Influenza-associated MOG antibody-positive longitudinally extensive transverse myelitis: a case report
BMC Neurology
Influenza-associated MOG antibody-positive longitudinally extensive transverse myelitis: a case report
Haruka Amano 0
Nobukazu Miyamoto 0
Hideki Shimura 0
Douglas Kazutoshi Sato 1
Kazuo Fujihara 1
Shinichi Ueno 0
Ryota Nakamura 0
Yuji Ueno 0
Masao Watanabe 0
Nobutaka Hattori 2
Takao Urabe 0
0 Department of Neurology, Juntendo University Urayasu Hospital , 2-1-1 Tomioka, Urayasu, Chiba 279-0021 , Japan
1 Department of Neurology, Tohoku University Graduate School of Medicine , Miyagi , Japan
2 Department of Neurology, Juntendo University School of Medicine , Tokyo , Japan
Background: Myelin-oligodendrocyte glycoprotein antibody (MOG antibodies) was found in various demyelinated diseases. This is the first report of a patient with longitudinally extensive transverse myelitis with an extremely high titer of MOG antibodies after an influenza infection. This case supports the view that MOG antibodies are linked to longitudinally extensive transverse myelitis and that influenza infection might trigger the MOG antibodies. Case presentation: A 32-year-old healthy male developed high fever, dysesthesia and paraesthesia below the C2 area, muscle weakness of the bilateral lower extremities, and urinary retention ten days after an influenza type A infection. Magnetic resonance imaging revealed a longitudinal lesion in the spinal cord extending from C2 to the spinal conus. There were no lesions in the brain or optic nerves. Established cell-based immunoassays revealed that he was positive for MOG antibodies (titer = 65,536) and negative for anti-aquaporin 4 antibodies (AQP4 antibodies). He fully recovered after steroid pulse therapy followed by 60 mg prednisolone. Conclusion: This is the first report of influenza A-associated longitudinally extensive transverse myelitis with a high titer anti-MOG antibodies. Our case report supports a relationship between anti-MOG antibodies and longitudinally extensive transverse myelitis, which was triggered by influenza infection. Further studies are needed to establish the clinical significance of anti-MOG antibodies for diagnosis, treatment, and prognosis.
Myelin-oligodendrocyte glycoprotein antibody; Longitudinal extensive transverse myelitis; Neuromyelitis optica; Aquaporin-4 antibody
Background
Myelin oligodendrocyte glycoprotein (MOG) localizes
on the outermost surface of the myelin sheath and
oligodendrocytes in the central nervous system (CNS).
Autoantibodies against MOG are reportedly found in patients
with a spectrum of inflammatory demyelinating diseases
of the CNS, including acute disseminated
encephalomyelitis, multiple sclerosis, transverse myelitis, and
neuromyelitis optica (NMO) [
1
]. NMO is a severe inflammatory
disorder of the central nervous system that predominantly
affects the optic nerves and spinal cord. Most patients
with NMO have antibodies against the water channel
aquaporin-4 (AQP4 antibodies), which are thought to be
pathogenic [
2-4
]. However, some patients are seronegative
for AQP4 antibody; the lack of a biomarker makes
diagnosis and management of these patients difficult. Many
clinicians perceive these patients to be similar to those with
AQP4-Abs and manage them in the same way. Recently,
some groups showed that some AQP4 antibody
seronegative patients have antibodies against MOG [
5-7
]. They also
demonstrated differences in clinical phenotypes and
responsiveness of therapy between the AQP4 antibody
seronegative and MOG antibody seropositive patients with
NMO/NMO spectrum disorder (NMOSD). Detection of
MOG antibodies is important for patients management.
Mice that are transgenic for MOG-specific T-cell and
B-cell receptors develop spontaneous experimental
autoimmune encephalomyelitis (EAE) [
8-11
]. Recent studies
suggest that MOG-related EAE can mimic a neurological
syndrome closely resembling NMO. Although the clinical
spectrum of MOG IgG associated diseases in humans is
reflected in different experimental models, the role of
MOG antibodies in pathogenesis is still unclear. Here we
describe a patient who suffered from longitudinal
extended TM (LETM) with high-titer MOG antibodies
following an influenza-A infection and his AQP4
antibody was negative.
Case presentation
A 32-year-old male, without any relevant medical
history, felt general fatigue and had a high fever of 38.9
degrees Celsius. The following day, he was diagnosed with
influenza type A by nasal swab test at a clinic, and
prescribed oseltamivir. His symptoms were ameliorated
sufficiently that he was able to return to work from day 5
and go winter climbing on days 6–7. On day 9, however,
he experienced whole body pain, urinary retention, and
weakness of the bilateral lower extremities; these
symptoms then became exacerbated. Eventually, he was
unable to walk by himself, and came to our hospital on day
10. His body temperature was 39.4 degrees Celsius and
there was marked bilateral lower extremity weakness.
Deep tendon reflexes were normal in both the upper
an (...truncated)