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Olivopontocerebellaratrophy (OPCA) is a neurological degenerative disorder characterized by atrophy of the midfoot and cerebellial hemispheres in the pontine cerebellum and is a more common type
of hereditary ataxia.
Etiology is unknown, but family history
may be present.
The first symptoms are most common in progressive ataxia and weakness of both lower extremities, and eye movement disorders are common
.
Cruciform mechanism: the pontine nucleus and its fibrosis through the cerebellar mid-foot to reach the cerebellum, the pontine neuron, the ponto-transverse fiber, the cerebellar mid-foot are seriously reduced due to degeneration, and the glial hyperplasia increases the amount of water, thus forming a cross-shaped high signal of MRI T2 like the upper pontons; However, the fibres and pyramidal bundles (cortical nucleus bundles, cortical spinal tracts), medial thalamus, and dorsal caps of the ponts that emit the upper foot (binding arm) of the cerebellar are not damaged by the dentate nucleus, and this differential involvement is precisely the important reason for the appearance of crucifixions on MRI
.
The appearance of "cross signs" from the image alone can only be considered MSA, but the case for OPCA, SND, or Shy-Drager syndrome needs to be closely combined with the clinic
.
Generally speaking:
When Parkinson's syndrome is predominant, MSA is striatal substantia nigra degeneration (SND);
When cerebellar symptoms are dominant, MSA is OPCA;
When autonomic disorders are dominant, such as sexual dysfunction and dizziness, MSA is Shy-Drager syndrome
.