Multiple system atrophy (MSA) is one of the main neurodegenerative diseases occurring in adults. It often presents with progressive Parkinson’s disease (p-MSA) in which akinesia and rigidity predominate and which is rarely associated with resting tremor. Other common symptoms of multiple system atrophy, according to neurology specialists, include urination problems (urinary incontinence or urine retention), erectile dysfunction and orthostatic hypotension (drops in blood pressure when standing up).
In multiple system atrophy, speech is also impaired early. However, hallucinations or cognitive impairment rarely occur. A characteristic symptom of multiple system atrophy is stridor, which is a kind of high-pitched, intense nocturnal snoring that occurs when breathing in. Another common nocturnal symptom is nightmares associated with nocturnal agitation, known as REM behavior disorder.
Causes of multiple system atrophy
Multiple system atrophy is associated with synuclein deposits in the brain, as is Parkinson’s disease, and is therefore also considered a synucleinopathy. The cause of this neurodegenerative disease is unknown. No genetic alterations causing the disease have been detected.
Multiple system atrophy: relationship to Parkinson’s disease
Multiple system atrophy is often confused with Parkinson’s disease. This confusion occurs especially in the early stages, as slowness of movement and flexed body posture are characteristic of both diseases. In addition, urination problems and erectile dysfunction may occur in both diseases. In multiple system atrophy, typical resting tremor is very infrequent and speech and gait problems are early and frequent, the latter being the cause of frequent falls. There may be marked neck flexion. Onset of the disease before the age of 30 years or after 70 years is very rare.
Multiple system atrophy may sometimes present as a progressive cerebellar syndrome (cerebellar variant of multiple system atrophy (MSA-c). In these cases, the lesions occur in the cerebellum and its connections, rather than in the substantia nigra as is the case in variant p-multiple system atrophy. The patient suffers from progressive gait instability, known as ataxia, which is also associated with urination problems, blood pressure, and erectile and sleep dysfunction.
Diagnosis of multiple system atrophy
Magnetic resonance imaging is a useful tool in the diagnosis of multiple system atrophy. MRI can rule out other diseases that could be confused with multiple system atrophy, such as the presence of multiple cerebral infarcts. Other diagnostic tests are generally of little use.
Treatment of multiple system atrophy
At present, there is no drug that slows down the progressive course of this disease. The treatment of multiple system atrophy-p focuses on the administration of drugs to treat Parkinson’s disease. In general, the response is only positive in 20% of patients.
There are no drugs that alleviate the ataxia of c-multi-system atrophy. Orthostatic hypotension may cause brief and recurrent unconsciousness requiring non-pharmacological treatments such as the use of elastic stockings. Treatment with fluorhydrocortisone or other drugs is also frequently resorted to. On the other hand, stridor often requires nocturnal CPAP. Deep brain stimulation, successful in advanced Parkinson’s disease, is not indicated in patients with multiple system atrophy.