Anna Camós-Carreras, Marc Figueras-Roca, Marina Dotti-Boada, Rafel Alcubierre, Ricardo Pedro Casaroli-Marano, Esteban Muñoz, Bernardo Sánchez-Dalmau
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Seventeen SCA3 patients with an average follow-up of 44.9 months were recruited. Clinical features with significant baseline correlations with SD-OCT measurements included disease duration (CMT r = - 0.590; GCC r = - 0.585), SARA score (CMT r = - 0.560; RNFL r = - 0.390), and number of CAG repeats (MV r = - 0.552; RNFL r = - 0.503; GCC r = - 0.493). The annual rate of change of the SARA score during follow-up was associated with that of both the MV (r = - 0.494; p = 0.005) and GCC thickness (r = - 0.454; p = 0.012). High disability (stages 2 and 3) was independently inversely associated with the annual change in MV (ß coefficient - 17.09; p = 0.025). This study provides evidence of an association between clinical features and objective anatomical measurements obtained by SD-OCT in SCA3 patients. MV and GCC thickness could serve as potential biomarkers of disease severity, as their rates of decrease seem to be related to a worsening in the SARA score. These findings highlight the potential of SD-OCT as a noninvasive tool for assessing disease severity and progression in SCA3 patients.</p>","PeriodicalId":50706,"journal":{"name":"Cerebellum","volume":null,"pages":null},"PeriodicalIF":2.7000,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11269372/pdf/","citationCount":"0","resultStr":"{\"title\":\"Progression of Retinal Ganglion Cell and Nerve Fiber Layer Loss in Spinocerebellar Ataxia 3 Patients.\",\"authors\":\"Anna Camós-Carreras, Marc Figueras-Roca, Marina Dotti-Boada, Rafel Alcubierre, Ricardo Pedro Casaroli-Marano, Esteban Muñoz, Bernardo Sánchez-Dalmau\",\"doi\":\"10.1007/s12311-023-01634-1\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Spectral domain optical coherence tomography (SD-OCT) allows noninvasive measurements of retinal neuron layers. 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引用次数: 0
摘要
光谱域光学相干断层扫描(SD-OCT)允许视网膜神经元层的非侵入性测量。在这里,我们评估脊髓小脑性共济失调3型(SCA3)患者的临床特征与解剖SD-OCT测量之间的关系以及它们如何随时间变化。对SCA3患者进行回顾性研究。临床变量如病程、CAG重复次数、共济失调评定量表(SARA)评分与SD-OCT测量结果相关,包括视网膜神经纤维层(RNFL)厚度、神经节细胞复合体(GCC)厚度、黄斑体积(MV)和黄斑中央厚度(CMT)。17例SCA3患者,平均随访时间为44.9个月。与SD-OCT测量具有显著基线相关性的临床特征包括病程(CMT r = - 0.590;GCC r = - 0.585), SARA评分(CMT r = - 0.560;RNFL r = - 0.390), CAG重复数(MV r = - 0.552;RNFL r = - 0.503;GCC r = - 0.493)。随访期间SARA评分的年变化率与MV的年变化率相关(r = - 0.494;p = 0.005)和GCC厚度(r = - 0.454;p = 0.012)。高度残疾(2期和3期)与MV年变化呈独立负相关(ß系数- 17.09;p = 0.025)。本研究为SCA3患者的临床特征与SD-OCT获得的客观解剖测量之间的关联提供了证据。MV和GCC厚度可以作为疾病严重程度的潜在生物标志物,因为它们的下降率似乎与SARA评分的恶化有关。这些发现强调了SD-OCT作为评估SCA3患者疾病严重程度和进展的无创工具的潜力。
Progression of Retinal Ganglion Cell and Nerve Fiber Layer Loss in Spinocerebellar Ataxia 3 Patients.
Spectral domain optical coherence tomography (SD-OCT) allows noninvasive measurements of retinal neuron layers. Here, we evaluate the relationship between clinical features and anatomical SD-OCT measurements in patients with spinocerebellar ataxia type 3 (SCA3) and how they change with time. A retrospective review was conducted on SCA3 patients. Clinical variables such as disease duration, number of CAG repeats, and the Scale for the Assessment and Rating of Ataxia (SARA) score were correlated with SD-OCT measurements, including retinal nerve fiber layer (RNFL) thickness, ganglion cell complex (GCC) thickness, macular volume (MV), and central macular thickness (CMT). Seventeen SCA3 patients with an average follow-up of 44.9 months were recruited. Clinical features with significant baseline correlations with SD-OCT measurements included disease duration (CMT r = - 0.590; GCC r = - 0.585), SARA score (CMT r = - 0.560; RNFL r = - 0.390), and number of CAG repeats (MV r = - 0.552; RNFL r = - 0.503; GCC r = - 0.493). The annual rate of change of the SARA score during follow-up was associated with that of both the MV (r = - 0.494; p = 0.005) and GCC thickness (r = - 0.454; p = 0.012). High disability (stages 2 and 3) was independently inversely associated with the annual change in MV (ß coefficient - 17.09; p = 0.025). This study provides evidence of an association between clinical features and objective anatomical measurements obtained by SD-OCT in SCA3 patients. MV and GCC thickness could serve as potential biomarkers of disease severity, as their rates of decrease seem to be related to a worsening in the SARA score. These findings highlight the potential of SD-OCT as a noninvasive tool for assessing disease severity and progression in SCA3 patients.
期刊介绍:
Official publication of the Society for Research on the Cerebellum devoted to genetics of cerebellar ataxias, role of cerebellum in motor control and cognitive function, and amid an ageing population, diseases associated with cerebellar dysfunction.
The Cerebellum is a central source for the latest developments in fundamental neurosciences including molecular and cellular biology; behavioural neurosciences and neurochemistry; genetics; fundamental and clinical neurophysiology; neurology and neuropathology; cognition and neuroimaging.
The Cerebellum benefits neuroscientists in molecular and cellular biology; neurophysiologists; researchers in neurotransmission; neurologists; radiologists; paediatricians; neuropsychologists; students of neurology and psychiatry and others.