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酷似2型夏科-马里-图斯病的迟发性弗里德赖希共济失调:异同之处何在?

Late-Onset Friedreich's Ataxia (LOFA) Mimicking Charcot-Marie-Tooth Disease Type 2: What Is Similar and What Is Different?

作者信息

Salomão Rubens Paulo A, Gama Maria Thereza Drumond, Rezende Filho Flávio Moura, Maggi Fernanda, Pedroso José Luiz, Barsottini Orlando G P

机构信息

Division of General Neurology and Ataxia Unit, Department of Neurology, Universidade Federal de São Paulo, São Paulo, Brazil.

出版信息

Cerebellum. 2017 Apr;16(2):599-601. doi: 10.1007/s12311-016-0822-9.

Abstract

Herein, we report a patient that presented with late-onset progressive steppage gait, neuropathy and pes cavus, suggesting Charcot-Marie-Tooth (CMT) disease. Subsequent genetic investigation confirmed Friedreich's ataxia (FRDA). We demonstrate that late-onset Friedreich's ataxia (LOFA) may be a CMT mimicker. This case reinforces that other genetic conditions may clinically resemble CMT. The clinical similarities between CMT and FRDA include a symmetrical neuropathy (axonal in FRDA), steppage gait, and eventually scoliosis. We suggest that late-onset forms of hereditary neuropathies should be carefully evaluated, since LOFA may be a CMT mimicker.

摘要

在此,我们报告一名患者,其表现为迟发性进行性跨阈步态、神经病变和高弓足,提示为夏科-马里-图斯(CMT)病。随后的基因检测确诊为弗里德赖希共济失调(FRDA)。我们证明迟发性弗里德赖希共济失调(LOFA)可能是一种CMT的模仿者。该病例强调其他遗传疾病在临床上可能与CMT相似。CMT和FRDA之间的临床相似之处包括对称性神经病变(FRDA为轴索性)、跨阈步态以及最终出现脊柱侧弯。我们建议应仔细评估迟发性遗传性神经病变,因为LOFA可能是CMT的模仿者。

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