Dataset Information

Noninvasive Assessment of Neuromuscular Disease in Dogs: Use of the 6-minute Walk Test to Assess Submaximal Exercise Tolerance in Dogs with Centronuclear Myopathy.

ABSTRACT: BACKGROUND:Noninvasive methods of quantitating exercise tolerance in dogs with neuromuscular disease are needed both for clinical and research use. The 6-minute walk test (6MWT) has been validated as a reliable test of exercise tolerance in dogs with pulmonary and cardiac disease, but not in dogs with neuromuscular disease. HYPOTHESIS/OBJECTIVES:Distance walked and number of steps taken during 6MWT will differ between Labrador retriever dogs with centronuclear myopathy (CNM) and control (ie, healthy) littermates. ANIMALS:Eight purebred Labrador retrievers were drawn from a purpose-bred research colony (status: 3 clear, 2 carrier, and 3 homozygous mutants for the protein tyrosine phosphatase-like A (PTPLA) gene mutation associated with CNM). METHODS:Pilot, prospective, Case-controlled study. Researchers were blinded to disease status. Each dog was leash-trained and acclimatized to the testing area (length, 12.8 m). At the start of testing, each animal was fitted with a pedometer, a timer was started, and dogs were allowed to walk at their own pace for 6 minutes. Distance walked and pedometer readings were recorded. RESULTS:Degree of paresis varied among affected dogs, and was reflected by significant differences in distance walked between CNM-affected dogs and those with clear and carrier genotypes (P = .048). Pedometer readings did not vary according to genotype (P = .86). CONCLUSIONS:The 6MWT appears to differentiate between the ambulatory capacity of normal and CNM-affected dogs. Additional studies are needed to confirm this relationship in a larger number of dogs, and to evaluate the ability of the 6MWT to differentiate between dogs with variable severity of neuromuscular disease-associated exercise intolerance.

SUBMITTER: Cerda-Gonzalez S

PROVIDER: S-EPMC4913584 | biostudies-literature | 2016 May

REPOSITORIES: biostudies-literature

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Noninvasive Assessment of Neuromuscular Disease in Dogs: Use of the 6-minute Walk Test to Assess Submaximal Exercise Tolerance in Dogs with Centronuclear Myopathy.

Cerda-Gonzalez S S Talarico L L Todhunter R R

Journal of veterinary internal medicine 20160325 3

<h4>Background</h4>Noninvasive methods of quantitating exercise tolerance in dogs with neuromuscular disease are needed both for clinical and research use. The 6-minute walk test (6MWT) has been validated as a reliable test of exercise tolerance in dogs with pulmonary and cardiac disease, but not in dogs with neuromuscular disease.<h4>Hypothesis/objectives</h4>Distance walked and number of steps taken during 6MWT will differ between Labrador retriever dogs with centronuclear myopathy (CNM) and c ...[more]

PMID: 27012153

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Project description:Centronuclear myopathy (CNM) is an inherited neuromuscular disorder characterised by clinical features of a congenital myopathy and centrally placed nuclei on muscle biopsy.The incidence of X-linked myotubular myopathy is estimated at 2/100000 male births but epidemiological data for other forms are not currently available.The clinical picture is highly variable. The X-linked form usually gives rise to a severe phenotype in males presenting at birth with marked weakness and hypotonia, external ophthalmoplegia and respiratory failure. Signs of antenatal onset comprise reduced foetal movements, polyhydramnios and thinning of the ribs on chest radiographs; birth asphyxia may be the present. Affected infants are often macrosomic, with length above the 90th centile and large head circumference. Testes are frequently undescended. Both autosomal-recessive (AR) and autosomal-dominant (AD) forms differ from the X-linked form regarding age at onset, severity, clinical characteristics and prognosis. In general, AD forms have a later onset and milder course than the X-linked form, and the AR form is intermediate in both respects.Mutations in the myotubularin (MTM1) gene on chromosome Xq28 have been identified in the majority of patients with the X-linked recessive form, whilst AD and AR forms have been associated with mutations in the dynamin 2 (DNM2) gene on chromosome 19p13.2 and the amphiphysin 2 (BIN1) gene on chromosome 2q14, respectively. Single cases with features of CNM have been associated with mutations in the skeletal muscle ryanodine receptor (RYR1) and the hJUMPY (MTMR14) genes.Diagnosis is based on typical histopathological findings on muscle biopsy in combination with suggestive clinical features; muscle magnetic resonance imaging may complement clinical assessment and inform genetic testing in cases with equivocal features. Genetic counselling should be offered to all patients and families in whom a diagnosis of CNM has been made.The main differential diagnoses include congenital myotonic dystrophy and other conditions with severe neonatal hypotonia.Management of CNM is mainly supportive, based on a multidisciplinary approach. Whereas the X-linked form due to MTM1 mutations is often fatal in infancy, dominant forms due to DNM2 mutations and some cases of the recessive BIN1-related form appear to be associated with an overall more favourable prognosis.

Dataset Information

Noninvasive Assessment of Neuromuscular Disease in Dogs: Use of the 6-minute Walk Test to Assess Submaximal Exercise Tolerance in Dogs with Centronuclear Myopathy.

Publications

Noninvasive Assessment of Neuromuscular Disease in Dogs: Use of the 6-minute Walk Test to Assess Submaximal Exercise Tolerance in Dogs with Centronuclear Myopathy.

Similar Datasets

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