Multiple system atrophy is a rare progressive degenerative neurological disorder. In this disorder the area of brain which controls internal processes or autonomic functions such as blood pressure, bladder control, heart rate, control of muscles, sweat etc start degenerating. It is a disorder of adults affecting at an average age of 55 to 65 years, with more frequency in men. It manifests variety of symptoms some of them resemble those of Parkinson disease. However, along with it there is malfunction of the internal process which is not present in Parkinson’s.
The exact cause of disease is not known.
Possible Causes Of Multiple System Atrophy
Multiple system atrophy comprises of three disorders that were previously believed to be separate disorders. In 1969, Graham and Oppenheimer introduced them as single entity. The three disorders include:
- Olivopentocerebellar atrophy in which there is coordination loss and problem of balancing the body.
- The second is striatonigral degeneration which has symptom similarity with Parkinson’s disease.
- The third is Shy-Drager syndrome, in which besides symptoms of Parkinsonism, there is associated problem of profound change in blood pressure and urinary problem etc.
Multiple system atrophy begins with development of one of the three disorders. Gradually symptoms of other two disorders develop. Within 5 to 6 years all disorders become one entity referred as multiple system atrophy.
The exact cause of multiple system atrophy is unknown. Some researchers believe that genetic factor and exposure to environmental toxins such as organic solvents, plastic monomers, pesticides and additives are the causative factors. However, it is not yet confirmed and more research is needed.
One thing is sure; the disorder causes degeneration and shrinkage of brain cells in certain part of brain called brain stem, cerebellum and basal ganglia. This portion of brain regulates involuntary functions in the body such as digestion, blood pressure, sweating etc. The brain tissue when seen under microscope reveals too much accumulation of protein called alfa-synuclein.
Symptoms Of Multiple System Atrophy
Multiple system atrophy affects people over the age of 50 with predominance among men. The symptoms have been classified into two categories; Parkinsonian and cerebellar. The signs and symptoms of Parkinsonism may appear early. They develop due to degeneration of brain cells in basal ganglia. Symptoms include rigidity of muscles, slow and shaky movement with difficulty in bending arms and legs. The unsteady gait may lead to fall. Patient often stoops. Unlike Parkinson’s the typical tremors are absent in this condition.
The symptoms of cerebellar variety include difficulty in coordination of muscles as a result the patient walks awkwardly with wide steps. Patient is slow to talk and has slurred speech. Double vision and difficulty on focusing an object is common. In addition patient has malfunction of several body processes. There is dramatic rise of blood pressure when a person stands from sitting or reclining position. He feels giddy and heavy headed. The phenomenon is called orthostatic hypotension.
Patient may also present with urinary problems such as incontinence, increased frequency, and urinary retention. Constipation is common due to sluggish bowel movement. Patient also suffers from loss of libido. Patient experiences difficulty in swallowing food. Often there is loss of control over bowel movement.
Symptomatic Treatment Of Multiple System Atrophy
Multiple system atrophy is incurable condition. Gradually after 5 to 10 years most patient die, but sometimes a person may survive longer. Hence the main aim of treatment is focused on making patients day to day life easier and to alleviate symptoms with multidisciplinary approach.
- Since orthostatic hypotension is a major concern, patient is prescribed medications that will raise the blood pressure. Also patient is advised to stand up slowly from sitting position so as to reduce the episodes of sudden fall in blood pressure. Wearing abdominal binder and compression stocking increase blood flow from limbs towards heart which will help to maintain blood pressure.
- Medicines are prescribed to reduce Parkinson’s disease like symptoms.
- Due to reduced sweating patient must avoid staying in warm climate. He may have to use artificial tears to relieve dry eyes and visit dentist regularly if dry mouth is a concerning problem.
- Pacemaker may be implanted in the heart to increase the electrical impulse and heart beat. This may help to stabilize blood pressure to normal level.
- In case of loss of libido, impotence drugs are effective.
- Physical therapy will be useful to maintain proper posture and balance.
- Speech therapy is useful if the patient has slurred speech.