A case of neuromyelitis optica spectrum disorder associated with a limited cutaneous systemic sclerosis and Sjögren syndrome

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  • Iwanaga Yasutaka
    Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University
  • Hayashi Shintaro
    Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University
  • Kawamura Nobutoshi
    Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University
  • Ohyagi Yasumasa
    Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University
  • Kira Jun-ichi
    Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University

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Other Title
  • 限局皮膚硬化型全身性強皮症とシェーグレン症候群にneuromyelitis optica spectrum disorderを合併した1例
  • 症例報告 限局皮膚硬化型全身性強皮症とシェーグレン症候群にneuromyelitis optica spectrum disorderを合併した1例
  • ショウレイ ホウコク ゲンキョク ヒフ コウカガタ ゼンシンセイ キョウ ヒショウ ト シェーグレン ショウコウグン ニ neuromyelitis optica spectrum disorder オ ガッペイ シタ 1レイ

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Abstract

A 51-year-old woman was referred to our department for a precise examination of her neuromyelitis optica spectrum disorder (NMOSD) symptoms. She had recurrent attacks of consciousness disturbance, cerebellar ataxia and diplopia (10 years ago), paraparesis and dysesthesia in four limbs (7 years ago), and consciousness disturbance and paraparesis (4 years ago). Neurological examination disclosed bilateral temporal pallor of the optic disc, atrophy and fasciculation of the right side of the tongue, dysesthesia in four limbs, mild motor weakness of both lower limbs, hyperreflexia in the right leg, pathological reflexes in bilateral lower limbs, and spastic bladder. T2-weighted cranial MRI showed lesions in the bilateral hypothalami and the dorsal portion of the medulla oblongata on the right side. T2-weighted spinal MRI revealed longitudinally extensive spinal cord lesions at T2–T8. A visual-evoked potential study disclosed prolonged latency of P100 bilaterally. During the examination, slight skin changes on the lower extremities indicative of scleroderma were observed, with no evidence of organ involvement. Skin biopsy showed increased numbers and swelling of collagen fibers. Thus, the patient was diagnosed with limited cutaneous systemic sclerosis (lcSSc). She also clinically manifested Sjögren syndrome. Her serum was positive for anti-nuclear, anti-centromere, and anti-aquaporin-4 antibodies. Following the administration of corticosteroids (25 mg/alternative day) the patient became stable. A variety of collagen diseases or autoimmune disorders have been reported to be major complications of NMOSD; however, the coexistence of lcSSc and NMOSD is extremely rare. To the best of our knowledge, this is the first description of a case with the coexistence of both conditions. Physicians should be aware of scleroderma in patients with NMOSD, even if patients do not complain of skin symptoms.

Journal

  • Rinsho Shinkeigaku

    Rinsho Shinkeigaku 53 (9), 695-700, 2013

    Societas Neurologica Japonica

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