Motor Neuron Disease

Sunday, December 16, 2007

There are four main types of MND, which influence people by various ways. Though there is a great difference imposed among all these forms and it is useful to separate various types of illness, in practice it is not always probably so definite.

Amyotrophic a lateral sclerosis (ALS).

This is the most common form, with and top and lower motor neuron participation. This form of illness is characterized by weakness and s wasting in the limbs.

Progressive bulbar the paralysis (PBP)

Influences for about one fourth of the diagnosed people, and involves both top and lower motor neurons. The symptoms can include inarticulate speech swallowing difficulties or speeches. Life expectancy is between six months and three years from a beginning of symptoms.

Progressive muscular atrophy (PMA)

Influences only a small proportion of the people, mainly causing damage. The early symptoms can be noticed as weakness or awkwardness of a hand. The majority of the people live during more than five years.

Primary lateral sclerosis (PLS)

A rare form of MND involving the upper motor neurons only, generating mainly weakness in lower limbs, though some people can test awkwardness in problems of speech or hands. The life expectancy could be in essence normal, although it may be life-limiting, depending on whether it remains as pure PLS or develops into ALS.

Signs and symptoms

Neurological examinations represent the certain signatures connected to upper and lower motor neuron degeneration. The signature of the upper motor neuron damage includes spasticity, brisk reflections and the Babinski sign. The signatures of lower motor neuron damage include atrophy of a muscle and weakness.

Pay attention that each group of muscles in a body requires both upper and lower motor neurons to function. It is a common misconception that "upper" motor neurons control the arms, whilst "lower" motor neurons control the legs. The signs described above can occur in any muscle group, including the arms, legs, torso, and bulbar region.

The symptoms usually represent between ages of 50-70, and include progressive weakness, expenditure of a muscle, and muscle fasciculation; spasticity or immovability in hands and legs; and superactive reflections of a sinew. Patients may present with symptoms as diverse as a dragging foot, unilateral muscle wasting in the hands, or slurred speech.
The symptoms described above can resemble set of other rare illnesses known, as "MND Mimic Disorders". They include, but are not limited to the multifocal motor neuropathy, hereditary spastic paraplegia, Kennedy's disease, spinal muscular atrophy and monomelic amyotrophy. Small subset of familial MND cases occur in children, such as "juvenile ALS", Madras syndrome, and individuals who have inherited the ALS2 gene. However, these are not typically referred to as MND, but by their specific names.

The Diagnosis

The diagnosis of MND is clinical, established neurologist on the basis of a history and neurological examination. There is no diagnostic test on MND. Researches such as tests of blood, electromyography (EMG), magnetic resonance displaying (MRI), and sometimes genetic test is useful to exclude other disorders, which can imitate MND. However, the diagnosis MND remains clinical. Excluding other illness, rather fast progression of attributes is strong diagnostic factor. “Although an individual's progression may sometimes "plateau", it will not recover or slow down. A set of diagnostic criteria called the El Escorial criteria have been defined by the World Federation of Neurologists for use in research, particularly as inclusion/exclusion criteria for clinical trials. Due to a lack of clinical diagnostic criteria, some neurologists use the El Escorial criteria during the diagnostic process, although strictly speaking this is functionality creep.”

MND at presence of both upper and lower motor neuron degeneration is ALS. Where the illness influences only the upper motor neurons it is PLS, and where there is only an influences of lower motor neuron it is PMA. Progressive bulbar palsy is degeneration of the lower motor neurons innervating the bulbar region (face, mouth, and throat), while pseudobulbar palsy refers to degeneration of the upper motor neurons to the same region).

The Prognosis

The majority of cases MND progresses rather quickly, with noticeable decline occurring over the course of months. Though the attributes can represent in one region, they will be distributed typically If restricted to one side of the body they are more likely to progress to the same region on the other side of the body before progressing to a new region. After several years, the majority of the patients require the help be executed actions of daily residing of a type by taking care, submission, and transportation.

MND is typically fatal within the limits of 2-5 years. Around 50 % die within the limits of 14 months from the diagnosis. The preservation 50 % not necessarily will die within the limits of following14 months as distribution, is significant is deformed. As a rough estimation, 1 in 5 patients survive within 5 years, and 1 in 10 patients experience 10 years. Stephen Hawking is a well-known example of a person with MND, and has lived for more than 40 years with the disease.

Death comes to an end, when the muscles, which operate breath, are not capable any more to expel carbon dioxide. The single exception is PLS, which can last for upwards 25 years. Taking into account typical age of a beginning, it effectively leaves most PLS patients with normal life expectancy. PLS can progress to ALS, a decade later.


Now there is no treatment for ALS. “The only drug that affects the course of the disease is riluzole. The drug functions by blocking the effects of the neurotransmitter glutamate, and is thought to extend the lifespan of an ALS patient by only a few months.”

Lack of effective treatments to slow down a ALS progression does not mean, that for the patients with ALS it is impossible with the medical point of view to care. Instead of it, treatment of the patients with ALS is focused to the aid (simplification) of attributes connected to illness. This involves a variety of health professionals including neurologists, speech-language pathologists, physical therapists, occupational therapists, dieticians, respiratory therapists, social workers, palliative care specialists, specialist nurses and psychologists.

“The researchers continuously investigate new processings (treatment) for MND. They include:
* antioxidants, such as co-enzyme Q10, to "mop up" waste molecules before they damage nerve cells;
* the anti-cancer drug tamoxifen;
* insulin-like growth factor 1 (IGF-1), which encourages the growth of nerves;
* the antibiotic minocycline, which extends survival in animal models of MND.
One day, scientists hope to use stem cells to treat the condition. Stem cells are the body’s master cells which can divide and turn themselves into any type of cell.”


"Pain is just weakness leaving your body." Keri Schrimpf






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