Cerebellar Abiotrophy in Nelore: First Report in Zebu Cattle (Bos taurus indicus)

Abstract

Cerebellar abiotrophy (CA) is a degenerative disorder of the central nervous system (CNS) that has been reported in humans and animals. In cattle, CA had been reported in Bos taurus taurus and crossbreed cattle. CA is characterized by degeneration and loss of Purkinje cells and decrease in the population of granule cells. The pathogenesis of this process is unknown, but it is believed that there is an autosomal recessive heritable factor involved. A 15-month-old Nelore ox (Bos taurus indicus) was sent to a slaughterhouse, where it presented cerebellar ataxia, symmetrical hypermetria, spasticity, ptyalism, and incoordination. There were no macroscopic changes. CNS samples were negative for BSE, rabies, and other infectious pathogens. Microscopically, there was atrophy of the molecular, granular, and Purkinje cell layers of the cerebellar cortex, and a marked and diffuse loss of the Purkinje cells. No other microscopic lesions were observed in CNS. These findings were consistent with cerebellar abiotrophy. To the best of our knowledge, no cases of CA have ever been reported in Bos taurus indicus. Therefore, the present case of CA in Nelore is the first report of the disease in Zebu cattle.

Full text

Oliveira et al; Cerebellar Abiotrophy in Nelore: First Report in Zebu Cattle (Bos taurus indicus).
Braz J Vet Pathol, 2011, 4(3), 235-238.
Brazilian Journal of Veterinary Pathology. www.bjvp.org.br . All rights reserved 2007.
235
Case Report
Cerebellar Abiotrophy in Nelore: First Report in Zebu Cattle
(Bos taurus indicus)
Taismara S. Oliveira1,2, Safira R. D. Lima1, Ronaldo Furtini1,
Valquiria Bull2, Érica A. Costa2, Tatiane A. Paixão3, Renato L. Santos2
1Laboratório de Saúde Animal do Instituto Mineiro de Agropecuária, Minas Gerais, Brazil.
2 Departamento de Clínica e Cirurgia Veterinárias, Escola de Veterinária, Universidade Federal de Minas Gerais, Brazil.
3 Departamento de Patologia Geral, Instituto de Ciências Biológicas, Universidade Federal de Minas Gerais, Brazil.
Corresponding author: Renato L. Santos. Departamento de Clínica e Cirurgia Veterinária, Escola de Veterinária, Universidade Federal de Minas
Gerais, Av. Presidente Antônio Carlos, 6627 – CEP 30161-970, Belo Horizonte, MG, Brazil. Phone:55-31-3409-2239. Fax: 55-31-3409-2230.
E-mail: rsantos@vet.ufmg.br
Submitted July 4th 2011, Accepted September 2nd 2011
Abstract
Cerebellar abiotrophy (CA) is a degenerative disorder of the central nervous system (CNS) that has been
reported in humans and animals. In cattle, CA had been reported in Bos taurus taurus and crossbreed cattle. CA is
characterized by degeneration and loss of Purkinje cells and decrease in the population of granule cells. The
pathogenesis of this process is unknown, but it is believed that there is an autosomal recessive heritable factor involved.
A 15-month-old Nelore ox (Bos taurus indicus) was sent to a slaughterhouse, where it presented cerebellar ataxia,
symmetrical hypermetria, spasticity, ptyalism, and incoordination. There were no macroscopic changes. CNS samples
were negative for BSE, rabies, and other infectious pathogens. Microscopically, there was atrophy of the molecular,
granular, and Purkinje cell layers of the cerebellar cortex, and a marked and diffuse loss of the Purkinje cells. No other
microscopic lesions were observed in CNS. These findings were consistent with cerebellar abiotrophy. To the best of
our knowledge, no cases of CA have ever been reported in Bos taurus indicus. Therefore, the present case of CA in
Nelore is the first report of the disease in Zebu cattle.
Key Words: Cerebellar cortical abiotrophy, cerebellum, Bos taurus indicus.
Introduction
Cerebellar abiotrophy (CA) is a degenerative
disorder of the central nervous system that has been
reported in humans and animals. CA is the most
common manifestation of abiotrophy in domestic
animals, and it is also referred to as cerebellar cortical
abiotrophy (8). In domestic animals, CA has been
reported in cattle, horse, dog, cat, sheep and pig (1, 2, 3,
7, 9, 10, 11, 12, 13, 14, 15, 17, 19, 21, 24, 25, 26). In
addition, CA has also been diagnosed in nonhuman
primates, laboratory mice and exotic animals (18, 23).
In cattle, CA has been described in Holstein, Angus,
Ayrshire, Shorthorn, Hereford, Charolais, Aquitanica
and Bos taurus taurus crossbred cattle (7, 14, 16, 17,
20, 25, 26), but not in Zebu cattle (Bos taurus indicus).
CA is characterized by degeneration and loss of
Purkinje cells and decrease in the population of granule
cells (23). The integrity of the granule cell neurons is
dependent on its synaptic relationship with the dendritic
zone of the Purkinje neurons, therefore, loss of these
neurons usually results in a secondary depletion of
granule cells (6). The pathogenesis of this process is
unknown, however it is believed that there is a
autosomal recessive heritable factor involved (4, 5, 9,
10, 22).
Case Report
A 15-month-old Nelore ox (Bos taurus
indicus) was sent to a slaughterhouse in Presidente
Olegário (State of Minas Gerais, Brazil), where it
presented cerebellar ataxia with head tremor,
symmetrical hypermetria, spasticity, ptyalism, and

Oliveira et al; Cerebellar Abiotrophy in Nelore: First Report in Zebu Cattle (Bos taurus indicus).
Braz J Vet Pathol, 2011, 4(3), 235-238.
Brazilian Journal of Veterinary Pathology. www.bjvp.org.br . All rights reserved 2007.
236
incoordination. The ox was slaughtered and no
macroscopic changes were observed in the central
nervous system (CNS) or elsewhere. The CNS was
collected by a veterinarian belonging to the official
inspection service, and sent to the official diagnostic
laboratory. CNS samples were negative for rabies by
immunofluorescence and inoculation in mice; negative
for BSE by histopathology and immunohistochemistry;
and PCR (polymerase chain reaction) negative for
bovine herpesvirus type-5, bovine herpesvirus type-1,
ovine herpesvirus type-2, swine herpesvirus type-1,
Listeria monocytogenes, and Histophilus somni.
Microscopically, there was atrophy of the molecular,
granular, and Purkinje cell layers of the cerebellar
cortex. There was also a marked and diffuse loss of the
Purkinje cells, with moderate attenuation and
astrogliosis of the molecular cell layer, and depletion of
granule cells, with marked thinning of granular cell
layer, which contained degenerative Purkinje cells. The
remaining Purkinje cells were either shrunken and
hyperchromatic or swollen and pale with cytoplasmic
vacuolization, often exhibiting central chromatolysis,
with peripheral displacement of Nissl substance. No
other lesions were observed in any other area of the
CNS (i.e. cerebrum, thalamus and brainstem). These
microscopic findings were consistent with cerebellar
abiotrophy (Figure 1-3).
Figure 1. Cerebellum, ox. Cerebellar cortex with
atrophy of molecular, granular, and Purkinje cell layers.
Marked and diffuse loss of the Purkinje cell layer, with
moderate attenuation and astrogliosis of the molecular
cell layer (H&E, 10x).
Figure 2. Cerebellum, ox. Remaining Purkinje cells are
shrunken and hyperchromatic, with moderate
attenuation and astrogliosis of the molecular cell layer.
(H&E, 40x).
Figure 3. Cerebellum, ox. Depletion of granule cells,
with marked thinning of the granular cell layer (H&E,
40x).
Discussion
The progressive degeneration and loss of
cerebellar cortical neurons that occurs in CA usually
results in clinical changes in young patients, i.e. during
early stages of life. Therefore, the affected animal is
normal at birth and develops progressive cerebellar
deficits during the postnatal period (7). In this case,
there was no record of clinical signs prior to slaughter.
The clinical manifestations of CA are cerebellar ataxia
characterized by head tremor, symmetrical hypermetria,
spasticity, broad-based stance, and loss of balance,
similar to that observed in this case. The cerebellum is
grossly normal, and microscopic changes do not have
an uniform distribution. These changes usually develop
first in the vermis and paramedian lobules and then
spread to the lateral lobules (23).
The cause of CA is unknown, however is
presumed to be an intrinsic metabolic effect related to
inherited recessive genetic defect (7). One hypothesis
involves excytotoxic degeneration of neurons that have
glutamate receptors and receive axon terminals with

Oliveira et al; Cerebellar Abiotrophy in Nelore: First Report in Zebu Cattle (Bos taurus indicus).
Braz J Vet Pathol, 2011, 4(3), 235-238.
Brazilian Journal of Veterinary Pathology. www.bjvp.org.br . All rights reserved 2007.
237
glutamate as the excitatory transmitter (7). Excessive
glutamate stimulation could cause degeneration of the
neuron. Possible explanations for excessive glutamate
stimulation include excessive glutamate release,
decreased glutamate uptake and clearance, or increased
glutamate receptor sensitivity (7).
CA must be differentiated from multisystemic
neuronal abiotrophy and cerebellar hypoplasia. In
multisystemic neuronal abiotrophy, neuropathological
changes develop during early to middle adulthood. In
this disease, there is usually a concurrent degeneration
of other neuronal populations (cerebellar, brain stem,
and spinal forms), and it is a rare condition in the
veterinary medicine (23). Cerebellar hypoplasia is one
of the most common congenital anomalies in cattle. It
can occur sporadically or be caused by viral infections
such as the bovine viral diarrhea virus (BVD),
bluetongue virus, and Akabane virus. In the Shorthorn
breed there is evidence that the disease is hereditary.
Clinically, the disease is characterized by the birth of
animals with tremors, incoordination and hypermetria,
but in contrast to CA, clinical signs are not progressive
(20).
CA have been described in most domestic
animal species, including dog, cattle, sheep, horse, pig,
and cat (7). It has also been reported in nonhuman
primates, laboratory mice, and exotic animal species
(18, 23). Most CA reports in domestic animals involve
dogs. Canine CA was first reported in the Kerry Blue
Terrier, in which it has been characterized as an
autosomal recessive heritable disease (9). Other canine
breeds also have heritable CA, including Gordon
Setters (10), Rough-coated Collies (12), Border Collies
(20) and Australian Kelpies (22, 24). CA has also been
diagnosed in Boxers (11), Schnauzers (2), Beagles (15),
Airedale Terriers, Finnish Harriers, Bernese Mountain
Dogs, Miniature Poodles, Brittany Spaniels, Cocker
Spaniels, Labrador Retrievers, Golden Retrievers, and
Great Danes (7). CA is uncommon in cats when
compared to dogs (1, 3, 7, 19). In horses, CA has been
described in the Swedish Gotland Pony, and Arabian or
Part-arabian foals. Equine CA has been characterized as
an autosomal recessive heritable disease, a possible
mutation (4) that is linked to the Arabian ancestry (5,
23). In Yorkshire pigs, CA has also been diagnosed,
appearing between the fourth and fifth weeks of live
(23). In sheep, a genetic cause for CA with putative
inheritance as an autosomal recessive trait has been
reported in Wiltshire sheep (13).
There are several reports of CA in cattle,
especially in Bos taurus taurus, mainly affecting
Aberdeen Angus and Holstein (14, 16, 17, 25, 20, 26).
In cattle, it is presumed that CA is inherited as an
autosomal dominant disorder with incomplete
penetrance. In Brazil, CA has been described in
Holstein (20) and Aquitanica (16). To the best of our
knowledge, no cases of CA have ever been reported
affecting Bos taurus indicus. Therefore, the present case
of CA in Nelore is the first report of the disease in Zebu
cattle. In this case, the ox belonged to a dealer, and
there was no information available concerning its
genetic background. Therefore, it was impossible to
verify any pattern of genetic inheritance. Yet, this is an
important report since it demonstrates for the first time
that Zebu cattle are also affected by CA.
Acknowledgments
Work in RLS lab is funded by CNPq and
FAPEMIG.
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