Neuromuscular involvement in nephrogenic systemic fibrosis.
BACKGROUND
Nephrogenic fibrosing dermopathy causes fibrotic skin changes in the setting of renal failure. Although this condition was initially felt to be limited to the skin, recent studies have described widespread involvement, and the term nephrogenic systemic fibrosis (NSF) has been suggested.


OBJECTIVE
To describe the clinical, electrophysiologic, and pathologic findings in three NSF patients with neuromuscular involvement.


METHODS
Case report.


RESULTS
Three patients developed progressive hardening of the skin and underlying fascia and muscles. Strength was difficult to assess because of limited joint mobility. electromyography/nerve conduction studies suggested involvement of both nerve and muscle. Muscle biopsy demonstrated primarily myopathic changes with variable fibrosis.


CONCLUSIONS
Neuromuscular involvement occurs in NSF and may be difficult to appreciate clinically because of joint and skin fibrosis.
Keyrouz
Salah
S
From the Department of Neurology, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USA. sarudnicki@uams.edu
Rudnicki
Stacy A
SA
D005260:Female; D005355:Fibrosis / Q000150:complications; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009468:Neuromuscular Diseases / Q000209:etiology*; D009469:Neuromuscular Junction / Q000473:pathology* / Q000503:physiopathology; D012871:Skin Diseases / Q000150:complications* / Q000473:pathology