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Primary lateral sclerosis (PLS) usually presents with gradual-onset, progressive, lower-extremity stiffness and pain due to muscle spasticity. Onset is often asymmetrical. [ 2 ] Although the muscles do not appear to atrophy as in ALS (at least initially), the disabling aspect of PLS is muscle spasticity and cramping, and intense pain when those ...
Progressive disease or progressive illness is a disease or physical ailment whose course in most cases is the worsening, growth, or spread of the disease. This may happen until death, serious debility, or organ failure occurs. [1] Some progressive diseases can be halted and reversed by treatment (surgical, dietary, or lifestyle interventions).
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Anticentromere antibody is more common in the limited form (80–90%) than in the diffuse form (10%), and anti-scl70 is more common in the diffuse form (30–40%) and in African-American patients (who are more susceptible to the systemic form). [26] Other conditions may mimic systemic sclerosis by causing hardening of the skin.
The importance of correctly recognizing progressive muscular atrophy as opposed to ALS is important for several reasons. The prognosis is a little better. A recent study found the 5-year survival rate in PMA to be 33% (vs 20% in ALS) and the 10-year survival rate to be 12% (vs 6% in ALS). [1]
sIBM causes progressive muscle weakness. [1] How sIBM affects individuals is variable, including the age of onset (which generally varies from the forties upwards) and rate of progression. Because of this variability, there is no "textbook case". [13] Common early symptoms include frequent tripping and falling and difficulty going up stairs.
Immunoglobulin and steroids are the first line choices for treatment. [citation needed] In severe cases of CIDP, when second-line immunomodulatory drugs are not efficient, autologous hematopoietic stem cell transplantation (HSCT) is sometimes performed. The treatment may induce long-term remission even in severe treatment-refractory cases of CIDP.
In 1850, François-Amilcar Aran was the first to describe a disorder he named "progressive muscular atrophy", a form of ALS in which only the lower motor neurons are affected. [126] In 1869, the connection between the symptoms and the underlying neurological problems was first described by Jean-Martin Charcot , who initially introduced the term ...