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目的:报道3例散发性神经棘红细胞病患者的临床表现。方法:对经电镜证实的3例外周血红细胞中棘红细胞>5%患者的临床表现和分型,结合复习相关文献予以分析。结果:神经棘红细胞病3例,其中男2例、女1例;年龄35~79岁,平均年龄(56.6±22.0)岁;平均病程(2.6±1.0)年(7个月至5年)。均为隐匿起病,缓慢进展,表现为明显的小脑共济失调体征和步态。MRI示小脑萎缩。1例伴智能减退和异动症,1例高密度脂蛋白高于正常。扫描电镜发现3例患者周围血红细胞中,棘红细胞均>5%。结论:散发性小脑性共济失调的成人患者应考虑神经棘红细胞病可能。
OBJECTIVE: To report the clinical manifestations of 3 patients with sporadic neuro-acanthocytosis. Methods: The clinical manifestations and classification of 3 cases of erythrocyte erythrocytes in 3 cases of peripheral blood erythrocytes confirmed by electron microscopy were analyzed, and the related literatures were reviewed. Results: There were 3 cases of neurobasal cell disease, including 2 males and 1 females. The average age was 56.6 ± 22.0 years. The average duration was 2.6 ± 1.0 years (ranged from 7 months to 5 years). Are hidden onset, slow progress, manifested as obvious signs and gait of cerebellar ataxia. MRI showed cerebellar atrophy. 1 case with hypothyroidism and dyskinesia, 1 case of high-density lipoprotein higher than normal. Scanning electron microscopy found that 3 cases of peripheral red blood cells, erythrocytes were> 5%. CONCLUSIONS: Adult patients with sporadic cerebellar ataxia should consider the possibility of neuropathic spirocytosis.