دورية أكاديمية
Progression of Retinal Ganglion Cell and Nerve Fiber Layer Loss in Spinocerebellar Ataxia 3 Patients.
| العنوان: | Progression of Retinal Ganglion Cell and Nerve Fiber Layer Loss in Spinocerebellar Ataxia 3 Patients. |
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| المؤلفون: | Camós-Carreras A; Ophthalmology Department, Seu Maternitat, Hospital Clínic de Barcelona, Universitat de Barcelona, Sabino de Arana 1, 08028, Barcelona, Spain. annacamos@gmail.com.; Faculty of Medicine and Health Sciences, Universitat de Barcelona, Casanova 143, 08036, Barcelona, Spain. annacamos@gmail.com., Figueras-Roca M; Ophthalmology Department, Seu Maternitat, Hospital Clínic de Barcelona, Universitat de Barcelona, Sabino de Arana 1, 08028, Barcelona, Spain.; Fundació Per La Recerca Biomèdica-IDIBAPS, Villarroel 170, 08036, Barcelona, Spain., Dotti-Boada M; Ophthalmology Department, Seu Maternitat, Hospital Clínic de Barcelona, Universitat de Barcelona, Sabino de Arana 1, 08028, Barcelona, Spain.; Faculty of Medicine and Health Sciences, Universitat de Barcelona, Casanova 143, 08036, Barcelona, Spain., Alcubierre R; Ophthalmology Department, Seu Maternitat, Hospital Clínic de Barcelona, Universitat de Barcelona, Sabino de Arana 1, 08028, Barcelona, Spain., Casaroli-Marano RP; Ophthalmology Department, Seu Maternitat, Hospital Clínic de Barcelona, Universitat de Barcelona, Sabino de Arana 1, 08028, Barcelona, Spain.; Faculty of Medicine and Health Sciences, Universitat de Barcelona, Casanova 143, 08036, Barcelona, Spain.; Fundació Per La Recerca Biomèdica-IDIBAPS, Villarroel 170, 08036, Barcelona, Spain., Muñoz E; Faculty of Medicine and Health Sciences, Universitat de Barcelona, Casanova 143, 08036, Barcelona, Spain.; Fundació Per La Recerca Biomèdica-IDIBAPS, Villarroel 170, 08036, Barcelona, Spain.; Neurology Department, Seu Villarroel, Hospital Clínic de Barcelona, Villarroel 170, 08036, Barcelona, Spain., Sánchez-Dalmau B; Ophthalmology Department, Seu Maternitat, Hospital Clínic de Barcelona, Universitat de Barcelona, Sabino de Arana 1, 08028, Barcelona, Spain.; Faculty of Medicine and Health Sciences, Universitat de Barcelona, Casanova 143, 08036, Barcelona, Spain.; Fundació Per La Recerca Biomèdica-IDIBAPS, Villarroel 170, 08036, Barcelona, Spain. |
| المصدر: | Cerebellum (London, England) [Cerebellum] 2024 Aug; Vol. 23 (4), pp. 1348-1354. Date of Electronic Publication: 2023 Nov 30. |
| نوع المنشور: | Journal Article |
| اللغة: | English |
| بيانات الدورية: | Publisher: Springer Country of Publication: United States NLM ID: 101089443 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1473-4230 (Electronic) Linking ISSN: 14734222 NLM ISO Abbreviation: Cerebellum Subsets: MEDLINE |
| أسماء مطبوعة: | Publication: <2006->: New York : Springer Original Publication: London : Martin Dunitz, c2002- |
| مواضيع طبية MeSH: | Machado-Joseph Disease*/diagnostic imaging , Machado-Joseph Disease*/pathology , Retinal Ganglion Cells*/pathology , Tomography, Optical Coherence*/methods , Nerve Fibers*/pathology , Disease Progression*, Humans ; Male ; Female ; Middle Aged ; Retrospective Studies ; Adult ; Aged ; Follow-Up Studies |
| مستخلص: | 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. (© 2023. The Author(s).) |
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| فهرسة مساهمة: | Keywords: Machado-joseph disease; Optical coherence tomography; Retinal ganglion cell; Spinocerebellar ataxia type 3 |
| تواريخ الأحداث: | Date Created: 20231129 Date Completed: 20240724 Latest Revision: 20240727 |
| رمز التحديث: | 20240727 |
| مُعرف محوري في PubMed: | PMC11269372 |
| DOI: | 10.1007/s12311-023-01634-1 |
| PMID: | 38030858 |
| قاعدة البيانات: | MEDLINE |
Find this article in full text from Springer Verlag. 
Full Text Finder | تدمد: | 1473-4230 |
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| DOI: | 10.1007/s12311-023-01634-1 |