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Longitudinally extensive myelopathy in Caucasians: a West Australian study of 26 cases from the Perth Demyelinating Diseases Database
  1. W Qiu1,
  2. J-S Wu1,
  3. M-N Zhang2,
  4. T Matsushita3,
  5. J-i Kira3,
  6. W M Carroll1,
  7. F L Mastaglia1,
  8. A G Kermode1
  1. 1Centre for Neuromuscular and Neurological disorders, University of Western Australia, and Department of Neurology, Sir Charles Gairdner Hospital, Queen Elizabeth II Medical Centre, Perth, Western Australia
  2. 2First Hospital of Shanxi Medical University, Taiyuan, PR China
  3. 3Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
  1. Correspondence to Professor A G Kermode, Australian Neuromuscular Research Institute, Sir Charles Gairdner Hospital; Centre for Neuromuscular and Neurological Disorders, University of Western Australia, Queen Elizabeth II Medical Centre, 6009, Perth, WA, Australia; kermode{at}me.com

Abstract

Objectives To characterise West Australian cases of longitudinally extensive myelopathy (LEM).

Methods Twenty six patients with LEM were identified from a cohort of 983 patients with demyelinating disease. Clinical and MRI data and AQP4-IgG results were reviewed.

Results LEM cases were classified as conventional MS (CMS) 13, neuromyelitis optica (NMO) 7, and isolated LEM 6. LEM was the initial presentation in 13/26 cases. In CMS cases lesions were mainly in the lower cervical cord (C4–C7) whereas in NMO and isolated LEM they were more often thoracic and were longer. The severity of disability was highly variable but was greater in the NMO than the CMS group. Only one of 20 patients tested was seropositive for AQP4-IgG.

Conclusion LEM occurred as part of CMS or NMO or in isolation. Patients with LEM had highly heterogeneous clinical characteristics and a low rate of AQP4-IgG seropositivity.

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Footnotes

  • WQ and J-SW contributed equally to this manuscript

  • Funding W Qiu was supported by Endeavour International Postgraduate Research Scholarships (EIPRS) of Australia and Postgraduate Award from University of Western Australia. J-S Wu was supported by Medical Postgraduate Scholarship from Multiple Sclerosis Research Australia and the National Health & Medical Research Council of Australia.

  • Competing interests None.

  • Ethics approval Ethics approval was provided by University of Western Australia.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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