Multiple system atrophy is a rare progressive neurological disorder impairing the body’s autonomic (involuntary) functions including blood pressure, heart rate, digestion and bladder function. It is an adult-onset, sporadic and rapidly progressive fatal disease. The Autonomic American Society and American Academy of Neurology have issued a statement in 2007 in which MSA is categorized as MSA with predominant parkinsonism (MSA-P) and MSA with predominant cerebellar features (MSA-C).

Causes

No exact cause of MSA has been identified

Potential causes (but no conclusive evidence has been found):

Genetics

Environmental toxins

The damaged brain tissue of patients with MSA have been shown to contain an abnormally large amount of a protein called alpha-synuclein, research indicating that the onset of MSA might be associated with the overproduction of said protein

Alpha-synucleins are a family of highly charged proteins with significant functions related to nerve cell activity

One of these proteins, alpha-syn is the major building block of pathological sites of viral multiplication that characterize many neurodegenerative disorders

The process of viral multiplication refers to the fact that in order to multiply within a host, a virus must firstly infect a cell. During this phase of infection the virus introduces into the healthy cell its genetic material (RNA or DNA), a process assisted in many instances with help from essential proteins

Symptoms and signs

MSA is associated with the deterioration and shrinkage of the cerebellum, basal ganglia and brain stem, which regulate internal body functions, motor control and digestion

 

MSA-P symptoms:

Rigidity of muscles and difficulty in bending arms and legs

Slow movement (bradykinesia)

Tremors (rarer compared to classic Parkinson’s disease)

Impaired posture and balance

 

MSA-C symptoms:

Lack of muscle coordination (ataxia)

Impairment of movement and coordination, unsteady gait and loss of balance

Slurred, slow or low-volume speech

Visual disturbances such as blurred or double vision, difficulty focusing the eyes

Difficulty swallowing or chewing

 

General signs and symptoms:

Postural (orthostatic) hypotension:

A form of low-blood pressure causing dizziness, or even fainting when standing up from sitting or lying down

Dangerously high blood pressure levels when lying down

Urinary and bowel dysfunctions:

Constipation

Urinary incontinence

Sweating abnormalities:

Reduction in the production of perspiration, tears and saliva

Difficulty in regulating body temperature, causing cold hands and feet or heat intolerance

Sleeping difficulties:

Agitated sleep

Abnormal breathing during sleep

Sexual dysfunctions:

Impotence

Loss of libido

Irregular heartbeat

Difficulty controlling emotions

Advice

There is no cure for MSA

Treatment aims at ameliorating symptomatology and maintaining body functions and capabilities:

Medications to raise blood-pressure

Medications to reduce parkinsonism

Pacemaker implant to keep the heart beating at a regular rate

Impotence medications

Gastrostomy tube to alleviate feeding and swallowing

Catheter insertion to alleviate bladder function

Physical therapy to retain muscle control and function

MISCONCEPTIONS
  • MSA symptoms are often confused with Parkinson’s disease signs

Statistics

The disease progresses rapidly with median survival rates of 6.2-9.5 years from the onset of first symptoms

The prevalence of MSA is reported to be between 3.4-4.9 cases per 100,000 population, in the U.S.

MSA meets orphan disease status, meaning that MSA is a condition affecting fewer than 200,000 people nationwide

In the European Union (EU), the prevalence rates show 4-5 cases per 100,000 persons

In the United Kingdom, the crude prevalence of MSA, including all probable and possible cases, is 3.3 per 100,000 population.

In Iceland, the incidence is 0.6 per 100,000 and prevalence is 3.1 per 100,000

In Japan, the prevalence is 13.1 per 100,000 individuals

In Western countries, MSA-P predominates, occurring in 66-82% of patients

In Eastern countries (e.g., Japan), MSA-C is common, occurring in 67% of patients

The disease more often affects men than women

The mean age at onset in MSA is 52.5-55 years

The disease progresses over intervals of 1-18 years

Did you know?

Multiple system atrophy was formerly known as Shy-Drager syndrome