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Atrofia muscular espinal - Overview

Nombres alternativos

Enfermedad de Werdnig-Hoffmann

Definición:

Es un grupo de enfermedades hereditarias que provocan degeneración y debilidad muscular progresiva, que finalmente llevan a la muerte.

Causas, incidencia y factores de riesgo:

La atrofia muscular espinal (AME) o amiotrofia espinal es un grupo de diferentes enfermedades musculares. Tomadas en grupo, es la segunda causa principal de enfermedad neuromuscular. La mayor parte del tiempo, una persona tiene que heredar el gen defectuoso de ambos padres para estar afectado. Aproximadamente 4 de cada 100,000 personas padecen la afección.

La forma más grave es la atrofia muscular espinal tipo I, también llamada enfermedad de Werdnig-Hoffman. Los bebés con atrofia muscular espinal tipo II tienen síntomas menos intensos a comienzos del período de lactancia, pero se debilitan de forma progresiva con los años. La atrofia muscular espinal tipo III es la forma menos grave de la enfermedad.

La atrofia muscular espinal puede aparecer en la adultez en ocasiones poco frecuentes, usualmente como una forma más leve de la enfermedad.

Los antecedentes familiares de este tipo de atrofia son un factor de riesgo para todos los tipos de este trastorno.

  • 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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