Multiple system atrophy is a rare neurodegenerative disorder characterized by autonomic dysfunction, tremors, slow movement, muscle rigidity, and postural instability and ataxia.
Definition. 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.
There's currently no cure for MSA and no way of slowing its progression. People with the condition typically live for 6 to 9 years after their symptoms start and may get worse quickly during this time. Some people may live for more than 10 years after being diagnosed.
The main signs and symptoms are problems with muscle coordination (ataxia), but others may include: Impaired movement and coordination, such as unsteady gait and loss of balance. Slurred, slow or low-volume speech (dysarthria) Visual disturbances, such as blurred or double vision and difficulty focusing your eyes.
The cause of MSA is unknown. The vast majority of cases are sporadic, meaning they occur at random. A distinguishing feature of MSA is the accumulation of the protein alpha-synuclein in glia, the cells that support nerve cells in the brain.
Multiple system atrophy (MSA) is a rare neurological disease that causes certain brain areas to deteriorate. Over time, this disrupts abilities and functions handled by those brain areas. This disease is ultimately fatal.
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.
MSA affects men and women equally, with an average age of onset of approximately 55 years [2, 3].
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.
In MSA there may be several stages -- alpha-synuclein accumulates in the oligodendroglial cells, then there is failure of mitochondrial function as well as loss of trophic factor support. Then the oligodendroglia degenerate, followed by microglia and astroglial activation.
Most cases of multiple system atrophy are sporadic, which means they occur in people with no history of the disorder in their family. Rarely, the condition has been reported to run in families; however, it usually does not have a clear pattern of inheritance.
Multiple system atrophy (MSA) is a rapidly progressive neurodegenerative disease with a mean survival of 6–10 years from disease onset1.
PD and MSA patients are prone to pain with insufficient treatment.
There's no cure for multiple system atrophy. Managing the disease involves treating signs and symptoms to make you as comfortable as possible and to maintain your body functions. To treat specific signs and symptoms, your doctor may recommend: Medications to raise blood pressure.
Sleep and breathing problems are very common in MSA patients. Sleep apnea (momentary lapses in breathing), respiratory stridor (noisy breathing), REM behavior disorder (shouting and acting out dreams) and excessive daytime sleepiness can be among the earliest symptoms of MSA.
Neither smoking history nor heavy alcohol use influenced overall survival in MSA patients.
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.
What bowel problems can occur when you have MSA? Constipation is the most common bowel problem experienced by people with MSA. However, a number of people with MSA will experience diarrhoea and some people have fluctuation between constipation and diarrhoea (see points 5, 7 and 9 below).
MSA Life Expectancy (Prognosis)
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.
MRI is useful and indispensable in the diagnosis of MSA and also possibly for monitoring disease progression.
At present, there are no therapies that can reverse or slow the progression of MSA.
An additional manifestation of MSA is sudden death, in which patients die of no apparent reason 24 h after the onset of symptoms [9]. Sudden death in MSA usually occurs during sleep [10] and may occur at an early disease stage [11].
This past March, the FDA granted the drug fast track designation for the treatment of MSA. “Biohaven sees the potential for verdiperstat to be the first disease-modifying treatment for MSA. No disease-modifying treatments currently exist for MSA; only symptomatic and palliative therapies are available,” Qureshi said.
These results suggest distinct autoimmune patterns in MSA and PD. These findings suggest a specific autoimmune physiological mechanism involving responses toward α-syn, differing in neurodegenerative disease with overlapping α-syn pathology.
In general, life expectancy is shorter than usual for Cerebellar Degenerative Ataxia patients. Many, however, may live into their 50s or even their 60s.