Cognition is affected in only a small percent of cases.1 Throughout the course of the illness patients should be encouraged to participate in all decisions concerning their health. Patient and family may benefit by participating in local and national support groups. They provide the opportunity to share ideas on ways to manage the physical, psycho-social, and financial aspects of this complex disease.
The medical interdisciplinary health team plays a critical role in assisting the patient and caregivers in the discussion of end-of-life issues. As the disease progresses the struggle to make choices is an on-going process. The primary care giver is the spouse in the vast majority of cases and needs to be aware of what is driving their choices. Hospice is an excellent resource for all involved, both during and following the illness. The chaplain may be able to help individuals recognize the difference between giving up and letting go. Learning to live encompasses the ability to let go. It is an acceptance of our mortality and humanity. MSA is definitely a very demanding, challenging and life-shortening disorder.

Jack and his immediate family were fortunate in being able to address advance directives together. He was very spiritual and his religious faith played a major role in his ability to accept the challenge of living and dying with MSA. He selected Hospice and comfort measures were instituted in the nursing home. Increased respiratory secretions and the inability to cough effectively required frequent suctioning. Jack’s restlessness and discomfort were relieved by IV morphine and he died peacefully with his family present.

Support groups
The SDS/MSA Support Group. 2004 Howard Lane, Austin, TX.78728
(1-800-288-5582).
http://www.Shy-drager.com
American Academy of Neurology, 1080 Montreal Ave., St.Paul, MN 55116-612-695-1940. http://www.rarediseases.org
Anatomic Dysfunction Center, Vanderbilt University Medical Center.
Nashville, TN 37232-2195. Phone:615-343-8649.
References

1. Gilman S, Wenning GK, et.al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology. 2008;71(9):670-76.

2. Bradley WG, Daroff, RB, Fenichel GM, Jankovic J. eds. Neurology in Clinical Practice. (4th ed), 2004. Philadelphia, Pa: Butterworth-Heinemann/Elsevier).

3. Shlossmacher M, Hamann C, Cole A, et.al. A 79 year-old woman with disturbances in gait, cognition and autonomic function. New England Journal of Medicine. 2004;351(9):912-922.

4. Low PA, Singer W. Up date on management of neurogenic orthostatic hypotension. Lancet Neurology. 2008;7(5):451-58.

5. Olanow CW, Stern MB, Serbi K. The scientific and clinical basis for the treatment of Parkinson disease. Neurology. 2009;72(Suppl 4):S6,S80-33.
 
6. Lee Y-C, Liu CS, et.al. The ‘hot cross bun’ sign in the patients with spinocerebellar ataxia. Eurpean .Journal of Neurology. 2009;16(4):513-16.

7. Van de Warrenburg BP, Bhati KP, Quin NP. Causes of death in multiple system atrophy. Journal of neurosurgery-psychiatry. 2001;78(3):327-29.

8. Pavior DC, Williams D, et.al. Is sphincter electromyography helpful in the diagnosis of multiple system atrophy? Mov. Disord. 2005;20(11):1425-30.

9. Iranzo A, Santamaria J, Tolosa E. The clinical and pathophysiologic relevance of REM sleep behavior disorder in neurodeenerative diseases.
Sleep Medical Review. 2009;April 8.(pre pub on internet)

10. Sachin S, Shukla G. Clinical speech impairment in Parkinson’s disease, progressive supranuclear palsy, and multiple system atrophy. Neurology India. 2008;56:122-26.

Review of the brains’s anatomy

Cerebrum, the largest area of the brain, is located above the brain stem. It has 2 hemispheres, right and left, which are divided into lobes, each having a particular function.

The frontal lobe plays an important role in developing of one’s reasoning, decision making skills, intelligence, memory, behavior and personality. The left frontal lobe, known as Broca’s area, coordinates speech muscles and major body movements.

The temporal lobes in the lower part of the cerebrum manage most of
the auditory activities e.g. translating words into meaning. It is involved in behavior, emotions and speech development. In most right handed people the left temporal lobe controls language comprehension, thus it is called the dominant lobe.

The parietal lobes, located in the upper central area, process messages being sent to and from the lobes concerning physical sensations. It aids knowing the right from left, spatial orientation, and awareness of body parts. It facilitates one’s ability to calculate, read and write.

The occipital lobes, in the back portion of the cerebrum, process what is seen by the retinas, allowing one to judge distances and spatial
relationships. Like the temporal lobes, the right lobe processes what is seen in the left field of vision.

The cerebellum, located posterior to the brain stem, helps to coordinate movement, balance, posture and fine muscle control. Current research reveals the cerebellum may enhance one’s ability to listen to speech and music and develop greater awareness of various stimuli.

Brain stem- All functions controlled by the cerebrum pass through the brain stem which is located at the base of the brain. It is composed of 3 parts: midbrain, pons and medulla oblongata. This area controls involuntary functions: breathing, blood pressure, heart rate, and swallowing. It aids in developing sleep/wake patterns, and involuntary functions. It has 12 cranial nerves which control hearing, vision, sense of smell and balance.

The spinal cord, a continuation of the brain stem, receives and transmits sensory information and motor commands to the body via neurons. This transfer of impulses is an electro-chemical reaction which requres neuro-transmission of chemicals e.g. dopamine, acetycholine or serotonin. The axons are covered with a myelinated sheath which enhances the speed of the impulse. A neuron can receive and transmits multi impulses over a variety of pathways simultaneously. In MSA the glia cells have an abnormal protein called alpha-synctein, which interferes with the production of the myelin sheath that protects the neuron, causing dysfunction of the neuron.

The autonomic nervous system (ANS) is comprised of 2 divisions: sympathetic nervous system( SNS) and the parasympathetic nervous system (PNS). They maintain one’s dynamic equilibrium by having an opposing, but complimentary effect on the body’s organs During times of stress or emergencies, the SNS, stimulates the vital organs, e.g. heart, lungs, etc. The PNS , sometimes referred to as the “rest and digest” system because it mainly stimulates the GU, GIT and sexual function.
 
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