Severe demyelination but no astrocytopathy in clinically definite neuromyelitis optica with anti-myelin-oligodendrocyte glycoprotein antibody

2014 ◽  
Vol 21 (5) ◽  
pp. 656-659 ◽  
Author(s):  
Kensuke Ikeda ◽  
Naoki Kiyota ◽  
Hiroshi Kuroda ◽  
Douglas Kazutoshi Sato ◽  
Shuhei Nishiyama ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Neuromyelitis Optica ◽  
Basic Protein ◽  
Transverse Myelitis ◽  
Acute Attack ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
High Dose ◽  
Longitudinally Extensive Transverse Myelitis ◽  
High Dose Methylprednisolone ◽  
Extensive Transverse Myelitis

We report a patient with neuromyelitis optica (NMO) presenting anti-myelin-oligodendrocyte glycoprotein (MOG)-seropositive, in whom biomarkers of demyelination and astrocyte damage were measured during an acute attack. A 31-year-old man developed right optic neuritis followed by longitudinally extensive transverse myelitis, fulfilling the criteria for definite NMO. He was anti-MOG-seropositive and anti-aquaporin-4 seronegative. The myelin basic protein level was markedly elevated whereas glial fibrillary acidic protein was not detectable in cerebrospinal fluid during an acute attack. His symptoms quickly improved after high-dose methylprednisolone therapy. This case suggests that NMO patients with anti-MOG may have severe demyelination in the absence of astrocyte injury.

scholarly journals P.049 Medically refractory longitudinally extensive transverse myelitis successfully treated with cyclophosphamide induction

2016 ◽  
Vol 43 (S2) ◽  
pp. S32-S33
Author(s):  
LJ Baxter ◽  
S Chen ◽  
JM Burton
Keyword(s):  
Respiratory Failure ◽  
Plasma Exchange ◽  
Immune Therapy ◽  
Transverse Myelitis ◽  
Acute Disseminated Encephalomyelitis ◽  
High Dose ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Intravenous Methylprednisolone ◽  
Intensive Care Admission ◽  
Extensive Transverse Myelitis

Background: Longitudinally extensive transverse myelitis (LETM) is a demyelinating condition that is associated with diseases such as neuromyelitis optica spectrum disorder (NMOSD), acute disseminated encephalomyelitis, collagen vascular disease, or can be idiopathic. LETM can be severe enough to cause quadraparesis, marked sensory dysfunction, and respiratory failure. Rarely, these patients are unresponsive to conventional immune therapy. Methods: We report two cases of severe LETM with acute development of quadraparesis and respiratory failure requiring intensive care admission and failure to respond to high-dose corticosteroids, plasma exchange, IVIg and rituximab. Disease cessation and ultimately, significant recovery, was achieved after an 8-day cyclophosphamide induction. Results: A 21 yo female with antibody positive NMOSD and a 19 yo male with idiopathic LETM remained quadraparetic and ventilator dependent with active MRIs despite multiple courses of intravenous methylprednisolone, plasma exchanges, and in the NMOSD patient, IVIg and a 4-week course of rituximab. Both patients ultimately improved significantly and are now ambulatory with subsequent cyclophosphamide induction. Conclusions: In patients with severe LETM of presumed immune origin, who fail to respond to corticosteroids and plasma exchange, cyclophosphamide induction should be considered. This agent provides a more robust immunosuppressive response and can be induced rapidly. Cyclophosphamide effects and supportive evidence are further discussed.

Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis

2006 ◽  
Vol 59 (3) ◽  
pp. 566-569 ◽  
Author(s):  
Brian G. Weinshenker ◽  
Dean M. Wingerchuk ◽  
Sandra Vukusic ◽  
Linda Linbo ◽  
Sean J. Pittock ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Extensive Transverse Myelitis

Occurrence of acute large and edematous callosal lesions in neuromyelitis optica

2009 ◽  
Vol 15 (6) ◽  
pp. 695-700 ◽  
Author(s):  
M Nakamura ◽  
T Misu ◽  
K Fujihara ◽  
I Miyazawa ◽  
I Nakashima ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Medical Records ◽  
Brain Magnetic Resonance Imaging ◽  
Transverse Myelitis ◽  
Resonance Imaging ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Lower Margin ◽  
Chronic Stage ◽  
The Brain ◽  
Extensive Transverse Myelitis

Background The corpus callosum is commonly involved in multiple sclerosis (MS), but the characteristics of callosal lesions in neuromyelitis optica (NMO) are unknown. Objective To reveal the features of callosal lesions in NMO in comparison to MS. Methods We retrospectively reviewed the medical records and the brain magnetic resonance imaging films of 56 patients with MS and 22 patients with NMO. Results In MS, 36 (64.3%) of 56 patients had callosal lesions, but only four patients had acute lesions. All such acute lesions were small, isolated and non-edematous, and the intensity was homotonic. Chronic lesions were observed in 34 patients with MS, and 32 (94%) of them presented small lesions located at the callosal lower margin (“hemi-oval pattern”). Meanwhile, four (18.2%) patients with NMO had callosal lesions, and three of them had acute lesions. Those acute lesions were multiple, large edematous ones with heterogeneous intensity (“marbled pattern”). In the chronic stage, the lesions shrank or disappeared. Conclusions Acute large, edematous callosal lesions occasionally occur in NMO. Similar to longitudinally extensive transverse myelitis, such callosal lesions may reflect severe edematous inflammation in NMO, and may provide additional evidence that the pathogenesis in NMO is different from that in MS.

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