The diagnosis of nemaline rod myopathy (NM) is based on the presence of num
erous pathognomonic rods within a fresh frozen muscle biopsy specimen. Thre
e forms of congenital NM have been described in humans, and rods have been
found to occur in various other conditions. A similar myopathy was describe
d in 1986 in a family of cats. In this report, we describe a case of congen
ital NM in a 10-month-old Border Collie, an adult-onset NM in an 11-year-ol
d Schipperke, and 2 acquired myopathies with nemaline rods in adult dogs as
sociated with hypothyroidism and Gushing's syndrome. Common clinical featur
es included exercise intolerance, abnormal electromyography, and the presen
ce of nemaline rods in fresh, frozen, and glutaraldehyde-fixed biopsies fro
m proximal appendicular limb muscles. Staining of cryostat sections of musc
le biopsy specimens by the modified Gomori trichrome technique disclosed nu
merous rod bodies that were localized to type 1 fibers by the histochemical
adenosine triphosphatase reaction. Accumulation of rods also was demonstra
ted by electron microscopy in 2 of the cases with localized enlargement and
streaming of Z lines. Documentation of NM in a young Border Collie and the
adult-onset form in the Schipperke alerts clinicians to the existence of t
his disorder in these breeds.