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Multiple System Atrophy

The neurological disorder multiple system atrophy (MSA) affects different parts of the brain and causes a variety of symptoms. There is no cure for MSA, but treatment can improve your quality of life and help manage the symptoms. MSA can affect your movement, balance, coordination, blood pressure, breathing, digestion, and other bodily functions.

Causes

The exact cause of MSA is unknown, but it involves the degeneration of certain brain cells that produce a protein called alpha-synuclein. As a result of this protein accumulation in the brain, abnormal deposits known as glial cytoplasmic inclusions (GCIs) form. These deposits interfere with brain cell function and kill them.

It is not inherited and it does not appear to be contagious. There are no clear risk factors for developing MSA, but it may be related to age, environment, or genetics.

Symptoms

MSA symptoms vary depending on the type and the area of the brain affected. There are two main types of MSA:

  • The symptoms of this type can be similar to those of Parkinson's disease, including tremors, stiffness, slowness, and difficulty walking.

  • It is characterized by ataxia (lack of coordination), dysarthria (slurred speech), and nystagmus (involuntary eye movements).

The autonomic nervous system regulates the involuntary functions of the body, such as blood pressure, heart rate, sweating, digestion, and urination. The symptoms of autonomic dysfunction include:

  • Standing up causes dizziness, fainting, or falling due to orthostatic hypotension.

  • Symptoms include headaches, chest pain, or shortness of breath when lying down due to supine hypertension.

  • Symptoms of urinary problems include difficulty starting or stopping urination, incontinence, or retention of urine.

  • Problems with the bowels: Constipation, diarrhea, or incontinence.

  • The symptoms of sexual dysfunction include erectile dysfunction, libido loss, and vaginal dryness.

  • Problems related to thermoregulation: Reduced or excessive sweating, heat intolerance, or cold extremities.

  • A sleep disorder characterized by sleep apnea, snoring, restless legs syndrome, or REM sleep behavior disorder (acting out dreams).

In general, MSA symptoms begin in the 50s or 60s and worsen over time. MSA symptoms vary from person to person, but there is a seven- to ten-year life expectancy after diagnosis.

Diagnosis

MSA is difficult to diagnose because its symptoms overlap with those of other neurological disorders, including Parkinson's disease, cerebellar ataxia, and multiple sclerosis. MSA cannot be diagnosed with a specific test, but it is diagnosed by clinical characteristics, medical history, and excluding other possible causes.

MSA can be diagnosed using the following tests:

  • Tests for infections, inflammations, or metabolic disorders that can cause similar symptoms in the blood.

  • These tests include MRIs, CT scans, or PET scans to detect brain atrophy, lesions, or abnormalities that may indicate MSA or rule out other conditions.

  • Tests of autonomic function: To measure the response of the autonomic nervous system to various stimuli, such as changes in posture, temperature, or breathing.

  • During sleep, sleep studies monitor breathing, heart rate, oxygen levels, and brain activity to detect sleep disorders, such as sleep apnea and REM sleep behavior disorders.

Treatment

Treatment options for MSA depend on the type and severity of the symptoms, but they may include:

  • Fludrocortisone, midodrine, sildenafil, carbidopa-levodopa, clonazepam, or melatonin are some of the drugs used to treat the symptoms of MSA.

  • An exercise program, stretching, massage, or assistive devices, such as walkers, canes, or wheelchairs, may be part of physical therapy to maintain mobility, strength, balance, and flexibility and prevent falls, contractures, or pressure sores.

  • MSA may cause changes in daily activities, such as dressing, eating, bathing, or writing. Occupational therapy may include adaptive equipment, strategies, or modifications, such as special utensils, clothing, or furniture.

  • In speech therapy, exercises, techniques, or devices, such as amplifiers, artificial saliva, or feeding tubes, may be used to improve speech, voice, and swallowing.

  • The purpose of respiratory therapy is to improve breathing and oxygenation and prevent respiratory infections, such as pneumonia. Respiratory therapy may include breathing exercises, inhalers, or mechanical ventilation.

  • Providing comfort, support, and symptom relief to people with advanced MSA and their families. Palliative care may include pain management, emotional counseling, or hospice care.

Prevention

Although MSA cannot be prevented, some lifestyle changes may help reduce the risk of complications and improve quality of life. These include:

  • Consuming a balanced and nutritious diet and drinking enough fluids to prevent dehydration, constipation, and malnutrition.

  • Alcohol, caffeine, tobacco, and other substances that can worsen symptoms or interact with medications should be avoided.

  • The blood pressure or sweating can be affected by extreme temperatures, hot baths, or saunas.

  • To prevent orthostatic hypotension, elevate the head of the bed, wear compression stockings, or take salt tablets.

  • To prevent infections, ulcers, and dental problems, practice good hygiene, skin care, and oral care.

  • If new or worsening symptoms occur, such as fever, chest pain, difficulty breathing, or confusion, seek medical attention immediately.

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