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首页> 外文期刊>Neuro-degenerative diseases >Transcranial Sonography Reveals Cerebellar, Nigral, and Forebrain Abnormalities in Friedreich's Ataxia
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Transcranial Sonography Reveals Cerebellar, Nigral, and Forebrain Abnormalities in Friedreich's Ataxia

机译：经颅超声检查显示弗里德里希共济失调的小脑，黑色和前脑异常。

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Background: Friedreich's ataxia (FA) is essentially characterized by degeneration of the dorsal root ganglia, the dorsal nuclei of Clarke, and the long spinal fiber tracts, yet there is accumulating evidence that neurodegeneration extends beyond these predilection sites. Transcranial sonography (TCS) has evolved as a valuable complementary neuroimaging tool in the assessment of neurodegenerative diseases due to its capacity to well depict structural changes and the accumulation of heavy metals. Its use for assessing cerebellar neurodegeneration, however, has not yet been investigated. Here we investigated whether TCS allows to assess particular features of cerebellar as well as midbrain and fore-brain abnormalities in FA. Methods: Comprehensive TCS imaging of 34 FA patients and 34 age-matched healthy controls. Results: Hyperechogenicity of the dentate nucleus was very frequent in FA patients (85%) and could even be observed in patients with short disease duration, suggesting that dentate alterations are a common and probably early feature of FA. Substantia nigra was significantly hypoechogenic, possibly indicating regional changes in subcellular brain iron regulation. FA patients showed significantly enlarged 4th, 3rd, and lateral ventricles, thus corroborating earlier MRI and postmortem findings of substantial cerebellar and forebrain atrophy in FA. Conclusions: TCS provides a quick-to-apply and inexpensive in vivo assessment of both cerebellar and noncerebellar abnormalities in FA, in particular highlighting dentate hyperechogenicity as a core feature. It might serve as a promising tool for imaging aspects of cerebellar neurodegeneration also in other neurodegenerative disorders.
机译：背景：弗里德里希共济失调（FA）的主要特征是背根神经节，克拉克背核和长脊椎纤维束变性，但越来越多的证据表明神经变性已超出这些好发部位。经颅超声检查（TCS）已经发展成为评估神经退行性疾病的一种有价值的补充神经影像工具，因为它能够很好地描绘出结构变化和重金属的积累。然而，其用于评估小脑神经变性的用途尚未进行研究。在这里，我们调查了TCS是否允许评估FA中小脑以及中脑和前脑异常的特定特征。方法：对34名FA患者和34名年龄匹配的健康对照者进行全面的TCS成像。结果：FA患者的齿状细胞核具有很高的回生性（85％），疾病持续时间短的患者甚至可以观察到，这表明齿状体改变是FA的常见特征，可能是其早期特征。黑质具有明显的低回声性，可能表明亚细胞脑铁调节的区域变化。 FA患者的第4、3和侧脑室显着增大，从而证实了早期的MRI和FA的尸检结果，即小脑和前脑实质性萎缩。结论：TCS为FA中的小脑和非小脑异常提供了一种快速应用且便宜的体内评估方法，尤其强调了齿状高回声性是其核心特征。它也可以作为在其他神经退行性疾病中对小脑神经变性方面进行成像的有前途的工具。

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