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1.
Hypertrophic muscular dystrophy was diagnosed in a 10-month-old male Rat Terrier with hypersalivation, dysphagia, gait abnormalities, and generalized weakness. Serum creatine kinase activity was high, and electromyography revealed myotonic discharges. Histologic examination of a muscle biopsy specimen revealed muscle fiber degeneration, clusters of basophilic regenerating fibers, and endomysial fibrosis. Staining for dystrophin, a sarcolemmal protein, was decreased, compared with that in muscle specimens from clinically normal dogs. Treatment with mexilitene hydrochloride and procainimide hydrochloride resulted in temporary improvement in clinical signs, but the disease became refractory to treatment, and the dog was euthanatized. Clinical and histologic characteristics of this dystrophin deficiency-related muscular dystrophy were similar to those of X-linked muscular dystrophy in dogs, hypertrophic muscular dystrophy in cats, and Duchenne muscular dystrophy in humans.  相似文献   

2.
A mature female Rhodesian Ridgeback was determined to have a progressive, degenerative myopathy associated with myotonia, dysphagia, and marked muscle wasting. Clinical findings revealed a diffuse muscular disease with percussion dimpling, dysphagia, and creatine kinase elevation. A paroxysmal atrial tachycardia was found. Electromyography revealed a diffuse myopathy with high-frequency bizarre waves, myotonic discharges especially in the masticatory, laryngeal, and pharyngeal muscles. A few positive sharp waves were found in some of the appendicular muscles. Histopathologic and histochemical stains on skeletal muscle biopsy specimens demonstrated moderate fiber-size variation, myofiber architectural changes, muscle-fiber splitting, focal necrosis and phagocytosis, high percentage of internal nuclei, and atrophy of type-2 muscle fibers. A review of myotonic myopathies in the dog is presented. The clinical, electrophysiologic, and histochemical findings are similar to those for myotonic muscular dystrophy in man.  相似文献   

3.
Determining the anatomic and functional origin for dysphagia is critical for development of an appropriate therapeutic plan and determination of the prognosis. The purpose of this retrospective study was to report the quantitative and qualitative outcome of contrast videofluoroscopic swallowing studies in a large cohort of dysphagic dogs presenting to a tertiary veterinary care hospital. The videofluoroscopic swallowing studies were reviewed to generate values for pharyngeal constriction ratio, timing of swallowing events (maximum pharyngeal contraction, opening of upper esophageal sphincter, closing of upper esophageal sphincter, and reopening of epiglottis), type of esophageal peristalsis generated, and esophageal transit time. One or more anatomic locations for origin of dysphagia were assigned (pharyngeal, cricopharyngeal, esophageal (primary motility disorder), other esophageal (stricture, vascular ring anomaly, mass), lower esophageal sphincter/hiatus. Sixty‐one of 216 studies (28%) were deemed unremarkable. Twenty‐seven of 216 dogs (13%) had pharyngeal dysphagia, 17/216 dogs (8%) had cricopharyngeal dysphagia, 98/216 dogs (45%) had dysphagia secondary to esophageal dysmotility, 19/216 dogs (9%) had dysphagia secondary to focal esophageal disorders, and 97/216 dogs (45%) had dysphagia of lower esophageal sphincter/hiatus origin. Multiple abnormalities were present in 82/216 (38%) dogs. Elevated pharyngeal constriction ratio was associated with pharyngeal, cricopharyngeal, and esophageal motility disorders, delayed upper esophageal sphincter opening was associated with cricopharyngeal disorders, a lower percentage of primary esophageal peristaltic waves was associated with cricopharyngeal, pharyngeal, or primary esophageal motility disorders. In conclusion, videofluoroscopic swallowing studies was pivotal in the diagnosis of dysphagia with 155/216 (72%) dogs receiving a final diagnosis.  相似文献   

4.
本文旨在探讨VE缺乏对鲤骨骼肌损伤的形态学影响。在半纯合日粮中分别添加0、25、50和100 mg/kg的VE,投喂体重约60 g的鲤鱼20周。结果表明:鲤鱼摄入VE缺乏的饲料后,出现“瘦背症”、脊柱弯曲及以突眼、竖鳞和腹水为特征的渗出性素质样病变。病理剖解见体壁肌肉萎缩,特别是背部两侧肌肉萎缩变薄,其厚度仅为对照组的1/4~1/2,似刀刃状;体侧红肌纤维褪色变白,呈白肌肉外观。组织学变化主要表现为肌营养性不良,骨骼肌变性、坏死,淋巴细胞和单核细胞浸润,大量结缔组织增生,残存的肌纤维萎缩变细。超微结构上表现为骨骼肌细胞核膜间隙增宽,呈锯齿状,并发生局部或广泛性的断裂,核变形,电子密度降低;肌浆内糖原颗粒明显减少,线粒体肿胀,嵴断裂,甚至溶解呈空囊泡状,肌原纤维的横纹模糊不清或完全消失。结论:日粮中0、25和50 mg/kgVE水平会导致鲤出现VE缺乏症,其骨骼肌主要表现为变性、坏死、肌萎缩和炎症细胞浸润的肌营养性不良。  相似文献   

5.
Sex-linked muscular dystrophy associated with dystrophin deficiency has been reported in several breeds of dogs and is best characterized in the golden retriever. In this case report, a young, male Labrador retriever with dystrophin-deficient muscular dystrophy is presented. Clinical signs included generalized weakness, lingual hypertrophy, and dysphagia. Electromyographic abnormalities including complex repetitive discharges were present. Serum creatine kinase concentration was dramatically elevated. Histopathological changes within a muscle biopsy specimen confirmed a dystrophic myopathy, and dystrophin deficiency was demonstrated by immunohistochemical staining. While X-linked muscular dystrophy has not previously been reported in the Labrador retriever, a hereditary myopathy with an autosomal recessive mode of inheritance has been characterized. A correct diagnosis and classification of these two disorders are critical for breeders and owners since both the mode of inheritance and the prognosis differ.  相似文献   

6.
Cinefluorography and videofluorography were used to record and analyze functional swallowing deficits of 12 dogs with spontaneously occurring oropharyngeal dysphagias and six experimental dogs with selected neurectomies. Ten of the 12 dogs had dysphagias affecting the cricopharyngeal stage of the oropharyngeal phase of swallowing. Two dogs had mixed oropharyngeal dysphagias. Clinical signs of cricopharyngeal dysphagia could not be differentiated from those of dysphagias due to pharyngeal or mixed oropharyngeal deficits. Signs of cricopharyngeal dysphagia consisted of: 1) repeated attempts to swallow; 2) excessive head movement; 3) dropping food from the mouth after unsuccessful swallowing attempts; 4) reingestion of dropped food. Nine of these dogs had cinefluorographic evidence of asynchrony between the normal pharyngeal contraction and relaxation, and subsequent cricopharyngeal relaxation and contraction. Only one dog demonstrated a consistent cricopharyngeal non-opening (achalasia). Seven of the dogs responded dramatically to cricopharyngeal myotomy. Two dogs with mixed oropharyngeal dysphagias had poor contractility of the pharyngeal muscles in addition to cricopharyngeal dysphagia. Clinical and cinefluorographic evaluation following cricopharyngeal myotomy of one dog verified exacerbation of functional deficits due to the iatrogenic cricopharyngeal chalasia. Esophagopharyngeal reflux accentuated the contrast medium retention in the pharynx and laryngotracheal aspiration. The need was stressed for careful differentiation between cricopharyngeal dysphagia and dysphagias involving the pharyngeal stage. Four experimental dogs with selective bilateral neurectomies of branches of the glossopharyngeal (IX) and vagus (X) nerves were evaluated clinically and cinefluorographically in an attempt to identify the pathogenesis of cricopharyngeal dysphagia. The variable results in the four dogs and the observed recovery in two dogs suggested that peripheral motor nerve deficits are not a major cause of cricopharyngeal dysphagia. Glossopharyngeal neurectomy in two dogs induced a profound functional disorder involving the pharyngeal and cricopharyngeal stages and the esophageal phase of swallowing. This would support a new hypothesis that the glossopharyngeal nerve is sensory to the esophagus as well as the pharynx, and may play a major role in disorders of the pharynx, upper esophageal sphincter, and esophagus, including congenital or acquired megaesophagus.  相似文献   

7.
A 2-year-old, male Weimaraner with muscular dystrophy was presented with generalized muscle atrophy of the limbs; hypertrophy of the neck, infraspinatus, and lingual muscles; dysphagia; and regurgitation. Unilateral cryptorchidism, unilateral renal agenesis, and hiatal hernia were also detected. Spontaneous muscle activity was identified on myography. Serum creatine kinase was markedly elevated. Immunohistochemical staining for dystrophin was restricted to suspected revertant (characteristics of immaturity) fibers. Histologically, skeletal myofiber degeneration, endomysial fibrosis, and mineralization were present. Following euthanasia, necropsy revealed hypertrophy of the diaphragm and cardiac muscle fibrosis. This case of muscular dystrophy represents a slowly progressive form with organ agenesis.  相似文献   

8.
The most common form of muscular dystrophy in dogs and humans is caused by mutations in the dystrophin gene. The dystrophin gene is located on the X chromosome, and, therefore, disease-causing mutations in dystrophin occur most often in males. Therefore, females with dystrophin deficiency or other forms of muscular dystrophy may be undiagnosed or misdiagnosed. Immunohistochemistry was used to analyze dystrophin and a number of other muscle proteins associated with muscular dystrophy in humans, including sarcoglycans and laminin alpha2, in muscle biopsy specimens from 5 female dogs with pathologic changes consistent with muscular dystrophy. The female dogs were presented with a variety of clinical signs including generalized weakness, muscle wasting, tremors, exercise intolerance, gait abnormalities, and limb deformity. Serum creatine kinase activity was variably high. One dog had no detectable dystrophin in the muscle; another was mosaic, with some fibers normal and others partly dystrophin-deficient. A 3rd dog had normal dystrophin but no detectable laminin alpha2. Two dogs could not be classified. This study demonstrates the occurrence of dystrophin- and laminin alpha2-associated muscular dystrophy and the difficulty in clinical diagnosis of these disorders in female dogs.  相似文献   

9.
A skeletal myopathy is found in approximately 100% of rasH2 mice. To confirm detailed features of the rasH2 skeletal myopathy, the biceps femoris, diaphragm, triceps brachii, gastrocnemial (types I and II fiber-mixed muscles) and soleus muscle (type I fiber-dominant muscle) obtained from male rasH2 and non-transgenic littermates aged 10-13 and 34 weeks were examined. Variations in the muscle fiber size, early-scattered degeneration/necrosis and regeneration of muscle fibers were detected in 10-13-week-old rasH2 mice. The severity of the above muscular lesions was more prominent in older rasH2 mice. These lesions were noted in the type II myofiber dominant muscles (biceps femoris, triceps brachii and gastrocnemial). NADH-TR stain clearly demonstrated a disorganized intermyofibrillar network and necrotic change in muscle fibers. No specific morphological changes, like rod structure or tubular aggregation seen in some types of myopathy, were noted in Gomori trichrome and NADH-TR stains in the rasH2 mouse like in many types of muscular dystrophy. Electronmicroscopically, occasional muscle fiber degeneration/regeneration, invaded phagocytic cells, indistinct Z-band suggesting excessive contraction and dilatation of the sarcoplasmic reticulum were observed. In summary, the skeletal myopathy occurring in rasH2 mice is consistent with muscular dystrophy characterized morphologically by progressive degeneration and regeneration of myofibers. The myopathy is confined to the type II myofiber predominant muscles and is not associated with any pathognomonic lesions. These characteristics will provide us with a useful model for research in muscular dystrophy of diverse myofibers.  相似文献   

10.
Serum activity of alanine aminotransferase (ALT) was consistently increased in dogs with canine X-linked muscular dystrophy (CXMD), a primary myopathy characterized by profound and on-going skeletal muscle necrosis. In order to determine whether the ALT was of liver origin, serum activity of creatine kinase (CK), aspartate aminotransferase (AST), ALT, and sorbitol dehydrogenase (SDH) obtained from dystrophic dogs was compared with enzyme activity present in clinically normal dogs. In dystrophic dogs at all ages tested, serum activity of CK, AST, and ALT was increased, and significant increases were present in dogs four weeks or older. In contrast, SDH activity in dystrophic dogs was not statistically different from values in clinically normal dogs. Ultrastructural examination of liver tissue revealed no evidence of hepatic degeneration in dystrophic dogs. It was concluded that increased serum activity of ALT in the dog may be associated with severe skeletal muscle degeneration, without concurrent hepatocellular necrosis.  相似文献   

11.
Cricopharyngeal achalasia is a rare cause of dysphagia in the dog. However it must be differentiated from other causes of dysphagia as it is treatable with surgery. It is a disruption of the cricopharyngeal phase of the oropharyngeal phase of deglutition. There appears to be an incoordination in the swallowing process between the relaxation of the rostral, middle pharyngeal muscles and the caudal pharyngeal muscles. It is seen as a primary condition in young animals presenting soon after weaning onto solid food. The dogs appear clinically healthy unless there is secondary aspiration pneumonia or emaciation. These dogs may present as respiratory emergencies and require intensive support and treatment prior to corrective surgery. The diagnosis is made on videofluoroscopy. The condition carries a good prognosis for cure with surgical myectomy of the cricopharyngeal muscle and the thyropharyngeal muscle, which make up the upper oesophageal sphincter. Temporary relief prior to surgery can be achieved by injection of the cricopharyngeal muscle with botulism toxin. Surgical treatment for dysphagia secondary to an underlying neurological, neuromuscular or pharyngeal weakness carries a guarded prognosis and will make aspiration pneumonia worse.  相似文献   

12.
Two juvenile Rottweiler siblings were presented with the complaint of decreased activity and various postural abnormalities, including plantigrade and palmigrade stance and splayed forepaw digits. The neurologic examinations were otherwise normal. Electromyography revealed rare fibrillation potentials and positive sharp waves. Motor nerve conduction velocities were normal, whereas compound muscle action potentials from the interosseous muscles were decreased. These findings were consistent with a primary myopathy. A 3rd pup from a different litter and a 4th pup from a litter with 3 of 8 affected dogs had similar clinical presentations. Histopathologic changes in fresh-frozen muscle biopsy samples were similar in all pups and consisted of myofiber atrophy with mild myonecrosis, endomysial fibrosis, and replacement of muscle with fatty tissue. These changes were more severe in distal muscles than in proximal muscles. Plasma carnitine concentrations (total and free) were decreased in all pups. Muscle carnitine concentrations (total and free) were decreased in 3 of 4 pups and the least affected pup had a borderline low free muscle carnitine concentration. Abnormalities involving major metabolic pathways were not found on quantification of organic and amino acids. Dystrophin immunocytochemistry was normal in 2 dogs tested. Distal myopathies in humans are classified under the dystrophic group of muscle disorders. These 4 cases represent a form of muscular dystrophy apparently not previously reported in dogs.  相似文献   

13.
Medical records of seven dachshunds with congenital nasopharyngeal stenosis from abnormally thickened palatopharyngeal muscles were reviewed. The intrapharyngeal ostium in all cases consisted of only a narrow slit. Dogs were presented with various clinical signs--the most common being dyspnea, expiratory cheek puff, salivation, pharyngeal dysphagia, oral dysphagia (to a lesser extent), and macroglossia. Diagnostic procedures included direct pharyngeal inspection, pharyngeal and thoracic radiography, fluoroscopy, lingual ultrasonography, biopsies in two dogs, and a postmortem examination in one dog. Diagnoses were readily made with radiographs and visual examinations. Macroglossia was confirmed with transcutaneous ultrasonography or a transmandibular finger test.  相似文献   

14.
Golden Retriever (GR) muscular dystrophy is an inherited degenerative muscle disease that provides an excellent model for Duchenne muscular dystrophy in humans. This study defined the histopathologic lesions, including the distribution of type I and II muscle fibers (FTI and FTII), in 12 dystrophic and 3 nondystrophic dogs between 7 and 15 months of age. The authors were interested in studying the influence on disease phenotype from crossing the base GR breed with Yellow Labrador Retrievers. The dystrophic dogs were divided according to breed: GRs and Golden Labrador Retrievers (GLRs). On hematoxylin and eosin staining, histopathologic lesions were more severe in GRs than GLRs. Six of eight GR muscles (75%) had a severe lesion grade (grade 3). In contrast, seven GLR muscles (87.5%) had mild lesions (grade 2), and only one had severe lesions (grade 3). Changes in fiber-type distribution were more pronounced in GRs versus GLRs. FTI:FTII ratio inversion was observed in three dystrophic GRs but only one GLR. The mean diameter of FTI and FTII was smaller in GRs and GLRs than in nondystrophic dogs (P < .01). The FTI of five GR muscles (62.5%) were larger than those of GLRs, whereas only one GLR muscle was larger (P < .05). The differential was less pronounced for FTII, with four GR muscles being larger and three GLR being larger. Observations indicate that crossing the base GR breed with Labrador Retrievers lessened the severity of the GR muscular dystrophy phenotype.  相似文献   

15.
Two cases of dystrophin‐deficient muscular dystrophy in 16‐week‐old male lurcher siblings are reported. The myopathies were characterised by regurgitation, progressive weakness and muscle wastage. The dogs had generalised weakness in all four limbs, with more pronounced weakness in the pelvic limbs. Reduced withdrawal in all limbs, muscle contracture and lingual hypertrophy were noted. Serum creatine kinase activities were markedly elevated. Electromyographic abnormalities included fibrillation potentials. Histopathological and immunohistochemical staining were consistent with dystrophin‐deficient muscular dystrophy. Clinical improvement was noted in one of the cases with l ‐carnitine supplementation and supportive therapy. Genetic transmission of the disease was postulated as the dogs were siblings.  相似文献   

16.
Skeletal muscles from healthy dogs and Labrador Retrievers with hereditary muscular dystrophy were examined morphologically and histochemically and were analyzed biochemically for Na+, K+, Ca2+, Mg2+, Zn2+, Cu2+, Cl-, total muscle water, and total neutral lipid content. Flame atomic absorption spectrophotometer was used for elemental quantitation of hydrochloric acid tissue extracts. Muscle samples from dystrophic dogs contained substantially increased concentrations of Na+, Ca2+, Zn2+, Cu2+, and Cl-, and a considerable reduction in the content of K+ and Mg2+ compared with samples from healthy dogs. Total muscle water and total fat content was higher in muscles from dystrophic dogs. Most muscle samples from dystrophic dogs had a type-2 fiber deficiency and an increase in number of fibers with internalized nuclei.  相似文献   

17.
Golden retriever and Labrador retriever muscular dystrophy are inherited progressive degenerative myopathies that are used as models of Duchenne muscular dystrophy in man. Thoracic lesions were reported to be the most consistent radiographic finding in golden retriever dogs in a study where radiographs were performed at a single-time point. Muscular dystrophy worsens clinically over time and longitudinal studies in dogs are lacking. Thus our goal was to describe the thoracic abnormalities of golden retriever and Labrador retriever dogs, to determine the timing of first expression and their evolution with time. To this purpose, we retrospectively reviewed 390 monthly radiographic studies of 38 golden retrievers and six Labrador retrievers with muscular dystrophy. The same thoracic lesions were found in both golden and Labrador retrievers. They included, in decreasing frequency, flattened and/or scalloped diaphragmatic shape (43/44), pulmonary hyperinflation (34/44), hiatal hernia (34/44), cranial pectus excavatum (23/44), bronchopneumonia (22/44), and megaesophagus (14/44). The last three lesions were not reported in a previous radiographic study in golden retriever dogs. In all but two dogs the thoracic changes were detected between 4 and 10 months and were persistent or worsened over time. Clinically, muscular dystrophy should be included in the differential diagnosis of dogs with a combination of these thoracic radiographic findings.  相似文献   

18.
Two adult Boxers were evaluated because of chronic dysphagia of several years' duration. Serum creatine kinase activity was high in both dogs, but other hematologic or serum biochemical abnormalities were not detected. Esophagraphy revealed abnormalities of the cricopharyngeal phase of swallowing in both dogs, and electromyography of the pharyngeal and laryngeal muscles revealed complex repetitive discharges, positive sharp waves, and fibrillation potentials characteristic of primary myopathy or neuropathy. Because of the severity of their condition, both dogs were euthanatized. Histologically, mixed-cell infiltrates were seen in sections of the masseter and thyropharyngeal muscles. Results of indirect immunofluorescence staining for proteins associated with dystrophic myopathy were unremarkable, except for decreased staining for integrin alpha7. A diagnosis of chronic inflammatory myopathy was made. The clinical importance of reduced staining for integrin alpha7 could not be determined but was considered to be a result of the myopathy.  相似文献   

19.
The diagnostic utility of contrast‐enhanced videofluoroscopic esophagography in dysphagic cats has been rarely studied relative to dogs. Current literature regarding feline dysphagia typically consists of individual case reports or small case series. This retrospective study analyzed the imaging findings in 11 cats undergoing 15 videofluoroscopic swallow studies. Hiatal hernia (n = 5), esophageal stricture (n = 3), and esophageal dysmotility (n = 7) were the most common diagnoses (some cats having more than 1 diagnosis) in dysphagic cats that underwent videofluoroscopic swallow studies. Esophageal dysmotility appeared to be associated with a higher percentage of swallows from which no peristaltic waves were generated. Oropharyngeal and cricopharyngeal causes of dysphagia were not identified in any cat and quantitative assessment of the swallowing reflex (pharyngeal constriction ratio = 0.17 ± 0.09; time to maximum pharyngeal contraction = 0.13 ± 0.02 s; time to proximal esophageal sphincter opening = 0.07 ± 0.02 s; time to proximal esophageal sphincter closed = 0.23 ± 0.05 s; time to opening of the epiglottis = 0.27 ± 0.04 s) was similar to quantitative swallowing parameters previously reported in healthy dogs. In conclusion, videofluoroscopy is a diagnostic tool that can identify esophageal abnormalities that are not readily apparent on survey radiographs. Limitations include the potential need for multiple studies, and the possibility of poor compliance in the feline patient. Results of this study are intended to help veterinarians define a prioritized differential diagnosis list for dysphagic cats.  相似文献   

20.
A four‐month‐old female Dobermann presented with myalgia, dysphagia, progressive weakness and loss of body condition. Diagnostic evaluation at nine months of age revealed markedly elevated serum creatine kinase activity, electromyographic abnormalities and histological evidence of chronic‐active muscle necrosis. Imaging confirmed dysphagia and aspiration pneumonia. Muscular dystrophy was suspected and immunohistochemical staining of muscle cryosections demonstrated reduced sarcoglycans. Treatment consisted of gastrostomy, and over the next 5 months the dog gained weight, despite continued loss of muscle mass. The dog died at 14 months of age after developing clinical signs of aspiration pneumonia. To the authors’ knowledge, this is the first report of muscular dystrophy in a Dobermann and only the second detailed report of a canine sarcoglycanopathy. Supportive care resulted in an acceptable quality of life for 10 months after clinical signs were first observed.  相似文献   

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