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

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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