Saturday, February, 11, 2012

Spinal muscular atrophy

Table of Contents

Alternative Names

Werdnig-Hoffmann disease


Treatment

There is no treatment for the progressive weakness caused by the disease. Supportive care is important. Attention must be paid to the respiratory system because affected people have difficulty protecting themselves from choking. Breathing complications are common.

Physical therapy is important to prevent contractions of muscles and tendons and abnormal curvature of the spine (scoliosis). Bracing may be necessary.


Support Groups


Expectations (prognosis)

The lifespan in SMA type I is seldom longer than 2 - 3 years. Survival time with type II is longer, but the disease kills most of those affected while they are still children. Children with type III disease may survive into early adulthood. However, people with all forms of the disease have worsening weakness and debility.


Complications
  • Aspiration
  • Contractions of muscles and tendons
  • Respiratory infections
  • Scoliosis

Calling your health care provider

Call your health care provider if your child:

  • Appears weak
  • Develops any other symptoms of spinal muscular atrophy
  • Has difficulty feeding

Breathing difficulty can rapidly become an emergency condition.


Review Date: 03/09/2010
Reviewed By: 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.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)