What causes Shy-Drager syndrome?
Shy-Drager syndrome is a rare degenerative condition. It is a condition that appears similar to Parkinson's disease in that patients may be slow moving, tremulous and have a shuffling gait but with more widespread neurologic damage and
damage to the autonomic nervous system (the portion of the nervous system that controls involuntary functions).
There is neuronal loss and gliosis in the inferior olives, pons, cerebellum, substantia nigra, locus ceruleus, striatum and the intermediolateral column of the spinal cord. Typical but not specific to MSA are argyrophilic inclusions containing accumulations of tubules. Alpha-synuclein has recently been shown to be accumulated in glial cytoplasmic incusions. Alpha synuclein is also a component of Lewy bodies in sporadic Parkinsonism, dementia with Lewy bodies, and a rare variant of Alzheimer's disease with abundant Lewy bodies. This is in distinction to the "tau" rich inclusion bodies found in most types of Alzheimer's disease and progressive supranuclear palsy. Depletion of catecholamine neurons in the rostral ventrolateral medulla is a consistant finding in patients with MSA and autonomic failure.
The cause is unknown. There is progressive damage (degeneration) of the nervous system, with damage to all parts of the nervous system. The disorder develops gradually. It is most often diagnosed in men over 60 years old. |
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