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At least 127 records · Page 7Linked to original sources
PubMed1984

[Myocardial lesions in several forms of progressive muscular atrophy].

Using the clinical, electrocardiographic, biochemical and morphological findings, the authors assessed the cardiovascular system in 30 patients with Duchenne's myopathy and spinal amyotrophy. An analysis of the material revealed considerable metabolic-dystrophic changes in the myocardium, being more pronounced with Duchenne's myopathy. The early diagnosis of myocardial damage in the above diseases can be made on the basis of evidence from the isoenzymic spectrum of creatine kinase. Recommendations are offered on the pathogenetic therapy with due regard for myocardial involvement.

Adolescent↗
PubMedDate not supplied

[Satellite potentials in electromyograms in patients with Duchenne progressive muscular atrophy].

CN EMG study was performed in 30 patients with Duchenne Muscle Dystrophy. The frequency of Motor Unit Potential (MUP) with satellite components was reviewed. The amplitude and duration of individual components as well as the distance between main MUAP and satellite components were measured. The results were correlated with clinical data (duration of the disease, muscle force and wasting) and morphological (obtained from rectus femoris open biopsy). Satellite components were found in 34% of the MUP number. A positive correlation was found (p 0.001) between the duration of the complex MUP (main MUP with satellite components) and muscle force. There was no evident correlation between the morphological findings (muscle fibre regeneration and necrosis) and occurrence of satellite potentials. The diagnostic yield of satellite potentials in neuromuscular diseases in discussed.

Child↗
PubMed1986

Canine storage disease characterized by hereditary progressive neurogenic muscular atrophy: breeding experiments and clinical manifestation.

Progressive neurogenic muscular atrophy due to storage of a compound lipid in the lower motor neurons was diagnosed in 3 English Pointers that were littermates. Using 2 clinically normal littermates of these 3 affected dogs and 2 clinically normal dogs of the 2nd litter from the parents of the original 3 affected dogs as the initial breeding stock, a breeding experiment was performed, resulting in a breeding line of 26 dogs, 4 of which had the disease and 6 of which died before 3 months of age. Results indicated that the disease may have an autosomal recessive mode of inheritance. The clinical manifestation and electrophysiologic findings indicated lower motor neuron involvement in the affected dogs produced by breeding consistent with findings in the original 3 affected dogs. Upper motor neurons or the sensory system was not involved. The disease appeared to be distinct from other canine storage diseases previously reported.

Animals↗
PubMed2002

Mimic syndromes in sporadic cases of progressive spinal muscular atrophy.

Described are patients initially diagnosed with progressive spinal muscular atrophy (PSMA), in whom further evaluation established another diagnosis. The authors prospectively investigated incident and prevalent cases of PSMA. Seventeen of 89 patients, after initial registration, were later excluded because reassessment revealed a diagnosis other than PSMA. In 11 of the 17 patients with a revised diagnosis, a potential treatment was available: multifocal motor neuropathy (7), chronic inflammatory demyelinating polyneuropathy (2), inflammatory myopathy (1), and MG (1). Other misdiagnoses included myopathy, syringomyelia, ALS, idiopathic chronic axonal polyneuropathy, and idiopathic brachial plexus neuropathy. One patient with a possible herniated lumbar disk recovered spontaneously.

Adult↗