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KMID : 0359720140320010026
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Journal of the Korean Neurological Association
2014 Volume.32 No. 1 p.26 ~ p.29
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Chronic Inflammatory Demyelinating Polyneuropathy Combined With Limited Cutaneous Systemic Sclerosis
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Lee Eun-Kyoung
Yun Dong-Joo
Ko Young-Chai
Jang Sang-Hyun
Yoon Soo-Jin
Oh Gun-Sei
Lee Soo-Joo
Park Sung-Hye
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Abstract
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Chronic inflammatory demyelinating polyneuropathy (CIDP) has been rarely reported in systemic sclerosis (SSc). We describe the clinical, electrophysiological, and pathologic findings in a 73-year-old female with established limited cutaneous SSc who later developed CIDP. The patient had progressive limb weakness, sensory loss, and slow nerve conduction velocities. Sural nerve biopsy showed evidence of demyelination and remyelination. This case demonstrates that chronic demyelinating polyneuropathy can be an unusual manifestation of SSc, presumably resulting from an immune-mediated process.
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KEYWORD
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Chronic inflammatory demyelinating polyneuropathy, Systemic sclerosis, Autoimmunity
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