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• Is myelitis associated with Sjogren's syndrome a neuromyelitis optica spectrum disorder?
  Masahiro Mori
  Published on: 13 April 2016
• Acute myelitis or anterior spinal artery occlusion?
  Nicola Latronico
  Published on: 13 April 2016

• Published on: 13 April 2016

  Is myelitis associated with Sjogren's syndrome a neuromyelitis optica spectrum disorder?

  • Masahiro Mori, Doctor
  • Other Contributors:
    • Shoichi Ito, Satoshi Kuwabara

  Dear Editor,

  In 2005, our case report entitled 'Acute longitudinal myelitis as the initial manifestation of Sjogren's syndrome' was published in this journal (1). In that report, we described the case of a 31-year-old woman who presented with acute longitudinal myelitis extending along the entire spinal cord. She was also diagnosed with Sjogren's syndrome on the basis of positive anti-SS-A antibody test results a...

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  Dear Editor,

  In 2005, our case report entitled 'Acute longitudinal myelitis as the initial manifestation of Sjogren's syndrome' was published in this journal (1). In that report, we described the case of a 31-year-old woman who presented with acute longitudinal myelitis extending along the entire spinal cord. She was also diagnosed with Sjogren's syndrome on the basis of positive anti-SS-A antibody test results and findings of lip biopsy. We concluded that this condition was myelitis associated with Sjogren's syndrome, considering that it was unlikely to be Devic's syndrome or neuromyelitis optica (NMO), because of the absence of optic nerve lesions, and on the basis of the conventional diagnostic criteria at that time. After this paper was published, we received an e-mail from Dr. Weinshenker who recommended that the patient be tested for the presence of NMO-IgG. Hence, we collected her serum sample on 22 July 2004, and sent it for NMO- IgG testing to the Neuroimmunology Laboratory, Mayo Clinic; however, the results of the NMO-IgG test were negative. Thereafter, the patient further experienced 4 episodes of myelitis but none of optic neuritis. Her brain magnetic resonance (MR) image obtained during the first episode was normal, but that obtained during the second episode showed periventricular lesions with gadolinium enhancement. Recently, we developed an enzyme-linked immunosorbent assay (ELISA) for detecting anti-aquaporin-4 antibody (2), and we measured this antibody in the same serum sample that was used for testing NMO-IgG; the results of the ELISA were strongly positive. Moreover, the extended disease entity 'NMO spectrum disorder' was proposed by Wingerchuk et al. in 2007 (3), including idiopathic single or recurrent episodes of longitudinally extensive myelitis. Eventually, we revised the diagnosis of 'myelitis associated with Sjogren's syndrome' as 'neuromyelitis optica spectrum disorder'. This was supported by the fact that the serum sample that had been negative for NMO-IgG in the original test at the Neuroimmunology Laboratory was found to be positive for NMO-IgG/anti-aquaporin-4 antibody in a retest using their novel and improved assays by Dr. Pittock. There are many reports concerning the involvement of the central nervous system (CNS), including myelitis associated with Sjogren's syndrome (4). However, the association between CNS involvement, especially longitudinally extensive myelitis, and Sjogren's syndrome remains controversial. Firstly, most of the reports were published before the discovery of NMO-IgG or anti-aquaporin-4 antibody. Secondly, NMO-IgG or anti-aquaporin-4 antibody test results should be carefully interpreted. Many techniques were used for the detection of these antibodies, but the sensitivities of these techniques were thought to differ. Further, sequential analysis of anti-aquaporin-4 antibody titres in the same patient revealed dynamic changes in the antibody levels related to the disease activity and treatments. A negative result in 1 test does not exclude the possibility of a positive result in another test. Lastly, it has been known that not a few NMO patients concurrently present with anti- SS-A antibody in their serum or Sjogren's syndrome but without any disease activity of Sjogren's syndrome. Moreover, it has been reported that NMO- IgG is detected only in patients with NMO spectrum disorder, but not in Sjogren's syndrome patients who do not have optic neuritis or myelitis (5). From these points, it is necessary to carefully rule out NMO in order to evaluate CNS involvement associated with Sjogren's syndrome. Further investigation is needed to clarify whether longitudinal myelitis in patients with Sjogren's syndrome is a condition caused by Sjogren's syndrome or neuromyelitis optica spectrum disorder, and whether 'myelitis associated with Sjogren's syndrome' truly exists. P.S. We thank Dr. Weinshenker for his helpful advice, and Dr. Pittock for confirmation of the positivity of NMO-IgG/anti-aquaporin-4 antibody by novel and improved assays.

  References

  1. Yamamoto T, Ito S, Hattori T. Acute longitudinal myelitis as the initial manifestation of Sjogren's syndrome. J Neurol Neurosurg Psychiatry 2006;77:780.

  2. Hayakawa S, Mori M, Okuta A, et al. Neuromyelitis optica and anti- aquaporin-4 antibodies measured by an enzyme-linked immunosorbent assay. J Neuroimmunol 2008;196:181-7.

  3. Wingerchuk DM, Lennon VA, Lucchinetti CF, et al. The spectrum of neuromyelitis optica. Lancet Neurol 2007;6:805-15.

  4. Rabadi MH, Kundi S, Brett D, et al. Primary Sjogren syndrome presenting as neuromyelitis optica. J Neurol Neurosurg Psychiatry 2010;81:213-4.

  5. Pittock SJ, Lennon VA, de Seze J, et al. Neuromyelitis optica and non-organ-specific autoimmunity. Arch Neurol 2008;65:78-83.

  Conflict of Interest:

  None declared

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  Conflict of Interest:
  None declared.

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• Published on: 13 April 2016

  Acute myelitis or anterior spinal artery occlusion?

  • Nicola Latronico, MD

  Dear Editor,

  I read with interest the case report by Yamamoto and colleagues on acute onset paraplegia in a young woman, which was interpreted as a case of acute longitudinal myelitis (1). Sudden onset without any inflammatory prodromal symptom or sign is evocative of arterial occlusion, and the MRI may be compatible with anterior spinal artery occlusion (2). Although lancinating radicular pain was absent, I wond...

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  Dear Editor,

  I read with interest the case report by Yamamoto and colleagues on acute onset paraplegia in a young woman, which was interpreted as a case of acute longitudinal myelitis (1). Sudden onset without any inflammatory prodromal symptom or sign is evocative of arterial occlusion, and the MRI may be compatible with anterior spinal artery occlusion (2). Although lancinating radicular pain was absent, I wonder whether the authors investigated this diagnosis, as prompt intravenous heparin treatment can be resolutive in such cases. Procoagulant states in young women are not rare, pregnancy and the postpartum, the contraceptive pill may play a role.

  References

  1)Yamamoto T, Ito S, Hattori T. Acute longitudinal myelitis as the initial manifestation of Sjo�gren�s syndrome. J Neurol Neurosurg Psychiatry 2006;77:780.

  2) Latronico N, Fassini P, Antonini B, Gasparotti R. A pain in the neck. Lancet 2002; 359: 1206.

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  Conflict of Interest:
  None declared.

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