| Amyotrophic Lateral Sclerosis (ALS) (Also known as
Lou Gehrig's Disease) |
| Onset: |
Adulthood. |
| Symptoms: |
Generalized weakness and muscle wasting with cramps and muscle twitches
common. |
| Progression: |
ALS first affects legs, arms and/or throat muscles. Usually progresses
rapidly with 3 to 5 year average survival. |
| Inheritance: |
|
Infantile Progressive Spinal Muscular
Atrophy (SMA, SMA1 or WH)
(Also known as SMA Type 1, Werdnig-Hoffman) |
| Onset: |
Before birth to 3 months. |
| Symptoms: |
Generalized muscle weakness, weak cry, trouble swallowing as well as sucking,
and breathing distress. Cannot sit up. |
| Progression: |
Progresses very rapidly with early childhood death. |
| Inheritance: |
Autosomal recessive. |
| Intermediate Spinal Muscular Atrophy (SMA or SMA2)
(Also known as SMA Type 2) |
| Onset: |
6 months to 3 years. |
| Symptoms: |
Weakness in arms, legs, upper and lower torso, often with skeletal deformities.
|
| Progression: |
Disease usually progresses rapidly and respiratory problems may develop. |
| Inheritance: |
Autosomal recessive. |
| Juvenile Spinal Muscular Atrophy (SMA, SMA3 or KW)
(Also known as SMA Type 3, Kugelberg-Welander) |
| Onset: |
1 to 15 years. |
| Symptoms: |
Weakness in leg, hip, shoulder, arm and sometimes respiratory muscles. |
| Progression: |
Disease progresses slowly. Wheelchair often required later in life. Life
span usually not affected. |
| Inheritance: |
Autosomal recessive. |
Spinal Bulbar Muscular
Atrophy (SBMA) (Also known as Kennedy's Disease and X-Linked SBMA) |
| Onset: |
Adulthood (20 to 50 years - variable severity). |
| Symptoms: |
Weakness and muscle wasting of bulbar region (mouth and throat) and skeletal
muscles. Usually affects only men -- women as carriers may have a mild form.
Facial fasciculations and mild sensory involvement are common. |
| Progression: |
Slow, variable progression, sometimes accompanied by breast development,
infertility and testicular wasting in men. Normal life span. |
| Inheritance: |
X-linked recessive (females are carriers). |
| Adult Spinal Muscular Atrophy (SMA) |
| Onset: |
18 to 50 years. |
| Symptoms: |
Generalized weakness and muscle wasting with muscle twitches common.X-linked
form affects men only and involves muscles of mouth and throat as well as
other muscles. |
| Progression: |
Variable disease progression. Relatively mild form of SMA with little
impact on life expectancy. |
| Inheritance: |
Autosomal dominant, autosomal recessive. |
