Motor Neuron Disease

Excerpt

Motor neuron disease (MND) is said to be a progressive neurological disorder that presents with both lower motor neurons (anterior horn cells that project from the brainstem and the spinal cord to the muscle) and upper motor neuron signs (neurons that project to the brainstem and spinal cord from higher cortical centers).

While the anterior horn cell and the corticospinal tract have been shown to be the primary site of involvement, the involvement of other parts of the nervous system (cortical, autonomic, cerebellar, and extrapyramidal system) has also been documented. The four main phenotypes of motor neuron disease, based upon the site of origin and the severity of neurological involvement, are as follows: Amyotrophic lateral sclerosis, progressive bulbar palsy, progressive muscular atrophy, and primary lateral sclerosis.

Other systems used for the classification of this disease have used the site of onset (spinal or bulbar onset), degree of adherence to the El-Escorial (and Airlie house) criteria, and pattern of heritability (sporadic versus familial) as criteria for characterizing this neurodegenerative illness with a complex genetic basis. MND has been shown to be a disease of middle age with a mean age of 58 to 63 years at the time of onset for sporadic Amyotrophic lateral sclerosis (ALS), and 40-60 years of age for familial ALS.

There has been a growing ethical debate along with pleas to formulate policies pertaining to the provision of Euthanasia and physician-assisted suicide by the families of patients and their advocates, which has brought the discussion surrounding this disease into the public domain.