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Brain Nerve. 2007 Oct;59(10):1099-107.

[Skin changes in amyotrophic lateral sclerosis].

http://www.ncbi.nlm.nih.gov/pubmed/17969350

[Article in Japanese]

Abstract

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.

 

 

Ann Neurol. 1992 Mar;31(3):305-10.

Collagen cross-linking of skin in patients with amyotrophic lateral sclerosis.

http://www.ncbi.nlm.nih.gov/pubmed/1637138

Abstract

Collagen cross-links of skin tissue (left upper arm) from 11 patients with amyotrophic lateral sclerosis (ALS) and 9 age-matched control subjects were quantified. It was found that patients with ALS had a significant reduction in the content of an age-related, stable cross-link, histidinohydroxylysinonorleucine, that was negatively correlated with the duration of illness. The contents of sodium borohydride-reducible labile cross-links, dehydro-hydroxylysinonorleucine and dehydro-histidinohydroxymerodesmosine, were significantly increased and were positively associated with the duration of illness (r = 0.703, p less than 0.05 and r = 0.684, p less than 0.05, respectively). The results clearly indicate that during the course of ALS, the cross-linking pathway of skin collagen runs counter to its normal aging, resulting in a "rejuvenation" phenomenon of skin collagen. Thus, cross-linking of skin collagen is affected in ALS.

 

Skin involvement in amyotrophic lateral sclerosis
May 4, 1996
http://www.thelancet.com/journals/lancet/article/PIIS0140-6736(96)90737-0/abstract
Summary
Background Patients with sporadic amyotrophic lateral sclerosis (ALS) show disorganised collagen and elastin of the dermis. We looked for inflammatory alterations to cutaneous blood vessels.
Patients and findings Seven patients with sporadic ALS were investigated; five were confined to bed, but none had bedsores. Light and electron microscopy of skin showed an oedematous dermis with collagen fibrils of irregular diameter. Small blood vessels were characterised by duplicated basement membranes and deposition of β-amyloid protein, the main component of the neuronal and non-neuronal amyloid deposits in Alzheimer's disease. These skin changes were seen in all degrees of disability, but none was found in age-matched and sex-matched controls.
Interpretation The skin in ALS is characterised by a distinctive pattern of alterations of connective tissue and blood vessels. Examination of skin is an additional and easily accessible investigation which may help elucidate the pathogenesis of ALS.
a Department of Dermatology, Virchow-Clinics, Humboldt-University of Berlin, D-13353, Berlin, Germany

 Abundant FUS-immunoreactive pathology in the skin of sporadic amyotrophic lateral sclerosis

Mar 1, 2013

http://onlinelibrary.wiley.com/doi/10.1111/ane.12114/abstract

Objectives

The fused in sarcoma (FUS) protein is a 526 amino acid and its expression is ubiquitous. Recently, mutations in a gene coding FUS have been identified in familial amyotrophic lateral sclerosis (ALS). Also, FUS has been found in neuronal cytoplasmic inclusions in sporadic forms of ALS, suggesting that FUS has an important role in the neurodegeneration occurring in sporadic disease. However, there has been no study of FUS in ALS skin.

Material and methods

We made a quantitative immunohistochemical study of the expression of FUS in the skin from patients with sporadic ALS and controls.

Results

The proportion of FUS-immunoreactive (ir) cells in the epidermis in ALS patients was significantly higher (< 0.001) than in controls. There was a significant positive relationship (r = 0.78, < 0.001) between the proportion and duration of illness in ALS patients. The optical density of FUS-ir cells in the epidermis in ALS patients is markedly stronger (P < 0.001) than in controls. There was a significant positive relation (r = 0.49, P < 0.05) between the immunoreactivity and duration of illness in ALS patients.

Conclusions

These data suggest that changes of FUS in ALS skin are related to the disease process, and that metabolic alterations of FUS may take place in the skin of patients with ALS.