Multiple system atrophy

Multiple system atrophy (MSA) is a degenerative neurological disorder.

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Presentation

MSA is characterized by a combination of the following:

• Progressive damage to the autonomic nervous system, commonly leading to low blood pressure upon standing, difficulty urinating, and/or abnormal breathing during sleep
• Muscle rigidity +/ tremor and slow movement (Parkinsonism)
• Poor coordination / unsteady walking (ataxia)

When autonomic failure predominates, the term Shy-Drager syndrome is often used, although this term is no longer current, given the recent terminology changes which are explained below.

MSA is associated with the 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.

Nerve cells in the affected areas of the brain shrink (atrophy). When brain tissue of a person with MSA is examined under a microscope, structures called glial cytoplasmic inclusion bodies or Papp-Lantos bodies. It is the presence of these inclusion bodies in the movement, balance and automatic control centres of the brain that confirms a diagnosis of MSA.

Symptoms

For men, the first sign is often erectile dysfunction (unable to achieve or sustain an erection). Both men and women often experience problems with their bladders including urgency, frequency, incomplete bladder emptying or an inability to pass urine (retention).

As the disease progresses three groups of symptoms predominate. These are:

• parkinsonism (slow, stiff movement, writing becomes small and spidery)
• cerebellar dysfunction (difficulty coordinating movement and balance)
• autonomic dysfunction (impaired automatic body functions) including:

— postural or orthostatic hypotension, resulting in dizziness or fainting upon standing up

— urinary incontinence

— impotence

— constipation

— dry mouth and skin

— trouble regulating body temperature due to abnormal sweating

— abnormal breathing during sleep

Not all patients experience all of these symptoms.

Prognosis

MSA usually progresses more quickly than Parkinson's disease.^[1] Almost 80% of patients are disabled within 5 years of onset of the motor symptoms, and only 20% survive past 12 years.^{[citation needed]} The mean survival is roughly 6 years.^{[citation needed]} Rate of progression differs in every case and speed of decline may vary widely in individual patients.

Treatment

There is no 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.

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.

Levdopa (L-Dopa) often only transiently or does not alleviate the parkinsonian symptoms of most MSA patients. In fact, poor response to L-Dopa has been suggested as a possible element in the differential diagnosis of MSA from Parkinson's disease.

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.

Hospice/homecare services can be very useful as disability progresses.

Terminology

Other terms have been used to refer to this disorder, based on the predominant systems presented. These terms and their distinctions have been dropped in recent (1996 onwards) medical usage^[2] and replaced with MSA subtype naming, but are helpful to understanding the older literature about this disease:

References

1. ^ Bower J, Maraganore D, McDonnell S, Rocca W (1997). "Incidence of progressive supranuclear palsy and multiple system atrophy in Olmsted County, Minnesota, 1976 to 1990". Neurology 49 (5): 1284-8. PMID 9371909.
2. ^ The Consensus Committee of the American Autonomic Society and the American Academy of Neurology. Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy. Neurology 1996;46:1470. PMID 8628505.
3. ^ Shy GM, Drager GA. A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study. Arch Neurol 1960;2:511-27. PMID 14446364.