Short myelitis lesions in aquaporin-4-IgG-positive neuromyelitis optica spectrum disorders.
OBJECTIVE
Short transverse myelitis (STM; <3 vertebral segments) is considered noncharacteristic of neuromyelitis optica (NMO) spectrum disorders (NMOSDs). Nonappreciation of the potential for STM to occur in NMOSD may lead to increased disability from delay in diagnosis and appropriate treatment.


OBJECTIVE
To determine the frequency of short lesions at the initial myelitis manifestation of NMOSD and to compare the demographic, clinical, and radiological characteristics of aquaporin-4-IgG (AQP4-IgG) seropositive and seronegative STM.


METHODS
We reviewed the records and images of patients at the Mayo Clinic who were identified as AQP4-IgG positive from 1996 to 2014. Inclusion criteria were first STM episode, magnetic resonance imaging performed 90 days or less from symptom onset, spinal cord T2-hyperintense lesion less than 3 vertebral segments, AQP4-IgG seropositivity, and a final diagnosis of NMO or NMOSD. Patients with an initial longitudinally extensive transverse myelitis were excluded (n = 151). Patients with STM who were seronegative for AQP4-IgG among an Olmsted County population-based cohort of inflammatory demyelinating disorders of the central nervous system were used as a control group.


METHODS
Delay to diagnosis in months, clinical and radiological characteristics, and disability measured by ambulatory status.


RESULTS
Twenty-five patients who were AQP4-IgG seropositive with an initial STM represented 14% of initial myelitis episodes among patients with NMOSD. The STM episode was defined as the first manifestation of NMOSD in 10 patients (40%) preceded by optic neuritis in 13 patients (52%) and preceded by a nausea and vomiting episode in 2 patients (8%). In comparison with the excluded patients with NMOSD who had an initial longitudinally extensive transverse myelitis, delay to diagnosis/treatment was greater when initial lesions were short (P = .02). In AQP4-IgG-positive STM cases, subsequent myelitis episodes were longitudinally extensive in 92%. Attributes more common in patients with AQP4-IgG-positive STM than in 27 population-based patients with AQP4-IgG-negative STM included the following: nonwhite race/ethnicity; tonic spasms; coexisting autoimmunity; magnetic resonance imaging (central cord lesions, T1 hypointensity, and a brain inconsistent with multiple sclerosis); and cerebrospinal fluid (oligoclonal bands lacking).


CONCLUSIONS
Short transverse myelitis is not uncommon in NMOSD and, when it is present, delays diagnosis and treatment. Clinical and radiological characteristics identified in this study may help select patients with STM who are at the highest risk for an NMOSD. Short transverse myelitis does not exclude consideration of AQP4-IgG testing or NMOSD diagnosis.
Department of Neurology, Mayo Clinic, Rochester, Minnesota.
Eoin P
EP
Flanagan
Department of Neurology, Mayo Clinic, Rochester, Minnesota.
Brian G
BG
Weinshenker
Department of Radiology, Mayo Clinic, Rochester, Minnesota.
Karl N
KN
Krecke
Department of Neurology, Mayo Clinic, Rochester, Minnesota3Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota4Department of Immunology, Mayo Clinic, Rochester, Minnesota.
Vanda A
VA
Lennon
Department of Neurology, Mayo Clinic, Rochester, Minnesota.
Claudia F
CF
Lucchinetti
Department of Neurology, Mayo Clinic, Rochester, Minnesota3Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.
Andrew
A
McKeon
Department of Neurology, Mayo Clinic, Scottsdale, Arizona.
Dean M
DM
Wingerchuk
Department of Neurology, Mayo Clinic Jacksonville, Florida.
Elizabeth A
EA
Shuster
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.
Yujuan
Y
Jiao
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.
Erika S
ES
Horta
Department of Neurology, Mayo Clinic, Rochester, Minnesota3Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.
Sean J
SJ
Pittock
D000328:Adult; D000368:Aged; D051401:Aquaporin 4 / Q000276:immunology*; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009187:Myelitis / Q000209:etiology* / Q000473:pathology; D009471:Neuromyelitis Optica / Q000097:blood* / Q000150:complications*; D013116:Spinal Cord / Q000473:pathology; D018709:Statistics, Nonparametric
D051401:Aquaporin 4; D007074:Immunoglobulin G