[Skin changes in amyotrophic lateral sclerosis].
It has been repeatedly noted, but never as yet fully explained, that patients with amyotrophic lateral sclerosis (ALS) do not develop bedsores even at the terminal stage. Furthermore, the skin of ALS patients feels supple, like tanned leather, and loses elasticity. When the skin is stretched, it returns only sluggishly to its original position. We termed this property of skin "delayed return phenomenon (DRP)"; it is usually seen more than 2.5 years after the onset of symptoms. Although it is thought that a phenomena such as DRP and the absence of bedsores are characteristic of this disease, little attention has been paid to these unique features in ALS patients. In this review we summarize recent developments in research on skin from ALS patients. From our own works cited in this review it is clear that not only the motor neuron but also the skin is affected in ALS, and that abnormalities of collagen, glycosaminoglycans, vascular endotherial growth factor (VEGF) and neurotrophic factors like ciliary neurotrophic factor (CNTF), neurotrophin-3 (NT-3) and insulin-like growth factor-1 (IGF-1) do occur in the skin of ALS. Examination of the skin in patients with ALS would be easy to carry out as an additional examination. Further analysis of the complex skin abnormalities will be useful in elucidating the basic pathological mechanism of ALS.
Ono
Seiitsu
S
Department of Neurology, Teikyo University Chiba Medical Center, 3426-3 Anegasaki, Ichihara-shi, Chiba 299-0111, Japan.
D000690:Amyotrophic Lateral Sclerosis / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D020934:Ciliary Neurotrophic Factor / Q000378:metabolism / Q000502:physiology; D024022:Fibrillar Collagens / Q000378:metabolism / Q000502:physiology; D006025:Glycosaminoglycans / Q000378:metabolism / Q000502:physiology; D006801:Humans; D007150:Immunohistochemistry; D007334:Insulin-Like Growth Factor I / Q000378:metabolism / Q000502:physiology; D020933:Neurotrophin 3 / Q000378:metabolism / Q000502:physiology; D012867:Skin / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D042461:Vascular Endothelial Growth Factor A / Q000378:metabolism / Q000502:physiology
D020934:Ciliary Neurotrophic Factor; D024022:Fibrillar Collagens; D006025:Glycosaminoglycans; D020933:Neurotrophin 3; D042461:Vascular Endothelial Growth Factor A; D007334:Insulin-Like Growth Factor I