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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 ...
The disease is characterized by slowly progressive weakness and wasting of both proximal muscles (located on or close to the torso) and distal muscles (close to hands or feet), most apparent in the finger flexors and knee extensors. [3] IBM is often confused with an entirely different class of diseases, called hereditary inclusion body ...
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]
When a patient presents with a non-length-dependent demyelinating polyneuropathy which either develops chronically over several months or progresses over more than a month, CIDP may be diagnosed. There may be a secondary progressive course along with a progressive course that follows, or it may be relapsing and remitting.
Spinal and bulbar muscular atrophy (SBMA), popularly known as Kennedy's disease, is a rare, adult-onset, X-linked recessive lower motor neuron disease caused by trinucleotide CAG repeat expansions in exon 1 of the androgen receptor (AR) gene, which results in both loss of AR function and toxic gain of function.
Idiopathic primary BSS is a late-onset myopathy with progressive muscular weakness that is detected on the spinal extensor muscles in elderly patients and is more predominant in females. [2] The pathogenesis of primary BSS is typically related to fibrosis and fatty infiltration of muscular tissues and to mitochondrial changes due to the aging ...
Multiple organ dysfunction syndrome (MODS) is altered organ function in an acutely ill patient requiring immediate medical intervention. [1]There are different stages of organ dysfunction for certain different organs, both in acute and in chronic onset, whether or not there are one or more organs affected.
Causes progressive weakness, initially in the muscles of the face, shoulders, and upper arms. Additional muscles are often affected. [20] Affected individuals can become severely disabled, with 20% requiring a wheelchair by age 50. [21] 30% of cases involve spontaneous mutations. [21] Penetrance and severity seem to be lower in females compared ...