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

Yasutaka Iwanaga, Shintaro Hayashi, Nobutoshi Kawamura, Yasumasa Ohyagi, Jun-Ichi Kira

Research output: Contribution to journalArticle

3 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)695-700
Number of pages6
JournalClinical Neurology
Volume53
Issue number9
DOIs
Publication statusPublished - Sep 1 2013

Fingerprint

Neuromyelitis Optica
Systemic Scleroderma
Skin
Paraparesis
Lower Extremity
Paresthesia
Consciousness
Extremities
Aquaporin 4
Fasciculation
Pallor
Optic Atrophy
Collagen Diseases
Abnormal Reflexes
Cerebellar Ataxia
Medulla Oblongata
Diplopia
Muscle Spasticity
Visual Evoked Potentials
Centromere

All Science Journal Classification (ASJC) codes

  • Clinical Neurology

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A case of neuromyelitis optica spectrum disorder associated with a limited cutaneous systemic sclerosis and Sjögren syndrome. / Iwanaga, Yasutaka; Hayashi, Shintaro; Kawamura, Nobutoshi; Ohyagi, Yasumasa; Kira, Jun-Ichi.

In: Clinical Neurology, Vol. 53, No. 9, 01.09.2013, p. 695-700.

Research output: Contribution to journalArticle

Iwanaga, Yasutaka ; Hayashi, Shintaro ; Kawamura, Nobutoshi ; Ohyagi, Yasumasa ; Kira, Jun-Ichi. / A case of neuromyelitis optica spectrum disorder associated with a limited cutaneous systemic sclerosis and Sjögren syndrome. In: Clinical Neurology. 2013 ; Vol. 53, No. 9. pp. 695-700.
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