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The Challenge of MSA

by Margo Quinlan, RN, MSN
Laboure College, Boston, Ma.

I visit my brother Jack four to five times a year. After a recent visit I asked my husband if he noticed that Jack seemed very quiet, distant and had little to add to the conversation. Jack’s affect was flat. I thought he might be depressed since he had recently retired and there had been a death in the family. Four months later Jack was diagnosed with Parkinson’s Disease (PD) by his primary physician and started on Sinemet. I was surprised since he didn’t have tremors, but his walk was slower.

Five months later his wife called and said “Jack has been diagnosed with MSA and there’s not much they can do.” I asked if she meant MRSA. “No, it’s like Parkinson’s but there’s no known medication that helps.” The diagnosis was made by a neurologist who specializes in multiple system atrophy (MSA).
 
As time passed, Jack’s staring expression (mask-like fascies) became more evident. Difficulty with balance and walking progressed quickly, and he developed a wide stance as a compensatory measure for “freezing”. Jack was never troubled with tremors, but his generalized bradykinesia and stiffness were noticeable. Despite the physical therapy and safety precautions that were instituted, his hupotensive episodes and falls were more numerous. As the months progressed his hoarseness, hypophonia and dysarthria became troublesome. Jack’s speech was difficult for the family and caregivers to understand and frustrating for Jack. Toward the end of this battle with MSA he had difficulty in swallowing, which led to aspiration pneumonia several times. His breathing was stridorous and he required frequent suctioning. Thanks to his family’s support and the wonderful care by Hospice, he died quietly in his sleep from pneumonia.

In the early 1960’s, two doctors from Texas recognized that clinical manifestations of parkinsonism, in conjunction with, autonomic dysfunction was a unique entity, and called it Shy-Drager’s, now referred to as MSA.

MSA is a heterogeneous neurodegenerative disorder with clinical presentation of extrapyradmidal, cerebellar, autonomic or pyramidal symptoms. There are two major subtypes:
MSA-P is primarily caused by degeneration of the striatonigral pathways with a clinical predominance of bradykinesia, rigidity, stiffness and postural imbalance.
MSA-C manifests a predominance of cerebellar symptoms. due to atrophy of the cerebellum, olives and pons This is evidenced by ataxia,, lack of coordination and loss of balance.1

Autonomic dysfunction is the outstanding, defining feature of MSA and is present in both types. The autonomic nervous system is composed of two major divisions; sympathetic nervous system ( SNS) and parasympathetic nervous system(PNS) which oppose, yet balance each other to maintain the body’s equilibrium. Clinical signs vary in terms of areas of the body involved, severity and rapidity of progression. The most frequent signs in MSA are: urinary dysfunction, impotence, orthostatic hypotension, respiratory, gastrointestinal and sleep abnormalities.2

“Definite MSA requires neuropathologic demonstration of CNS alpha-synuclein-positive glial cytoplasmic inclusions with neurodegenerative changes in striatonigral or olivopontocerebellar structure. Probable MSA requires a sporadic, progressive adult onset disorder including rigorously defined autonomic failure and poorly levodopa-responsive parkinsonism or cerebellar ataxia. Possible MSA requires a sporadic, progressive adult onset disease including parkinsonism or cerebellar ataxia and at least one feature suggesting autonomic dysfunction plus, one other feature that may be a clinical or neuroimaging abnormality”

What actually causes MSA?

A misprocessed protein, alpha-synuclein-positive, is present in the oligodendrocytes cells. It is known as glial cytoplasmic inclusions (GCI) and interferes with the normal production of the myelin sheath, a protective layer around the axons.1 No known treatment prevents or slows the progression of MSA, which is usually faster than idiopathic Parkinson’s. 1

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