Cervical cord and ventricle affection in neuromyelitis optica

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Neuromyelitis optica (NMO) is an inflammatory demyelinating central nervous system (CNS) disorder primarily affecting the optic nerve and the spinal cord. Magnetic resonance imaging (MRI) findings in NMO include the presence of contiguous spinal cord lesions extending up to more than three vertebral segments and involvement of the optical nerve and anterior visual pathway 1. Previously, the lack of brain lesions was an important diagnostic criterion but was recently challenged by new data, as brain MRI abnormalities in NMO have been observed more frequently than expected 2. Thus, the absolute restriction of CNS involvement was removed in the revised diagnostic criteria for NMO 4. Recent MRI studies have focused on lesion localization and regional CNS atrophy to characterize different patterns of MRI abnormalities in NMO in delineation of multiple sclerosis (MS). Infratentorial lesions, especially within the medulla oblongata seem to be of important clinical relevance in NMO 5. Furthermore, a recent study describing correlations between Gadolinium ring enhancing spinal cord lesions and markers for demyelination in the cerebrospinal fluid (CSF), supported the hypothesis of associations between inflammatory processes affecting the spinal cord and the entire CNS 7.
However, controversial results have been published regarding the involvement of cerebral grey and white matter (GM, WM) in NMO 8. GM atrophy in NMO has been found to be restricted to cortical areas, while in contrast to MS, the deep grey matter nuclei seemed to be unaffected 10. Contrary to MS, cortical lesions were not found in NMO corresponding to histopathological findings like the absence of cortical demyelination 11. A different study using voxel‐based morphometry (VBM) analysis reported significant reduction in WM volume without alteration of GM in patients with NMO 12. By contrast, in MS, atrophy of the cortex and of deep grey matter is a common finding already in early disease phases, corresponding to clinical progression 14.
Ventricular enlargement, especially widening of the third ventricle, has been described as an early marker of disease progression in MS, reflecting deep GM involvement and periventricular tissue loss 15. So far, enlargement of the ventricular system has not been observed in NMO.
The aim of this study was to determine different regional patterns of CNS involvement, including supra‐ and infratentorial brain regions, as well as the upper cervical cord in NMO and MS patients using quantitative MRI and additionally to complement ventricular volumetry results by VBM of the CSF 16.
Furthermore, the relationship of regional CNS atrophy and ventricular alteration on clinical disability was investigated.
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