Researchers have also examined environmental factors that could contribute to the risk of multiple system atrophy. Initial studies suggested that exposure to solvents, certain types of plastic or metal, and other potential toxins might be associated with the condition.
For a diagnosis of “probable MSA” a patient must have autonomic dysfunction, including otherwise unexplained urinary urgency, frequency, or incomplete emptying, erectile dysfunction in males, or orthostatic blood pressure drop by at least 30 mmHg systolic or 15 mmHg diastolic within 3 min of standing.
They suspect it has to do with genetic mutations that affect how certain cells use α-synuclein. There's also evidence that MSA-C runs in families.
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.
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.
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.
The first cases of MSA were presented as olivopontocerebellar atrophy (OPCA) about a century ago. The Shy-Drager syndrome with features of parkinsonism and autonomic failure with OH was described in 1960. The term MSA was introduced to unify different forms of MSA in 1996.
Sleep disorders in patients with MSA include rapid eye movement sleep behavior disorder (RBD), excessive daytime sleepiness (EDS), and nocturnal sleep disturbances.
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.
As already mentioned, individuals with MSA undergo motor and muscle degeneration as the disease progresses. Physiotherapists can help maintain muscle range of motion and tone by using passive range of motion in combination with an exercise program that includes resistance training and/or gait/balance training.
The progression of MSA varies, but the condition does not go into remission. As the disorder progresses, daily activities become more difficult. Possible complications include: Breathing problems during sleep.
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.
Blood Tests
Individuals with MSA have near normal levels of a neurotransmitter called norepinephrine, which is used in the autonomic nervous system. This chemical is often decreased in PAF, and is used in some centers for diagnosis of PAF (peripheral)5. There are no diagnostic blood tests for MSA at present.
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.
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.
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.
However, in the last few years, cognitive impairment was found to be a frequent feature in MSA based on evidence from qualitative neuropsychological assessment. Dementia in MSA is now reported in 14-16% of cases.
Approximately 90 percent of individuals with MSA will experience parkinsonism symptoms including slowness of voluntary movements (bradykinesia), muscle stiffness (rigidity) which may make it difficult to bend the arms and/or legs, and impaired balance (postural instability).
The incidence of MSA is 3:100,000 in adults aged over 50 years, and the course of illness lasts 5 to 10 years. There are 2 known groups: MSA-P (parkinsonian) and MSA-C (cerebellar) that have clinical presentations associating them closely with more common movement disorders (eg, Parkinson's disease [PD] and ataxia).
MSA is broken down into two main subtypes based on the predominant symptom: MSA-predominant Parkinsonism (MSA-P), and MSA-predominant cerebellar ataxia (MSA-C). A patient diagnosed with MSA-P may over time appear to have MSA-C and vice versa, so these categorizations are not always set in stone.
The diagnostic accuracy was 71% in probable MSA and 60% in possible MSA. Correctly diagnosed patients with MSA had a younger age at onset and age at death than patients with PD or PSP, but duration of symptoms did not differ.
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.
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.
Several MRI abnormalities on conventional MRI already are included in the current diagnostic criteria for MSA along with abnormalities of functional neuroimaging, including 18F‐flurodeoxyglucose positron‐emission tomography (FDG‐PET) and presynaptic dopaminergic imaging.