Epidemiology and Clinical Description of Amyotrophic Lateral Sclerosis in Low Income Setting: A Syndrome with Short Survival

Abstract

Abstract:Background: Amyotrophic lateral sclerosis (ALS) is a degenerative motor neuron disease. It evolves to loss of autonomy and death. Objective: To describe the cases of ALS clinically definite observed in hospital field. Patients and methods: The retrospective study has covered a period of 10 years (2003)(2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012)) and a total of 10,128 patient's files were analyzed. The neurology department of our level 3 hospital has located the current study. Six patients with no particular medical history have been reported. The inclusion patients are cases where the diagnosis of ALS was clinically definite according to the modified El Escorial classification. About 978 of likely, possible and probable cases of ALS were not included. Results: The mean age was 49 years (24 and 67), all males, with a frequency of 0.59 ‰ and an incidence rate of 0.6 cases per year. The clinical signs were marked by the pyramidal syndrome and peripheral neuropathic motor syndrome. MRI or CT scan signs were marked by the bulbar light atrophy. EMG had shown spontaneous activities with reinnervation signs. The histology exam has found neurogenic fascicular atrophy. The average duration of progression of the disease from the diagnostic to death was 17.6 months (6 to 36). The median of survival all cases combined was 42 weeks. Symptomatic treatment was associated to rehabilitation. Riluzole has been established despite its high cost, but without success. Conclusion: This observation highlighted the major difficulties encountered in the management of ALS and its increasing frequency in south Saharan Africa.