Globoid cell leukodystrophy (Krabbe's disease) in a Japanese domestic cat

Abstract

A male Japanese domestic cat developed progressive limb paralysis from 4 months of age. The cat showed visual disorder, trismus and cognitive impairment and died at 9 months of age. At necropsy, significant discoloration of the white matter was observed throughout the brain and spinal cord. Histologically, severe myelin loss and gliosis were observed, especially in the internal capsule and cerebellum.In the lesions, severe infiltration of macrophages with broad cytoplasm filled with PAS-positive and nonmetachromatic granules (globoid cells) was evident. On the basis of these findings, the case was diagnosed as feline globoid cell leukodystrophy (Krabbe’s disease). Immunohistochemical observation indicated the involvement of oxidative stress and small HSP in the disease.

Morphometrical study of the cat cerebellum using unbiased design‐based stereology

“…Pathological effects of this viral disease include hydrocephalus, cerebellar hypoplasia and cerebellar cortical dysplasia resulting in reduced and deformed cellular layers (Brown, Baker, & Barker, 2007; Resibois, Coppens, & Poncelet, 2007). Other disorders targeting the cerebellum in cats include feline morbillivirus (Sieg et al, 2019), saccadic oscillations (Ives, MacKillop, & Olby, 2018), globoid cell leukodystrophy (Krabbe's disease) (Ogawa et al., 2014) and feline herpesvirus‐1 (Townsend et al., 2013).…”

Clinical and MRI findings in two unrelated cats with globoid cell leucodystrophy

“…Tremors of the head and trunk were reported only in two of six cats,2 5 and they were present in both cases of this report, although milder in case 2, despite the severe cerebellar white matter degeneration. As in other degenerative diseases, there is a mismatch between the severity of the histological changes in GLD and the severity of the clinical signs; in fact prosencephalic signs vary from mild to absent, and so far cognitive impairment has been reported in one cat,24 despite wide involvement of the telencephalic white matter.…”

“…GLD is a very rare and fatal condition in all species, including in people. It has been reported in six cats—three in USA,2 23 two in Italy5 and one in Japan24—although only clinical and histopathological features were described. As in the present cases, pelvic limb involvement is a predominant feature and inevitably progresses to paraplegia, with loss of nociception in all affected patients.…”

“…The disease has been reported in people,7–10 monkeys ( Macaca mulatta ),11 mouse,12 sheep,13 14 dogs15–22 and cats 2 5 23 24…”

“…In cats, the onset of clinical signs is between five weeks and four months of age and is typically characterised by progressive paraparesis or tetraparesis evolving to paraplegia, weakness, and focal or generalised tremors, sometimes accompanied by segmental spinal reflex loss and other CNS signs 2 5 23 24…”