Based on the provided information, the question is a little unclear. "Hoffman's disease" is typically understood to refer to Werdnig-Hoffmann disease, which is a severe form of Spinal Muscular Atrophy (SMA) typically diagnosed in infants. The references provided focus on Werdnig-Hoffmann disease. Adult-onset SMA exists, but it is not typically referred to as "Hoffman's disease." Therefore, I will address this question by discussing Werdnig-Hoffmann disease in the context of what adult-onset SMA may present.
Understanding Spinal Muscular Atrophy (SMA)
Spinal Muscular Atrophy (SMA) is a genetic disorder affecting the motor neurons, which are nerve cells in the spinal cord and brainstem that control muscle movement. This leads to muscle weakness and atrophy (wasting).
Werdnig-Hoffmann Disease (SMA Type 1)
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Definition: As the reference states, Werdnig-Hoffmann disease is a form of spinal muscular atrophy. It is also known as SMA Type 1, is the most severe form.
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Onset: Typically diagnosed in infancy.
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Symptoms:
- Significant muscle weakness
- Difficulty with motor skills, such as:
- Standing
- Walking
- Sitting
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Treatment:
- As cited in the reference, there is no cure.
- Physical therapy and supportive care can help manage symptoms.
Adult-Onset SMA
While "Hoffmann's disease" primarily refers to the infantile form (SMA Type 1), adult-onset SMA does exist as a distinct condition:
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Onset: Symptoms begin in adulthood.
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Symptoms:
- Progressive muscle weakness, primarily in the limbs.
- Muscle atrophy.
- Fasciculations (muscle twitching).
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Diagnosis: Diagnosis involves neurological examination, genetic testing, and electromyography (EMG).
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Treatment: Similar to infantile SMA, treatment focuses on managing symptoms and improving quality of life.
| Feature | Werdnig-Hoffmann (SMA Type 1) | Adult-Onset SMA |
|---|---|---|
| Onset | Infancy | Adulthood |
| Severity | Severe | Variable (often less severe) |
| Common Term | Hoffman's Disease | Adult-Onset SMA |
