissues. The ophthalmic pathology of this syndrome has been
investigated less extensively than other tissues and organs. In this
report, we present the postmortem ocular findings, including light,
fluorescence, and electron microscopy, from a child with the
Jansky-Bielschowsky form of this syndrome.
