Although the initial stimulus in the MSA remains unknown, the inflammation mechanism is common (12). Extensive microglial activation and neuroinflammation with diffuse T-cell infiltration have been found in postmortem studies of MSA brains (8, 13–18).
Potential toxic products include inflammatory cytokines and other inflammatory markers, produced and released by activated microglia. Therefore, it is speculated that there is an inflammatory state in the brain of MSA, which may be associated with the neurophysiological cause of the disease.
Slowness of movement and feeling stiff
Movement is hard to initiate, and the person will often have a distinctive slow, shuffling walk with very small steps. Some people may also have stiff and tense muscles. This can make it even more difficult to move around and cause painful muscle cramps (dystonia).
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.
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.
Currently, there are no treatments to stop or slow the progression of MSA, and there is no cure. However, there are treatments to help people cope with the symptoms. For some individuals, levodopa (a drug used to treat Parkinson's symptoms) may help improve motor function, but the benefits are often short-lived.
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.
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.
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.
The progression of MSA varies, but the condition does not go into remission. As the disorder progresses, daily activities become more difficult.
Its symptoms often mimic those of Parkinson's disease and ataxia. There is no cure, and many physicians are not familiar with the condition – meaning MSA is often misdiagnosed. However, symptoms can be managed, which is why it's important to be evaluated and treated by physicians who have experience dealing with MSA.
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.
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.
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.
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 the worst cases, failure of the breathing muscles and/or heart can lead to death. A therapy to combat the muscle wasting and weakness in MSA is needed urgently.
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.
Typical ocular features of MSA include blepharospasm, excessive square-wave jerks, mild to moderate hypometria of saccades, impaired vestibular-ocular reflex (VOR), nystagmus and impaired event-related evoked potentials.
Autonomic Symptoms
Symptoms can include: Cold hands or feet and heat intolerance, because control of body temperature is impaired.
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.
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.
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.
Attention, execution, verbal and visual memory, verbal fluency, and new learning were impaired in patients with MSA. MSA-P had more impairment in motor and mental speed, working memory, executive functions, and focused attention compared to MSA-C.
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 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.