Multiple system atrophy (MSA) with orthostatic hypotension

Overview

A progressive neurological disorder involving the central and autonomic nervous system

Symptoms

Orthostatic hypotension Urinary dysfunction Bowel dysfunction Sexual impotence Absence of sweating Parkinson-like symptoms Cerebellar incoordination Muscle wasting Involuntary muscle contractions Coarse leg tremors

Prognosis

MSA usually progresses more quickly than Parkinson's disease.There is no remission from the disease. The remaining lifespan after the onset of symptoms is on average about 9 years.Almost 80% of patients are disabled within 5 years of onset of the motor symptoms, and only 20% survive past 12 years. Rate of progression differs in every case and speed of decline may vary widely in individual patients.

Treatment

There is no discovered cure for MSA, so treatment involves treating the symptoms. Management by rehabilitation professionals (physiotherapists, occupational therapists, speech therapists, and others) for problems with walking/movement, daily tasks, and speech problems is essential. Also social workers can help with coping with disability and access to health care services, both for the person with MSA as well as his/her family caregivers. Ongoing care from a neurologist specialized in "movement disorders" is recommended as the complex symptoms of MSA are often not familiar to less-specialized health care professionals. One particularly serious problem, the drop in blood pressure upon standing up (with risk of fainting thus injury from falling) often responds to fludrocortisone, a synthetic mineralocorticoid. Another common drug treatment is midodrine (an alpha-agonist.) 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. Avoidance of triggers of low blood pressure (e.g. hot weather, alcohol, dehydration) are crucial. Hospice/homecare services can be very useful as disability progresses. Levdopa (L-Dopa) (a drug used in the treatment of Parkinson's disease)fails to improve the parkinsonian symptoms of most MSA patients. A recent trial reported that only 1.5% of MSA patients experienced a >50% improvement when taking levodopa, and even this was a transient effect lasting less than one year. Poor response to L-Dopa has been suggested as a possible element in the differential diagnosis of MSA from Parkinson's disease. A recent study conducted in Europe failed to find an effect for the drug riluzole in treating MSA or PSP