Shy-Drager Syndrome

Multiple system atrophy (MSA) is a rare neurodegenerative disorder characterized by autonomic dysfunction, tremors, slow movement, muscle rigidity, and postural instability (collectively known as parkinsonism) due to dysfunction of the basal ganglia, and ataxia. This is caused by progressive degeneration of neurons in several parts of the brain including the basal ganglia, inferior olivary nucleus, and cerebellum. Multiple system atrophy (MSA) is a rare neurodegenerative disorder characterized by autonomic dysfunction, tremors, slow movement, muscle rigidity, and postural instability (collectively known as parkinsonism) due to dysfunction of the basal ganglia, and ataxia. This is caused by progressive degeneration of neurons in several parts of the brain including the basal ganglia, inferior olivary nucleus, and cerebellum. Many people affected by MSA experience dysfunction of the autonomic nervous system, which commonly manifests as orthostatic hypotension, impotence, loss of sweating, dry mouth and urinary retention and incontinence. Palsy of the vocal cords is an important and sometimes initial clinical manifestation of the disorder. A modified form of the alpha-synuclein protein within affected neurons may cause MSA. About 55% of MSA cases occur in men, with those affected first showing symptoms at the age of 50-60 years. MSA often presents with some of the same symptoms as Parkinson's disease. However, those with MSA generally show little response to the dopamine medications used to treat Parkinson's disease, and only about 9% of MSA patients with tremor had a true parkinsonian pill-rolling tremor. MSA is distinct from multisystem proteinopathy, a more common muscle wasting syndrome. It should also not be confused with multiple organ dysfunction syndrome, sometimes referred to as multiple organ failure, or with multiple organ system failure, an often-fatal complication of septic shock or other very severe illnesses or injuries. MSA is characterized by the following, which can be present in any combination: A variant with combined features of MSA and Lewy body dementia may also exist. There have also been occasional instances of frontotemporal lobar degeneration associated with MSA. The most common first sign of MSA is the appearance of an 'akinetic-rigid syndrome' (i.e. slowness of initiation of movement resembling Parkinson's disease) found in 62% at first presentation. Other common signs at onset include problems with balance (cerebellar ataxia) found in 22% at first presentation, followed by genito-urinary symptoms (9%): both men and women often experience urgency, frequency, incomplete bladder emptying, or an inability to pass urine (retention). About 1 in 5 MSA patients experience a fall in their first year of disease. For men, the first sign can be erectile dysfunction. Women have also reported reduced genital sensitivity.