Progressive muscle atrophy

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Unclear presently if a variant of motor neuron disease or spinal muscular atrophy but progress to death can be much slower than classic motor neuron disease. It can evolve into classic motor neuron disease. There are only lower motor neuron signs. Some variants are familial with specific SOD1 mutations adding to the potential controversy on classification[1]. It is clinically distinguished from motor neurone disease by:

  • Advanced extremity paresis and atrophy
  • Absent or late affection of bulbar function

References

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