What is Multiple System Atrophy? Multiple System Atrophy (MSA) is a rare, progressive neurological disorder, caused by cell loss in certain areas of the brain leading to a variety of symptoms affecting especially the functions of the autonomic nervous system and the motor system. Depending on the brain areas most predominantly involved, a subset of disorders have been described including sporadic olivopontocerebellar atrophy (sOPCA), which is characterized primarily by disturbances of balance, coordination and speech, striatonigral degeneration (SND), where the patient initially suffers from parkinsonian features like bradykinesia (=slowed movements), rigidity (=stiffness) and tremor, and Shy-Drager syndrome, with marked changes in blood pressure regulation, urinary difficulties and, (in male patients) disturbance of sexual function predominating the initial presentation. As this variety of names often caused confusion not only among patients, but also physicians, MSA is nowadays referred to as MSA-P type if parkinsonian features predominate or MSA-C type if cerebellar symptoms predominate, replacing the terms SND and sOPCA .The term Shy-Drager syndrome should not be used anymore as almost every patient is affected by autonomic/urinary dysfunction. MSA affects both man and woman, with an average symptom onset in the 6th decade and a prevalence rate of 3 to 4.4/100.000 / about 4/100.000. The majority of patients diagnosed with MSA do not have a good prognosis with an average survival rate of 9 years following disease onset. The cause of the cell loss leading to MSA still remains unknown, but there is no evidence for a hereditary component and it is not contagious. Diagnosis Due to the variety of different ways MSA can manifest, it is often difficult to differentiate it from other neurodegenerative disorders like Parkinson's disease, progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD). Diagnosis is made on careful history, physical examination and some tests including several types of brain imaging and autonomic function tests. However, a definite diagnosis can still only be accomplished by post-mortem examination of the brain. Symptoms The most common symptoms include the following: · Stiffness/Rigidity · Bradykinesia · Poor balance, clumsiness · Urinary difficulties · In male patients, impotence · Orthostatic hypotension = a significant fall in blood pressure upon standing, causing dizziness or even fainting, tiredness, blurred vision and head or neck pain · Speech difficulties · Sleep disturbances · Swallowing difficulties · Constipation · (Mild intellectual impairment) · Loss of sweating NOTE: Being diagnosed with MSA does not mean that you develop all the symptoms Treatment At the moment there is no cure for MSA. Symptomatic treatment is aimed at reducing the disabling effects of each symptom associated with MSA. Cerebellar ataxia (incoordination and tremulous goal-directed movements of the limbs, gait unsteadiness, slurred speech) is difficult to control by drugs, however, all patients with parkinsonism should receive dopaminergic replacement therapy including levodopa and dopamine agonists since there is a 30% chance of benefit. A range of pharmacological and physical measures can be tried to alleviate the symptoms of orthostatic hypotension, in most instances a salt-rich diet, head-up tilt at night, elastic stockings, low dose fludrocortisone and midodrine will improve patients. Urogenital symptoms can also often be treated effectively; patients should be referred to expert uroneurologists and incontinence advisors. Sofar, the relentless disease progression cannot be halted or stopped by any medication, however, with increasing understanding of cell death mechanisms in MSA novel strategies will become available. A multicentre trial of riluzole, a glutamate release blocker, has just been launched to address the neuroprotective potential of antiglutamatergic therapy in MSA.