Étude LENSLA : caractérisation des patients atteints de sclérose latérale amyotrophique évoluant depuis plus de 5 ans suivis au centre de référence de Bordeaux
Year: 2014
Type: dissertation
Study design: Observational Study
Abstract: Background: Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease which preferentially affects motor neurons, death occurring generally before 5 years of evolution. Long survivors are not that rare and few studies have described them. Objectives: Clinical and paraclinical description of ALS long survivors followed in the referral centre of Bordeaux. Evaluation of revised El Escorial and Awaji diagnostic criteria in this context. Methods: Retrospective and descriptive observational study, including all patients followed in the referral centre with a disease progression of five years or more. Results: 44 patients were included, with a clear male predominance (sex-ratio 2.7), a median age at diagnosis of 55 years and an increased diagnostic latency (median of 17 months). Three motor phenotypes were present: “classical” ALS (57%), lower motor neuron predominant (23%) and upper motor neuron predominant (20%). The cervical region (43%) and distal parts of the limbs (71%) were more frequently involved at diagnosis, with predominant signs of lower motor neuron. Bulbar symptoms were not less prevalent (52% at diagnosis), but unrelated to survival. Sensory abnormalities were present in 22% of the nerve conduction studies performed. The motor deficit propagation could not be explained by a contiguous propagation for 14% of the patients, but by a skipping pattern. Sensitivity of the revised El Escorial and Awaji criteria was 39% and 43% respectively. Conclusion: The evidence of phenotypic heterogeneity among ALS long survivors, the occurrence of sensory abnormalities and the diagnostic criteria insufficiency highlight the necessity to find biomarkers for ALS.
Source: HAL
Author: Cyrielle Coignion
Institution: Université de Bordeaux
Language: French
Subfield: Clinical Neurology
Field: Medicine
Domain: Health Sciences
Open Access status: green
OpenAlex ID: W1569467357