+ Resolve Article
+ Follow Us
Follow on FacebookFollow on Facebook
Follow on TwitterFollow on Twitter

+ Translate
+ Subscribe to Site Feed
GeoScience Most Shared ContentMost Shared Content

Chronic distal spinal amyotrophy or spinal forms of charcot marie disease a report on 6 sporadic adult cases

, : Chronic distal spinal amyotrophy or spinal forms of charcot marie disease a report on 6 sporadic adult cases. Revue Neurologique (Paris) 135(11): 815-826

Based on a personal series of 6 cases, and about 20 cases reported in the published literature, the principal characteristics of chronic distal amyotrophy of spinal origin are described: early onset or in young adults, initial disorder in lower limbs, especially anterolateral region of the legs, with secondary lesions in small muscles of the hands, absence of sensory disturbance, usually very slow progression, electrophysiological appearance of chronic denervation with normal conduction velocities and neurogenic muscle lesions with unaffected peripheral nerve. These forms can be compared to the spinal forms of Charcot-Marie-Tooth disease and appear to be degenerative in origin even though proof by autopsy is lacking. In spite of their common characteristics their genetic heterogenicity has to be noted; sporadic cases are seen most frequantly but some cases are related to recessive autosomal transmission and others to dominant autosomal transmission. From the clinical point of view, some cases are distinctive in that there is early or predominent atrophy in the lower limbs, while others have lesions in muscles of bulbar innervation, which is against their origin from spinal amyotrophy. Electrical signs at a distance from the atrophied muscles suggest extension of the pathological process. The clinical concept of the distal form of spinal amyotrophy should be retained together with the proximal and scapuloperoneal forms.


Other references

Spector, M., 2016: Decellularized tissues and organs: an historical perspective and prospects for the future. Biomedical Materials 11(2): 020201-020201

Carlton, B.E.; Tufts, E.; Girard, D.E., 1979: Water hemlock poisoning complicated by rhabdomyolysis and renal failure. A case of water hemlock poisoning associated with rhabdomyolysis and acute renal failure is reported. Water hemlock and its toxin along with the epidemiology and clinical features of Cicuta poisoning are discussed.

Goger, B.; Halden, Y.; Rek, A., 2002: Different affinities of glycosaminoglycan oligosaccharides for monomeric and dimeric interleukin-8: a model for chemokine regulation at inflammatory sites. The binding of interleukin-8 (IL-8) to heparan sulfate (HS) proteoglycans on the surface of endothelial cells is crucial for the recruitment of neutrophils to an inflammatory site. Fluorescence anisotropy measurements yielded an IL-8 dimerization...

Mora-Cristancho, J.A.; Arévalo-Ferro, C.; Ramos, F.A.; Tello, E.; Duque, C.; Lhullier, C.; Falkenberg, M.; Schenkel, E.Paulo., 2012: Antifouling activities against colonizer marine bacteria of extracts from marine invertebrates collected in the Colombian Caribbean Sea and on the Brazilian coast (Santa Catarina). The growth inhibition of 12 native marine bacteria isolated from Aplysina sponge surfaces, the shell of a bivalve, and Phytagel immersed for 48 h in sea water were used as indicator of the antifouling activity of the extracts of 39 marine organism...

Warren, R., 1906: White Wagtails in Co. Mayo. Zoologist London, 10 275

Bessa S.M.; Helmy I.; Mekky F.; Hamada S.; Sadek A.M., 1979: The patterns of acute abdomen seen in the university of alexandria hospital emergency department egypt. This prospective study of the different patterns of acute abdomen covered a 6 mo. period from Feb. to July 1977, in the emergency department of Alexandria University Hospital. Patients (1660) with the diagnosis of acute abdomen were studied. Acute...

Gilfillan, I.M.; Stevenson, J.A.; Holmden, E.; Ferreira, C.J.; Lee, A., 1980: Gibberellic acid for reducing creasing in navels in the Eastern Cape. In 2-year trials on the control of creasing in Navel oranges, the effects were compared of GA at 5 or 10 p.p.m. and urea phosphate at 0.5 or 1.0%, each applied either alone or together in various combinations at different times, and potassium nitr...

S.D.ngKai; Zhou YongBin; Zhou DaPeng; Zhang HongWei; Jin FuYu; Cao LiKe; Tang QingHua, 2005: Study on photosynthesis physioecological characteristics of hybrid popular clones under drought stress. A study was conducted to determine the net photosynthetic rate and transpiration rate and the diurnal course of Populus pseudosimonii and Populus x euramericana [P. canadensis] (W13) with Li-6400 P under drought stress. The result indicated that o...

Hoffmann, I.; de Molina, P.Malo.; Farago, B.; Falus, P.; Herfurth, C.; Laschewsky, Aé.; Gradzielski, M., 2015: Dynamics of microemulsions bridged with hydrophobically end-capped star polymers studied by neutron spin-echo. The mesoscopic dynamical properties of oil-in-water microemulsions (MEs) bridged with telechelic polymers of different number of arms and with different lengths of hydrophobic stickers were studied with neutron spin-echo (NSE) probing the dynamics...

Shen, J.; Watanabe, S.I.; Kaneko, A., 1994: Papain treatment in cell dissociation decreases the density of active cyclic GMP-activated channels in the rod outer segment membrane. Japanese Journal of Physiology 44(SUPPL 1): S207