Diagnosis Neuromyelitis optica

1 diagnosis

1.1 variants
1.2 tumefactive lesions
1.3 differential diagnosis

diagnosis

the mayo clinic proposed revised set of criteria diagnosis of devic s disease in 2006. new guidelines require 2 absolute criteria plus @ least 2 of 3 supportive criteria. in 2015 new review published international panel refining previous clinical case definition leaving main criteria unmodified:

absolute criteria:

supportive criteria:

variants

after development of nmo-igg test, spectrum of disorders comprising devic s disease expanded. spectrum believed consist of:

standard devic s disease, according diagnostic criteria described above
limited forms of devic s disease, such single or recurrent events of longitudinally extensive myelitis, , bilateral simultaneous or recurrent optic neuritis
asian optic-spinal ms—this variant can present brain lesions ms.
longitudinally extensive myelitis or optic neuritis associated systemic autoimmune disease
optic neuritis or myelitis associated lesions in specific brain areas such hypothalamus, periventricular nucleus, , brainstem
nmo-igg negative nmo: aqp4 antibody-seronegative nmo poses diagnostic challenge. cases related anti-myelin oligodendrocyte glycoprotein (mog) autoantibodies.

whether devic s disease distinct disease or part of wide spectrum of multiple sclerosis debated. devic s disease differs in has more severe sequelae after acute episode standard ms, ms infrequently presents transverse myelitis, , oligoclonal bands in csf, white matter lesions on brain mri, uncommon in devic s disease, occur in on 90% of ms patients.

recently, aqp4 has been found distinguish standard multiple sclerosis neuromyelitis optica, ms heterogeneous condition, , ms cases reported kir4.1 channelopathies (autoimmunity against potassium channels) still possible consider nmo part of ms spectrum. besides, nmo-aqp(−) variants not astrocytopathic, demyelinating.

tumefactive lesions

tumefactive demyelinating lesions in nmo not usual, have been reported appear in several cases mistakenly treated interferon beta.

differential diagnosis

aqp4-ab-negative nmo presents problems diagnosis. behavior of oligoclonal bands respect ms can establish more accurate diagnosis. oligoclonal bands in nmo rare , tend disappear after attacks, while in ms present , persistent.

it important notice differential diagnosis that, though uncommon, possible have longitudinal lesions in ms