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The session focused on the basis of selective motor neuron death in ALS. There was general agreement that the death of the motor neuron results from a cascade of toxic biological events triggered by a variety of factors. As such, ALS can be considered to be a multifactorial disorder that includes a population of genetically predisposed patients. The concept of a factor that renders the motor neuron vulnerable to damage in situations that a normal cell can easily resist is a theme common to many disease processes. Similar mechanisms have been implicated in Parkinson's disease, stroke, epilepsy, Alzheimer's disease, and muscular dystrophies. The importance of this concept lies in the possibility of developing rational treatment based on the prevention of the secondary cell death. Several key topics remain to be addressed: the mechanisms by which the cascade is triggered, the role of microglia and astrocytes, the reason for the age dependency of the disease, and the factors behind the involvement of different regions of the neuroaxis.
Motor neuron selective vulnerability.
An integrated view of ALS needs to consider not only the degenerative process of the motor neurons, but also the role of non-neuronal supporting cells. The characteristic loss of anterior horn cells, atrophy of ventral roots, and demyelination of the corticospinal tracts traditionally establish the diagnosis and indicate that it is a selective disease of the motor system. However, there can also be damage to other nerve cells, sensory neurons, sphincteric motor neurons, and cells of the substantia nigra and frontal lobe. These are more commonly seen in the patient who has been maintained on a respirator. [1-3] Whether this reflects the ultimate involvement of nonmotor systems or the effects of chronic respiratory insufficiency is uncertain. Either way, the early vulnerability of the motor neuron in ALS is likely to be a reflection of …
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