Canine cerebellar cortical abiotrophy in two mixed breed littermate

Abstract

La abiotrofia neuronal es una degeneración prematura de neuronas debida a una anomalía hereditaria del metabolismo celular, que comienza en los primeros meses de vida. La aparición de los signos ocurre semanas o incluso años más tarde, y el déficit es progresivo. El objetivo del trabajo fue describir 2 casos de abiotrofia en caninos hermanos mestizos. Un macho y una hembra de 45 días de una camada de 4 fueron examinados debido a temblores y ataxia de los 4 miembros. Los cachorros enfermos exhibían ampliación de la base de sustentación, pérdida del equilibrio, vocalización, inclinación de la cabeza hacia la izquierda, reacciones posturales enlentecidas y respuesta a la amenaza disminuída. Cuando fueron incapaces de mantenerse en estación se llevaron a cabo la eutanasia e inmediata necropsia. Se tomaron muestras para estudios histopatológico. Los análisis morfométrico (número de células de Purkinje) y lectinhistoquímico se llevaron a cabo en tejidos afectados y en un control normal. Se observó reducción del tamaño cerebelar y significativa disminución del número de células de Purkinje, que se observaron tumefactas y eosinófilas. Las lectinas no mostraron afinidad por componentes citoplasmáticos. Los diagnósticos diferenciales fueron excluidos debido al curso, examen clínico y análisis histopatológico, lectinhistoquímico y de imágenes.

Neuronal abiotrophy is an inherited premature degeneration of neurons due to an abnormality of the cell metabolism, which often begins in the first months of life. Onset of signs is progressive and occurs weeks or even years later. The aim of the work was to describe 2 cases of canine abiotrophy in mixed breed littermates. Two 45-day-old male and female puppies from a litter of four were examined due to muscle tremors and ataxia. Affected puppies exhibited ataxia, wide based stance, loss of balance, vocalization and head tilt to the left side. Postural reactions were slowed down and there was loss of menance response. When inability to stand worsened, euthanasia and necropsy were performed. Samples were collected for histopathological studies. Number of Purkinje cells was calculated by means of morphometric analysis. Lectinhistochemistry was performed in affected and normal tissues. Reduction in cerebellar size was observed. Comparing to control tissues, a marked decrease in Purkinje cells was found. They were eosinophilic and swollen. Differences in cellular count (normal/death) in normal and affected cerebellum were significant. None of the lectins tested showed affinity for cytoplasmic components. Differential diagnoses were excluded owing to course, clinical, pathological, image analysis and lectinhistochemical findings.