Neuro Diseases: Multiple System Atrophy

Multiple System Atrophy (MSA), also called as multisystem atrophy, as the name implies is associated with atrophy (thinning) of multiple systems within the brain.

It is a degenerative neurological disorder. Progressive atrophy of various structures (like extrapyramidal, cerebellar, brain stem, autonomic centers etc) within the brain tissue takes place in different combinations.

This disease is most often seen in individuals above 50. Men are affected slightly more than women. Any race or ethnicity can get this disease.

The cause is not yet clear. Some exposure to environmental toxin is a possibility but never confirmed. Most of these MSA are acquired without any genetic basis so far.

MSA was traditionally classified as;

• Shy drager syndrome
• OPCA (Olivoponto cerebellar atrophy)
• SN (Striato nigral degeneration)

But now a days generally it is divided into 2 categories;

1) MSA – p type (with predominant parkinson features)

2) MSA – c type (with predominant cerebllar type)

Patients will present with certain distinct clinical manifestations including;

• Extra pyramidal manifestation like parkinsonian features - rigidity or stiffness of the limbs & body, slowness of movements, tremors etc

• Cerebellar type manifestation - ataxia/in coordination, balance issues, speech disturbances etc

• Autonomic system disturbances - orthostatic hypotension (drop in blood pressure on sitting up or standing) causing dizziness, fainting mostly in an upright position

Diagnosis is made based on clinical manifestations plus some laboratory investigations. An imaging study of the brain like MRI or CT may reveal atrophy of cerebellum, brain stem, basal ganglial areas etc. Autonomic nervous system testing is indicated. There are different ways of doing it including ANSAR system, using traditional methods etc.

Unfortunately there is no cure yet for this disorder. So supportive & symptomatic intervention are provided.

The orthostatic hypotension may be treated with drugs like fludrocortisone, midodrine etc. There are many other measures that might help including this.

Parkinsnian features may be treated with medications like levodopa or similar drugs but they are not much helpful.

A neurologist is generally involved in the care of patients with Multiple System Atrophy.