Very late-onset neuromyelitis optica spectrum disorder beyond the age of 75

Abstract

Aquaporin-4 antibody (AQP4-Ab)-positive neuromyelitis optica spectrum disorder (NMOSD) is a rare but often severe autoimmune disease with median onset around 40 years of age. We report characteristics of three very-late-onset NMOSD (including complete NMO) patients >75 years of age, in whom this diagnosis initially seemed unlikely because of their age and age-associated concomitant diseases, and briefly review the literature. All three patients, aged 79, 82 and 88 years, presented with a spinal cord syndrome as the first clinical manifestation of AQP4-Ab-positive NMOSD. They all had severe relapses unless immunosuppressive therapy was initiated, and one untreated patient died of a fatal NMOSD course. Two patients developed side effects of immunosuppression. We conclude that a first manifestation of NMOSD should be considered even in patients beyond the age of 75 years with a compatible syndrome, especially longitudinally extensive myelitis. Early diagnosis and treatment are feasible and highly relevant. Special attention is warranted in the elderly to recognize adverse effects of immunosuppressive therapies as early as possible.

Fig. 1

MRI scans of case 1–3 showing longitudinally extensive spinal cord lesions. Yellow solid arrows indicate extensions of lesions. The lesion extended throughout the entire myelon and there were also lesions in the brainstem in case 1 (a) (upper inset in a). Blue dashed lines in the sagittal images indicate the levels of related axial scans. med. obl. axial scan at level of medulla oblongata, C and T indicate the respective cervical and thoracic vertebrae; T2 T2 weighed MRI sequence, T1 T1 weighed MRI sequence, +Gd gadolinium-enhanced sequence