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[Obstract] Objective: To show the clinical manifestations、 the results of auxiliary examinations and the factures of red-cell morphology in Chorea-acanthocytosis (ChAc), and in order to make a definitive diagnosis of ChAc.Method: 1、 Retrospective analysis of the diagnostic process of one patient with ChAc; 2、 Observing the red-cell morphology features and the proportion of acanthocytes in the blood with the scanning electron microscope and optical microscope in different treatment conditions.Results: the main symptom of ChAc were involuntary choreiform movement, increasing amount and proportion of acanthocytes in the peripheral blood was noted by the scanning electron microscope, which is completely different with the results in optical microscope.Conclusion: the accurate diagnosis depends on the clinical manifestations and the proportion of acanthocytes in the scanning electron microscope, the results of count in blood smear are not reliable.