Article
Paraspinal and limb motor neuron involvement within homologous spinal segments in ALS.
Department of Neurology, St. Maria Hospital, Institute of Molecular Medicine, Faculty of Medicine, Lisbon, Portugal.
Clinical Neurophysiology (impact factor:
3.41).
08/2008;
119(7):1607-13.
DOI:10.1016/j.clinph.2008.03.014
pp.1607-13
Source: PubMed
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Citations (0)
- Cited In (1)
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Article: Can regional spreading of amyotrophic lateral sclerosis motor symptoms be explained by prion-like propagation?
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ABSTRACT: Progressive accumulation of specific misfolded protein is a defining feature of amyotrophic lateral sclerosis (ALS), similarly seen in Alzheimer disease, Parkinson disease, Huntington disease and Creutzfeldt-Jakob disease. The intercellular transfer of inclusions made of tau, α-synuclein and huntingtin has been demonstrated, revealing the existence of mechanisms reminiscent of those by which prions spread through the nervous system. Evidence for such a prion-like propagation mechanism has now spread to the major misfolded proteins, superoxide dismutase 1 (SOD1) and the 43 kDa transactive response DNA binding protein (TDP-43), implicated in ALS. The focus in this review is on what is known about ALS progression in terms of clinical as well as molecular aspects. Furthermore, the concept of 'propagation' is dissected into contiguous and non-contiguous types, and this concept is expanded to the severity of the focal symptom as well as its regional spread which can be explained by cell to cell propagation in the local neuron pool.Journal of neurology, neurosurgery, and psychiatry 04/2012; 83(7):739-45. · 4.87 Impact Factor
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Keywords
11 patients
32 patients
46 non-neurogenic control subjects
abnormal motor unit potentials
affected muscle
amyotrophic lateral sclerosis
control subjects
distally predominant fibrillations
electrophysiological diagnosis
limb muscle innervated
motor neurons
ongoing denervation
paraspinal muscle
paraspinal muscle EMG
paraspinal muscles
peripheral neuropathy paraspinal muscles
quantitative concentric needle EMG
reinnervated distal axons
similar abnormalities
study denervation