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Optical coherence tomography in adult adrenoleukodystrophy: a cross-sectional and longitudinal study
- Source :
- Neurological Sciences. 42:235-241
- Publication Year :
- 2020
- Publisher :
- Springer Science and Business Media LLC, 2020.
-
Abstract
- Adrenoleukodystrophy (ALD) encompasses different neurological phenotypes, ranging from the most severe cerebral forms (C-ALD) to the less severe adrenomyeloneuropathy (AMN). As visual system can be varyingly involved, we aimed at exploring whether optical coherence tomography (OCT) may detect retinal abnormalities and their longitudinal changes in adult ALD patients. In this cross-sectional and longitudinal study, we measured the thicknesses of peripapillary retinal nerve fiber layer (pRNFL), macular ganglion cell complex (mGCC), and segmented inner and outer macula at baseline and their changes over time in 11 symptomatic adult ALD males and 10 age- and sex-matched healthy controls. Statistical analyses were performed for the patients as complete group, and splitting them into two subgroups, one (C-ALD) with and the other (AMN) without cerebral parieto-occipital white matter (WM) lesions. In the complete ALD group and in the C-ALD subgroup, the average pRNFL, mGCC, and inner macula were significantly thinner than in controls (p ≤ 0.01), whereas in the AMN subgroup, they were constantly, though non-significantly, thinner. Significant outer macula thinning was also observed (p
- Subjects :
- endocrine system
medicine.medical_specialty
genetic structures
Nerve fiber layer
Dermatology
White matter
03 medical and health sciences
chemistry.chemical_compound
0302 clinical medicine
Optical coherence tomography
Ophthalmology
medicine
030212 general & internal medicine
Neuroradiology
medicine.diagnostic_test
business.industry
Leukodystrophy
Retinal
General Medicine
medicine.disease
eye diseases
Ganglion
Psychiatry and Mental health
medicine.anatomical_structure
chemistry
Adrenoleukodystrophy
sense organs
Neurology (clinical)
business
030217 neurology & neurosurgery
Subjects
Details
- ISSN :
- 15903478 and 15901874
- Volume :
- 42
- Database :
- OpenAIRE
- Journal :
- Neurological Sciences
- Accession number :
- edsair.doi...........e0e2401f33defdd2092502bcec30ca42