Recurrent longitudinally extensive transversal myelitis in a patient with Sjögren’s syndrome

Jelena Šekarić; Željko Živanović; Aleksandra Lučić - Prokin; Timea Kokai - Zekić; Kosta Petrović; Ivan Turkalj; Zita Jovin; Slobodan Gvozdenović

doi:10.2298/1341

Jelena Šekarić Department of Neurology, Clinical Centre of Vojvodina, Novi Sad, Serbia
Željko Živanović Department of Neurology, Clinical Centre of Vojvodina, Novi Sad, Serbia
Aleksandra Lučić - Prokin Department of Neurology, Clinical Centre of Vojvodina, Novi Sad, Serbia
Timea Kokai - Zekić Department of Neurology, Clinical Centre of Vojvodina, Novi Sad, Serbia
Kosta Petrović Department of Radiology, Clinical Centre of Vojvodina, Novi Sad, Serbia
Ivan Turkalj Department of Radiology, Clinical Centre of Vojvodina, Novi Sad, Serbia
Zita Jovin Department of Neurology, Clinical Centre of Vojvodina, Novi Sad, Serbia
Slobodan Gvozdenović Department of Neurology, Clinical Centre of Vojvodina, Novi Sad, Serbia

DOI: https://doi.org/10.2298/1341

Keywords: myelitis, transverse, sjorgen’s syndrome, neuromyelitis optica, diagnosis, drug therapy, treatment outcome,

Abstract

Introduction. Longitudinally extensive transverse myelitis (LETM) is a transversal myelitis that extends through three or more vertebral segments in length. Case report. A 52-year-old woman was hospitalized due to pain in the lumbar region, difficulty in walking, hypoesthesia of the anogenital area and urinary retention. In the past medical history, two years earlier, the patient had been diagnosed with transversal myelitis confirmed by MRI of the cervical spine and six months earlier, the patient was diagnosed with primary Sjögren’s syndrome (SS). During the current hospitalization MRI of the spinal cord revealed extensive inflammatory lesions of almost the whole spinal cord. Lumbar puncture (LP) revealed mild pleocytosis and slightly increased protein level. Isoelectric focusing of cerebrospinal fluid (CSF) and serum proteins was normal. Visual evoked potentials were normal. Serological testing excluded acute viral infections. Corticosteroid therapy was applied with good therapeutic response. Control MRI revealed regression of pathological changes in the spinal cord. Conclusion. A wide range of disorders can cause LETM, but usually the first line diagnosis is neuromyelitis optica (NMO). Based on the detection of NMO immunoglobulin G in the serum of affected patients, a variety of allied disorders were grouped under the name of NMO spectrum disorders, including recurrent myelitis associated with LETM and myelitis associated with autoimmune disorders such as SS. There have been only a few cases reported in the literature with recurrent LETM associated with non-organ specific autoimmune disorder.

Author Biography

Jelena Šekarić, Department of Neurology, Clinical Centre of Vojvodina, Novi Sad, Serbia

Department of Neurology, Emergency Center of Clinical Center of Vojvodina, Novi Sad, Serbia,

M.D.

References

Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG. Revised diagnostic criteria for neuromyelitis optica. Neurology 2006; 66(10): 1485−9.

Kitley JL, Leite MI, George JS, Palace JA. The differential diagnosis of longitudinally extensive transverse myelitis. Mult Scler 2012; 18(3): 271−85.

Lennon VA, Wingerchuck DM, Kryzer TJ, Pittock SJ, Lucchinetti CF, Fujihara K, et al. A serum antibody marker of neuromyeli-tis optica: distinction from multiple sclerosis. Lancet 2004; 364(9451): 2106-2112.

Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Wein-shenker BG. The spectrum of neuromyelitis optica. Lancet Neurol 2007; 6(9): 805-815.

Weinshenker BG, Wingerchuk DM, Vukusic S, Linbo L, Pittock SJ, Lucchinetti CF, et al. Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol 2006; 59(3): 566−9.

Delalande S, de Seze J, Fauchais AL, Hachulla E, Stojkovic T, Ferriby D, et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore) 2004; 83(5): 280−91.

Pittock SJ, Lennon VA, de Seze J, Vermersch P, Homburger HA, Wingerchuk DM, et al. Neuromyelitis optica and non organ-specific autoimmunity. Arch Neurol 2008; 65(1): 78–83.

Yamamoto T, Ito S, Hattori T. Neurological picture. Acute longitudinal myelitis as the initial manifestation of Sjögren's syndrome. J Neurol Neurosurg Psychiatry 2006; 77(6): 780.

Lehnhardt FG, Impekoven P, Rubbert A, Burghaus L, Neveling M, Heiss WD, et al. Recurrent longitudinal myelitis as primary manifestation of SLE. Neurology 2004; 63(10): 1976.

Chen HC, Lai J H, Juan C J, Kuo SY, Chen CH, Chang DM. Longitudinal myelitis as an initial manifestation of systemic lupus erythematosus. Am J Med Sci 2004; 327(2): 105–8.