Contemporary aspects of spinal muscular atrophy diagnosis and the treatment strategies in children




spinal muscular atrophy, progressive muscular weakness, disease-modifying therapy


The article presents up-to-date data on the peculiarities of the diagnosis of spinal muscular atrophy (SMA) in childhood. It has been shown that the progressive motor neurons degeneration in the spinal cord and brainstem resulting in system damage, such as progressive muscle weakness, hypotonia, muscles atrophy, developmental delay, bulbar syndrome, paradoxical breathing, heart, respiratory failure, damage to the bones and internal organs, is caused by survival of motor neuron 1 gene mutation, which codes for a full-length survival motor neuron protein necessary for motor neurons. Consequently, genetic testing is important to confirm SMA diagnosis as early as possible to prescribe treatment for the patients in time. Moreover, prescribed SMA treatment according to contemporary standards using the new disease-modifying medicines have created a turning point in SMA management and it appeared to slow disease progression and prolong motor development and ability to walk compared to the known natural history.


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