Canine degenerative myelopathy (DM), recognized as a spontaneous model of amyotrophic lateral sclerosis, is known as a late-onset progressive degenerative disease of the spinal cord. Because of the progressive nature of DM, many dogs are elected to be euthanized, resulting in limited information on the end-stage clinical presentation. We investigated the long-term clinical course from diagnosis to natural death to further deepen our understanding of the entire clinical picture of this disease. Because curcumin was administered in some cases, the therapeutic effect of curcumin on DM was also examined. Forty dogs included in this study were client-owned Pembroke Welsh Corgis with a definitive diagnosis of DM by necropsy and histopathology. Dogs were excluded from this study if they died from another disease or were elected to be euthanized. Information on the long-term clinical symptoms of DM was investigated based on a questionnaire, which was collected from the dog owners. Urinary incontinence and respiratory disorder were observed in most dogs, as was respiratory impairment-correlated death. In contrast, signs consistent with brainstem dysfunction were noticed at the terminal stage in a small portion of dogs. Although further studies with more cases are needed, the results of this study suggest that administration of curcumin is effective in slowing the progression of DM.

Canine degenerative myelopathy (DM) is an adult-onset, chronic, progressive neurodegenerative disease reported in multiple canine breeds, including the German Shepherd Dog (GSD). Clinical signs include progressive motor neuron paralysis, which begins in the pelvic limbs and eventually leads to respiratory distress, which may necessitate euthanasia. A common DM-associated mutation is a single nucleotide substitution that causes an amino acid substitution (c.118G>A, p.E40K) in the canine SOD1 gene. This SOD1 mutation and the clinical progression rate of A/A risk genotype in the Japanese GSD population have not been analyzed before. Therefore, the aim of this study was to determine the frequency of the mutated allele and analyze the clinical progression rate in the Japanese GSD population. We studied 541 GSDs registered with the Japanese German Shepherd Dog Registration Society between 2000 and 2019. Genotyping was performed using real-time PCR with DNA extracted from the hair roots of each dog. The study revealed 330 G/G dogs (61%), 184 G/A dogs (34%), and 27 A/A dogs (5%), indicating a frequency of the mutant allele of 0.220, which are in Hardy–Weinberg equilibrium. We analyzed the clinical signs in A/A dogs with an age limit of 10 years based on information obtained from the dogs’ owners. Of the seven A/A dogs older than 10 years, owners reported DM-related clinical signs, indicating a clinical progression rate of 100%. These results, further genotyping, and thorough clinical examinations of SOD1 A/A risk genotype will help control and prevent DM in the Japanese GSD population.

Glycogen storage disease type II (Pompe disease: PD) is an autosomal recessively inherited fatal genetic disorder that results from the deficiency of a glycogen hydrolyzing enzyme, acid α-glucosidase encoded by the GAA gene. Here, we describe the molecular basis of genetic defects in an 8-month-old domestic short-haired cat with PD. The cat was previously diagnosed with PD based on the clinical and pathological findings of hypertrophic cardiomyopathy and excessive accumulation of glycogen in the cardiac muscles. Sanger sequencing was performed on 20 exons of the feline GAA gene using genomic DNA extracted from paraffin-embedded liver tissues. The affected cat was found to be homozygous for the GAA:c.1799G>A mutation resulting in an amino acid substitution (p.R600H) of acid α-glucosidase, a codon position of which is identical with three missense mutations (p.R600C, p.R600L, and p.R600H) causing human infantile-onset PD (IOPD). Several stability and pathogenicity predictors have also shown that the feline mutation is deleterious and severely decreases the stability of the GAA protein. The clinical, pathological, and molecular findings in the cat were similar to those of IOPD in humans. To our knowledge, this is the first report of a pathogenic mutation in a cat. Feline PD is an excellent model for human PD, especially IOPD.

Transforming growth factor-beta 1 (TGF-β1) plays a central role in the progression of chronic kidney disease (CKD). However, in feline CKD, renal expression of TGF-β1 and how it changes as the disease progresses have not been fully studied. In the present study, we immunohistochemically assessed the renal expression levels of TGF-β1 in cats with CKD and statistically analyzed its correlation with CKD severity. Clear immunosignals were detected in the glomerular mesangial cells, Bowman’s capsules, proximal tubules, distal nephrons, platelets, and vascular smooth muscles in the kidneys of cats with CKD. Statistically, luminal signals in the distal nephrons showed positive correlations with plasma creatinine levels and glomerulosclerosis, while those in the proximal tubules and platelets showed negative correlations with plasma urea and/or creatinine levels. Therefore, it was suggested that the changes in the renal expression of TGF-β1 could be associated with progression of feline CKD.

Salivary gland tumours are relatively uncommon and most of the tumours arise from parotid gland. Fine needle aspiration cytology (FNAC) is advised preoperatively as diagnostic tool but sometimes found to shown both false positive and false negative results. This study was aimed to find out distribution of neoplasm of major salivary glands and also to explore the sensitivity and specificity of FNAC. The present cross sectional study was done in the Dept. of Otolaryngology Head and Neck Surgery, BSMMU from January 2007 to December 2008. A total number of 60 gender-matched patients with major salivary neoplasm, confirmed by FNAC, were recruited in the study. Operated salivary gland specimens were sent for histopathological examination, histopathological findings were compared. Overall male to female ratio was 1:1. Out of 60 cases, 47 (78.3%) patients had parotid and 13 (21.7%) patients submandibular gland neoplasm. Male to female ratio for parotid tumour was1:1.1 and for submandibular 1.6:1. Mean age of the patients was 44.5 with range of 14-85 years. Of the total 60 cases 47 (78.3%) were benign and 13 (21.7%) malignant. Out of 47 parotid tumour 85.1% were benign and 14.9% malignant. Among the parotid tumour 97% were superficial lobe and 3.0% deep lobe. In case of submandibular gland 53.84% were benign and 46.15% tumour malignant. According to the sides of involvement, 25 (53.2%) cases of parotid neoplasm tumour were in the left and 22(46.8%) the right. In submandibular gland the distribution was 7 (53.8%) and 6 (46.16%) respectively. Statistically incidence of parotid tumour was significantly higher than submandibular tumour (p<0.05). Out of 60 cases in 56 (93.3%) preoperative FNAC and postoperative histopathological findings were same. There was 1.7% false positive and 5% were false negative results. Sensitivity, specificity of FNAC were 80% and 97.8% respectively. Positive predictive value was 92.3% and negative predictive 93.6% for FNAC.FNAC though cheap and safe but its diagnostic accuracy was 93.3%.Histopathologial examination remained to be of value for diagnostic confirmation of major salivary gland neoplasm. DOI: http://dx.doi.org/10.3329/bmrcb.v39i2.19645 Bangladesh Med Res Counc Bull 2013; 39: 69-73