Management of multiple system atrophy: state of the art

Abstract

Multiple system atrophy (MSA) is a sporadic neurodegenerative disease of undetermined aetiology presenting with parkinsonian, autonomic, cerebellar, and pyramidal signs. Despite the lack of any effective therapy to reverse MSA, some of the symptoms may be improved with adequate symptomatic therapies. Medical treatment is largely aimed at mitigating the parkinsonian and autonomic features. The therapeutic results of levodopa therapy in cases of MSA are difficult to interpret because of their variability. Nevertheless, the simple statement that patients with MSA do not respond to levodopa is false. Clinical and pathologically proven series document levodopa efficacy in about 40-60% of patients with MSA and predominant parkinsonian features. Other antiparkinsonian compounds (dopamine agonists, amantadine) may also be employed, but they are not more effective than levodopa. Orthostatic hypotension (OH) can be suspected from the patient s history and subsequently documented in the clinic by measuring lying and standing blood pressure. The diagnosis ideally should be confirmed with additional laboratory tests to determine the cause and evaluate the functional deficit, so as to aid treatment. A number of pharmacological agents with different mechanisms of action have been used in MSA to reduce OH when this is symptomatic. OH can also be alleviated by avoiding aggravating factors, such as the effects of food, micturition, exposure to a warm environment, and physiological diurnal changes, and by using other non-pharmacological strategies. The treatment of the very common genitourinary symptoms (incontinence, retention, impotence) should also be considered in order to improve the quality of life of these patients.