Spongy degeneration is seen in prion diseases and in a variety of progressive, invariably lethal neurodegenerative disorders. In various dog breeds it can involve the white or the grey matter. Amongst those involving the gray matter, cases are described in Bull Mastiff, Rottweiler, Saluki, Cocker Spaniel and Australian Cattle dog. A hereditary spongy degeneration has been reported in Malinois puppies, which suffer from congenital tremors with ataxia. In this report we describe two cases of spongy degeneration in Malinois puppies, emphasizing the morphological and immunohistochemical features of the CNS lesions. A 4-week-old female intact Belgian Malinois puppy was presented for mild depression, tremors of the head, cerebellar ataxia, and progressive ascending paraparesis. Complete blood count and serum biochemistry were normal. Serology for canine distemper virus, Toxoplasma spp. and Neospora spp. was negative. Cerebrospinal fluid showed no abnormalities. Because of the progressive ataxia and development of proprioceptive deficits on all limbs, and poor prognosis, the owners elected for humane euthanasia. No gross lesions were found in the body, including the brain. Brain, spinal cord, and samples of major organ were fixed in 4% buffered formaldehyde. Transverse sections of brain and spinal cord were embedded in paraffin, sectioned at 5 µm, and stained with hematoxylin and eosin, Luxol fast bleu. Immunohistochemistry for CNPase, Olig2, glial fibrillary acidic protein (GFAP), phosphorylated neurofilaments , heat shock protein (HSP) 70, ubiquitin, … were performed on selected sections. Lesions were restricted to the brain and were characterized by the presence of bilateral and symmetric large interneuronal vacuoles in the area of cerebellar nuclei, granular cell layer and foliate white matter of the cerebellum. Lesions detected in these puppies are similar to previously described cases (Cachin et al., 1991, Kleiter et al., 2011); the predominant lesions were found in cerebellar nuclei and flocculonodular cerebellar cortex, whereas spongy degeneration was not detected in the cerebral cortex. These are the first cases of spongy polioencephalopathy of Malinois dogs described in Italy, and the first time that immunohistochemical features are reported.
Spongy polioencephalopathy in two Belgian Malinois puppies
SALVADORI, CLAUDIA;CANTILE, CARLO
2014-01-01
Abstract
Spongy degeneration is seen in prion diseases and in a variety of progressive, invariably lethal neurodegenerative disorders. In various dog breeds it can involve the white or the grey matter. Amongst those involving the gray matter, cases are described in Bull Mastiff, Rottweiler, Saluki, Cocker Spaniel and Australian Cattle dog. A hereditary spongy degeneration has been reported in Malinois puppies, which suffer from congenital tremors with ataxia. In this report we describe two cases of spongy degeneration in Malinois puppies, emphasizing the morphological and immunohistochemical features of the CNS lesions. A 4-week-old female intact Belgian Malinois puppy was presented for mild depression, tremors of the head, cerebellar ataxia, and progressive ascending paraparesis. Complete blood count and serum biochemistry were normal. Serology for canine distemper virus, Toxoplasma spp. and Neospora spp. was negative. Cerebrospinal fluid showed no abnormalities. Because of the progressive ataxia and development of proprioceptive deficits on all limbs, and poor prognosis, the owners elected for humane euthanasia. No gross lesions were found in the body, including the brain. Brain, spinal cord, and samples of major organ were fixed in 4% buffered formaldehyde. Transverse sections of brain and spinal cord were embedded in paraffin, sectioned at 5 µm, and stained with hematoxylin and eosin, Luxol fast bleu. Immunohistochemistry for CNPase, Olig2, glial fibrillary acidic protein (GFAP), phosphorylated neurofilaments , heat shock protein (HSP) 70, ubiquitin, … were performed on selected sections. Lesions were restricted to the brain and were characterized by the presence of bilateral and symmetric large interneuronal vacuoles in the area of cerebellar nuclei, granular cell layer and foliate white matter of the cerebellum. Lesions detected in these puppies are similar to previously described cases (Cachin et al., 1991, Kleiter et al., 2011); the predominant lesions were found in cerebellar nuclei and flocculonodular cerebellar cortex, whereas spongy degeneration was not detected in the cerebral cortex. These are the first cases of spongy polioencephalopathy of Malinois dogs described in Italy, and the first time that immunohistochemical features are reported.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
