Bovine ceroid-lipofuscinosis (Batten's disease): The major component stored is the DCCD-reactive proteolipid,subunit c,of mitochondrial ATP synthase

The ceroid-lipofuscinoses (Batten's disease) are a group of recessively inherited lysosomal storage diseases of children and animals in which there is intracellular accumulation of a fluorescent lipopigment in a wide variety of cells. Lipopigment bodies isolated from pancreas, liver, kidney and brain tissue from a heifer affected with ceroid-lipofuscinosis contained between 55 and 62% protein. A dominant component comigrated on LDS-PAGE with the major low molecular weight protein stored in ovine ceroid-lipofuscinosis. It was identified by amino acid sequence and mass spectroscopy as the full subunit c of mitochondrial ATP synthase, normally found only in the inner mitochondrial membrane, where it is estimated to account for 2–4% of the membrane protein. In pancreatic lipopigment it accounted for at least 40% of the total lipopigment mass and this storage was considered specific to the disease. No other mitochondrial proteins were found in storage bodies. These results are similar to those found in studies on the ovine and the late infantile and juvenile human forms of the disease. It is concluded that bovine ceroid-lipofuscinosis is also a proteolipid proteinosis in which subunit c of mitochondrial ATP synthase is specifically stored in lysosome derived organelles.Abbrevations DCCD dicyclohexycarbodi-imide - LDS-PAGE lithium dodecyl sulphate polyacryl-amide gel electrophoresis - PTH phenylthiohydantoin - CsCl caesium chloride     

Bovine ceroid-lipofuscinosis: pathology of blindness

Five Devon cattle with suspected ceroid-lipofuscinosis and aged between 19 and 39 months of age were humanely slaughtered and subjected to post-mortem examination. There was severe atrophy of the cerebrum, particularly of the occipital cortex. Microscopy also showed severe atrophy of the retina with complete loss of photoreceptor cells, even in the youngest animal examined. Histopathologically the disease was characterised by accumulation of a fluorescent lipopigment in neurones, including those of the retina and a severe astrocytosis. The disease, which is characterised by the accumulation of subunit c of mitochondrial ATP synthase, is similar to that extensively described in South Hampshire sheep except that the retinal lesions were more severe. In contrast, tremors were not noted in the cattle. The clinical history and similarity to the disease in sheep and other species indicated inheritance was as an autosomal recessive trait.     

The effect of thiopental sodium, methoxyflurane and halothane on the acid-base status in sheep.

Experiments have been carried out on 20 adult fat-tailed ewes to determine the effects of thiopental sodium, methoxyflurane and halothane on acid-base status of the saliva loss during prolonged surgical anaesthesia. The rate of loss of base in saliva depends on the volume of saliva produced, which fell sharply at the onset of anesthesia with the volatile anaesthesia. Plasma pH and plasma pvCO2 excess were both increased by the volatile anaesthetics but fell sharply during thiopental anaesthesia. Plasma pH and plasma PvCO2 showed no consistent relationship.     

Generalised ceroid-lipofuscinosis and brown bowel syndrome in Cocker spaniel dogs

The ceroid-lipofuscinoses comprise a group of inherited storage diseases of human beings and animals that are characterised by progressive neurodegenerative disease and the accumulation within cells of a fluorescent lipopigment. A distinct syndrome occurs in Cocker spaniel dogs in which there is a generalised accumulation of a lipofuscin-like pigment, with such a heavy accumulation in smooth muscle that the intestine and other organs have a brown discolouration. Such discolouration is not observed in other forms of ceroid-lipofuscinosis. Dogs are clinically affected in adulthood and show progressive hind limb paresis, incoordination, and deficient postural reactions and proprioception. Spinal reflexes may be exaggerated. Behavioural or temperament changes, seizures or blindness may occur.     

Generalised ceroid-lipofuscinosis and brown bowel syndrome in Cocker spaniel dogs

The ceroid-lipofuscinoses comprise a group of inherited storage diseases of human beings and animals that are characterised by progressive neurodegenerative disease and the accumulation within cells of a fluorescent lipopigment. A distinct syndrome occurs in Cocker spaniel dogs in which there is a generalised accumulation of a lipofuscin-like pigment, with such a heavy accumulation in smooth muscle that the intestine and other organs have a brown discolouration. Such discolouration is not observed in other forms of ceroid-lipofuscinosis. Dogs are clinically affected in adulthood and show progressive hind limb paresis, into-ordination, and deficient postural reactions and proprioception. Spinal reflexes may be exaggerated. Behavioural or temperament changes, seizures or blindness may occur.     

Cortical function in cattle during slaughter: conventional captive bolt stunning followed by exsanguination compared with shechita slaughter

Brain function was examined in adult cattle after conventional captive bolt stunning or shechita slaughter, using eight animals in each treatment. The times to loss of evoked responses (visual and somatosensory) and spontaneous activity in the electro-corticogram were used to determine the onset of brain failure. Captive bolt stunning followed by sticking one minute later resulted in immediate and irreversible loss of evoked responses after the stun. Spontaneous cortical activity was lost before sticking in three animals, and in an average of 10 seconds after sticking in the remaining five animals. The duration of brain function after shechita was very variable, and particularly contrasted with captive bolt stunning with respect to the effects on evoked responses. These were lost between 20 and 126 seconds (means of 77 seconds for somatosensory and 55 seconds for visual evoked responses) and spontaneous activity was lost between 19 and 113 seconds (mean 75 seconds) after slaughter.     

Ischaemic stroke in dogs and humans: a comparative review

Cerebrovascular accidents, also known as strokes, are one of the major causes of disability and mortality among adult humans. The increased availability of magnetic resonance imaging in veterinary medicine means they are being increasingly recognised in dogs, too. Cerebrovascular accident is defined as the sudden onset of non-progressive, focal brain dysfunction as a result of ischaemic infarction or haemorrhage. Focal ischaemic stroke is caused by interruption of the arterial blood flow to a dependent area of brain parenchyma by a thrombus or an embolus. Once the diagnosis of ischaemic stroke is confirmed, potential sources of thrombosis or embolism should be investigated and treated accordingly. Dogs with ischaemic stroke tend to recover within several weeks with supportive care only.     

Histopathology of grossly normal mesenteric lymph nodes of New Zealand farmed red deer (Cervus elaphus) including identification of lipopigment

This article describes the histopathology of grossly normal mesenteric lymph nodes (MLNs) of New Zealand farmed red deer (Cervus elaphus). Eighty MLNs were sourced from 10 deer from 5 North Island herds and 5 South Island herds classified as low risk and high risk of Mycobacterium avium subspecies paratuberculosis (MAP) infection, respectively. Fixed sections were stained with hematoxylin and eosin; Ziehl-Neelsen; and, selectively, periodic acid-Schiff, Perl's, and Sudan black. Positive Ziehl-Neelsen stain, follicular hyperplasia, capsular eosinophil infiltration, focal granulomas, foci of macrophages containing lipopigment, parasitic granulomas, and calcified foci are described and severity graded where appropriate. Animal age, sex, and herd of origin are variably associated with the presence of one or more features. Trabecular fibrosis and dilated edema-filled sinusoids are described. These observations allow differentiation between likely nonpathologic histologic features in deer MLNs and features possibly attributable to infection with a pathogen such as MAP.     

Type I idiopathic non-erosive immune-mediated polyarthritis in a mixed-breed dog

A young adult dog was presented with an acute onset of stiffness, weakness, marked muscle atrophy and rapid weight loss. The dog was inappetent, lethargic, and had grossly visible distension of multiple joints. Synovial fluid analysis, routine bloodwork, and radiography confirmed immune-mediated polyarthritis; the dog responded favorably to treatment with prednisone and analgesics.     

The effect of the bont tick (Amblyomma hebraeum) on the weight gain of Africander steers

The effects of the bont tick Amblyomma hebraeum on the productivity of cattle need to be quantified in order to design economically optimal control programs. Liveweight gains (LWGs) of three groups of Africander steers, maintained in the same pasture and exposed to zero, medium or high numbers of larvae, nymphs and adults of the tick, were measured. Larvae and nymphs had no significant effect on LWG but adults had a large, statistically significant effect (P less than 0.05). The tick-free group had an average LWG of 20 kg more than the heavily infested group after 3.5 months. No mortality was recorded from ticks or tick-borne diseases during the experiment. There was no relationship between the number of engorging adult female ticks counted and loss of LWG of individual animals. The latter suggests that exposure to ticks as well as engorgement by female ticks causes losses. The loss per adult female that completed engorgement was estimated, by relating the LWGs of individual animals to their tick infestations, to be 4 +/- 2 g per adult female tick. An alternative estimate, made by comparing the average LWG and tick infestations of each treatment group, was equal to 10 +/- 4 g. This latter estimate includes the effect of challenge (and rejection) as well as tick feeding and so was accepted as giving the best estimate. Screw-worm fly (Chrysomya bezziana) struck an average of 7.5% of the cattle infested with ticks in any week but the effects on the productivity of the cattle were minimized by immediate treatment. There was a significant, positive correlation between the incidence of screw-worm fly strike and the numbers of adult ticks counted on the experimental animals. The results provide data for calculating losses caused by A. hebraeum in different parts of its geographical range.     

Responsiveness to Progestagen-eCG-Cloprostenol Treatment in Goat Food Restricted for Long Period and Refed

For 6 months, 10 adult Saanen crossbred goats were fed undernutrition diet (70% maintenance), and finally five goats were refed for 6 weeks with 150% maintenance. In all animals oestrus was synchronized using 45 mg FGA vaginal sponge for 11 days, 300 IU eCG and 50 microg cloprostenol 48 h prior to sponge removal. From oestrus onset, during a 24-h period, blood samples were collected for oestradiol and NEFA assay. Ovulation was verified by laparoscopy 3 days after sponge removal. Body mass loss was 18.62 +/- 3.03% of initial weight and in refed goats body weight recovery was 90.63 +/- 3.56%. NEFA level was higher in restricted goats (p < 0.05). Fifty per cent of underfed goats (2/4) and all refed goats (4/4) exhibited oestrus and ovulation. Significant relationship (p < 0.05) was found between weight loss and the interval sponge removal-oestrus onset (r = 0.91) or ovulation rate (r = 0.70). Only in the refed group was the ovulation rate related to the oestradiol amount (r = 0.99) (p < 0.05). Collectively results showed that a short period of improved feeding re-established the responsiveness of oestrus synchronization in chronically fasted goats.     

Congenital deafness and vestibular deficit in the dobermann

A condition characterised by the early onset of vestibular deficit and hearing loss was investigated in the dobermann breed of dog. Affected pups showed behavioural signs of head tilt, circling and ataxia and there was a total absence of vestibular response to rotation or caloric stimulation. Severe deafness, as assessed by brainstem auditory evoked response testing, was present by three weeks of age in all affected animals. The inner ears showed a progressive neuroepithelial type of cochlear degeneration with loss of the auditory sensory cells. In the vestibular system, however, there was no equivalent sensory cell loss and the only abnormal feature was the absence or abnormality of the otoconia in some of the affected animals. Pedigree analysis suggested that the condition was inherited as an autosomal recessive trait.     

Neuronal ceroid lipofuscinosis in Merino sheep

OBJECTIVE: To characterise neuronal ceroid lipofuscinosis (NCL) in Merino sheep. DESIGN: A prospective clinical, pathological, biochemical and genetic study. PROCEDURE: NCL cases were studied from a medium-wool Merino flock, the stud of origin of its replacement rams, and an experimental flock established at the University of Sydney. RESULTS: Behavioural changes and visual impairment were first detected at 7 to 12 months of age and progressed, with associated motor disturbances and at later stages seizures, to premature death by 27 months of age. At necropsy there was severe cerebrocortical atrophy associated with neuronal loss, astrocytosis and the presence in neurons of eosinophilic intracytoplasmic storage bodies with the characteristics of a lipopigment. In the retina there was progressive loss of photoreceptor cells. Storage bodies isolated from fresh brain, liver and pancreas formed electron-dense aggregates and coarse multilamellar and fine fingerprint profiles ultrastructurally, and consisted mainly of the hydrophobic protein, subunit c of mitochondrial ATP synthase. A homozygosity mapping approach localised the gene causing the disease in Merino sheep to the chromosomal region (OAR7q13-15) associated with NCL in South Hampshire sheep. CONCLUSION: NCL in Merino sheep is a subunit c-storing disease, clinically and pathologically similar to NCL in South Hampshire sheep. We propose that the disease in both breeds represents mutation at the same gene locus in chromosomal region OAR7q13-15.     

IMAGING DIAGNOSIS—NEURONAL CEROID LIPOFUSCINOSIS WITH A CHRONIC SUBDURAL HEMATOMA

A subdural hematoma was found to accompany neuronal ceroid lipofuscinosis in an 11‐month‐old Dachshund. Results from clinical, magnetic resonance (MR) imaging, histopathologic, ultrastructural, and molecular assessments are described. The dog had a 3‐month history of progressive neurologic signs. In MR images, there was severe asymmetric cerebral atrophy with a subdural hematoma. Histopathologically, there was autofluorescent, periodic acid–Schiff‐positive lipopigment in neurons and transmission electron microscopy confirmed a typical curvilinear profile of the storage bodies. We hypothesize that rapid brain atrophy contributed to the subdural hematoma formation, a complication not described previously in dogs with neuronal lipofuscinosis.     

Anti‐isthmus autoimmunity in a novel feline acquired alopecia resembling pseudopelade of humans*

Pseudopelade is a primary scarring (cicatricial) alopecia of humans characterized by lymphocyte‐rich inflammation centred around the hair follicle isthmus. Lymphocyte folliculotropism is associated with isthmus apoptosis and, ultimately, follicular destruction and dermal fibrosis. In a cat, an acquired alopecia was diagnosed as pseudopelade based on the following criteria: (i) an adult‐onset, patchy to diffuse nonpruritic hair loss; (ii) an early folliculo‐destructive phase in which lymphocytes and dendritic cells accumulated in and around the follicular isthmus; and (iii) a late stage in which the lower segments of hair follicles underwent atrophy and were replaced by fibrosing tracts. Additionally, immunological investigations characterized the cytotoxic phenotype of isthmotropic lymphocytes and demonstrated the presence of circulating IgG autoantibodies specific for multiple follicular antigens. Altogether, the results of the present study suggest an immune‐mediated pathogenesis for this case of feline pseudopelade, similarly to that causing alopecia areata in humans and other mammalian species.     

Late onset cerebellar degeneration in a middle-aged cat

Cerebellar degeneration (abiotrophy) (CD) is a spontaneous and accelerated degeneration of one or several mature cerebellar neuronal cell populations and has been described in many domestic animals, especially in dogs, with numerous breed-related cases. In cats, CD is mentioned as a rare sporadic entity. Late onset CDs are exceptionally uncommon and only two cases are reported in young adults, both aged 18 months. This report describes clinical and pathological findings of a late onset feline CD in a 9-year-old male Persian cat. The cat was presented with a history of progressive ataxia lasting 2 years. Neurological examination revealed severe neurological deficits such as generalised and severe ataxia, hypermetria in all four limbs, and bilateral absence of menace response. The lesion was diffusely localised in cerebellum. On gross pathology, the cerebellum appeared of normal size and shape and kidneys were characterised by mild hyperaemia. Histologically, lesions were limited to the cerebellum and kidneys. In the cerebellum, all cerebellar folia of both hemispheres and the vermis were affected. Changes were characterised by severe and diffuse loss of Purkinje cells, loss of cellularity in the granular layer, mild astrogliosis associated with moderate hypertrophy of Bergmann's glia. Immunohistochemistry for feline parvovirus antigen revealed a negative result. Renal lesions consisted of chronic fibrosis associated with chronic interstitial nephritis. CD is a rare disease and occurs commonly in puppies or young animals, who are clinically normal at birth and usually develop neurological signs within a few weeks or months after birth. This report represents the first case of CD in a middle-aged cat.     

Coordination of social signals and ovarian function during sexual development

Social cues, often in the form of priming pheromones, can retard or enhance the rate of sexual development in a variety of mammals. The complex interactions between the social environment and reproduction have been explored most thoroughly in the house mouse. A urinary pheromone produced by females in a group inhibits sexual development, and a urinary pheromone from adult males accelerates onset of puberty in juvenile females. These priming pheromones apparently are detected by the vomeronasal organ and induce the changes in ovarian function via changes in the hypothalamic-pituitary system. Accelerated onset of puberty is not accompanied by deficits in reproductive performance. Puberty acceleration can have important management implications for domestic farm animals. It already is proving useful for manipulation in rearing swine and in synchronizing seasonal reproductive recrudescence in sheep. In cattle, the results are less clear that signals from the bull can hasten onset of puberty in heifers. The effect may be operable only under certain nutritional or other interacting conditions. Presumably, the postpartum anestrus period in the cow can be shortened by stimulation from the bull, although pheromones have not been implicated in this effect.     

Injury of the origin of the gastrocnemius and superficial digital flexor muscles in a Thoroughbred racehorse: An atypical presentation of reciprocal apparatus failure

Damage to the origin of the gastrocnemius and superficial digital flexor muscles has been previously reported as an acute injury in both foals and adult horses. This case report describes, for the first time, the clinical onset of caudal reciprocal apparatus failure in a 2‐year‐old Thoroughbred. These signs were due to injury of the gastrocnemius and superficial digital flexor muscle origins sustained prior to the onset of lameness. This condition should be considered in cases demonstrating similar clinical signs, even in the absence of known trauma or acute injury to the region.     

Angiostrongylosis in a greyhound

Angiostrongylus vasorum infection was diagnosed at necropsy of a Greyhound imported from Ireland. Granulomatous masses and pulmonary arterial thrombosis were associated with adult and larval stages of metastrongyle nematodes. Hemorrhages, widespread in subcutaneous tissue and muscle masses and at serosal surfaces, were suggestive of the onset of the bleeding disorder that has been seen in angiostrongylosis in Europe. The mollusks that can serve as intermediate hosts are known to be common in the United States, and therefore establishment of this parasite from imported racing or breeding stock is possible.     

Recombinant human hepatitis B vaccine initiating alopecia areata: testing the hypothesis using the C3H/HeJ mouse model

Untoward effects of human vaccines suggest that recombinant hepatitis B vaccine may induce alopecia areata (AA) in some patients. Similar untoward immunological effects may also account for AA-like diseases in domestic species. In this study, the C3H/HeJ spontaneous adult onset AA mouse model was used to test the role, if any, of recombinant hepatitis B vaccine on the initiation or activation of AA. Initial experiments demonstrated no effect on induction of AA in young adult female C3H/HeJ mice ( P  = 0.5689). By contrast, older females, those at the age when AA first begins to appear in this strain, had a significant increase ( P  = 0.0264) in the time of onset of AA, suggesting that the vaccine may initiate disease in mice predisposed to AA. However, larger vaccine trials, which included diphtheria and tetanus toxoids as additional controls, did not support these initial result findings and suggest that AA associated with vaccination may be within the normal background levels of the given population.