What is ALS disease? is there a cure?

Answer 1

Amyotrophic lateral sclerosis (ALS), which is also called motor neuron disease, Charcot's disease, or Lou Gehrig's disease, is an age-dependent and generally fatal paralytic disorder caused by the degeneration of motor neurons in the motor cortex, brain stem, and spinal cord. About 10 percent of cases are familial (FALS) and the rest are sporadic (SALS).

Broadly, there are three types of ALS usually considered in epidemiological studies: SALS, FALS, and a variant of ALS, sometimes called Guamanian ALS, found in the Western Pacific and characterized by the occurrence of parkinsonism, dementia, or both. SALS has a worldwide incidence of 1 to 2 in 100,000 persons, with fairly uniform distribution worldwide and equal representation among racial groups. The occurrence of ALS before the age of 40 years is uncommon. The incidence is greatest between the ages of 50 and 70 years, and it seems to decline thereafter. The male-to-female ratio is about 1.3:1. Clusters of the disease have been identified, particularly in the Kii peninsula of Japan and the Mariana Islands. The only indisputable risk factor other than age and gender is genetic susceptibility, with familial cases occurring in about 10 percent of most case series. Many isolated potential etiologies have been proposed for SALS, with the only consistent associations thus far being long-term exposure to heavy metals, particularly lead, and a family history of parkinsonism and dementia. There is recent evidence of association between increased dietary fat and cigarette smoking. Curiously, cigarette smoking is negatively associated with the development of Parkinson's disease. Electrical injury has also been reported to increase the risk of ALS.

ALS is a syndrome of upper and lower motor neuron dysfunction at several levels of the neuraxis without involvement of other neurological systems. There is no definitive test that can diagnose ALS. In 1994, the El Escorial criteria were developed to standardize its diagnosis. Although the disease may present in many different ways and in different parts of the body, generally the patient seeks attention because of symptomatic weakness. Alternatively, there may be a history of fasciculations, muscle cramping, and atrophy before weakness is apparent. A frequent diagnostic feature is the presence of hyper-reflexia in segmental regions where muscles are starting to atrophy and an absence of sensory disturbance in the same distribution. Limb involvement occurs more often than bulbar involvement, and upper limbs are more often affected than lower limbs in SALS; this pattern is reversed in FALS. However, the pattern of involvement is frequently asymmetrical or focal. If more upper motor neurons are affected, the symptoms will be primarily clumsiness, stiffness, and fatigue, whereas lower motor neuron degeneration will present as weakness or atrophy and occasionally fasciculations. Bulbar symptoms include hoarseness, slurring of speech, choking on liquids, and difficulty initiating swallowing. Paresthesias and sensory symptoms affect up to 25 percent of patients, but if present, these complaints are mild. There is wide variation in disease progression and duration, neither of which can be accurately predicted from age or site of onset, although generally elderly patients have a shorter survival. Bowel, bladder, and sexual functioning are usually spared, with about 4 percent of patients experiencing loss of sphincter control. ALS patients have diminished skin elasticity and rarely develop bedsores, seemingly because of the accumulation of a basic protein there. Although fasciculations, especially of the tongue, are considered by some to be specifically associated with ALS, they may actually be seen in any disorder affecting the motor neuron or their axons. Remote effect of radiation damage to the hypoglossal nerve is a case in point.


Summary of El Escorial Criteria for the Diagnosis of Amyotrophic Lateral Sclerosis:

The diagnosis of ALS requires the presence of signs of lower motor neuron (LMN) degeneration by clinical, electrophysiological, or neuropathological examination and signs of upper motor neuron (UMN) degeneration by clinical examination, and the progressive spread of these signs within a region or to other regions, together with the absence of electrophysiological or neuroimaging evidence of other disease processes that might explain these signs.

Suspected ALS
LMN signs only in at least two regions

Possible ALS
UMN and LMN signs in only one region, or UMN signs only in at least two regions, or LMN signs rostral to UMN signs
Special cases: monomelic ALS, progressive bulbar palsy without spinal UMN and/or LMN signs, primary lateral sclerosis without spinal LMN signs

Probable ALS
UMN signs in at least two regions, with some UMN signs above LMN signs

Definite ALS
UMN signs and LMN signs in bulbar region and at least two spinal regions, or UMN and LMN signs in three spinal regions

Thus far, only the benzothiazole riluzole has been shown to extend life span, although in double-blind studies, the extension averages only 3 months and mortality rates are unaffected. In larger follow-up studies, the increase in life may be as long as 12 months. Riluzole acts to block voltage-activated sodium channels and to protect against glutamate toxicity. The life span of transgenic mice expressing mutant human SOD1 was prolonged by 22 percent with intrathecal administration of a caspase inhibitor, and human clinical trials are planned. Although there is no truly effective pharmacological treatment yet available for ALS, a supportive multidisciplinary team can provide care and symptom management that significantly improves the patient's quality of life. Drugs such as baclofen, quinine, and phenytoin can be used to reduce cramping; anticholinergic agents can be used to control sialorrhea. Amitriptyline or selective serotonin reuptake inhibitors may help control pseudobulbar symptoms in some patients. Occupational therapy can provide techniques and orthotics that can extend the patient's ability to function independently. A speech pathologist can provide instruction to prolong the patient's ability to communicate and swallow. Augmentative communication devices are now covered by Medicare but require formal evaluation. Durable medical equipment (e.g., power-driven wheelchairs, hospital beds) allow most patients to remain at home. Open, sympathetic discussion of feeding and ventilatory support options is required with the patient and family. Bilevel positive airway pressure may provide at least short-term symptomatic relief of respiratory fatigue and sleep apnea. Algorithms for nutritional and respiratory management are available at the Web site of the American Academy of Neurology (www.aan.com). ALS remains a fatal disease, with a mean survival of 3 years after the onset of symptoms.

Answer 2

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Answer 3

ALS (Amyotrophic lateral sclerosis)is a progressive, fatal neurodegenerative disease caused by the degeneration of motor neurons. ALS is marked by gradual degeneration of the nerve cells in the central nervous system that control voluntary muscle movement. The disorder causes muscle weakness and atrophy throughout the body. In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away (atrophy), and have fasciculations because of denervation. Eventually, the brain completely loses its ability to initiate and control voluntary movement. The disease does not necessarily debilitate the patient's mental functioning in the same manner as Alzheimer's disease or other neurological conditions. Instead, those suffering advanced stages of the disease may retain the same memories, personality, and intelligence they had before its onset.

No cure has yet been found for ALS. However, the Food and Drug Administration (FDA) has approved the first drug treatment for the disease: Riluzole (Rilutek). Riluzole is believed to reduce damage to motor neurons by decreasing the release of glutamate. Clinical trials with ALS patients showed that riluzole prolongs survival by several months, and may have a greater survival benefit for those with a bulbar onset. The drug also extends the time before a patient needs ventilation support. Riluzole does not reverse the damage already done to motor neurons, and patients taking the drug must be monitored for liver damage and other possible side effects. However, this first disease-specific therapy offers hope that the progression of ALS may one day be slowed by new medications or combinations of drugs

Answer 4

The formal name of ALS is amyotrophic lateral sclerosis, also known as Lou Gehrig's disease. It's a progressive, fatal neurodegenerative disease caused by the degeneration of motor neurons. ALS is marked by gradual degeneration of the nerve cells in the central nervous system that control voluntary muscle movement. The disorder causes muscle weakness and atrophy throughout the body. In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away (atrophy), and have fasciculations because of denervation. Eventually, the brain completely loses its ability to initiate and control voluntary movement. No cure has yet been found for ALS. However, the Food and Drug Administration (FDA) has approved the first drug treatment for the disease: Riluzole (Rilutek).

Answer 5

ALS stands for amyotrophic lateral sclerosis, and is often called Lou Gehrig's disease.

It is fatal, there is no cure at this time. A person with ALC will have gradual degeneration of his voluntary muscle movement. The muscles waste away and eventually, the brain completely loses its ability to initiate and control voluntary movement.

It is a physical disease, not mental (like Alzheimer's).

Answer 6

All the answers so far telling what ALS is are correct. My brother was diagnosed with ALS in 1993. He lived about 2 years after. It is a horrible illness, that destroys your body, but the most awful thing is you still have your mind. Our family watch as my brother struggled everyday, but I have to say, he never gave up, and had a sense of humor that probably helped all of us. Unfortunately he left behind all of us, as well as his only daughter. There really is no cure as of yet. My brother was involve in many clinical drug programs to see if this or that worked, obviously nothing did. He was back and forth from Cleveland Clinic, and actually ha to stay for months at a time. You would think in this wonderful country we live in, with all its resources someone would find a cure. Not only did my brother have ALS, my mom is now in a Nursing Home with Alzheimer's. How lucky can one family be?
Debi
I don't mean to sound angry, please don't take it that way. Perhaps my humor is just like my brother's was.

Answer 7

Amyotrophic lateral sclerosis (ALS), often referred to as "Lou Gehrig's disease," is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually lead to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed. Yet, through it all, for the vast majority of people, their minds remain unaffected.

There is no cure for this yet.

Answer 8

It is short for Amyotrophic Lateral Sclerosis. It was commonly known as 'Lou Gehrig's disease as he was the most famous person in sports history to die from this disease.
It is currently incurable. This disease is in the muscular dystrophy family of diseases. It affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually lead to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed. Yet, through it all, for the vast majority of people, their minds remain unaffected.
A-myo-trophic comes from the Greek language. "A" means no or negative. "Myo" refers to muscle, and "Trophic" means nourishment---"No muscle nourishment." When a muscle has no nourishment, it "atrophies" or wastes away. "Lateral" identifies the areas in a person's spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates it leads to scarring or hardening ("sclerosis") in the region.

Answer 9

Is Als Curable

Answer 10

als is a is a disease that makes your mind clear but you cant do anything on your own

Answer 11

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