Wessel K, Schroth G, Diener H C, Müller-Forell W, Dichgans J
Department of Neurology, Medical University, Lübeck, Federal Republic of Germany.
Eur Arch Psychiatry Neurol Sci. 1989;238(4):225-30. doi: 10.1007/BF00381470.
The severity of Friedreich's ataxia was graded in ten patients by clinical examination and in five by use of posturography. These data were compared with neuroradiology findings. CT-confirmed infratentorial atrophy occured only in advanced cases of Friedreich's ataxia; the correlation with the clinical score was poor. On mid-sagittal MRI planes the diameters of fourth ventricle, brain stem at the level of the inferior olive and spinal cord at the levels of the foramen magnum and C3 were measured. Patients with Friedreich's ataxia had significant MRI-confirmed atrophy of the cranial spinal cord as compared with a normal, age-matched control group. This was also observed in patients with Friedreich's ataxia in the early stages. A reliable correlation between atrophy of the cranial spinal cord and the clinical score, however, could again not be found. MRI exploration of the cranial spinal cord may be recommended as an additional diagnostic marker in Friedreich's ataxia.
通过临床检查对10例弗里德赖希共济失调患者的严重程度进行分级，5例通过姿势描记法进行分级。将这些数据与神经放射学检查结果进行比较。CT证实的幕下萎缩仅出现在弗里德赖希共济失调的晚期病例中；与临床评分的相关性较差。在矢状面MRI图像上，测量第四脑室、下橄榄水平脑干以及枕骨大孔和C3水平脊髓的直径。与年龄匹配的正常对照组相比，弗里德赖希共济失调患者经MRI证实存在明显的颅脊髓萎缩。在弗里德赖希共济失调早期患者中也观察到了这一情况。然而，再次未发现颅脊髓萎缩与临床评分之间存在可靠的相关性。对于弗里德赖希共济失调，建议将颅脊髓的MRI检查作为一项额外的诊断指标。
