Progressive muscle atrophy

ALS is a disorder of the motor neurons and the cortical neurons that provide their input. The disorder is characterized by rapidly progressive weakness and muscle atrophy. Most affected patients die of respiratory compromise and pneumonia after 2 to 3 years. There is prominent loss of motor neurons in the spinal cord and brainstem although the oculomotor neurons are spared. Large pyramidal motor neurons in layer V of motor cortex, which are the origin of the descending corticospinal tracts, are also lost. 

Motor neuron disease is characterized clinically by weakness, muscle atrophy and spasticity. This illness, often termed Lou Gehrig s disease in the United States, is the most common adult-onset form of MND with a prevalence of approximately 2-3 per 100,000 people [1-3,10, 25, 28]. Each year in the United States, in excess of 5,000 people are diagnosed with ALS. In parts of the United Kingdom, 1 in =500 deaths are attributed to some form of MND. The principal clinical signs of ALS include progressive limb weakness, which may be symmetrical or asymmetrical atrophy of appendicular, bulbar and respiratory muscles and spasticity [1,2,26,28]. The paralysis/muscle atrophy and spasticity are the result of degeneration of motor neurons in the spinal cord/brain stem and motor cortex respectively. The onset of this illness is typically in the fifth or sixth decade of life affected individuals usually... 

The gene involved in myotonic dystrophy encodes a protein kinase whose function is still uncharacterized, The disease is characterized by progressive muscle deterioration, cardiac amhythmia, frontal baldness, cataracts, and teaicular atrophy. Because the disease shows anticipation, symptoms range ftom mild to a severe neonatal condition. 

Thi autosomal dominant disorder, which affects approximately 1 in 8,000 individuals, is characterized by progressive muscle deterioration, cardiac arrhythmia, testicular atrophy, frontal baldness, and cataraas. As noted above, most cases are caused by a trinucleotide repeat expansion in the 3 UTR of a gene that encodes a protein kinase. Larger repeat numbers lead to earlier and more severe expression of the disease (anticipation). Especially large expansions sometimes occur in maternal transmission of the trinucleotide repeat, resulting in a severe neonatal form of the disorder. 

ALS is a neurological disease with damage to motor neurons in the brain and spinal cord. It is characterized by progressive muscle weakness, atrophy, and spasticity. Characteristic neuropathological features include the loss of anterior horn motor... 

Amyotrophic lateral sclerosis is an inexorably progressive motor neuron disease, in w hich both the upper motor neurons and the lov er motor neurons degenerate leading to muscle atrophy. Patients eventually experience respiratory failure, usually within three to five years from diagnosis. However, the onset of AES may be subtle and early symptoms are frequendy overlooked. As many as 20,000 Americans have ALS, and an estimated 5,000 people in the United States are diagnosed with the disease each year. Onset is usually in the 5th through 7th decade of life. 

Prognosis— inability to walk 10 years after onset, with progressive weakness, atrophy, and contracture of muscles leading to skeletal distortion, complete helplessness, and death in the second decade due to inanition or infection. 

Progressive muscle distrophy. As Stepanova and Grinio demonstrated [106] this disease in children is accompanied with decrease in muscle camosine content progressively and amount of camosine falls proportionally to depth of muscle atrophy. Other diseases accompanied with muscle atrophy (like spastic tetraparesis) are also characterized by progressive decrease in camosine content. As authors suggested, possible explanation of this phenomenon is a deficiency of system of camosine synthesis [106]. 

Motor neuron disease is also known as amyotrophic lateral sclerosis. It is a condition in which there is loss of spinal motor neurons and neurons of descending motor pathways from pyramidal cells of the cerebral cortex. The disease results in rapidly progressing muscular weakness, muscle atrophy, fasciculations, spasticity, difficulty speaking. 

With n-hexane and MEK, the clinical signs typically develop two or three months after abuse and show a fairly rapid progression involving a symmetrical and primarily distal motor neuropathy leading to paralysis and muscle atrophy in the legs and sometimes in the arms as well. Head and trunk muscles are not usually affected. Sensory disturbance is manifest as a diminution in perception of pain. 

Slowly progressive muscle weakness and atrophy selective pattern with quadriceps femoris and finger flexors affected first, followed by other muscle groups frequently asymmetric and more severe on nondominant side... 

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