| Popis: |
Multiple system atrophy (MSA) is an α-synucleinopathy clinically characterized by autonomic disturbance, parkinsonism, and cerebellar ataxia occurring in any combination. Other clinical features include pyramidal signs, dystonia, and REM sleep behaviour disorder (RBD) and early bladder involvement. Mean age at onset is mid to late fifties, earlier than the other atypical parkinsonian disorders (progressive supranuclear palsy and corticobasal syndrome) and rarely over 70 years. MSA can be subdivided into a predominantly parkinsonian type (MSA-P) and a predominantly cerebellar type (MSA-C) depending on the principal clinical features. MSA-P is the more common subtype found in Europe and is associated with a more rapid progression to loss of function; MSA-C is generally less common, but is the more common variant in Japan. Almost all patients, often later in the disease, will have symptoms of autonomic disturbance such as urinary dysfunction and orthostatic hypotension. Disease duration is typically 7–8 years. |
