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 brain cells of a person with MSA contain a protein called alpha-synuclein. A build-up of abnormal alpha-synuclein is thought to be responsible for damaging areas of the brain that control balance, movement and the body's normal functions.
The frequency of cognitive impairment in MSA varies among studies. In autopsy-confirmed cases, it ranges from 20% to 37%. b) We previously reported that 33 out of 102 MSA patients (32%) had cognitive impairment; however, only one patient showed cognitive impairment as an initial symptom.
Based on comprehensive neuropsychological assessment, cognitive impairment was found to be a frequent feature in MSA, and clinically-defined dementia is now reported in 14-16% of cases.
Brain imaging scans, such as an MRI , can show signs that may suggest MSA and also help determine if there are other causes that may be contributing to your symptoms.
Polyglutamine (polyQ) diseases such as spinocerebellar ataxias (SCA1, SCA2, SCA3, SCA6, SCA17, DRPLA) and also late-onset Huntington disease (HD) represent the most frequent genetic misdiagnosis due to the considerable clinical overlap with MSA [5].
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.
During the final stages of the disease, patients have trouble chewing, swallowing, speaking and breathing.
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 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.
Dementia is typically diagnosed when acquired cognitive impairment has become severe enough to compromise social and/or occupational functioning. Mild cognitive impairment (MCI) is a state intermediate between normal cognition and dementia, with essentially preserved functional abilities.
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].
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.
MRI is useful and indispensable in the diagnosis of MSA and also possibly for monitoring disease progression.
It is documented that fatigue is a symptom of MSA and is prevalent in the early stages while remaining persistent over time. Everyone experiences MSA symptoms differently, and fatigue is no exception. Here are some signs to be mindful of: Weakness or feelings of heaviness, especially in the arms and legs.
PD and MSA patients are prone to pain with insufficient treatment.
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.
Besides motor and autonomic symptoms, patients affected by MSA often complain of neuropsychiatric disorders, including depression, anxiety, emotional lability and pseudobulbar affect, which result in a worsening of quality of life (QoL) and an increase in disability (12–15).
When you feel safer, because the 'danger' has passed or because you no longer fear it, your body returns to a more relaxed state. This explains why some symptoms of MSA such as a tremor or speech difficulty can seem temporarily worse in stressful situations.
MSA affects men and women equally, with an average age of onset of approximately 55 years [2, 3].
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.
What are the symptoms of MSA? Most often, the first clinical symptom a patient will note will be lightheadedness, dizziness, and episodes of passing out, but the first symptoms in some patients may include difficulty initiating movement, body stiffness, urinary incontinence, and increased falls.
Disease Overview
Multiple system atrophy (MSA) is a rare sporadic progressive neurological disorder characterized by a varying combination of symptoms and signs. Onset is during adulthood (>30 years).
In this unselected referral autopsy series of patients with antemortem diagnoses of MSA, the diagnostic accuracy was about 62%, which is within the range of other autopsy series.