Atrofia muscular espinhal proximal tipo 4
Autor: Antonio Jorge de Melo | Publicado: 2017-03-18T05:20:00-07:00

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<![endif]--> A atrofia muscular espinhal proximal tipo
4 (SMA4) é a forma de atrofia espinhal muscular proximal de apresentação no
adulto (ver este termo), caracterizada por fraqueza muscular e hipotonia
resultante da degeneração e perda dos neurónios motores inferiores da medula
espinhal e do núcleo do tronco cerebral.





·&nbsp;&nbsp;&nbsp;&nbsp;

&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp; Sinônimo: Atrofia
muscular espinhal do adulto&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp; &nbsp; &nbsp; &nbsp; &nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp; 
Hereditariedade: Autossómica
     recessiva&nbsp;
Prevalência:
1-9 / 1 000 000 Idade de início: Idade
     adultaCID-10: G12.1







&nbsp;

Epidemiologia




A
prevalência está estimada em cerca de 1/300,000. A SMA4 manifesta-se geralmente
na segunda ou terceira década de vida.



&nbsp;

Descrição
clínica 



A
fraqueza muscular afeta predominantemente as pernas e os músculos da anca e
depois progride para os ombros e braços. A marcha cambaleante é comum. Pode
ocorrer tremor dos dedos, fasciculação e hipertrofia da panturrilha. O quadro
clínico é semelhante ao observado na SMA3 (ver este termo), mas a fraqueza
motora é menos grave na SMA4.



&nbsp;

Etiologia




Tal
como as outras formas de SMA, a SMA4 tem sido associada a deleções no gene SMN1
(5q12.2-q13.3) que codifica a proteína SMN (sobrevivência do neurónio motor).
As deleções do gene SMN1 são transmitidas de forma autossómica recessiva.
Embora haja alguma variabilidade, a gravidade da doença na SMA está
inversamente relacionada com o número de cópias do segundo gene SMN ( SMN2 ;
5q13.2) e alguns estudos têm mostrado que os doentes com a forma ligeira de
SMA4 têm várias cópias (quatro a seis) do SMN2 . No entanto, não foram encontradas
mutações no gene SMN1 em alguns doentes diagnosticados com SMA4 e nestes casos
as anomalias genéticas ainda não foram identificadas.



&nbsp;

Métodos
de diagnóstico 



O
diagnóstico é baseado na história e observação clínicas. Nos doentes com
anomalias do SMN1 , o diagnóstico pode ser confirmado por teste genético. Podem
ser necessário efectuar eletromiografia e biópsia muscular.



&nbsp;

Diagnóstico
diferencial 



Os
diagnósticos diferenciais incluem a esclerose lateral amiotrófica, esclerose
lateral primária, miastenia gravis, e perturbações do metabolismo dos
carboidratos (ver estes termos).



&nbsp;

Diagnóstico
pré-natal 



O
diagnóstico pré-natal é possível para as famílias em que foram identificadas
mutações no gene SMN1 . Nestes casos deve ser fornecido aconselhamento
genético.



&nbsp;

Controlo
da doença e tratamento 



A
gestão é sintomática, envolve uma abordagem multidisciplinar, e tem como
objetivo melhorar a qualidade de vida. São recomendadas terapia ocupacional e
fisioterapia. Estão a decorrer ensaios clínicos para identificar tratamentos
com fármacos específicos para SMA, e os estudos preliminares indicaram que o
ácido valpróico (como inibidor da histona desacetilase) pode melhorar a força
muscular quantitativa e função motora subjetiva nos doentes com SMA4.



&nbsp;



&nbsp;

&nbsp;

Prognóstico






A SMA4 é a forma mais
ligeira de SMA e, em geral o curso da doença é benigno, sendo que os doentes
têm uma esperança de vida normal.







Fonte: http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=PT&amp;Expert=83420