Multiple System Atrophy - Causes, Symptoms, Diagnosis, and Treatment

Introduction

Multiple system atrophy (MSA) is a rare neurological disorder that affects the autonomic nervous system, causing progressive damage to nerve cells in the brain. It is a debilitating disease that can significantly impact an individual's quality of life.

MSA is a relatively newly classified disease previously known as Shy-Drager Syndrome. The name was changed in 1996 to reflect the various symptoms and systems affected by the disease. MSA is categorized into two types, MSA-P and MSA-C, which affect different brain parts.

MSA-P primarily affects movement, causing symptoms similar to Parkinson's disease, such as tremors, rigidity, and problems with balance and coordination. MSA-C, on the other hand, primarily affects coordination and balance, causing difficulty walking and falling.

What Are the Causes of MSA?

The cause of multiple system atrophy (MSA) is currently unknown, although some researchers are investigating potential factors such as inherited genes or environmental toxins. However, there is little substantial evidence to support these theories.

MSA is characterized by the deterioration and shrinking (atrophy) of specific brain areas, including the cerebellum, basal ganglia, and brainstem. These areas are responsible for regulating internal body functions and controlling movement.

Upon examination of the damaged brain tissue of individuals with MSA, nerve cells (neurons) it was observed to contain an unusual amount of alpha-synuclein protein. There is some indication that an excessive buildup of this protein may be involved in the development of MSA.

What Are the Signs and Symptoms of MSA?

Multiple system atrophy (MSA) is a condition that impacts various body functions and typically arises in adulthood, commonly in one's fifties or sixties. There are two forms of MSA: parkinsonian and cerebellar, distinguished by the particular symptoms that manifest upon diagnosis.

• Parkinsonian Type: The most prevalent type of MSA is similar to Parkinson's disease, exhibiting comparable symptoms such as:

1. Stiff muscles.

2. Difficulty bending arms and legs.

3. Slow movement (bradykinesia).

4. Tremors that may happen at rest or while moving arms or legs.

5. A soft voice.

6. Issues with posture and balance.

• Cerebral Type: The primary symptoms of this type of MSA are difficulties with muscle coordination, known as ataxia. Other indications can consist of:

1. Impaired movement and coordination, resulting in an unstable gait and balance loss.

2. Slurred, slow, or quiet speech, referred to as dysarthria.

3. Visual impairments, such as blurry or double vision and difficulty with eye focus.

4. Difficulty in swallowing or chewing, known as dysphagia.

5. Alterations in speech, such as slurring of words.

Individuals with MSA may additionally experience the following:

• Shoulder and neck discomfort.

• Constipation.

• Cold extremities.

• Difficulties regulating sweating.

• Weakness in the body and limbs, which may be more noticeable in one arm or leg.

• Uncontrollable fits of laughter or crying.

• Sleep disturbances, such as insomnia, snoring, restless legs, or nightmares.

• Loud breathing and unintended sighing.

• A low and feeble voice.

How to Diagnose MSA?

The diagnosis of MSA can be challenging, particularly in the early stages, because many of the symptoms are similar to those of Parkinson's disease. A doctor may perform a brief neurological examination and review the patient's medical history to diagnose. The doctor may also order a variety of tests, including:

• Autonomic testing to evaluate blood pressure and heart rate control.

• Bladder function assessment.

• Neuroimaging, such as magnetic resonance imaging (MRI), which can identify changes that may suggest MSA or rule out other causes of the symptoms.

• Positron emission tomography (PET) scans, which show how organs and tissues are functioning and can detect reduced metabolic function in certain parts of the brain.

• DaTscanTM, a radiopharmaceutical that evaluates the dopamine transporter in the striatum, can help determine if a dopamine system disorder causes the condition; however, it cannot differentiate between MSA and Parkinson's disease. In general, people with MSA do not experience sustained symptom improvement with Levodopa, a drug used to treat Parkinson's disease, which is often indicative of MSA.

What Treatment Options Are Available for MSA?

At present, there are no cures or treatments that can halt the progressive neurodegeneration associated with MSA. However, interventions are available to help alleviate the condition's symptoms. Levodopa may temporarily relieve motor symptoms, but its efficacy may decrease as the disease progresses.

Orthostatic hypotension, which can lead to fainting and dizziness, can be treated with interventions such as wearing compression stockings, adding extra salt and water to the diet, and avoiding heavy meals. Fludrocortisone and Midodrine are medications that can be prescribed for this condition. The FDA has approved the medication Droxidopa for treating orthostatic hypotension seen in MSA. Dihydroxyphenylserine can also help replace neurotransmitters that are decreased in the autonomic nervous system in MSA.

Treatment of the bladder control problem depends on the underlying cause. Anticholinergic drugs, such as Oxybutynin or Tolteridine, can help reduce the sudden urge to urinate. Botulinum toxin injections may be used to control fixed abnormal muscle postures (dystonia). Medications like Clonazepam, Melatonin, or specific antidepressants can be used to manage sleep disorders like REM sleep behavior disorder. Medications such as Clonazepam, Melatonin, or certain antidepressants.

Some people with MSA may experience significant swallowing difficulties and require a feeding tube or nutritional support. Speech therapy can help with identifying strategies to address swallowing difficulties. Physical therapy can help maintain mobility and reduce contractures. Physical therapy can be beneficial in preserving mobility, diminishing muscle spasms and abnormal postures, and preventing contractures. As the condition progresses, individuals may require assistive devices like walkers and wheelchairs. In such cases, occupational therapists can aid in ensuring home safety and imparting alternative methods to perform daily activities like dressing and eating.

What Are the Complications of MSA?

MSA is a progressive disorder, meaning symptoms worsen over time and there is no remission. This progression can lead to difficulties in daily activities. Potential complications include breathing problems during sleep, injuries from falls due to poor balance or fainting, immobility, difficulty caring for oneself, vocal cord paralysis, and difficulty swallowing. The life expectancy for individuals with MSA is typically seven to ten years after the onset of symptoms, but this varies. Respiratory problems, infections, and lung blood clots are common causes of death in individuals with MSA.

Conclusion

In conclusion, multiple system atrophy (MSA) is a rare neurodegenerative disorder affecting various body parts, causing various symptoms that can make daily activities challenging. While there is no cure for MSA, treatments are available to help manage the symptoms and improve the quality of life for individuals with the condition. However, the progression of MSA varies widely, and the disorder can lead to various complications that can affect life expectancy.