Sudden death in MSA is hypothesized to be a consequence of disordered central respiration, suffocation caused by sputum and food, upper airway obstruction from NPPV acting on a floppy epiglottis, cardiac autonomic disturbance, or a combination of these factors.
People typically live about 7 to 10 years after multiple system atrophy symptoms first appear. However, the survival rate with MSA varies widely. Death is often due to respiratory problems, infections or blood clots in the lungs (pulmonary embolus).
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. alpha-synuclein misfolds in MSA.
MSA damages the nervous system. The disease tends to progress rapidly. About one half of people with MSA-P have lost most of their motor skills within 5 years of onset of the disease.
A person with MSA will have increased difficulty with movement and eventually become bedridden. People with MSA often develop swallowing problems that can lead to pneumonia in the later stages of the disease.
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.
Sleep disorders in patients with MSA include rapid eye movement sleep behavior disorder (RBD), excessive daytime sleepiness (EDS), and nocturnal sleep disturbances. Previous studies showed that 69% to 100% of patients with MSA experience RBD.
These include loss of consciousness, changes to skin colour, and changes to breathing. Read more on our page, final moments of life.
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.
The most common cause of sudden cardiac death is ventricular fibrillation, which is a kind of arrhythmia that causes the heart muscle to be unable to contract as usual. This situation makes regular heartbeats impossible, consequently preventing the pumping of blood throughout the body.
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.
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.
The first organ system to “close down” is the digestive system. Digestion is a lot of work! In the last few weeks, there is really no need to process food to build new cells. That energy needs to go elsewhere.
Terminal agitation is typically seen during the hours or days before death and can be distressing and overwhelming for caregivers.
This explains why some symptoms of MSA such as a tremor or speech difficulty can seem temporarily worse in stressful situations. Feeling anxious and worried is a familiar feeling for many people affected by MSA and it can easily become an unhelpful cycle.
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.
Alongside the bladder difficulties, people with MSA also experience bowel difficulties.
The disease was first known as Shy-Drager Syndrome. Currently, it is believed that MSA is “sporadic,” meaning that there are no established genetic or environmental factors that cause the disease. A few reports have described families with MSA, but this finding is probably very rare.
Red flags supporting the diagnosis of MSA include the following: Orofacial dystonia. Disproportionate antecollis. Severe anterior flexion of the spine (camptocormia)
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 rare, progressive, fatal, neurodegenerative disorder. There are two main types: the parkinsonian type (MSA-P) and cerebellar type (MSA-C). The disease usually presents with genitourinary dysfunction, orthostatic hypotension, and rapid eye movement (REM) sleep behavior disorder.
Deconditioning – having MSA means a greater effort is needed to be mobile, this can lead to deconditioning of the muscles and the cardiovascular system, which in turn can lead to fatigue.
Hallucinations have been reported to occur in 5–9% of patients with MSA but rarely in CBD.