Optical coherence tomography as a marker of neurodegeneration in patients with Wilson's disease
| Autor: | Anna Członkowska, Tomasz Litwin, Michał Karliński, Karolina Dzieżyc, Ewa Langwińska-Wośko |
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| Rok vydání: | 2016 |
| Předmět: |
Adult
Male Retinal Ganglion Cells Unified Wilson’s Disease Rating Scale medicine.medical_specialty Pathology Neurology genetic structures Wilson’s disease Nerve fiber layer Retina 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine Nerve Fibers Optical coherence tomography Hepatolenticular Degeneration Ophthalmology medicine Humans Macula Lutea Neurodegeneration Pathological Neuroradiology medicine.diagnostic_test business.industry Retinal General Medicine Middle Aged medicine.disease eye diseases Wilson's disease medicine.anatomical_structure chemistry 030221 ophthalmology & optometry Disease Progression Female Original Article Neurology (clinical) sense organs business 030217 neurology & neurosurgery Copper Tomography Optical Coherence |
| Zdroj: | Acta Neurologica Belgica |
| ISSN: | 2240-2993 |
| Popis: | Wilson's disease (WD) is an inherited autosomal recessive disorder that leads to pathological copper accumulation in different organs. Optical coherence tomography (OCT) is proposed as a marker of neurodegeneration in many neurological diseases. Thinning of the total retinal nerve fiber layer (RNFL) and macular thickness (Mth) examined by OCT was detected in patients with WD, especially those with brain magnetic resonance imaging changes. The aim of this study was to evaluate the relationship between OCT parameters and the progression of neurological signs measured by the Unified Wilson's Disease Rating Scale (UWDRS) in patients with WD. Consecutive patients with WD admitted to the Department of Neurology underwent OCT to assess the thickness of the macula and total RNFL. Patients also had neurologic assessments according to the UWDRS part III. Patients were divided into two groups based on the presence (UWDRS+) and absence (UWDRS-) of neurological symptoms. Fifty-eight patients (34 females, 24 males) were enrolled. Mean duration of treatment was 9 years (standard deviation [SD], ±10.8). The mean UWDRS score at the time of study was 8.4 (range 1-52; SD ±13.9) points. Total RNFL as well as macula thickness were significantly decreased in the UWDRS+ group versus the UWDRS- group. A significant negative correlation was found between OCT parameters (RNFL and Mth measurements) and neurological impairment according the UWDRS scale. This study confirms that OCT may be a useful tool for measuring the degree of neurodegeneration in patients with WD, and may play role in monitoring disease progression. |
| Databáze: | OpenAIRE |
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