PubMed Health⌕ Search

Biomedical subjects

A de Lahunta

Publications and source records attributed to A de Lahunta.

At least 55 records · Page 3Linked to original sources
PubMed1992

Canine X-linked muscular dystrophy as an animal model of Duchenne muscular dystrophy: a review.

Canine X-linked muscular dystrophy is a spontaneously occurring, progressive, degenerative myopathy of dogs that is clinically and pathologically similar to Duchenne muscular dystrophy in man. The molecular basis for the disease has been shown to be a lack of dystrophin, the protein product of the Duchenne muscular dystrophy gene. Breeding colonies of dystrophic dogs have been established. This report reviews the findings of genetic, clinical, pathologic, molecular biologic, and immunocytochemical studies of the canine model, and compares the features of the canine disease to those of Duchenne dystrophy in man.

Animals↗
PubMed1991

Sarcocystis neurona n. sp. (Protozoa: Apicomplexa), the etiologic agent of equine protozoal myeloencephalitis.

Sarcocystis neuronan n. sp. is proposed for the apicomplexan taxon associated with myeloencephalitis in horses. Only asexual stages of this parasite presently are known, and they are found within neuronal cells and leukocytes of the brain and spinal cord. The parasite is located in the host cell cytoplasm, does not have a parasitophorous vacuole, and divides by endopolygeny. Schizonts are 5-35 microns x 5-20 microns and contain 4-40 merozoites arranged in a rosette around a prominent residual body. Merozoites are approximately 4 x 1 micron, have a central nucleus, and lack rhoptries. Schizonts and merozoites react with Sarcocystis cruzi antiserum but not with Caryospora bigenetica. Toxoplasma gondii, Hammondia hammondi, or Neospora caninum antisera in an immunohistochemical test.

Animals↗
PubMed1990

Feline sphingolipidosis resembling Niemann-Pick disease type C.

A 9-week old domestic short-hair kitten with progressive neurological dysfunction had histopathological lesions consistent with a lysosomal storage disease. Light microscopy of the brain, spinal cord, and ganglia revealed distention and vacuolation of many neuronal populations, and extensive neuroaxonal dystrophy. Large numbers of foamy macrophages were observed in the liver, spleen, lymph nodes, and lung. Hepatocytes appeared pale and swollen. Ultrastructural examination of all affected tissues and organs revealed heterogeneous membranous inclusions. Lipid analysis of liver revealed an excess of cholesterol, glucosylceramide, lactosylceramide and phospholipids including sphingomyelin. There was some increase in the levels of brain GM2 and GM3 gangliosides. Sphingomyelinase activity in liver was partially deficient or low normal. Skin fibroblasts were cultured from two affected cats from the colony established with littermates of the subject of this report. The cultured skin fibroblasts had partially decreased sphingomyelinase activity and a greatly decreased ability to esterify exogenous cholesterol. Clinical, morphological, and biochemical findings suggest that this cat had sphingolipidosis similar to human Niemann-Pick disease type C, a disease not previously described in the cat. The feline form of this storage disease may provide a useful model for studies on the human disease.

Animals↗
PubMed1990

Canine X-linked muscular dystrophy: morphologic lesions.

Gross pathologic lesions and light microscopic and ultrastructural features of skeletal muscle lesions in canine X-linked muscular dystrophy (CXMD) were studied in dogs from 3 months to 6 years of age. Necrosis and regeneration were present at all ages, but were most prominent in the youngest dogs studied. Increased intracytoplasmic calcium, as evidenced by positive alizarin red S staining, was associated with fiber necrosis, but was also seen in small numbers of otherwise normal fibers. Progressive changes included development of severe fiber size variation, endomysial and perimysial fibrosis, prominent cytoplasmic disorganization, internalization of myonuclei, mitochondrial proliferation, mild fat infiltration, and alterations in the fiber-type pattern. The most consistent early ultrastructural changes were dilatation of the sarcoplasmic reticulum and focal subsarcolemmal areas of degeneration. Convincing sarcolemmal defects were not found. Z-band streaming was present at all ages, and Z-band duplication and nemaline rods were seen in older dogs. Evidence for abnormal regeneration was found in the oldest dog, and was associated with extensive fibrosis. These findings document the progression of lesions in CXMD, and illustrate the profound alterations in fiber organization and fiber type that may occur in late stages of dystrophin-deficient muscular dystrophy.

Aging↗
PubMed1990

Equine motor neuron disease; a preliminary report.

A spontaneous motor neuron disease or neuronopathy was identified in 10 horses from the northeastern United States. Signs of generalized weakness, muscle fasciculations, muscle atrophy and weight loss progressed over 1 to several months in young and old horses of various breeds. Pathologic studies revealed that degeneration and loss of motor neurons in the spinal cord and brain stem resulted in axonal degeneration in the ventral roots and peripheral and cranial nerves and denervation atrophy of skeletal muscle. Many spinal neurons were swollen, chromatolytic and contained neurofilamentous accumulations. Other cell bodies were shrunken and undergoing neuronophagia and some were lost and replaced by glia. This fatal equine motor neuron disease has not been reported previously and its cause has not been determined. The progressive weakness and wasting and the neuronal degenerative changes in these horses were similar to those described in people with sporadic amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease.

Animals↗
PubMed1990

Abiotrophy in domestic animals: a review.

This review of abiotrophies in domestic animals has been organized by the predominate anatomical location of the lesion. Secondary considerations include the major signs of the clinical disorder and special neuropathological features. Those abiotrophies that have an established genetic basis are identified but the review includes degenerative disorders in which the etiology is not yet established.

Animals↗
PubMed1989

Atlantoaxial subluxation and absence of transverse ligament of the atlas in a dog.

Absence of the transverse ligament of the atlas was diagnosed at necropsy in an 8-month-old Shih Tzu with radiographic signs of atlantoaxial subluxation. Symmetric ataxia, tetraparesis, and signs of pain in the vertebral canal suggested a lesion in the cervical portion of the spinal cord. Necropsy revealed absence of the transverse ligament of the atlas and malformation of dens and atlas. In addition, the alar ligaments were distinct and thick, and the atlanto-occipital and atlantoaxial joint capsules were markedly thicker than normal. Histologic examination revealed focal compressive myelopathy of the spinal cord at the level of the atlantoaxial joint.

Animals↗
PubMed1989

Idiopathic epilepsy in three gaur (Bos gaurus).

Idiopathic epilepsy was diagnosed in 3 gaur in a zoologic park collection during a 3-year period. Seizures were characterized by tonic rigidity of all limbs. Results of serum biochemical analysis, CBC, blood lead analysis, CSF analysis, and necropsy were within normal limits in all gaur. The role of heredity in development of seizures in these gaur is unclear because of limited founder stock of gaur in North America.

Animals↗
PubMed1989

Focal spinal muscular atrophy in two German shepherd pups.

Focal spinal muscular atrophy evolved rapidly in two German Shepherd pups 2 weeks after birth. By 1 month, the male and female littermates had developed a valgus deformity of the right forelimb, a flexed carpus, and arm and forearm muscle weakness and atrophy. In the male, less severe left forelimb weakness and wasting also occurred, and bilateral forelimb denervation potentials were recorded. Postmortem study of the male revealed asymmetric loss and degeneration of motoneurons in the cervical spinal cord intumescence. Degenerating neurons appeared vacuolated or chromatolytic. Chromatolysis was often peripheral and resulted from dispersion and loss of the free and attached ribosomes which normally from Nissl bodies. Although this focal neuronopathy was unlike motoneuron diseases described previously in animals, it resembled the asymmetric and unilateral, benign spinal muscular atrophies found in humans.

Animals↗
PubMed1989

Subclinical lumbar polyradiculopathy in aged domestic, laboratory, and exotic mammalian species--a light and selected electron microscopic study.

Lumbar polyradiculopathy, characterized by ballooning myelin sheaths and axonal distortion, was documented in multiple, aged, domestic, laboratory, and exotic mammalian species. Animals studied that exhibited this nerve rootlet change included the goat, sheep, pig, white-tailed deer, gerbil, vole, ferret, beaver, hedgehog, chinchilla, North Chinese leopard, lion, cheetah, mountain lion, llama, East African eland, Uganda giraffe, polar bear, and drill. Ultrastructurally, lumbar nerve rootlets from the North Chinese leopard revealed prominent and often multiple lamellar separations in the area of myelin bubbling. Clefts in myelin sheaths contained macrophages with engulfed fragments of myelin. Axons were sometimes variously attenuated, and the axoplasm contained densely packed neurofilaments. In all species, the lesion was subclinical and considered an incidental age-related finding.

Aging↗
PubMed1989

Occipitoatlantoaxial malformation with duplication of the atlas and axis in a half Arabian foal.

An unusual occipitoatlantoaxial malformation is described in a 2-week-old male part Arabian foal that was unable to stand at birth and showed signs of spastic tetraparesis due to a cervical spinal cord compression. There were 2 atlases present. One was fused to the occipital bones. The other articulated with the first atlas and an axis which had a long dens that projected into the vertebral canal. Examination of the ossification centers of the axis indicated partial duplication of that bone.

Abnormalities, Multiple↗
PubMed1988

Cerebellar infarction caused by arterial thrombosis in a dog.

A cerebellar infarction was found to be caused by a vascular thrombosis in a dog. The cause of the thrombosis was not determined. The dog had signs of a central vestibular disturbance. Scintigraphic imaging of the brain revealed evidence of increased uptake of the radionucleotide within the cranial vault. Analysis of CSF revealed albuminocytologic dissociation. Histologically, there was severe, locally extensive, well-demarcated hemorrhagic necrosis within the left cerebellar hemisphere. A mural thrombus was evident within a large meningeal artery. Although thrombosis of cerebrospinal vessels is rare in the dog, it should be considered in the differential diagnosis as a cause of acute CNS dysfunction.

Animals↗
PubMed1988

Morphology and embryological interpretation of a congenital occipito-atlanto-axial malformation in a dog.

A clinical, radiological, and morphological study of a congenital occipito-atlanto-axial malformation in a 13-week-old male Saint Bernard dog that became suddenly tetraplegic at 8 weeks is described. The dog was recumbent, had generalized muscle atrophy, but was alert and responsive. Pain was elicited when the head-neck junction and the cervical vertebrae were palpated, and a bony abnormality was palpated at the occiput and atlas. Clinical signs of upper motor neuron and general proprioceptive deficits in all four limbs were compatible with a focal lesion in the cervical spinal cord. Plain radiographs of the head and neck revealed malformation of the occipital bones, atlas, and axis, unilateral atlanto-occipital fusion, and atlanto-axial subluxation. At necropsy the right half of the atlantal neural arch was fused to the right exoccipital bone. On the axis, the dens was small, malformed, and deviated to the left; the transverse processes were enlarged; and the spinous process was small with a cleft caudally. The spinal cord was severely compressed at the level of the atlanto-axial articulation, and histological examination revealed extensive loss of neuronal cell bodies, axons, myelin, and the central canal. Reactive astrogliosis was also extensive. After a discussion of normal and abnormal development of the vertebral column and its joints, it was concluded that a failure of normal joint development at about 30 days of gestation in the dog could lead to congenital occipito-atlanto-axial malformation.

Animals↗
PubMed1988

Canine protozoan polyradiculoneuritis.

Four pups in a litter of eight Labrador Retrievers suddenly developed hind limb weakness. In three, paralysis ascended rapidly resulting in quadriplegia, cervical weakness, dysphagia and death. Postmortem examination revealed a severe polyradiculoneuritis in which roots, ganglia, and spinal and cranial nerves were heavily infiltrated by lymphocytes, plasma cells and macrophages and contained abundant protozoan pseudocysts. On sections of the brain and spinal cord protozoa were less frequent and appeared independent of the glial nodules which marked focal areas of necrosis. The organisms initially were thought to be Toxoplasma gondii, but this supposition was not supported by serological, immunocytochemical, or electron microscopic findings. Ultrastructurally the organisms resembled an unidentified sporozoan parasite, which has been reported in the CNS of dogs in Scandinavia. The inflamed spinal roots contained many degenerated and demyelinated axons. Electron microscopic studies indicated that the tachyzoite-like organisms, through their invasive and proliferative activities, brought about many of the degenerative changes in the Schwann cells and axons of the spinal roots and nerves.

Animals↗
PubMed1988

Suprasellar germ cell tumors in the dog: a report of five cases and review of the literature.

Suprasellar germ cell tumors were identified in five young adult to middle-aged dogs. Clinical signs included depression, mydriasis, ptosis and visual deficit. At necropsy large extramedullary masses were found on the ventral surface of the rostral brain stem. Histologically four were characterized by sheets and nests of moderately pleomorphic round cells resembling seminoma, admixed with larger vacuolated cells, glandular formations with secretory material, and occasional foci of squamous differentiation. The fifth case was more homogeneous, with germinomatous elements predominating. Immunohistochemical examination of three tumors revealed positive staining for alpha fetoprotein. Although only one case showed intracranial metastasis, these tumors were considered malignant due to the marked local invasion and destruction. The veterinary literature is reviewed, and we propose that two tumors previously reported as craniopharyngioma be reclassified as germ cell tumors. One other tumor previously reported as an unclassified suprasellar tumor was identified, which also demonstrated features of a germ cell tumor.

Animals↗
PubMed1988

A study of cerebellar and cerebral cortical degeneration in miniature poodle pups with emphasis on the ultrastructure of Purkinje cell changes.

In a litter of three Miniature Poodles, a normal female pup contrasted with two males which displayed severe equilibrium and postural disturbances soon after birth. In the male pups diffuse cerebral cortical degeneration accompanied cerebellar cortical atrophy, which was characterized by extensive loss and degeneration of Purkinje neurons. As in some prior histological descriptions of canine cerebellar atrophies, degenerating Purkinje cells appeared either pale, swollen and vacuolated or shrunken and hyperchromatic. Swollen vacuolated cell bodies and condensed necrotic neurons were scattered through the cerebral cortex. Ultrastructural studies revealed that the vacuolar degeneration in Purkinje and cerebral neurons was referable to marked dilation of endoplasmic reticulum and loss of ribosomes. In shrunken Purkinje neurons, increased cytoplasmic eosinophilia was associated with a decline in Nissl bodies and accumulations of mitochondria and especially lamellar bodies. The latter, stacked derivatives of endoplasmic reticulum, were not encountered in shrunken cerebral perikarya. Lamellar bodies reached giant proportions in the dendritic stems of degenerating Purkinje neurons. In Purkinje axons, however, these bodies often were overshadowed by aggregates of axoplasmic tubules. The cytological changes in these Poodle pups were notably different from those reported in earlier ultrastructural studies of canine inherited cerebellar degenerations.

Animals↗
PubMed1988

Canine X-linked muscular dystrophy. An animal model of Duchenne muscular dystrophy: clinical studies.

The progression of clinical disease and serum creatine kinase (CK) levels in canine X-linked muscular dystrophy (CXMD) was studied in 7 dogs from birth to 12-14 months and in 18 dogs at varying intervals from birth to 8 weeks. One affected male was studied from age 3.5 to 6 years, and all pups were descendants of this dog. A lethal neonatal form was recognized in some pups. In the more typical form, clinical signs of stunting, weakness and gait abnormalities were evident by 6-9 weeks and were progressive, leading to marked muscle atrophy, fibrosis and contractures by 6 months. Serum CK levels were markedly elevated, such that affected pups could be identified by 1 week. CK values increased until 6-8 weeks, then plateaued at approx. 100 times normal. Affected females and beagle-cross dogs were less severely affected than large breed-cross dogs. In the 2 adult dogs with cardiac insufficiency CK levels had decreased to 5-15 times normal. These studies show that CXMD and Duchenne muscular dystrophy have striking phenotypic as well as genotypic similarities. In addition, these studies of CXMD suggest that in females and in smaller dogs the same genetic defect results in a less severe clinical disease.

Age Factors↗