Congenital tremor in piglets: Is bovine viral diarrhea virus an etiological cause?

Congenital tremor in pigs involves several etiologies, including pestivirus, which may cause neurological injuries in different animal species. To evaluate whether bovine viral diarrhea virus (BVDV), an important pestivirus, is one of the etiological agents of congenital tremor in swine, gilts and the fetuses were challenged at 45 days of gestation with BVDV-2. Four pregnant gilts were inoculated oronasally, four gilts underwent fetal intrauterine inoculation, and two gilts constituted the control group. Antibody titers were determined by virus neutralization (VN), and viral RNA was detected by RT-PCR. Blood samples were collected from all gilts and piglets born to obtain whole blood and serum for analysis. One third of the neonates were euthanized at three days old, and samples of the encephalon, brain stem and spinal cord were collected for anatomopathological evaluation and viral RNA detection. The piglets that remained alive were clinically evaluated every day, and blood sampling was performed regularly for 35 days. The piglets from gilts in both inoculation treatment groups showed no clinical neurological signs and were born with no viral RNA in their blood and organs. Piglets born from oronasally inoculated gilts did not present antibodies against BVDV-2 at birth, although they were acquired by passive maternal transfer. In contrast, intrauterine-inoculated piglets were born with high antibody titers (80 to 640) against the agent, which remained high until the end of the experimental period. Microscopically, no noticeable changes were observed. Macroscopically, 29.5% of the total piglets euthanized, from both inoculation groups, were born with a low cerebellar:brain ratio. Nevertheless, some piglets had a high cerebellar:brain ratio, indicating the need for standardizing this value. Thus, it was concluded that BVDV is not an etiological agent for congenital swine tremor.

Multiple congenital encephalic malformations in a calf

Multiple congenital malformations of the central nervous system are uncommonly described in domestic animals. A 7-days-old, female, Holstein calf was submitted for necropsy with a history of opisthotonus and permanent lateral recumbence. Clinical signs included apathy, abdominal breathing, occasional flexion of the forelimbs, and decreasing of the superficial and deep sensibility in all limbs. Necropsy and histopathology revealed cerebellar hypoplasia, stenosis of the mesencephalic aqueduct, fusion of the rostral and caudal colliculi, secondary hydrocephaly, villous hypertrophy of the choroid plexus and heterotopy of cerebellar cellular components. Immunohistochemical labeling for the antibody anti-BVDV in fragments of brain resulted negative, suggesting that this virus did not participate in the pathogenesis of these malformations.(AU)

Multiple congenital encephalic malformations in a calf

Multiple congenital malformations of the central nervous system are uncommonly described in domestic animals. A 7-days-old, female, Holstein calf was submitted for necropsy with a history of opisthotonus and permanent lateral recumbence. Clinical signs included apathy, abdominal breathing, occasional flexion of the forelimbs, and decreasing of the superficial and deep sensibility in all limbs. Necropsy and histopathology revealed cerebellar hypoplasia, stenosis of the mesencephalic aqueduct, fusion of the rostral and caudal colliculi, secondary hydrocephaly, villous hypertrophy of the choroid plexus and heterotopy of cerebellar cellular components. Immunohistochemical labeling for the antibody anti-BVDV in fragments of brain resulted negative, suggesting that this virus did not participate in the pathogenesis of these malformations.

Variante de Dandy Walker: reporte de un caso / Dandy-Walker variant: Case report

Introducción: La variante de Dandy Walker se define como una hipoplasia variable del vermis cerebeloso, con o sin aumento de la fosa posterior y sin elevación del tentorio. Objetivo: Describir el caso de una enfermedad poco frecuente y hacer énfasis en la necesidad de precisar la etiología de malformaciones prenatales en niños que son clasificados erróneamente como parálisis cerebral secundaria a asfixia, así como su manejo multidisciplinario. Caso clínico: Paciente varón, de 8 años de edad, con antecedentes de parálisis cerebral infantil, epilepsia y retraso del desarrollo, que fue ingresado por historia de convulsiones tónico-clónicas. Durante su hospitalización presentó múltiples episodios convulsivos, controlados con anticonvulsivantes. Se realizó tomografía computarizada, observándose comunicación entre la cisterna magna y el cuarto ventrículo; este último aumentado de tamaño. Además, el vermis del cerebelo presentaba hipoplasia parcial, siendo estos hallazgos compatibles con una variante del síndrome Dandy Walker. Conclusión: La variante de Dandy Walker puede ser sintomática o asintomática, y las imágenes encontradas no necesariamente se relacionan con las alteraciones del desarrollo, debido a los múltiples síndromes y alteraciones cromosómicas vinculadas a este cuadro. La presentación clínica y el pronóstico dependen de las alteraciones presentes. Por ello, es importante un manejo multidisciplinario considerando que el tratamiento depende de los síntomas presentados.

Introduction: Dandy Walker variant is defined by a variable hypoplasia of the cerebellar vermix with or without posterior fossa increase and without tentorium elevation. Objective: describe the case of a rare disease and emphasise the need to clarify the aetiology of prenatal malformations, as well as its multidisciplinary management. Case report: A male patient, 8 years of age, with a history of Infantile Cerebral Palsy and epilepsy, who was admitted with a history of tonic-clonic seizures. He was admitted due to psycho-motor developmental delay. During his hospitalisation, he had multiple seizure episodes, controlled with anticonvulsants. A computerized tomography was performed, in which communication was observed between the cisterna magna and fourth ventricle (the latter increased in size). In addition, the cerebellar vermix showed a partial hypoplasia. All these findings were compatible with a variant of the Dandy Walker syndrome. Conclusion: Dandy Walker variant may be asymptomatic and the images found may not indicate them as the cause of developmental disorders, due to its association with multiple syndromes and chromosomal abnormalities. Clinical presentation and prognosis depends on the related disorders, and a multidisciplinary approach is important, because the treatment depends on the symptoms presented.

Lissencephaly-pachygyria and cerebellar hypoplasia in a calf / Lisencefalia-paquigiria e hipoplasia cerebelar em um bovino

A case of lissencephaly-pachygyria and cerebellar hypoplasia diagnosed in a Charolais x Tabapuã calf is described. The calf presented since birth, clinical signs characterized by apathy, prolonged recumbency, tremors of the head and neck, ataxia, hypermetria, difficulty walking, blindness and swelling of the joints of the four limbs. Due to the unfavorable prognosis, the animal was euthanized and necropsied at 34 days of age. At necropsy, a rudimentary development of the brain folds (gyri) and grooves (sulci) was observed, and the cerebellum was hypoplastic. The cut surface of the brain exhibited thickening of the gray matter (pachygyria) in the frontal, parietal, temporal and occipital cortices and narrowing of the white matter. In the organs of the thoracic and abdominal cavities, no significant lesions were observed. Histologically, cerebral cortex was thick and exhibited neuronal disorganization of the gray matter. The cerebellum had a thin molecular layer, and neuronal disorganization with ectopia of the Purkinje neurons in the region of the granular and molecular layers. There were no bacterial growths in cultures of joint swabs. This was the only case on the property, which suggests that this malformation, which has not previously been described in cattle, was a sporadic case, and it was not possible to determine its cause. Neurological lesions and clinical sings presented here should be considered in the differential diagnosis of congenital diseases of the central nervous systems of cattle.(AU)

Descreve-se um caso de lisencefalia-paquigiria e hipoplasia cerebelar diagnosticado em um bovino, macho, cruza Charolês x Tabapuã que apresentava, desde o nascimento, sinais clínicos caracterizados por apatia, decúbito prolongado, tremores da cabeça e do pescoço, ataxia, hipermetria, dificuldade na marcha, cegueira e aumento de volume nas articulações dos quatro membros. Devido ao prognóstico desfavorável, o bovino foi eutanasiado e necropsiado aos 34 dias de idade. Na abertura da caixa craniana, observou-se formação rudimentar dos giros do telencéfalo e cerebelo hipoplásico. Ao corte o encéfalo, apresentava espessamento da substância cinzenta (paquigiria) do córtex frontal, parietal, temporal e occipital e estreitamento da substância branca. Nos órgãos das cavidades torácica e abdominal, não foram observadas lesões significativas. Histologicamente, no córtex cerebral havia desorganização neuronal da substância cinzenta que estava espessa. No cerebelo, havia diminuição da camada molecular e desorganização neuronal com ectopia dos neurônios de Purkinje na região das camadas granular e molecular. Não houve crescimento bacteriano das culturas de suabes das articulações. O fato de ser o único caso na propriedade sugere que a malformação, sem descrição anterior em bovinos, trata-se de caso esporádico, não sendo determinada sua causa. As alterações neurológicas aqui observadas devem ser levadas em consideração no diagnóstico diferencial de enfermidades congênitas do sistema nervoso central de bovinos.(AU)

Lissencephaly-pachygyria and cerebellar hypoplasia in a calf / Lisencefalia-paquigiria e hipoplasia cerebelar em um bovino

A case of lissencephaly-pachygyria and cerebellar hypoplasia diagnosed in a Charolais x Tabapuã calf is described. The calf presented since birth, clinical signs characterized by apathy, prolonged recumbency, tremors of the head and neck, ataxia, hypermetria, difficulty walking, blindness and swelling of the joints of the four limbs. Due to the unfavorable prognosis, the animal was euthanized and necropsied at 34 days of age. At necropsy, a rudimentary development of the brain folds (gyri) and grooves (sulci) was observed, and the cerebellum was hypoplastic. The cut surface of the brain exhibited thickening of the gray matter (pachygyria) in the frontal, parietal, temporal and occipital cortices and narrowing of the white matter. In the organs of the thoracic and abdominal cavities, no significant lesions were observed. Histologically, cerebral cortex was thick and exhibited neuronal disorganization of the gray matter. The cerebellum had a thin molecular layer, and neuronal disorganization with ectopia of the Purkinje neurons in the region of the granular and molecular layers. There were no bacterial growths in cultures of joint swabs. This was the only case on the property, which suggests that this malformation, which has not previously been described in cattle, was a sporadic case, and it was not possible to determine its cause. Neurological lesions and clinical sings presented here should be considered in the differential diagnosis of congenital diseases of the central nervous systems of cattle.

Descreve-se um caso de lisencefalia-paquigiria e hipoplasia cerebelar diagnosticado em um bovino, macho, cruza Charolês x Tabapuã que apresentava, desde o nascimento, sinais clínicos caracterizados por apatia, decúbito prolongado, tremores da cabeça e do pescoço, ataxia, hipermetria, dificuldade na marcha, cegueira e aumento de volume nas articulações dos quatro membros. Devido ao prognóstico desfavorável, o bovino foi eutanasiado e necropsiado aos 34 dias de idade. Na abertura da caixa craniana, observou-se formação rudimentar dos giros do telencéfalo e cerebelo hipoplásico. Ao corte o encéfalo, apresentava espessamento da substância cinzenta (paquigiria) do córtex frontal, parietal, temporal e occipital e estreitamento da substância branca. Nos órgãos das cavidades torácica e abdominal, não foram observadas lesões significativas. Histologicamente, no córtex cerebral havia desorganização neuronal da substância cinzenta que estava espessa. No cerebelo, havia diminuição da camada molecular e desorganização neuronal com ectopia dos neurônios de Purkinje na região das camadas granular e molecular. Não houve crescimento bacteriano das culturas de suabes das articulações. O fato de ser o único caso na propriedade sugere que a malformação, sem descrição anterior em bovinos, trata-se de caso esporádico, não sendo determinada sua causa. As alterações neurológicas aqui observadas devem ser levadas em consideração no diagnóstico diferencial de enfermidades congênitas do sistema nervoso central de bovinos.

[Dandy-Walker variant: Case report]. / Variante de Dandy Walker: reporte de un caso.

INTRODUCTION: Dandy Walker variant is defined by a variable hypoplasia of the cerebellar vermix with or without posterior fossa increase and without tentorium elevation. OBJECTIVE: describe the case of a rare disease and emphasise the need to clarify the aetiology of prenatal malformations, as well as its multidisciplinary management. CASE REPORT: A male patient, 8 years of age, with a history of Infantile Cerebral Palsy and epilepsy, who was admitted with a history of tonic-clonic seizures. He was admitted due to psycho-motor developmental delay. During his hospitalisation, he had multiple seizure episodes, controlled with anticonvulsants. A computerized tomography was performed, in which communication was observed between the cisterna magna and fourth ventricle (the latter increased in size). In addition, the cerebellar vermix showed a partial hypoplasia. All these findings were compatible with a variant of the Dandy Walker syndrome. CONCLUSION: Dandy Walker variant may be asymptomatic and the images found may not indicate them as the cause of developmental disorders, due to its association with multiple syndromes and chromosomal abnormalities. Clinical presentation and prognosis depends on the related disorders, and a multidisciplinary approach is important, because the treatment depends on the symptoms presented.

Lissencephaly and cerebellar hypoplasia in a goat / Lisencefalia e hipoplasia cerebelar em caprino

A case of lissencephaly and cerebellar hypoplasia was observed in a 30-day-old goat. The goat presented with sternal recumbence, absence of a menace response, intention tremors, ataxia, and nystagmus. The goat was euthanized and necropsied after having been hospitalised for eleven days. At necropsy, the surface of the brain was found to be smooth, the cerebral sulci and gyri were absent, and the cerebellum was reduced in size. Histologically, the grey matter and white matter were thicker and thinner than normal in cortices, respectively. The neurons were randomly arranged in the grey matter. In the cerebellum, the layers were disorganised, and cells were heterotopics. The histologic and gross lesions observed in this animal are characteristic of lissencephaly associated with cerebellar hypoplasia. The presence of a single goat affected suggests that the malformation was not of infectious origin and because lissencephaly is a malformation not previously described in goats, it is unlikely this case was inherited.

Um caso de lisencefalia e hipoplasia cerebelar é descrito em um caprino com 30 dias de idade. No exame físico, apresentava decúbito esternal permanente, ataxia, ausência do reflexo de ameaça, tremores de intenção e nistagmo. Após 11 dias de internamento, o caprino foi eutanasiado e necropsiado. Na necropsia, o cérebro não apresentava giros nem sulcos e o cerebelo estava reduzido de tamanho. Histologicamente, em todo o córtex cerebral, a substância cinzenta estava mais espessa e a substância branca mais fina que o normal. Os neurônios estavam distribuídos de forma aleatória na substância cinzenta. No cerebelo, as camadas estavam desorganizadas, com localização heterotópica das células. Os achados macroscópicos e histológicos são característicos de lisencefalia e hipoplasia cerebelar. A presença de um só animal afetado sugere que a malformação não é de origem infecciosa e o fato da lisencefalia nunca ter sido descrita antes em caprinos sugere que não é uma doença hereditária.

Lissencephaly and cerebellar hypoplasia in a goat

A case of lissencephaly and cerebellar hypoplasia was observed in a 30-day-old goat. The goat presented with sternal recumbence, absence of a menace response, intention tremors, ataxia, and nystagmus. The goat was euthanized and necropsied after having been hospitalised for eleven days. At necropsy, the surface of the brain was found to be smooth, the cerebral sulci and gyri were absent, and the cerebellum was reduced in size. Histologically, the grey matter and white matter were thicker and thinner than normal in cortices, respectively. The neurons were randomly arranged in the grey matter. In the cerebellum, the layers were disorganised, and cells were heterotopics. The histologic and gross lesions observed in this animal are characteristic of lissencephaly associated with cerebellar hypoplasia. The presence of a single goat affected suggests that the malformation was not of infectious origin and because lissencephaly is a malformation not previously described in goats, it is unlikely this case was inherited.

Um caso de lisencefalia e hipoplasia cerebelar é descrito em um caprino com 30 dias de idade. No exame físico, apresentava decúbito esternal permanente, ataxia, ausência do reflexo de ameaça, tremores de intenção e nistagmo. Após 11 dias de internamento, o caprino foi eutanasiado e necropsiado. Na necropsia, o cérebro não apresentava giros nem sulcos e o cerebelo estava reduzido de tamanho. Histologicamente, em todo o córtex cerebral, a substância cinzenta estava mais espessa e a substância branca mais fina que o normal. Os neurônios estavam distribuídos de forma aleatória na substância cinzenta. No cerebelo, as camadas estavam desorganizadas, com localização heterotópica das células. Os achados macroscópicos e histológicos são característicos de lisencefalia e hipoplasia cerebelar. A presença de um só animal afetado sugere que a malformação não é de origem infecciosa e o fato da lisencefalia nunca ter sido descrita antes em caprinos sugere que não é uma doença hereditária.

Lissencephaly and cerebellar hypoplasia in a goat

A case of lissencephaly and cerebellar hypoplasia was observed in a 30-day-old goat. The goat presented with sternal recumbence, absence of a menace response, intention tremors, ataxia, and nystagmus. The goat was euthanized and necropsied after having been hospitalised for eleven days. At necropsy, the surface of the brain was found to be smooth, the cerebral sulci and gyri were absent, and the cerebellum was reduced in size. Histologically, the grey matter and white matter were thicker and thinner than normal in cortices, respectively. The neurons were randomly arranged in the grey matter. In the cerebellum, the layers were disorganised, and cells were heterotopics. The histologic and gross lesions observed in this animal are characteristic of lissencephaly associated with cerebellar hypoplasia. The presence of a single goat affected suggests that the malformation was not of infectious origin and because lissencephaly is a malformation not previously described in goats, it is unlikely this case was inherited.

Um caso de lisencefalia e hipoplasia cerebelar é descrito em um caprino com 30 dias de idade. No exame físico, apresentava decúbito esternal permanente, ataxia, ausência do reflexo de ameaça, tremores de intenção e nistagmo. Após 11 dias de internamento, o caprino foi eutanasiado e necropsiado. Na necropsia, o cérebro não apresentava giros nem sulcos e o cerebelo estava reduzido de tamanho. Histologicamente, em todo o córtex cerebral, a substância cinzenta estava mais espessa e a substância branca mais fina que o normal. Os neurônios estavam distribuídos de forma aleatória na substância cinzenta. No cerebelo, as camadas estavam desorganizadas, com localização heterotópica das células. Os achados macroscópicos e histológicos são característicos de lisencefalia e hipoplasia cerebelar. A presença de um só animal afetado sugere que a malformação não é de origem infecciosa e o fato da lisencefalia nunca ter sido descrita antes em caprinos sugere que não é uma doença hereditária.

HIPOPLASIA CEREBELAR E PORENCEFALIA EM BOVINOS CHAROLÊS NO SUL DO RIO GRANDE DO SUL

Two cases of cerebellar hypoplasia in Charolais calves from a herd of a farm located in the municipality of Capão do Leão, Southern Brazil are described. One calf with cerebellar clinical signs at birth, showed incoordination, hypermetria and intention tremors, mainly in the neck and head. The other calf was recumbent with opisthotonos. The gross lesions were characterized by cerebellar hypoplasia, with only remnants of cerebellar floculus and parafloculus and an expanded fluid-filled space covered by leptomeninges. Mild hydrocephalus and porencephaly were observed in the cerebrum. It was discussed the possible etiologies and suggested the possibility of the disease has been caused by bovine viral diarrhea-mucosal disease (BVD-MD) virus despite of negative results of imuno-histochemical technique for detection of this virus in cerebellar fragments of the affected calves.

Descrevem-se dois casos de hipoplasia cerebelar congênita observados em terneiros da raça Charolês, pertencentes a um estabelecimento localizado no município de Capão do Leão. Um dos animais apresentava sinais clínicos cerebelares ao nascimento, caracterizados por severa incoordenação motora, hipermetria e tremores de intenção, principalmente da cabeça e do pescoço. O outro animal não conseguia manter-se em estação e apresentava opistótono acentuado. As lesões macroscópicas caracterizaram-se por hipoplasia cerebelar, observando-se apenas remanescentes das regiões do flóculo e do paraflóculo cerebelares e uma cavidade preenchida por líquido e recoberta pelas leptomeninges, que estavam aderidas aos resquícios das folhas cerebelares. Ao corte do encéfalo, havia hidrocefalia discreta e a presença de cavidades císticas (porencefalia). São discutidas as possíveis etiologias, sugerindo-se a possibilidade de que a enfermidade tenha sido causada pelo vírus da diarréia viral bovina-doença das mucosas, apesar dos resultados negativos da imuno-histoquímica realizada em fragmentos do cerebelo dos animais doentes para detecção desse vírus.

Hipoplasia cerebelar e porencefalia em bovinos charolês no sul do rio grande do sul

Two cases of cerebellar hypoplasia in Charolais calves from a herd of a farm located in the municipality of Capão do Leão, Southern Brazil are described. One calf with cerebellar clinical signs at birth, showed incoordination, hypermetria and intention tremors, mainly in the neck and head. The other calf was recumbent with opisthotonos. The gross lesions were characterized by cerebellar hypoplasia, with only remnants of cerebellar floculus and parafloculus and an expanded fluid-filled space covered by leptomeninges. Mild hydrocephalus and porencephaly were observed in the cerebrum. It was discussed the possible etiologies and suggested the possibility of the disease has been caused by bovine viral diarrhea-mucosal disease (BVD-MD) virus despite of negative results of imuno-histochemical technique for detection of this virus in cerebellar fragments of the affected calves.

Descrevem-se dois casos de hipoplasia cerebelar congênita observados em terneiros da raça Charolês, pertencentes a um estabelecimento localizado no município de Capão do Leão. Um dos animais apresentava sinais clínicos cerebelares ao nascimento, caracterizados por severa incoordenação motora, hipermetria e tremores de intenção, principalmente da cabeça e do pescoço. O outro animal não conseguia manter-se em estação e apresentava opistótono acentuado. As lesões macroscópicas caracterizaram-se por hipoplasia cerebelar, observando-se apenas remanescentes das regiões do flóculo e do paraflóculo cerebelares e uma cavidade preenchida por líquido e recoberta pelas leptomeninges, que estavam aderidas aos resquícios das folhas cerebelares. Ao corte do encéfalo, havia hidrocefalia discreta e a presença de cavidades císticas (porencefalia). São discutidas as possíveis etiologias, sugerindo-se a possibilidade de que a enfermidade tenha sido causada pelo vírus da diarréia viral bovina-doença das mucosas, apesar dos resultados negativos da imuno-histoquímica realizada em fragmentos do cerebelo dos animais doentes para detecção desse vírus.