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Multiple System Atrophy

Neurological disorder with degeneration of nerve cells in certain brain areas, leading to movement, balance, and autonomic function problems.Encyclopedia Entry for Multiple System Atrophy :Multiple system atrophy cerebellar subtype. MSA-C can be passed down through families (inherited form). It can also affect people without a known family history (sporadic form). Researchers have identified certain genes that are involved in the inherited form of this condition. The cause of MSA-C in people with the sporadic form is not known. The disease slowly gets worse (is progressive). MSA-C is slightly more common in men than in women. The average age of onset is 54 years old.Symptoms of MSA-C tend to start at a younger age in people with the inherited form. The main symptom is clumsiness ( ataxia ) that slowly gets worse. There may also be problems with balance, slurring of speech, and difficulty walking. Other symptoms may include: Abnormal eye movements Abnormal movements Bowel or bladder problems Difficulty swallowing Cold hands and feet Lightheadedness when standing Headache while standing that is relieved by lying down Muscle stiffness or rigidity, spasms, tremor Nerve damage (neuropathy) Problems in speaking and sleeping due to spasms of the vocal cords Sexual function problems Abnormal sweating.A thorough medical and nervous system examination, as well as a symptom and family history are needed to make the diagnosis. There are genetic tests to look for the causes of some forms of the disorder. But, no specific test is available in many cases. An MRI of the brain may show changes in the size of affected brain structures, especially as the disease gets worse. But it is possible to have the disorder and have a normal MRI. Other tests such as positron emission tomography (PET) may be done to rule out other conditions. These may include swallowing studies to see if a person can safely swallow food and liquid.There is no specific treatment or cure for OPCA. The aim is to treat the symptoms and prevent complications. This may include: Tremor medicines, such as those for Parkinson disease Speech, occupational and physical therapy Ways to prevent choking Walking aids to help with balance and prevent falls.The following groups can provide more information on MSA-C: National Institute of Neurological Disorders and Stroke -- www.ninds.nih.gov/Disorders/All-Disorders/Multiple-System-Atrophy-Information-Page National Organization for Rare Disorders -- www.rarediseases.org/rare-diseases/multiple-system-atrophy.MSA-C slowly gets worse, and there is no cure. The outlook is generally poor. But, it may be years before someone is very disabled.Complications of MSA-C include: Choking Infection from inhaling food into the lungs ( aspiration pneumonia ) Injury from falls Nutrition problems due to difficulty swallowing.Call your health care provider if you have any symptoms of MSA-C. You will need to be seen by a neurologist. This is a doctor who treats nervous system problems.MSA-C; Cerebellar multiple system atrophy; Olivopontocerebellar atrophy; OPCA; Olivopontocerebellar degeneration.Central nervous system Central nervous system and peripheral nervous system.Ciolli L, Krismer F, Nicoletti F, Wenning GK. An update on the cerebellar subtype of multiple system atrophy. Cerebellum Ataxias. 2014;1-14. PMID: 26331038 www.ncbi.nlm.nih.gov/pubmed/26331038. Jancovic J. Parkinson disease and other movement disorders.Encyclopedia Entry for Multiple System Atrophy :Multiple system atrophy. The cause is unknown. MSA develops gradually and is most often diagnosed in men older than 60.MSA damages the nervous system. Symptoms may include: Face changes, such as a masklike appearance to the face and staring Difficulty chewing or swallowing (occasionally), not able to close the mouth Disrupted sleep patterns (often during rapid eye movement [REM] sleep late at night) Dizziness or fainting when standing up or after standing still Frequent falls Erection problems Loss of control over bowels or bladder Problems with activity that requires small movements (loss of fine motor skills), such as writing that is small and hard to read Loss of sweating in any part of the body Mild decline in mental function Movement difficulties , such as loss of balance, shuffling when walking Muscle aches and pains (myalgia), and stiffness Nausea and problems with digestion Posture problems, such as unstable, stooped, or slumped over Slow movements Tremors Vision changes , decreased or blurred vision Voice and speech changes Other symptoms that may occur with this disease: Confusion Dementia Depression Sleep-related breathing difficulties, including sleep apnea or a blockage in the air passage that leads to a harsh vibrating sound.Your health care provider will examine you, and check your eyes, nerves, and muscles. Your blood pressure will be taken while you are lying down and standing up. There are no specific tests to confirm this disease. A doctor who specializes in the nervous system (neurologist) can make the diagnosis based on: History of symptoms Physical examination results Ruling out other causes of symptoms Testing to help confirm the diagnosis may include: MRI of head Plasma norepinephrine levels Urine examination for norepinephrine breakdown products ( urine catecholamines ).There is no cure for MSA. There is no known way to prevent the disease from getting worse. The goal of treatment is to control symptoms. Dopaminergic medicines, such as levodopa and carbidopa, may be used to reduce early or mild tremors. But, for many people with MSA these medicines do not work well. Medicines may be used to treat low blood pressure. A pacemaker that is programmed to stimulate the heart to beat at a rapid rate (faster than 100 beats per minute) may increase blood pressure for some people. Constipation can be treated with a high-fiber diet and laxatives. Medicines are available to treat erection problems.Outcome for MSA is poor. Loss of mental and physical functions slowly get worse. Early death is likely. People typically live 7 to 9 years after diagnosis.Call your provider if you develop symptoms of this disorder. Call your provider if you have been diagnosed with MSA and your symptoms return or get worse. Also call if new symptoms appear, including possible side effects of medicines, such as: Changes in alertness/behavior/mood Delusional behavior Dizziness Hallucinations Involuntary movements Loss of mental functioning Nausea or vomiting Severe confusion or disorientation If you have a family member with MSA and their condition declines to the point that you are unable to care for the person at home, seek advice from your family member's provider.Shy-Drager syndrome; Neurologic orthostatic hypotension; Shy-McGee-Drager syndrome; Parkinson plus syndrome; MSA-P; MSA-C.Central nervous system Central nervous system and peripheral nervous system.Fanciulli A, Wenning GK. Multiple system atrophy. N Engl J Med. 2015;372(3):249-263. PMID: 25587949 www.ncbi.nlm.nih.gov/pubmed/25587949. Jankovic J. Parkinson disease and other movement disorders.