NMOSD诊断和治疗课件.pptxVIP

NMOSD诊断和治疗;主要内容;The Definition and Progression of NMOSD;NMOSD diagnostic criteria for adult patients;Core clinical characteristics 1. Optic neuritis 2. Acute myelitis 3. Area postrema syndrome: episode of otherwise unexplained hiccups or nausea and vomiting 4. Acute brainstem syndrome 5. Symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical diencephalic MRI lesions 6. Symptomatic cerebral syndrome with NMOSD-typical brain lesions ;Additional MRI requirements for NMOSD without AQP4-IgG and NMOSD with unknown AQP4-IgG status 1. Acute optic neuritis: requires brain MRI showing (a) normal findings or only nonspecific white matter lesions, OR (b) optic nerve MRI with T2-hyperintense lesion or T1-weighted gadolinium enhancing lesion extending over .1/2 optic nerve length or involving optic chiasm 2. Acute myelitis: requires associated intramedullary MRI lesion extending over 3 contiguous segments (LETM) OR 3 contiguous segments of focal spinal cord atrophy in patients with history compatible with acute myelitis 3. Area postrema syndrome: requires associated dorsal medulla/area postrema lesions 4. Acute brainstem syndrome: requires associated periependymal brainstem lesions;;;Spinal cord MRI, acute LETM lesion associated with acute TM Increased signal on sagittal T2-weighted (standard T2-weighted, proton density, or STIR sequences) extending over 3 or more complete vertebral segments Central cord predominance (more than 70% of the lesion residing within the central gray matter) Gadolinium enhancement of the lesion on T1-weighted sequences (no specific distribution or pattern of enhancement is required) Other characteristic features that may be detected Rostral extension of the lesion into the brainstem (figure 1, D and E) Cord expansion/swelling Decreased signal on T1-weighted sequences corresponding to region of increased T2-weighted signal ;Spinal cord MRI, chronic Longitudinally extensive cord atrophy (sharply demarcated atrophy extendi