Open AccessNeuronal Ceroid Lipofuscinosis in Rambouillet Sheep: Characterization of the Clinical Disease
Author(s) -
Woods Philip R.,
Storts Ralph W.,
Shelton Maurice,
Menzies Carl
Publication year - 1994
Publication title -
journal of veterinary internal medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.356
H-Index - 103
eISSN - 1939-1676
pISSN - 0891-6640
DOI - 10.1111/j.1939-1676.1994.tb03252.x
Subject(s) - neuronal ceroid lipofuscinosis , medicine , disease , purebred , blindness , physiology , pathology , clinical disease , biology , breed , optometry , genetics
A chronic progressive, neurodegenerative disease has been identified in 4 closely related flocks of purebred Rambouillet sheep. Blindness, circling, proprioceptive deficits, reduced cognition, and poor body condition are the main clinical signs. Prominent lesions include markedly decreased cerebral size and weight, enlarged cerebral ventricles, and intraneuronal accumulations of autofluorescent pigment. Affected sheep usually die between 1 and 2 years of age. The clinical signs and postmortem findings are consistent with neuronal ceroid‐lipofuscinosis.