Avaliação epidemiológica, clínica, anatomopatológica e etiológica de surtos de ataxia em cabritos e cordeiros / Epidemiological, clinical, etiological and histopatological evaluation of delayed enzootic ataxia outbreaks in kids and lambs

Nos anos de 2001 e 2002, 46 cabritos (CAB) e 35 cordeiros (COR) de uma propriedade do agreste do Estado de Pernambuco foram acometidos por ataxia enzoótica (AE) de forma tardia. Houve aumento da incidência do 1o ano (46,3 por cento - CAB; 24,2 por cento COR) para o ano subseqüente (100 por cento - CAB e COR). Somente no ano de 2001, houve maior incidência em CAB (P<0,002) que nos COR. Todos os animais acometidos sucumbiram, com exceção de um CAB e um de COR. O quadro clínico variou de acordo com a idade em que surgiram os sintomas, ou seja, de 30 a 45 dias os animais manifestaram paralisia dos membros anteriores e posteriores, acompanhada de espasticidade muscular e morte; com mais de 45 dias, ocorreu paralisia flácida ou espástica apenas dos membros posteriores e morte; somente os cabritos com menos de 45 dias apresentaram movimentos verticais de cabeça. As lesões histopatológicas evidenciadas foram: degeneração axonal, esferóides, gliose e dismielinização no segmento ventral e lateral da medula espinhal. Nos animais com menos de 45 dias, essas lesões foram evidentes na região cervical e nos animas mais velhos na região lombar. Apenas nos cabritos foi constatada lesão degenerativa no cerebelo. A dieta oferecida ao rebanho continha quantidades adequadas de cobre, porém os animais acometidos por AE apresentaram baixos teores de cobre no fígado (45,8mg kg-1 MS). Os elementos antagonizantes do cobre, Mo, S, e Zn, estavam normais, com exceção do ferro, que estava em alta concentração tanto no solo (8600mg kg-1) quanto nos alimentos (284mg kg-1). O excesso de ingestão de ferro sugere ter provocado a carência de cobre nos animais.

Histopathology of enzootic ataxia in Sika deer (Cervus nippon Temminck).

Thirteen Sika deer (Cervus nippon Temminck) showing ataxia on a stock farm in the north eastern district of China were examined histopathologically. The principal pathological changes were spongy vacuolation and myelin deficiency in the white matter of the spinal cord and brain stem, fibrosis and rupture of the elastic lamina of the spinal arterioles, and mesothelial hyperplasia in the spinal arachnoid. Other findings included defective formation of the elastic laminae of the aorta, and the blood vessels in the kidney and lung, hemosiderosis in the spleen and liver, and lymphocyte depletion in the systemic lymph nodes. Copper concentrations were low in the serum and liver. In the white matter of the spinal cord and brain stem, demyelination appeared to coexist with dysmyelination and secondary myelin breakdown. It was inferred that decreased activity of copper containing enzyme induced various lesions. The possible role of copper deficiency in the pathogenesis of the ataxic conditions is discussed.

Swayback in a blesbok (Damaliscus dorcas phillipsi) and a black wildebeest (Connochaetes gnou).

Ataxia associated with myelopathy and low liver copper concentrations is described in a blesbok (Damaliscus dorcas phillipsi) and black wildebeest (Connochaetes gnou) from the Karoo Nature Reserve, Graaff-Reinet. This syndrome, which occurs in neonatal and delayed forms, has been described in sheep, goats, pigs and domesticated red deer, but reports in other ungulates, including antelope, are very rare and generally unconfirmed by histopathological examination. The 2 animals examined exhibited a typical pattern of Wallerian degeneration in selected tracts of the spinal cord. Many blesbok in the reserve are markedly pale in colour. Fading of the hair is also associated with copper deficiency in ungulates. High lamb mortality occurs as a result of the ataxia and threatens the survival of the blesbok herd in the reserve. Confirmation of low liver copper concentrations in affected animals offers direction towards the solution of the problem.

Ultrastructure of hepatocytes in copper-deficient Sika deer (Cervus nippon Temminck).

The livers of 13 Sika deer (Cervus nippon Temminck) aged 4 to 9 years and suffering from copper deficiency (enzootic ataxia) were examined histologically, histochemically and by electron microscopy. In addition, the serum and liver copper concentrations, measured in three animals, were found to be low. Histologically, the hepatocytes exhibited cloudy swelling, and numerous haemosiderin deposits were seen in the hepatocytes and Kupffer cells. Staining with p-dimethyl amino-benzylidene-rhodamine revealed distinctly fewer copper granules than normal. Histochemically, 3,3'-diaminobenzidine-H2O2 staining revealed increased numbers of catalase-positive granules around nuclei. Electron microscopically, "giant" and bizarre-shaped mitochondria, irregular depression of the mitochondrial membrane, and fusion of cristae were noted. Disorders of copper-containing enzymes, including cytochrome oxidase, caeruloplasmin and monoamine oxidase, may have been responsible for the mitochondrial abnormalities.

A farm investigation into swayback in a herd of goats and the result of administration of copper needles.

Clinical, biochemical and pathological findings led to a diagnosis of swayback in a herd of goats. Trace element values in soil, pasture and supplementary feed were measured, and copper oxide needles administered to the adult goats. This form of treatment had a more limited effect in these goats than is reported in sheep.

Delayed swayback in goat kids, a study of 23 cases.

The results of a retrospective study of 23 goat kids with delayed swayback are reported. Principal clinical signs were ataxia, loss of postural control, spasticity of the hindlimbs, and muscular weakness, often progressing to permanent recumbency. Denervation of skeletal muscles was demonstrated by electromyography in 2 kids. Three kids slowly recovered during hospitalisation. Histopathological changes were characterized by degeneration of selected neuronal populations with their processes within the central and the peripheral nervous system. Affected systems included upper motor neuron, vestibular, general proprioceptive, and lower motor neuron pathways, with additional involvement of the cerebellar cortex in some animals. Our findings, including limited ultrastructural observations, support the notion that the neuraxon rather than the myelin sheath is the prime target of disease in delayed swayback. The available copper values of affected kids and their unaffected herd mates were significantly lower than those of random control goats, which provides further support for a role of copper deficiency in the aetiology of this disease in the goat.

Menkes' disease and swayback. A comparative study of two copper deficiency syndromes.

The neuropathological findings in two siblings with Menkes' disease were compared with representative material obtained from lambs suffering from swayback (enzootic ataxia). The aim of the study was to demonstrate the similarity of lesions in a genetic and a nutritional form of copper deficiency in support of the view that all lesions in Menkes' disease could be ascribed to simple hypocupraemia. All lesions of Menkes' disease were shown to have their counterpart in swayback, with exception of the abnormal arborisations of the Purkinje cell dendrites. These have often been interpreted as malformations and cited in evidence of the prenatal origin of the cerebral lesions. They are, however, non-specific and similar lesions have been reported in conditions arising in later life. While there is abundant collateral evidence of disturbed copper metabolism in utero, the problem of the prenatal versus postnatal origin of cerebral damage remains unresolved.

Studies on viral leukoencephalomyelitis and swayback in goats.

Six kid goats with CNS disease were studied by a variety of techniques. All had morphological evidence of Viral Leukoencephalomyelitis of Goats. (VLG) characterized by demyelinating encephalomyelitis and pneumonitis. Attempts were made to isolate a virus from 5 of these goats by direct culture of CNS and visceral tissues. Syncytial formation was found in some lung, spleen and bone marrow cultures but syncytia were found to be free of complete virus by electron microscopy. When newborn goats were inoculated with syncytia forming cells, a visna-like virus was found in bone marrow cultures of 1 goat 8 days post inoculation. Bone marrow cells from a twin goat 3 months after inoculation produced syncytia without complete virus. it is suggested that complete virus might be present in goats shortly after infection, while persisting in an incomplete form. Three of the 6 goats studied also had lesions of swayback with diffuse encephalomyelopathy and low liver copper levels. Control copper levels in kids with non-neurological disease were variable, occasionally being as low as swayback animals. The experience of others that swayback in the goat and low copper status are poorly correlated is confirmed. Discrepancies inherent in assaying formalin preserved tissues for copper are described.

California goats with a disease resembling enzootic ataxia or swayback.

In a retrospective study typical signs and lesions of enzootic ataxia or swayback were found in 16 young dairy goats from eight widely scattered herds in California. In addition to the constant appearance of chromatolytic neurons in brainstem and spinal cord, and myelin deficiency in certain tracts of the cord, cerebellar hypoplasia was found frequently. Liver copper was subnormal in six of nine kids tested. The disease is viewed as a developmental defect in which failure of neuronal perikaryon metabolism leads to distal axonopathy with secondary demyelination.