Multiple system atrophy is a rare neurodegenerative disorder characterized by autonomic dysfunction, tremors, slow movement, muscle rigidity, and postural instability and ataxia.
Syncope, urinary incontinence, impotence, constipation, fecal incontinence, cardiac arrythmias as well as other symptoms occur as a result of widespread pathological changes in multiple areas of the central and autonomic nervous system.
Today, Shy-Drager Syndrome (now known as called Multiple System Atrophy) is a neurological disease resulting from degeneration of certain nerve cells in the brain and spinal cord. Body functions controlled by these areas of the brain and spinal cord function abnormally in patients with this disease.
In general, life expectancy is shorter than usual for Cerebellar Degenerative Ataxia patients. Many, however, may live into their 50s or even their 60s.
There is no cure for hereditary forms of cerebellar degeneration. Treatment is usually supportive and is based on the person's symptoms or on disorders that may contribute to the cerebellar degeneration.
The symptoms of cerebral atrophy vary depending on which area of the brain is affected. Depending on the disease or disorder causing the cerebral atrophy, symptoms can include: Dementia (the loss of the ability to think, reason, or remember to the extent that it interferes with a person's daily life and activities)
Multiple system atrophy- parkinsonian type (MSA-P) is a rare condition that causes symptoms similar to Parkinson disease. However, people with MSA-P have more widespread damage to the part of the nervous system that controls important functions such as heart rate, blood pressure, and sweating.
Appetite reduces and weight loss is apparent. Communication becomes too effortful and breathing more bubbly or shallow. Dying is very rarely a dramatic event. In the majority of cases it is an increasing winding down of all bodily functions and everything stopping, death occurring in a peaceful and dignified manner.
Multiple system atrophy (MSA) is a rare, degenerative neurological disorder affecting your body's involuntary (autonomic) functions, including blood pressure, and motor control. MSA was formerly called Shy-Drager syndrome, olivopontocerebellar atrophy or striatonigral degeneration.
MSA progresses faster.
People with Parkinson's disease usually take years to develop autonomic dysfunction. With MSA, autonomic dysfunction can start within a year.
Symptoms of multiple system atrophy (MSA) Symptoms of MSA usually start when someone is between 50 and 60 years of age, but they can begin at any time after 30. The symptoms are wide-ranging and include muscle control problems, similar to those of Parkinson's disease.
Prognosis is currently guarded, with most MSA patients passing away from the disease or its complications within 6-10 years after the onset of symptoms.
Listen, listen, listen: Living with MSA can be very isolating. The family may be eager to talk about what they are going through so listening and showing empathy can be one of the most helpful things you can do. Or they may just want a light, fun evening with laughter. Pay attention to their cues and follow their lead.
Outlook / Prognosis
People with FD have reduced life expectancies. About half of people with this condition live into their 30s.
Sleep disorders in patients with MSA include rapid eye movement sleep behavior disorder (RBD), excessive daytime sleepiness (EDS), and nocturnal sleep disturbances.
Though dementia is not considered a common characteristic of MSA, cognitive impairment occurs in some patients in the form of loss of verbal memory and verbal fluency1.
Higher H-Y stage indicates a more severe neuromuscular state in MSA-P and is considered to be related to higher energy expenditure and decrease of BMI. Patients with MSA-P lose weight as the disease progresses.
We found that 30 MSA patients (46.15%) suffered from pain. There was a trend towards a higher prevalence in MSA-P compared to MSA-C patients although the difference was not significant, which might be due to the small sample size. Few studies have investigated the pain mechanism in MSA patients.
Multiple system atrophy is a complex condition that is likely caused by the interaction of multiple genetic, environmental, and lifestyle factors. Some of these factors have been identified, but many remain unknown.
Our findings show that smoking history and/or heavy alcohol use is associated with younger age of onset in MSA but do not influence survival.
Different drugs can have neurotoxic and destructive effects on brain cells. Substances that are associated with neurological damage include but are not limited to alcohol, heroin, amphetamines, marijuana, opioids, inhalants, and cocaine.
Mild cases of brain atrophy may have little effect on daily functioning. However, brain atrophy can sometimes lead to symptoms such as seizures, aphasia, and dementia. Severe damage can be life threatening.
Some degree of atrophy and subsequent brain shrinkage is common with old age, even in people who are cognitively healthy. However, this atrophy is accelerated in people with mild cognitive impairment and even faster in those who ultimately progress from mild cognitive impairment to Alzheimer's disease.