Vestibular dysfunction in neurofibromatosis type 2-related schwannomatosis.
Vestibular dysfunction in neurofibromatosis type 2-related schwannomatosis.
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神经纤维瘤病的前庭功能障碍与2型与造型瘤病相关。
DOI：
10.1093/braincomms/fcad089
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发表时间：
2023
影响因子：
4.8
通讯作者：
Lewis, Richard F.
中科院分区：
文献类型：
--
作者：
Madhani, Amsal S.;King, Susan;Zhu, Jennifer;Karmali, Faisal;Welling, D. Bradley;Cai, Wenli;Jordan, Justin T.;Lewis, Richard F.
NF2相关神经鞘瘤病是一种以神经系统肿瘤为特征的遗传性疾病，最典型的是起源于前庭耳蜗神经的前庭神经鞘瘤。尽管前庭症状可能会导致失能，但从未仔细分析过 NF2 相关神经鞘瘤病的前庭功能。此外，化疗（例如贝伐珠单抗）可以减少 NF2 相关神经鞘瘤病的肿瘤体积并改善听力，但对其前庭效应尚不清楚。在本报告中，我们研究了 8 名未经治疗的 NF2 相关神经鞘瘤病患者的三种主要前庭介导的行为（眼球运动、运动知觉和平衡）、临床前庭功能障碍（头晕和共济失调）以及影像学和听力，并将他们的结果与正常受试者和散发性单侧前庭神经鞘瘤患者进行了比较。我们还研究了贝伐单抗如何影响两名 NF2 相关神经鞘瘤病患者。 NF2相关神经鞘瘤病中的前庭神经鞘瘤会降低前庭精度（变异性倒数，反映中心信噪比降低），但不会降低前庭精度（相对于理想幅度的幅度，反映中心信号幅度）并导致临床残疾。贝伐单抗改善了两名 NF2 相关神经鞘瘤病患者的前庭精确度和临床残疾，但不影响前庭精确度。这些结果表明，我们的 NF2 相关神经鞘瘤病人群中的前庭神经鞘瘤会降低中央前庭信噪比，而贝伐单抗则改善信噪比，这些变化可以通过传入神经噪声的增加（神经鞘瘤）和抑制（贝伐单抗）来机械地解释。马达尼等人。评估了 2 型神经纤维瘤病相关神经鞘瘤病患者的前庭功能障碍。前庭神经鞘瘤和贝伐珠单抗分别降低和提高了前庭精度，但不影响前庭准确性，这与生长在前庭神经上的神经鞘瘤产生传入神经噪声并被贝伐珠单抗抑制的新机制一致。
NF2–related schwannomatosis is a genetic disorder characterized by neurologic tumours, most typically vestibular schwannomas that originate on the vestibulo-cochlear nerve(s). Although vestibular symptoms can be disabling, vestibular function has never been carefully analysed in NF2–related schwannomatosis. Furthermore, chemotherapy (e.g. bevacizumab) can reduce tumour volume and improve hearing in NF2–related schwannomatosis, but nothing is known about its vestibular effects. In this report, we studied the three primary vestibular-mediated behaviours (eye movements, motion perception and balance), clinical vestibular disability (dizziness and ataxia), and imaging and hearing in eight untreated patients with NF2–related schwannomatosis and compared their results with normal subjects and patients with sporadic, unilateral vestibular schwannoma tumours. We also examined how bevacizumab affected two patients with NF2–related schwannomatosis. Vestibular schwannomas in NF2–related schwannomatosis degraded vestibular precision (inverse of variability, reflecting a reduced central signal-to-noise ratio) but not vestibular accuracy (amplitude relative to ideal amplitude, reflecting the central signal magnitude) and caused clinical disability. Bevacizumab improved vestibular precision and clinical disability in both patients with NF2–related schwannomatosis but did not affect vestibular accuracy. These results demonstrate that vestibular schwannoma tumours in our NF2–related schwannomatosis population degrade the central vestibular signal-to-noise ratio, while bevacizumab improves the signal-to-noise ratio, changes that can be explained mechanistically by the addition (schwannoma) and suppression (bevacizumab) of afferent neural noise. Madhani et al. assessed vestibular dysfunction in patients with neurofibromatosis type 2–related schwannomatosis. Vestibular schwannomas and bevacizumab, respectively, degraded and improved vestibular precision without affecting vestibular accuracy, consistent with a novel mechanism whereby afferent neural noise is generated by schwannoma(s) growing on the vestibular nerve and is suppressed by bevacizumab.
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DOI：
10.1093/noajnl/vdaa153
发表时间：
2021-01
期刊：
Neuro-oncology advances
影响因子：
--
作者：
Ren Y;Chari DA;Vasilijic S;Welling DB;Stankovic KM
通讯作者：
Stankovic KM
影响因子：
8.8
作者：
Plotkin, Scott R.;Messiaen, Ludwine;Evans, D. Gareth
通讯作者：
Evans, D. Gareth
影响因子：
2.5
作者：
FETTER, M;ZEE, DS
通讯作者：
ZEE, DS
影响因子：
1.4
作者：
Dimitri, PS;Wall, C;Oas, JG
通讯作者：
Oas, JG
影响因子：
3.4
作者：
Patel, Neil S.;Huang, Alice E.;Carlson, Matthew L.
通讯作者：
Carlson, Matthew L.