Multiple System Atrophy (MSA)

Multiple system atrophy (MSA) is a rare, progressive neurological condition that affects the autonomic nervous system, which controls automatic functions like blood pressure, heartbeat, and the bladder. Because the autonomic nervous system is involved in many essential bodily functions, patients with MSA experience a wide variety of symptoms.

Physicians at Massachusetts General Hospital specialize in diagnosing and treating multiple system atrophy. To learn more or explore treatment options, call 617-726-5532 or submit our online form to request an appointment.

What Is MSA?

Multiple system atrophy is a complex condition that affects the brain, leading to symptoms that impact movement, balance, and automatic bodily functions.

Multiple system atrophy primarily affects three key areas of the brain:

• Basal ganglia: These structures help connect different brain regions and play an important role in movement, learning, and memory.
• Brainstem: This area controls automatic functions like breathing and blood pressure.
• Cerebellum: Located at the back of the brain, the cerebellum helps with balance, posture, and smooth, coordinated movements.

Types of MSA

While MSA is considered a single disorder, it is often divided into two subtypes based on the early symptoms and areas of the brain most affected. Both types affect the brainstem, and there is crossover between them.

• MSA-P (Parkinsonian): This form of MSA can look similar to Parkinson's disease at first, with symptoms such as stiffness, slowness of movement, and tremors.
• MSA-C (Cerebellar): This form of multiple system atrophy affects the part of the brain responsible for balance, coordination, and muscle control. People with MSA-C often experience issues with walking, speaking, and fine motor movements.

Multiple System Atrophy Symptoms

The two types of MSA share some symptoms while others are unique to each type.

Shared MSA Symptoms

Both types of MSA have symptoms related to the automatic functions regulated by the brainstem. These symptoms of MSA include:

• Orthostatic hypotension: A drop in blood pressure when standing, leading to dizziness, lightheadedness, fainting, trouble thinking, fatigue, or falls.
• Bladder control issues: Difficulty with urination.
• Digestive problems: Common issues such as constipation or slow digestion.
• Sexual dysfunction: Both men and women may experience challenges such as erectile dysfunction or decreased lubrication. Not all issues, like low sex drive, are physical.
• Sleep problems: Disruptions like REM sleep behavior disorder (acting out dreams) and sleep apnea, which can affect sleep quality.
• Hypohidrosis (insufficient sweating): Reduced sweating that can make it difficult to tolerate heat and regulate body temperature, potentially leading to cold hands or feet.
• Cognitive difficulties: Some people with MSA experience trouble focusing or concentrating, though typically thinking is relatively preserved.
• Pain: Many people with MSA experience muscle pain, joint stiffness, or nerve-related pain.
• Breathing abnormalities: Some individuals develop breathing issues, including stridor (a high-pitched sound when breathing), sleep apnea, or irregular breathing patterns.

Symptoms of MSA-C

In addition to the shared symptoms of MSA, patients with cerebellar MSA may experience:

• Movement problems: Poor coordination and balance can make walking difficult or cause falls.
• Speech problems: You may notice slurred speech, slow speech patterns, or speaking at a very low volume.
• Vision problems: Blurred vision, double vision, or trouble focusing may occur.

Symptoms of MSA-P

MSA-P symptoms can be similar to Parkinson's disease. Common symptoms include:

• Low vocal volume
• Muscle stiffness that makes it difficult to bend your arms and legs
• Posture and balance issues
• Slow movement
• Abnormal movements

MSA Diagnosis and Staging

Diagnosing multiple system atrophy can be challenging since MSA is relatively rare, and symptoms often resemble more common neurological disorders. Identifying MSA may take time, as your doctor works to rule out other conditions.

To help confirm a diagnosis, your doctor will perform a physical examination and talk with you about your symptoms, how your symptoms have progressed, and the details of your medical history. If your doctor suspects MSA, your doctor may order tests to check for signs typical of the condition, and to rule-out other similar conditions:

• Automatic functions tests: These tests evaluate automatic functions controlled by the brainstem, such as blood pressure responses to breathing or changes in position (tilt).
• Blood and urine tests: These tests can look for rare genetic or immune conditions that can mimic MSA or identify biomarkers that might suggest MSA.
• MRI, DaT, or PET: Imaging tests can show irregularities in brain structure or function that might suggest MSA.
• Skin biopsy: A small sample of skin cells can be tested for high levels of alpha-synuclein, a protein that accumulates in MSA.

MSA Disease Progression Stages

Unlike some other diseases (like cancer), multiple system atrophy doesn't have clearly defined stages. However, MSA tends to progress steadily after diagnosis.

• One of the earliest symptoms for many people is sexual dysfunction, such as erectile dysfunction in men or decreased sensitivity in women. However, most cases of sexual dysfunction are not caused by MSA. Other early symptoms can include orthostatic hypotension, urinary retention, and REM sleep behavior disorder.
• End-stage complications often include pneumonia and issues related to prolonged bedrest or breathing problems, such as aspiration pneumonia or pulmonary embolus

Multiple System Atrophy Treatment

Treatment for MSA focuses on improving your quality of life and managing symptoms effectively. With the right care, therapies, and support, you can continue to live meaningfully and maintain independence for as long as possible.

Treatments for multiple system atrophy include:

• Increasing water and salt intake: Helps to maintain blood flow to the brain.
• Botox injections: Can help reduce muscle spasms and tightness.
• Compression garments: Aid in stabilizing blood pressure by improving circulation.
• Medications: Drugs such as midodrine, fludrocortisone, and droxidopa may be prescribed to help increase blood pressure and blood flow to the brain.
• Levodopa: A medication commonly used for Parkinson’s disease that can sometimes help with motor symptoms like stiffness and slow movement.
• Medications for individual symptoms: Your doctor may prescribe medications to manage sleep disorders, bladder incontinence, or other specific symptoms.
• Physical therapy: Helps maintain mobility, balance, and strength, reducing the risk of falls and improving daily function.
• Positioning during fainting episodes: Lying down immediately when feeling faint allows gravity to restore blood flow to the brain.

For more information or to explore treatment options, contact our Parkinson's Disease and Movement Disorders Center, which is recognized as a center of excellence by the Parkinson’s Foundation.

In addition to symptom management, patients may consider participating in clinical trials to explore potential new treatments.

FAQs About Multiple System Atrophy