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Spinal Muscular Atrophy, Pediatric

Jessica Rose Nance, MS, MD Reviewed 10/2018
 


BASICS

DESCRIPTION

  • Spinal muscular atrophy (SMA) is a progressive disorder of motor neurons in the spinal cord and brainstem.

  • Major symptom is proximal weakness.

  • Three forms are described based on clinic...

DIAGNOSIS

HISTORY

  • Hypotonia and weakness are the primary features. Infants with SMA I will be floppy and less active and have delayed motor milestones, with preserved language/social interaction (a bri...

TREATMENT

ALERT

An apparently minor respiratory infection may carry a higher risk of respiratory failure in SMA I and later stages of SMA II and III. Depending on family/patient wishes regarding respir...

ONGOING CARE

PATIENT EDUCATION

PROGNOSIS

  • Su...

ADDITIONAL READING

  • Arnold WD, Burghes AH. Spinal muscular atrophy: development and implementation of potential treatments. Ann Neurol.  2013;74(3):348–362. [View Abstract on OvidInsights]

  • Chung BH...

CODES

ICD9

  • 335.10 Spinal muscular atrophy, unspecified

  • 335.0 Werdnig-Hoffmann disease

  • 335.11 Kugelberg-Welander disease

  • 335.19 Other spinal muscular atrophy

ICD10

  • G12.0 Infantile spinal muscular atrophy, ty...

FAQ

  • Q: Can routine vaccinations be given to children with SMA?

  • A: Yes. In addition to routine vaccinations, yearly influenza and respiratory syncytial virus (RSV) vaccinations are recommended.

  • Q: How mu...

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