Neuronal ceroid-lipofuscinosis in Borderdale sheep.
OBJECTIVE
To describe the gross and histological lesions of a neurological disease in Borderdale sheep characterised clinically by blindness and circling, as a basis to its classification.


METHODS
Formalin-fixed tissues were processed into paraffin wax and epoxy resin for light and electron microscopy of variously stained sections.


RESULTS
Lesions were those of a lysosomal storage disease with severe neurodegeneration of the cerebral cortex. The staining reactions, autofluorescence and ultrastructure of storage material allowed the diagnosis of neuronal ceroid-lipofuscinosis associated with the accumulation of subunit-c of mitochondrial ATP synthase.


CONCLUSIONS
The severity of neurodegeneration and minor differences in the ultrastructure of storage material implied that this was a different disease from other forms of ovine ceroid-lipofuscinosis that accumulate subunit-c of mitochondrial ATP synthase. An autosomal recessive mode of inheritance is considered probable. Although of only minor economic importance, this disease may be important to research into the group of ceroid-lipofuscinoses as a whole.
Jolly
R D
RD
Institute of Veterinary Animal and Biomedical Sciences, Massey University, Private Bag 11222, Palmerston North, New Zealand. R.D.Jolly@massey.ac.nz
Arthur
D G
DG
Kay
G W
GW
Palmer
D N
DN