Multisystematrophie

Abstract

Multiple system atrophy (MSA) is an adult-onset sporadic degenerative disease of the CNS that can cause parkinsonism, autonomic failure, cerebellar and pyramidal signs in any combination. Often, the diagnosis of clinically probable MSA can be made without the need for additional investigations, MSA of SND or OPCA type being diagnosed according to whether parkinsonism or cerebellar signs predominate. However, in other patients a pathological anal or urethral sphincter EMG may enable the diagnosis to be established. The cerebral pathology of this condition comprises cell loss and gliosis in the nigrostriatal and/or olivopontocerebellar systems. In addition there is loss of sympathetic neurons of the intermediolateral cell columns, and of anterior horn cells from Onuf's nucleus, in the spinal cord. Oligodendroglial intracytoplasmic inclusions are found in many brain areas in all cases of MSA.