Demyelinating Diseases: Multiple Sclerosis and Neuromyelitis Optica



Fig. 58.1
Neuromyelitis optica (NMO). (a) Coronal fat-saturated T1-weighted gadolinium-enhanced MR image. (b) Coronal inversion recovery image. (c) Sagittal T1-weighted gadolinium-enhanced image. The left optic nerve demonstrates edema (b) and enhancement (a) consistent with neuritis. There is also focal enhancement of the lower thoracic cord (c) (arrow)



A307296_1_En_58_Fig2_HTML.gif


Fig. 58.2
NMO. (a) Sagittal T1-weighted gadolinium-enhanced MR image. (b) Coronal T1-weighted gadolinium-enhanced image. This young woman with vision loss and hypopituitarism was found to have NMO. There is slight enhancement of the optic chiasm (arrow) and infundibulum



  • MRI in some patients with NMO is normal.


  • Optical coherence tomography (OCT) may be used to measure the thickness of the retinal nerve fiber layer in MS and NMO.






      58.3 Histopathology






      • White matter plaques with demyelination and extensive perivenous infiltration of lymphocytes, macrophages, and plasma cells are commonly seen.


      • Axonal tracts are typically preserved.


      • Astrogliosis is often seen.


      • In severe cases, necrotizing inflammation of the white matter can be seen.


      • IgG and IgM antibodies are often positive in affected tissue.


      58.4 Clinical Management






      • Although not all patients with NMO are seroconverted, serum evidence of aquaporin-4 antibodies confirm the diagnosis (NMO-IgG antibody). This test has a sensitivity of 75 % in diagnosing NMO [18].


      • Diagnostic criteria for NMO currently require optic neuritis, acute myelitis, and two of the following three characteristics [19]:



        • MRI that is nondiagnostic for MS


        • A longitudinal spinal cord lesion extending over three vertebral segments


        • NMO-IgG seropositive status


      • Treatment for acute NMO includes high-dose corticosteroids and plasmapheresis.


      • Long-term treatment may involve drugs such as azathioprine, mofetil mycophenolate, and rituximab [20].


      • A frequent relapsing course is observed in over 80 % of patients with NMO [19].


      References



      1.

      Ysrraelit MC, Gaitán MI, Lopez AS, Correale J. Impaired hypothalamic-pituitary-adrenal axis activity in patients with multiple sclerosis. Neurology. 2008;71:1948–54.CrossRefPubMed


      2.

      Fassbender K, Schmidt R, Mössner R, Kischka U, Kühnen J, Schwartz A, Hennerici M. Mood disorders and dysfunction of the hypothalamic-pituitary-adrenal axis in multiple sclerosis: association with cerebral inflammation. Arch Neurol. 1998;55:66–72.CrossRefPubMed


      3.

      Huitinga I, Erkut ZA, van Beurden D, Swaab DF. Impaired hypothalamus-pituitary-adrenal axis activity and more severe multiple sclerosis with hypothalamic lesions. Ann Neurol. 2004;55:37–45.CrossRefPubMed

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      Mar 11, 2017 | Posted by in NEUROSURGERY | Comments Off on Demyelinating Diseases: Multiple Sclerosis and Neuromyelitis Optica

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