ABSTRACT
@#Objective Report and analyze the clinical features，diagnosis，and prognosis of 3 patients with elderly-onset（age>75 years） neuromyelitis Optica spectrum disorders（NMOSDs），reviewed previous pieces of literature to improve the risk and understanding of NMOSD in elderly patients. Methods We searched the domestic and international databases in the past 10 years，to collect the case report of patients with NMOSDs over the age of 75 years，along with three cases discovered in the neurology department of the First Affiliated Hospital of Zhengzhou University. The clinical features，laboratory tests and treatment，were retrospectively analyzed. Results A total of 12 patients with NMOSDs>75 years old were enrolled in this study，including 7 females and 5 males，with an average age of onset（82.67±4.42） years. All patients were diagnosed with longitudinally extensive transverse myelitis（LETM），and the single-phase course was the majority. The AQP4 test was positive in all cases and the MRI was illustrated that mainly caused the thoracic spinal cord. Twelve cases were treated with intravenous methylprednisolone（IVMP）1 g daily for 3 to 5 days，3 cases were combined with plasma exchange（PLEX），4 cases were treated with immunosuppression，and 5 cases had a relapse，9 cases were effective，2 cases were markedly effective，3 cases were worsened. Three patients died during treatment and follow-up. Conclusion Patients with very late-onset NMOSDs are more likely to have LETM，higher disability rates. IVMP plus PLEX is more effective than IVMP alone，But the benefit of immunosuppression is unclear in clinical practice and observational studies.
ABSTRACT
Neuromyelitis optica (NMO) has been traditionally described as the association of recurrent or bilateral optic neuritis and longitudinally extensive transverse myelitis (LETM). Identification of aquaporin-4 antibody (AQP4-IgG) has deeply changed the concept of NMO. A spectrum of NMO disorders (NMOSD) has been formulated comprising conditions which include both AQP4-IgG seropositivity and one of the index events of the disease (recurrent or bilateral optic neuritis and LETM). Most NMO patients harbor asymptomatic brain MRI lesions, some of them considered as typical of NMO. Some patients with aquaporin-4 autoimmunity present brainstem, hypothalamic or encephalopathy symptoms either preceding an index event or occurring isolatedly with no evidence of optic nerve or spinal involvement. On the opposite way, other patients have optic neuritis or LETM in association with typical lesions of NMO on brain MRI and yet are AQP4-IgG seronegative. An expanded spectrum of NMO disorders is proposed to include these cases.
Neuromielite óptica (NMO) tem sido tradicionalmente caracterizada como associação de neurite óptica recorrente ou bilateral e mielite tranversa longitudinalmente extensa (MTLE). O conceito de NMO tem mudado desde a identificação do anticorpo antiaquaporina-4. A NMO é atualmente considerada como um espectro de condições contendo pelo menos um dos eventos índices da doença (neurite óptica recorrente ou bilateral e MTLE) e soropositividade para AQP4-IgG. A maioria dos pacientes apresenta lesões cerebrais à imagem por ressonância magnética (IRM), algumas delas típicas de NMO. Pacientes soropositivos podem desenvolver sintomas de tronco encefálico, hipotálamo e de encefalopatia precedendo os eventos índices, ou isoladamente, na ausência de qualquer evidência de alteração visual ou espinal. Por outro lado, há pacientes soronegativos que apresentam ou neurite óptica ou MTLE associada a lesões cerebrais típicas de NMO à IRM. Todas essas situações estão incluídas no espectro expandido de NMO aqui proposto.