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Atrofia muscular espinal - Treatment

Nombres alternativos

Enfermedad de Werdnig-Hoffmann

Tratamiento:

No hay tratamiento para la debilidad progresiva causada por la enfermedad. Los cuidados complementarios son importantes. Se debe prestar atención al sistema respiratorio debido a que las personas afectadas tienen dificultad para protegerse a sí mismas de la asfixia. Las complicaciones respiratorias son comunes.

La fisioterapia es importante para prevenir contracciones de los músculos y tendones y la curvatura anormal de la columna (escoliosis). Puede ser necesario el uso de dispositivos ortopédicos (corsé).

Expectativas (pronóstico):

El período de vida en la atrofia muscular espinal tipo I rara vez es mayor a 2 a 3 años. El tiempo de supervivencia con el tipo II es mayor, pero la enfermedad mata a la mayoría de las personas afectadas cuando todavía son niños. Los niños con el tipo III de la enfermedad pueden sobrevivir hasta comienzos de la edad adulta. Sin embargo, las personas con todas las formas de la enfermedad sufren de debilidad y flojera cada vez mayor.

Complicaciones:

  • Aspiración
  • Contracciones de músculos y tendones
  • Infecciones respiratorias
  • Escoliosis

Situaciones que requieren asistencia médica:

Llame al médico si su hijo:

  • Parece débil
  • Desarrolla algún otro síntoma de una atrofia muscular espinal
  • Tiene dificultad al alimentarse

La dificultad respiratoria puede convertirse rápidamente en una situación de emergencia.

  • Reviewed last on: 3/9/2010
  • Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine; Daniel B. Hoch, PhD, MD, Assistant Professor of Neurology, Harvard Medical School, Department of Neurology, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Referencias

Kliegman RM, Behrman RE, Jenson HB, Stanton BF. The hip. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Elsevier; 2007:chap 606.

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