What is Spinal Muscular Atrophy?
What is Spinal Muscular Atrophy?
- Spinal Muscular Atrophy: A rare condition characterized by progressive degeneration of the spinal and brainstem motor neurons. During fetal development excess primary neurons are formed. The body automatically destroys the extra primary neurons so that only some survive and mature into neurons. In spinal muscular dystrophy, the process that destroys the excess primary neurons doesn't switch off and continues destroying the neurons resulting in progressive motor problems. Various types of the condition range from mild to severe enough to cause death within a couple of years of birth.
Spinal Muscular Atrophy is listed as a "rare disease" by the Office of
Rare Diseases (ORD) of the National Institutes of Health
(NIH). This means that Spinal Muscular Atrophy, or a subtype of Spinal Muscular Atrophy,
affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)
Ophanet, a consortium of European partners,
currently defines a condition rare when it affects 1 person per 2,000.
They list Spinal Muscular Atrophy as a "rare disease".
Source - Orphanet
Spinal Muscular Atrophy: Introduction
Types of Spinal Muscular Atrophy:
Types of Spinal Muscular Atrophy:
Broader types of Spinal Muscular Atrophy:
How many people get Spinal Muscular Atrophy?
Prevalance of Spinal Muscular Atrophy: 1 per 6,000 - 10,000 people suffer from spinal muscular atrophy, Genetics Home Reference website
Prevalance Rate of Spinal Muscular Atrophy: approx 1 in 6,000 or 0.02% or 45,333 people in USA [about data]
Who gets Spinal Muscular Atrophy?
Gender Ratio for Spinal Muscular Atrophy: males almost exclusively
How serious is Spinal Muscular Atrophy?
Complications of Spinal Muscular Atrophy:
see complications of Spinal Muscular Atrophy
Prognosis of Spinal Muscular Atrophy:
The
prognosis for individuals with SMA varies depending on the type of SMA and
the degree of respiratory function. Some may appear to be stable for
protracted periods, but improvement is not to be expected.
(Source: excerpt from NINDS Spinal Muscular Atrophy Information Page: NINDS)
What causes Spinal Muscular Atrophy?
Causes of Spinal Muscular Atrophy: see causes of Spinal Muscular Atrophy
Cause of Spinal Muscular Atrophy: Progressive degeneration of certain spinal cord cells (anterior horn cells).
What are the symptoms of Spinal Muscular Atrophy?
Symptoms of Spinal Muscular Atrophy:
see symptoms of Spinal Muscular Atrophy
Complications of Spinal Muscular Atrophy:
see complications of Spinal Muscular Atrophy
Onset of Spinal Muscular Atrophy: prenatal
Can anyone else get Spinal Muscular Atrophy?
Contagion of genetic conditions:
generally not; see details in contagion of genetic diseases.
Inheritance:
see inheritance of Spinal Muscular Atrophy
Inheritance of genetic conditions: see details in inheritance of genetic diseases.
Spinal Muscular Atrophy: Testing
Diagnostic testing: see tests for Spinal Muscular Atrophy.
Misdiagnosis: see misdiagnosis and Spinal Muscular Atrophy.
How is it treated?
Treatments for Spinal Muscular Atrophy:
see treatments for Spinal Muscular Atrophy
Research for Spinal Muscular Atrophy:
see research for Spinal Muscular Atrophy
Society issues for Spinal Muscular Atrophy
Hospitalization statistics for Spinal Muscular Atrophy:
The following are statistics from various sources about hospitalizations and Spinal Muscular Atrophy:
- 0.0003% (43) of hospital consultant episodes were for infantile spinal muscular atrophy type 1 or Werdnig-Hoffman in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
- 80% of hospital consultant episodes for infantile spinal muscular atrophy type 1 or Werdnig-Hoffman required hospital admission in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
- 55% of hospital consultant episodes for infantile spinal muscular atrophy type 1 or Werdnig-Hoffman were for men in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
- 45% of hospital consultant episodes for infantile spinal muscular atrophy type 1 or Werdnig-Hoffman were for women in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
- 39% of hospital consultant episodes for infantile spinal muscular atrophy type 1 or Werdnig-Hoffman required emergency hospital admission in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
- 7.2 days was the mean length of stay in hospitals for infantile spinal muscular atrophy type 1 or Werdnig-Hoffman in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
- 3 days was the median length of stay in hospitals for infantile spinal muscular atrophy type 1 or Werdnig-Hoffman in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
- more statistics...»
Organs Affected by Spinal Muscular Atrophy:
Organs and body systems related to Spinal Muscular Atrophy include:
Name and Aliases of Spinal Muscular Atrophy
Main name of condition: Spinal Muscular Atrophy
Class of Condition for Spinal Muscular Atrophy: genetic
Other names or spellings for Spinal Muscular Atrophy:
Werdnig-Hoffman Disease, Kennedy Disease, SMA, Kennedy-Alter-Sung disease, bulbospinal muscular atrophy, X-linked, spinal-bulbar muscular atrophy, muscular atrophy, spinal bulbar, spinal and bulbar muscular atrophy, Bulbospinal amyotrophy, X-linked, KD, SMAX1, Spinal and bulbar muscular atrophy, X-linked 1, Bulbospinal neuronopathy, X-linked recessive, XBSN, Kennedy spinal and bulbar muscular atrophy, SBMA, X-linked bulbospinal muscular atrophy
HMN (hereditary motor neuropathy) proximal type 1, Infantile spinal muscular atrophy type 1, Werdnig-Hoffmann disease
Source - Diseases Database
Bulbospinal muscular atrophy, X-linked, Bulbospinal neuronopathy, X-linked recessive, KD(Kennedy disease), Kennedy spinal and bulbar muscular atrophy, SBMA, SMAX1, Spinal and bulbar muscular atrophy, Spinal and bulbar muscular atrophy, X-linked 1, X-linked bulbospinal muscular atrophy, XBSN
Source - Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)
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