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Published on: August 21, 2017
Idiopathic severe recurrent transverse myelitis: a restricted variant of neuromyelitis optica
Koon Ho Chan1, Kin Lun Tsang, Gardnia Chung Yan Fong
1Division of Neurology, University Department of Medicine, 4th Floor, Professorial Block, Pokfulam Road, Queen Mary Hospital, The University of Hong Kong, Hong Kong, SAR, China. koonhochan@hotmail.com
Abstract:
Recurrent idiopathic transverse myelitis occur in multiple sclerosis (MS) and neuromyelitis optica (NMO). In NMO, acute optic neuritis and myelitis occur, either monophasic or relapsing, without clinical manifestations of involvement of other parts of the central nervous system (CNS). Recent evidence suggests that NMO is different from multiple sclerosis. The authors reported two patients having severe recurrent transverse myelitis sparing the optic nerves and cerebral hemispheres. Both patients had longitudinally extensive myelitis in some attacks with poor neurological outcome despite aggressive immunomodulatory therapy. One patient had prominent clinical features of brainstem injury with radiological and histological confirmation of brainstem involvement, and the other patient had trigeminal neuralgia suggestive of possible brainstem dysfunction. Histologically, prominent necrosis and neutrophilic infiltration of spinal cord tissue without eosinophils or hyalinized vessels were observed, and oligoclonal bands were absent in their cerebrospinal fluid. It is likely to be a distinct idiopathic inflammatory demyelinating disorder restricted to the spinal cord and brainstem different from MS, but within the spectrum of NMO with probably an autoimmune basis.
Insights
Recurrent transverse myelitis can occur in multiple sclerosis (MS) and neuromyelitis optica (NMO). This study identifies a distinct spinal cord and brainstem inflammatory disorder, potentially within the neuromyelitis optica spectrum.
Area of Science:
- Neuroimmunology
- Neurology
- Demyelinating Diseases
Background:
- Recurrent idiopathic transverse myelitis is observed in multiple sclerosis (MS) and neuromyelitis optica (NMO).
- Neuromyelitis optica (NMO) is characterized by acute optic neuritis and myelitis, distinct from MS, often without other central nervous system (CNS) involvement.
- Recent evidence supports NMO as a separate entity from MS.
Observation:
- Two patients presented with severe, recurrent transverse myelitis that spared optic nerves and cerebral hemispheres.
- Both patients experienced longitudinally extensive myelitis with poor outcomes despite immunomodulatory therapy.
- One patient exhibited brainstem injury signs, while the other had trigeminal neuralgia, suggesting brainstem dysfunction.
Findings:
- Histological examination revealed spinal cord necrosis and neutrophilic infiltration, without eosinophils or hyalinized vessels.
- Cerebrospinal fluid analysis showed an absence of oligoclonal bands.
- The condition appears to be a distinct idiopathic inflammatory demyelinating disorder affecting the spinal cord and brainstem.
Implications:
- This disorder differs from MS but may fall within the spectrum of NMO.
- The findings suggest a potential autoimmune basis for this distinct neurological condition.
- Further research is warranted to understand the specific pathophysiology and treatment strategies for this NMO-spectrum disorder.
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