SciELO - Scientific Electronic Library Online

vol.85 issue5Psychosocial adjustment and physical health in children of divorce author indexsubject indexarticles search
Home Pagealphabetic serial listing  

Services on Demand




Related links


Jornal de Pediatria

Print version ISSN 0021-7557On-line version ISSN 1678-4782


STREHLE, Eugen-Matthias. Long-term management of children with neuromuscular disorders. J. Pediatr. (Rio J.) [online]. 2009, vol.85, n.5, pp.379-384. ISSN 0021-7557.

OBJECTIVE: Duchenne muscular dystrophy is the commonest genetic myopathy but there exist a large number of inherited neuromuscular diseases which individually are very rare and where clinical information is not widely available. This review is based on the author's experience in a pediatric muscle clinic and provides practical guidance and treatment plans for frequently encountered problems. SOURCES: A MEDLINE search was conducted to retrieve recent articles relevant to the management of children with inherited myopathies and neuropathies. A patient cohort (n = 200) was evaluated using descriptive statistics. SUMMARY PF THE FINDINGS: Duchenne muscular dystrophy accounted for almost half of the diagnoses, followed by spinal muscular atrophy (12%), Becker muscular dystrophy and myotonic dystrophy (7% each). Sixteen patients (9%) had an unknown myopathy. CONCLUSIONS: As with other chronic illnesses, these patients should be regularly reviewed by health professionals from an early age to increase life expectancy and improve quality of life. It is useful for physicians to take a structured approach when looking after children with neuromuscular disorders and to monitor all affected organ systems.

Keywords : Neuromuscular disorders; myopathy; neuropathy; children; management; follow-up.

        · abstract in Portuguese     · text in English | Portuguese     · English ( pdf epdf ) | Portuguese ( pdf epdf )


Creative Commons License All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License