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Denervation processes are strongly associated with the symptoms experienced in post-polio syndrome. Individuals with post-polio syndrome undergo a continuous cycle of denervation and reinnervation that occurs after acute poliomyelitis. Over time, this cycle leads to an increase in the size of motor units in skeletal muscle fibers. Eventually ...
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.
Motor neuron diseases affect both children and adults. [5] While each motor neuron disease affects patients differently, they all cause movement-related symptoms, mainly muscle weakness. [6] Most of these diseases seem to occur randomly without known causes, but some forms are inherited. [2]
Treatment approaches include impacting the signaling pathways that induce muscle hypertrophy or slow muscle breakdown as well as optimizing nutritional status. [ citation needed ] Physical activity provides a significant anabolic muscle stimulus and is a crucial component to slowing or reversing muscle atrophy. [ 3 ]
Occasionally, biopsy of skin, nerve, or muscle may be performed, which can show signs of denervation and amyloid deposition with response to anti-TTR antibodies. [8] Additional testing should be performed to identify involvement of the heart or kidneys. [6]
Muscle wasting, fasciculations and fibrillations are typically signs of end-stage muscle denervation and are seen over a longer time period. Another feature is the segmentation of symptoms – only muscles innervated by the damaged nerves will be symptomatic.
An initial diagnosis of PMA could turn out to be slowly progressive ALS many years later, sometimes even decades after the initial diagnosis. The occurrence of upper motor neuron symptoms such as brisk reflexes, spasticity, or a Babinski sign would indicate a progression to ALS; the correct diagnosis is also occasionally made on autopsy. [4] [5]
Muscle weakness is a lack of muscle strength. Its causes are many and can be divided into conditions that have either true or perceived muscle weakness. True muscle weakness is a primary symptom of a variety of skeletal muscle diseases, including muscular dystrophy and inflammatory myopathy.