Multiple system atrophy is a degenerative neurological disorder. It is marked by a combination of symptoms affecting movement, blood pressure and other body functions; hence it is termed as “multiple system” atrophy.
Cause
In multiple system atrophy there is damage to autonomous nervous system with degeneration of nerve cells in specific areas of the brain. This cell degeneration causes problems with movement, balance and automatic functions of the body such as bladder control. However, the exact cause of MSA is unknown and no specific risk factors have been identified with it.
In some cases, a diagnosis of MSA can only be confirmed post-mortem. When brain tissue of a person with MSA is examined under a microscope, structures called glial cytoplasmic inclusion bodies are visible. These are also known as Papp Lantos bodies. These are the only significant findings which form the hallmark of MSA.
Symptoms
Multiple system atrophy presents with a wide range of symptoms including:
- Orthostatic hypotension: low blood pressure when standing with dizziness, fainting or blurred vision.
- Urinary disturbances: urgency, frequency, incomplete bladder emptying or an inability to pass urine (retention).
- Muscle rigidity and/or tremors with slow movement. It can also present as Parkinsonism, which includes slow, stiff movement, writing becomes small and spidery, involuntary shaking of head and hands.
- Postural instability: loss of balance, in coordination in movements
- Impotency: In men, the first sign to be observed is erectile dysfunction
- Speech and swallowing difficulties.
- Constipation
- Dry mouth and skin.
- Trouble regulating body temperature due to abnormal sweating.
- Abnormal breathing during sleep.
When autonomic failure is pronounced along with predominating Parkinsonism it is termed as Shy Drager Syndrome. This term is now not used often.
It must be noted that all patients with MSA may not experience these symptoms; some may experience a few or some in combination with the other.
Prognosis
There is no remission from the disease. The rate of progression differs in every case and speed of decline may vary widely in individual patients. It has been observed that MSA relatively progresses more quickly than Parkinson’s disease.
Treatment
There is no cure for MSA, so treatment involves treating the symptoms. It is extremely important to manage the blood pressure which falls on standing, which shall lead to serious injuries if the patient collapses. So proper care and medication has to be taken in such cases.
Non-drug treatments include “head-up tilt” (elevating the head of the whole bed by about 10 degrees), salt tablets, generous intake of fluids, and pressure (elastic) stockings. Avoiding of triggers of low blood pressure (e.g. hot weather, alcohol, dehydration) are crucial.
For predominant symptoms of Parkisonism, the drug Levodopa transiently helps the condition. The difficulties in walking/movement, daily tasks, speech can be managed well with physiotherapy, occupational therapy and speech therapy.
we have recorded a patient with MSA who is under care since early 2008. We have yet to achieve some significant results with this case.
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