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1.
Eur J Neurol ; 22(1): 86-92, 2015 Jan.
Article in English | MEDLINE | ID: mdl-25104178

ABSTRACT

BACKGROUND AND PURPOSE: Some 3%-10% of patients with multiple sclerosis (MS) experience disease onset before the age of 18 years ('early' onset MS, EOMS). Optical coherence tomography is a non-invasive method to measure retinal nerve fibre layer thickness (RNFLT) and total macular volume (TMV) and may be useful to differentiate axonal and neuronal damage in the retina of patients with a history of EOMS. Here RNFLT and TMV in EOMS patients after a mean disease duration of 11.6 years were compared with patients with age- or disease-duration-matched later onset MS (LOMS) and healthy controls (HCs). METHODS: In this observational cross-sectional study at two German academic MS centres, RNFLT and TMV were measured by spectral-domain optical coherence tomography in 32 HCs, 36 EOMS (mean age at onset 15.5 ± 2.0 years) and 58 LOMS patients. RESULTS: In comparison with HCs, EOMS patients displayed a significant reduction of RNFLT and TMV independently of a history of optic neuritis. In particular, RNFLT loss in EOMS was similar to that in LOMS and TMV loss was slightly higher compared with disease-duration-matched LOMS. In a generalized estimating model, the EOMS group also displayed a similar correlation between disease duration and RNFLT or TMV loss to LOMS patients. CONCLUSIONS: These data argue for a significant amount of axonal and neuronal damage in the retina of EOMS patients and may provide a structural basis for the observation that EOMS patients reach states of irreversible disability at a younger age than patients with LOMS.


Subject(s)
Multiple Sclerosis/pathology , Retina/pathology , Tomography, Optical Coherence/methods , Adult , Age of Onset , Cross-Sectional Studies , Female , Humans , Macula Lutea/pathology , Male , Middle Aged , Nerve Fibers/pathology , Retinal Neurons/pathology
2.
Eur J Neurol ; 20(8): 1170-6, 2013 Aug.
Article in English | MEDLINE | ID: mdl-23582075

ABSTRACT

BACKGROUND AND PURPOSE: In recent years a possible non-motor involvement of the nervous system in amyotrophic lateral sclerosis (ALS) has come into the focus of research and has been investigated by numerous techniques. Optical coherence tomography (OCT) - with its potential to reveal neuroaxonal retinal damage - may be an appropriate tool to investigate whether the anterior visual pathway is involved. Our aim was to determine whether OCT-based measures of retinal nerve fiber layer, ganglion cell layer, inner nuclear layer and outer nuclear layer thickness are abnormal in ALS, or correlated with disease severity. METHODS: Seventy-six ALS patients (144 eyes) and 54 healthy controls (108 eyes; HCs) were examined with OCT, including automated intraretinal macular segmentation. ALS disease severity was determined with the Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised. RESULTS: There was no significant difference between ALS patients and HCs in any of the examined OCT measures. Moreover, OCT parameters showed no correlation with clinical measures of disease severity. CONCLUSIONS: These findings indicate that involvement of the anterior visual pathway is not one of the non-motor manifestations of ALS.


Subject(s)
Amyotrophic Lateral Sclerosis/pathology , Optic Nerve/pathology , Tomography, Optical Coherence/methods , Adult , Aged , Aged, 80 and over , Cohort Studies , Disease Progression , Female , Humans , Male , Middle Aged , Retinal Ganglion Cells/pathology , Retinal Neurons/pathology , Retinal Photoreceptor Cell Inner Segment/pathology , Retinal Photoreceptor Cell Outer Segment/pathology
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