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However, because of the common parkinsonian features, the disorders have been collectively named Parkinson-plus syndromes.
Though the main features of OPCA are cerebellar manifestations, mild parkinsonism and pyramidal signs are also typically present.
Glial cytoplasmic inclusions (GCIs) and a neuronal multisystem degeneration are the pathologic hallmarks of this clinically variable disorder (see the image below).
Dejerine and Thomas first used the term olivopontocerebellar atrophy (OPCA) in 1900 when they described 2 patients with a degenerative disorder leading to progressive cerebellar dysfunction and parkinsonism.
Several primary neurodegenerative disorders share parkinsonian features, such as bradykinesia, rigidity, tremor, and gait disturbances.
These disorders have complex clinical presentations that reflect degeneration in various neuronal systems.