title: Aquaporin-4 antibody negative recurrent isolated optic neuritis: clinical evidence for disease heterogeneity.
abstract: Recurrent optic neuritis is frequently observed in multiple sclerosis (MS) and is a typical finding in neuromyelitis optica (NMO). Patients that lack further evidence of demyelinating disease are diagnosed with RION (recurrent isolated optic neuritis) or CRION (chronic relapsing inflammatory neuropathy) if they require immunosuppressive therapy to prevent further relapses. The etiology and disease course of this rare condition are not well defined. We studied a series of 10 patients who presented with recurrent episodes of isolated optic neuritis (ON, n=57) and were followed over a median of 3.5 years. Visual acuity was severely reduced at the nadir of the disease (20/200 to 20/800). All patients had MRI non-diagnostic for MS/NMO and were aquaporin-4 antibody negative. Six patients fulfilled the CRION criteria. In two of these a single ON followed by a long disease-free interval preceded development of CRION for years, suggesting the conversion of an initially "benign" isolated ON into the chronic relapsing course. Cerebrospinal fluid (CSF) analysis revealed mild pleocytosis in 5 patients, identical oligoclonal bands in serum and CSF were observed in 2 patients, while the others remained negative. In conclusion, recurrent ON is a disease entity that requires aggressive glucocorticoid and eventually long-term immunosuppressive therapy to prevent substantial visual impairment.
authors.affiliation: Dept. of Neurology, Friedrich-Alexander University of Erlangen, Erlangen, Germany. Anne.Waschbisch@uk-erlangen.de
authors.forename: A
authors.initials: A
authors.lastname: Waschbisch
authors.forename: M
authors.initials: M
authors.lastname: Atiya
authors.forename: C
authors.initials: C
authors.lastname: Schaub
authors.forename: T
authors.initials: T
authors.lastname: Derfuss
authors.forename: S
authors.initials: S
authors.lastname: Schwab
authors.forename: D H
authors.initials: DH
authors.lastname: Lee
authors.forename: M
authors.initials: M
authors.lastname: Müller
authors.forename: R A
authors.initials: RA
authors.lastname: Linker
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000906:Antibodies / Q000378:metabolism*; D051401:Aquaporin 4 / Q000276:immunology*; D002648:Child; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007167:Immunotherapy; D007964:Leukocytosis / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D043025:Oligoclonal Bands / Q000134:cerebrospinal fluid; D009902:Optic Neuritis / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy; D014792:Visual Acuity / Q000502:physiology; D055815:Young Adult
chemical_list: C496030:AQP4 protein, human; D000906:Antibodies; D051401:Aquaporin 4; D005938:Glucocorticoids; D043025:Oligoclonal Bands
keywords: CRION; Inflammatory neuropathy; Multiple sclerosis; Neuromyelitis optica; Optic neuritis; RION