Análise observacional de aquisições motoras em crianças portadoras de Amiotrofia Espinal Tipo I submetidas à intervenção medicamentosa com ácido valpróico / Observatory analyse of motor gain in type I spinal amyotrophy children with valproic acid use

AUTOR(ES)

Erika Christina Gouveia da Conceição

DATA DE PUBLICAÇÃO

2008

RESUMO

The spinal muscle atrophy (SMA) is the most frequent neuromuscular disease responsible for Hypotonic Children Syndrome (HCS). SMA is caused by anterior horn cells degeneration in the narrow. The neurological impairment is quickly and strongly established in the first months of life, so, after few months, the only movement that the children can do is moving the eyes. The Valproic acid is a drug easy to buy and to manage, and seems to avoid exon 7 deletion, which could prolong the neurons life. By this new therapeutic knowledge, and for dont exist a validate and easy to reproduce way to evaluate the motor function, there were made a functional evaluation protocol to follow the group of patients with SMA and other patients with HCS, with the purpose to qualify and to quantify the motor gain in these patients, creating a new form to evaluate, using film to exam in the end. Its easy to apply in the office and can became the only way to evaluate the results in different trials. There were evaluate five patients with SMA type 1, ages from 04 to 18 months, without respiratory assistance use, underwent to Valproic acid intervention. They were evaluated in five visits, in a total 2 years and a half of following. The motor acquisitions were made from the film and scores. The results showed that no one off al children presented a motor loss, which would be expected for the natural evolution of SMA type 1. Consequently, the patients that underwent to valproic acid trial presented a tendency to gain motor abilities when compared to the natural evolution of the disease and the film as an analyze method seem to be an efficient way to follow the motor evolution of these patients.

ASSUNTO(S)

amiotrofia espinal do tipo i movimentação. aquisição motora amiotrofia espinal spinal muscle atrophy type i motor acquisition valproic acid therapeutic use avaliação motora motor evaluation movements spinal muscle atrophy Ácido valpróico/uso terapêutico

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