Lamarche J B, Lemieux B, Lieu H B
Can J Neurol Sci. 1984 Nov;11(4 Suppl):592-600. doi: 10.1017/s0317167100035113.
We present the pathological data from the autopsies performed on 6 Friedreich's disease patients since the start of the Quebec Cooperative Study. All patients met the strict diagnostic criteria of the QCSFA. The anatomical lesions found in the peripheral and central nervous system were similar in all 6 cases and do not differ from those described in the literature. The clinical findings correlate closely with the histological lesions found in the peripheral nervous system and spinal cord. The evidence of segmental demyelination and remyelination in the spinal ganglia and posterior roots further supports the dying-back axonopathy hypothesis.
自魁北克合作研究开始以来，我们展示了对6例弗里德赖希共济失调病患者进行尸检得到的病理数据。所有患者均符合魁北克合作研究弗里德赖希共济失调病标准的严格诊断标准。在所有6例患者中，外周和中枢神经系统发现的解剖学病变相似，与文献中描述的病变并无差异。临床发现与外周神经系统和脊髓中的组织学病变密切相关。脊髓神经节和后根中节段性脱髓鞘和再髓鞘化的证据进一步支持了轴突逆行性变性假说。
