Neuroaxonal Dystrophy in Dogs: Case Report in 2 Litters of Papillon Puppies

Díaz, José Vargas; Duque, Carolina; Geisel, Rachel

doi:10.1892/0891-6640(2007)21[531:ndidcr]2.0.co;2

Cited by 9 publications

(9 citation statements)

References 17 publications

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“…To our knowledge, there is no other animal model of FNAD. Spontaneously occurring familial disorders characterized by neuroaxonal dystrophy have been described in various dog breeds (Cork et al, 1983; Griffiths et al, 1986; Sacre et al, 1993; Diaz et al, 2007; Jäderlund et al, 2007; Nibe et al, 2007, 2009), cats (Carmichael et al, 1993), sheep (Harper and Morton, 1991), horses (Miller and Collatos, 1997), and mice (Bouley et al, 2006). Each of these differs in at least some aspect of distribution of CNS lesions, integrity of myelin sheaths, or spheroid ultrastructure from what we report here.…”

Section: Discussionmentioning

confidence: 99%

Inherited neuroaxonal dystrophy in dogs causing lethal, fetal‐onset motor system dysfunction and cerebellar hypoplasia

Fyfe

Al-Tamimi

Castellani

et al. 2010

J of Comparative Neurology

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Neuroaxonal dystrophy in brainstem, spinal cord tracts, and spinal nerves accompanied by cerebellar hypoplasia was observed in a colony of laboratory dogs. Fetal akinesia was documented by ultrasonographic examination. At birth, affected puppies exhibited stereotypical positioning of limbs, scoliosis, arthrogryposis, pulmonary hypoplasia, and respiratory failure. Regional hypoplasia in the central nervous system was apparent grossly, most strikingly as underdeveloped cerebellum and spinal cord. Histopathologic abnormalities included swollen axons and spheroids in brainstem and spinal cord tracts; reduced cerebellar foliation, patchy loss of Purkinje cells, multifocal thinning of the external granular cell layer, and loss of neurons in the deep cerebellar nuclei; spheroids and loss of myelinated axons in spinal roots and peripheral nerves; increased myocyte apoptosis in skeletal muscle; and fibrofatty connective tissue proliferation around joints. Breeding studies demonstrated that the canine disorder is a fully penetrant, simple autosomal recessive trait. The disorder demonstrated a type and distribution of lesions homologous to that of human infantile neuroaxonal dystrophy (INAD), most commonly caused by mutations of PLA2G6, but alleles of informative markers flanking the canine PLA2G6 locus did not associate with the canine disorder. Thus, fetal-onset neuroaxonal dystrophy in dogs, a species with welldeveloped genome mapping resources, provides a unique opportunity for additional disease gene discovery and understanding of this pathology.

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Section: Discussionmentioning

confidence: 99%

Inherited neuroaxonal dystrophy in dogs causing lethal, fetal‐onset motor system dysfunction and cerebellar hypoplasia

Fyfe

Al-Tamimi

Castellani

et al. 2010

J of Comparative Neurology

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“…196 Neuroaxonal Dystrophy. 204,205 NAD has been sporadically described in cats. They can be histopathologically distinguished from other diseases by the presence of spheroids that involve distal or preterminal parts of the axon and are found in gray matter with proximity to the neuronal cell bodies in the spinal cord and brainstem.…”

Section: Primary Degenerative Spinal Cord Disordersmentioning

confidence: 99%

Tetraparesis, Hemiparesis, and Ataxia

Lorenz

2011

Handbook of Veterinary Neurology

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“…Progressive neurologic abnormalities included severe obtundation, gait abnormalities with postural reaction deficits, and absence of menace response. 5,[11][12][13]24,25,28,35 The progression of signs was often very rapid and dogs with NAD had short survival. The early age of onset in our dogs is similar to that reported in other cases of NAD in Chihuahua and Papillon puppies.…”

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confidence: 99%

“…The early age of onset in our dogs is similar to that reported in other cases of NAD in Chihuahua and Papillon puppies. 12,24,35 The development of the disease in at least 2 puppies from the same litter suggests a familial background.…”

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confidence: 99%

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Neuroaxonal Dystrophy and Cavitating Leukoencephalopathy of Chihuahua Dogs

et al. 2017

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A novel form of neuroaxonal dystrophy is described in 3 Chihuahua pups, 2 of which were from the same litter. It was characterized not only by accumulation of numerous and widely distributed axonal swellings (spheroids) but also by a severe cavitating leukoencephalopathy. The dogs presented with progressive neurological signs, including gait abnormalities and postural reaction deficits. Magnetic resonance images and gross examination at necropsy revealed dilation of lateral ventricles and cerebral atrophy, accompanied by cavitation of the subcortical white matter. Histopathologically, severe axonal degeneration with formation of large spheroids was found in the cerebral and cerebellar white matter, thalamus, and brainstem nuclei. Small-caliber spheroids were observed in the cerebral and cerebellar gray matter. The telencephalic white matter had severe myelin loss and cavitation with relative sparing of the U-fibers. Different from previously reported cases of canine neuroaxonal dystrophy, in these Chihuahuas the spheroid distribution predominantly involved the white matter with secondary severe leukoencephalopathy.

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Neuroaxonal Dystrophy in Dogs: Case Report in 2 Litters of Papillon Puppies

Cited by 9 publications

References 17 publications

Inherited neuroaxonal dystrophy in dogs causing lethal, fetal‐onset motor system dysfunction and cerebellar hypoplasia

Inherited neuroaxonal dystrophy in dogs causing lethal, fetal‐onset motor system dysfunction and cerebellar hypoplasia

Tetraparesis, Hemiparesis, and Ataxia

Neuroaxonal Dystrophy and Cavitating Leukoencephalopathy of Chihuahua Dogs

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