Wisdom of Life

The Ice Bucket Challenge! Any Relationship between ALS and MSA?

There is a buzz going on right now. Individuals are voluntarily drenching themselves in cold water, and the videos are going viral. Many more are joining in the campaign. The “Ice bucket challenge” is not new to anyone who has spent some time online recently. It involves the nomination of participants to be filmed dumping a bucket of ice water on their heads and nominating others to do the same in order to raise awareness for ALS and to encourage donations to research. Nominated participants have 24 hours to comply or forfeit by way of a charitable donation. However, many individuals take the challenge and still go on to make a generous donation.

While the ice bucket challenge is going on, campaigns for Multiple System Atrophy are tagging along nicely with the "Purple Rain Challenge". The same rule applies here although this time one must put a purple shirt on (purple symbolizes MSA, and the purple passion flower is the floral emblem of MSA Awareness)  and dance under a rain or sprinkler with friends, colleagues or family members whilst holding a sign with "Multiple System Atrophy" and the website – www.multiplesystematrophy.org. scribbled on it. Click HERE to join in.

By the way, there is an anonymous donor who pledges to MATCH all donations to the #MSAPurpleRain Video Challenge up to a total of $25, 000.

There are a number of similarities between MSA and ALS, although there are also a number of differences. So I went online to do a little comparative study and thanks to the vast information accessible via numerous sites, especially Wikipedia I got a few information to share:

 

WHAT IS?

Multiple-system atrophy (MSA) is a degenerative neurological disorder associated with the degeneration of nerve cells in specific areas of the brain. This cell degeneration causes problems with movement, balance, and other autonomic functions of the body such as bladder control or blood-pressure regulation.

Amyotrophic lateral sclerosis (ALS)—also referred to as motor neurone disease (MND), Lou Gehrig's disease in the United States, and rarely Charcot disease—is also with various causes. A  degenerative neurological disorder like MSA, ALS is characterised by musclespacity rapidly progressive weakness due to muscle atrophy and difficulty in speaking, swallowing, and breathing. ALS is the most common of the five motor neuron diseases.

 

PREVALENCE

MSA: The cause of MSA is unknown and no specific risk factors have been identified. Around 55% of cases occur in men, with typical age of onset in the late 50s to early 60s. According to Bower J, the overall prevalence of MSA is estimated at 4.6 cases per 100,000 people. This disease is more common in men than in women, with studies showing ratios ranging from between 1.4:1 to ratios as high as 1.9:1.

ALS: According to www.alsa.org, In the U.S., more than 5,600 are diagnosed every year, and up to 30,000 Americans are currently affected. ALS is responsible for 2 deaths per 100,000 people per year. Median survival time from onset to death is 39 months, and only 4% survive longer than 10 years, although rare cases survive 50 years or more. Most die from respiratory failure, usually within three to five years from onset of symptoms (Turner MR, 2003).

 

SIGNS AND SYMPTOMS

MSA is characterized by a combination of the following, which can be present in any combination: Because people with MSA often suffer slowness of initiation of movement resembling Parkinson's disease as found in 62% at first presentation it is sometimes grouped with diseases referred to as Parkinson's plus syndrome.

–         Autonomic dysfunctions, Parkinsonism (muscle rigidity, tremor and slow movement) and Ataxia (Poor coordination / unsteady walking) are the major symptoms of MSA.

ALS causes muscle weakness and atrophy throughout the body. Individuals affected by the disorder may ultimately lose the ability to initiate and control all voluntary movement, but unlike in MSA, bladder and bowel function and the muscle responsible for eye movement are usually spared until the final stages of ALS. (Dugdale DC, 2010)

Cognitive function is generally spared for most patients, although according to Phukan J, about 5% also develop frontotemporal dementia. A higher proportion of patients (30–50%) also have more subtle cognitive changes which may go unnoticed, but are revealed by detailed neuropsychological testing. Infrequently ALS coexists in individuals who also experience dementia, degenerative muscle disorder, and degenerative bone disorder as part of a syndrome called multisystem proteinopathy. (Johnson, 2010) Sensory nerves and the autonomic nervous system are generally unaffected, meaning the majority of people with ALS will maintain hearing, sight, touch, smell and taste.

 

INITIAL PRESENTATION

MSA: The most common first sign of MSA is rigidity and slowness of initiation of movement. Other common signs at onset include problems with balance, followed by genito-urinary problems. For men, the first sign can be an inability to achieve or sustain an erection. Both men and women often experience problems with their bladders including urgency, frequency, incomplete bladder emptying, or an inability to pass urine.

ALS: The earliest symptoms of ALS are weakness and/or muscle atrophy. Other presenting symptoms include trouble swallowing, cramping, or stiffness of affected muscles; muscle weakness affecting an arm or a leg; and/or slurred and nasal speech. Patients may experience awkwardness when walking or running or notice that they are tripping or stumbling, often with a "dropped foot" which drags gently along the ground. Patients may also experience difficulty with tasks requiring manual dexterity such as buttoning a shirt, writing, or turning a key in a lock. Occasionally, the symptoms remain confined to one limb for a long period of time or for the whole length of the illness.

About 25% of cases notice difficulty speaking clearly or swallowing. Speech may become slurred, nasal in character, or quieter. Other symptoms include difficulty swallowing and loss of tongue mobility.

 

PROGRESSION

MSA: As the disease progresses, one of three groups of symptoms predominate. These are:

Parkinsonism (slow, stiff movement, writing becomes small and spidery)

Cerebellar dysfunction (difficulty coordinating movement and balance)

Autonomic nervous system dysfunction (impaired automatic body functions) including: dizziness or fainting, urinary incontinence, impotence, constipation, vocal cord paralysis, dry mouth, dry skin, trouble regulating body temperature due to sweating deficiency in all parts of the body, loud snoring, etc

Other symptoms such as double vision can occur. Not all patients experience all of these symptoms.

Some patients (20% in one study) experience significant cognitive impairment as a result of MSA. (Brown 2010)

 

PROGNOSIS

MSA: There is no remission from MSA. The average remaining lifespan after the onset of symptoms in patients with MSA is 7.9 years. Almost 80% of patients are disabled within five years of onset of the motor symptoms, and only 20% survive past 12 years (Watanabe 2002). Rate of progression differs in every case and speed of decline may vary widely in individual patients.

ALS: Most people with ALS die from respiratory failure, usually within three to five years from the onset of symptoms. The median survival time from onset to death is around 39 months (3 years plus), and only 4% survive longer than 10 years.Guitarist Jason Becker has lived since 1989 with the disorder, while physicist Stephen Hawking has survived for more than 50 years, but they're considered unusual cases

 

CAUSES

There are no known causes of MSA, and it is not hereditary unlike familial ALS which has a known hereditary factor where the condition is known to run in the family, although, this is quite rare. Where there is no history of the disease in the family, there is no known cause of ALS.

Other potential causes for ALS which has inconclusive evidence include: head trauma, frequent drug use, and participation in contact sports.

 

TREATMENT

There is really no known cure for MSA, however the symptoms can be managed and controlled. While Riluzole is used to treat people with ALS, and has been found to improve survival but only to a modest extent, this drug is ineffective against MSA (Bensimon G, 2008). Alternatively, Levadopa (L-Dopa), a drug used to treat Parkinsonism is also used in the treatment of some of the Parkinsonism symptoms of MSA but with very little effect in comparism.

 

 

 

 

REFERENCES

 Bensimon G, Ludolph A, Agid Y, Vidailhet M, Payan C, Leigh PN (2008)."Riluzole treatment, survival and diagnostic criteria in Parkinson plus disorders: The NNIPPS Study"Brain 132 (Pt 1): 156–71

Bower J, Maraganore D, McDonnell S, Rocca W (1997). "Incidence of progressive supranuclear palsy and multiple system atrophy in Olmsted County, Minnesota, 1976 to 1990"Neurology 49 (5): 1284–8

Brown RG, Lacomblez L, Landwehrmeyer BG, Bak T, Uttner I, Dubois B, Agid Y, Ludolph A, Bensimon G, Payan C, Leigh NP; for the NNIPPS Study Group (August 2010). "Cognitive impairment in patients with multiple system atrophy and progressive supranuclear palsy". Brain 133 (Pt 8): 2382–93Dugdale DC, Hoch DB, Zieve D (27 August 2010). "Amyotrophic lateral sclerosis"A.D.A.M. Medical Encyclopedia

 Johnson JO, Mandrioli J, Benatar M, Abramzon Y, Van Deerlin VM, Trojanowski JQ, Gibbs JR, Brunetti M, Gronka S, Wuu J, Ding J, McCluskey L, Martinez-Lage M, Falcone D, Hernandez DG, Arepalli S, Chong S, Schymick JC, Rothstein J, Landi F, Wang YD, Calvo A, Mora G, Sabatelli M, Monsurrò MR, Battistini S, Salvi F, Spataro R, Sola P, Borghero G, Galassi G, Scholz SW, Taylor JP, Restagno G, Chiò A, Traynor BJ (2010). "Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS".Neuron 68 (5): 857–864

 Phukan J, Pender NP, Hardiman O (2007). "Cognitive impairment in amyotrophic lateral sclerosis"Lancet Neurol 6 (11): 994–1003

Turner MR, Parton MJ, Shaw CE, Leigh PN, Al-Chalabi A (2003). "Prolonged survival in motor neuron disease: a descriptive study of the King's database 1990–2002"J Neurol Neurosurg Psychiatry 74 (7): 995–997.

 Watanabe H, Saito Y, Terao S, et al. (May 2002). "Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients"Brain 125 (Pt 5): 1070–83

www.wikipedia.org

 

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