TY - JOUR
T1 - The heterogeneity of amyotrophic lateral sclerosis
T2 - A possible explanation of treatment failure
AU - Beghi, Ettore
AU - Mennini, Tiziana
AU - Bendotti, Caterina
AU - Bigini, Paolo
AU - Logroscino, Giancarlo
AU - Chiò, Adriano
AU - Hardiman, Orla
AU - Mitchell, Douglas
AU - Swingler, Robert
AU - Traynor, Bryan J.
AU - Al-Chalabi, Ammar
PY - 2007/12
Y1 - 2007/12
N2 - Amyotrophic lateral sclerosis (ALS) is a severe clinical condition characterized by upper and lower motor neuron degeneration for which there is no truly effective treatment. The absence of an effective treatment can be explained in part by the complex and heterogeneous genetic, biochemical, and clinical features of ALS. While ALS accounts for the majority of the motor neuron diseases, the recognition of disease variants and mimic syndromes may lead to further insights into possible causes for the generality of ALS. From a biochemical perspective, the process of motor neuron degeneration is complex and the multifactorial influences and potential biomarkers of ALS have never been assessed in the light of the clinical heterogeneity of ALS. Several genes and environmental influences have been suggested as possible risk factors of ALS. A better understanding of interactions between these risk factors, potential biomarkers and heterogeneous clinical features may lead to more clearly defined pathological profiles among individuals or groups of ALS patients and in turn lead to more focused therapeutic trials.
AB - Amyotrophic lateral sclerosis (ALS) is a severe clinical condition characterized by upper and lower motor neuron degeneration for which there is no truly effective treatment. The absence of an effective treatment can be explained in part by the complex and heterogeneous genetic, biochemical, and clinical features of ALS. While ALS accounts for the majority of the motor neuron diseases, the recognition of disease variants and mimic syndromes may lead to further insights into possible causes for the generality of ALS. From a biochemical perspective, the process of motor neuron degeneration is complex and the multifactorial influences and potential biomarkers of ALS have never been assessed in the light of the clinical heterogeneity of ALS. Several genes and environmental influences have been suggested as possible risk factors of ALS. A better understanding of interactions between these risk factors, potential biomarkers and heterogeneous clinical features may lead to more clearly defined pathological profiles among individuals or groups of ALS patients and in turn lead to more focused therapeutic trials.
KW - Amyotrophic lateral sclerosis
KW - Biochemistry
KW - Epidemiology
KW - Genetics
KW - Heterogeneity
KW - Motor neuron disease
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U2 - 10.2174/092986707782793862
DO - 10.2174/092986707782793862
M3 - Article
C2 - 18220753
AN - SCOPUS:37549000493
VL - 14
SP - 3185
EP - 3200
JO - Current Medicinal Chemistry
JF - Current Medicinal Chemistry
SN - 0929-8673
IS - 30
ER -