[Recurrent acute rhombencephalomyelitis in an adult or neuromyelitis optica? Presentation of a case]

Neurologia. 2012 Apr;27(3):154-60. doi: 10.1016/j.nrl.2011.04.006. Epub 2011 Jun 8.
[Article in Spanish]

Abstract

Introduction: The lack of accepted homogeneous criteria for the definition of some demyelinating diseases makes diagnostic characterization difficult and limits data interpretation and therapeutic recommendations. Recurrent encephalomyelitis (ADE-R) along with borderline cases of neuromyelitis optica (NMO) are especially controversial.

Objective: To describe the clinical and radiological evolution of an adult-onset ADE-R versus NMO case throughout 9 years of follow-up.

Patient and methods: Our patient presented with severe symptoms of rhombencephalomyelitis and the cranial and spinal magnetic resonance imaging (MRI) showed large lesions, with gadolinium enhancement in brainstem and spinal cord, correlating with the clinical picture. Infectious aetiology was excluded, IgG index was normal and NMO antibodies were negative. After treatment with intravenous corticosteroids and plasmapheresis, there was excellent recovery in the acute phase. During follow-up, seven relapses have occurred, mainly in the spinal cord, with good recovery and the same symptomatology, albeit with different severity. Immunosuppressive treatment was introduced since the beginning.

Conclusions: Our case shares common features of both ADE-R and NMO, illustrating that diagnostic characterization is not easy in spite of current criteria.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Azathioprine / therapeutic use
  • Brain Stem / pathology
  • Corticosterone / therapeutic use
  • Encephalitis / diagnosis*
  • Encephalitis / pathology
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunosuppressive Agents / therapeutic use
  • Magnetic Resonance Imaging
  • Methylprednisolone / therapeutic use
  • Neuromyelitis Optica / diagnosis*
  • Neuromyelitis Optica / pathology
  • Plasmapheresis
  • Recurrence
  • Spinal Cord / pathology
  • Young Adult

Substances

  • Immunoglobulins, Intravenous
  • Immunosuppressive Agents
  • Azathioprine
  • Corticosterone
  • Methylprednisolone