01/28/2011 - Atualizado em 07/02/2013
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterised by progressive muscular paralysis reflecting degeneration of motor neurones in the primary motor cortex,corticospinal tracts, brainstem and spinal cord.
The mean age of onset for sporadic ALS is about 60 years. Overall, there is a slight male prevalence (M:F ratio~1.5:1). Approximately two thirds of patients with typical ALS have a spinal form of the disease (limb onset) and present with symptoms related to focal muscle weakness and wasting, where the symptoms may start either distally or proximally in the upper and lower limbs.
Gradually, spasticity may develop in the weakened atrophic limbs, affecting manual dexterity and gait. Patients with bulbar onset ALS usually present with dysarthria and dysphagia for solid or liquids, and limbs symptoms can develop almost simultaneously with bulbar symptoms, and in the vast majority of cases will occur within 1?2 years.
Paralysis is progressive and leads to death due to respiratory failure within 2?3 years for bulbar onset cases and 3/5 years for limb onset ALS cases. The diagnosis is based on clinical history, examination, electromyography, and exclusion of 'ALS-mimics' by appropriate investigations. The management of ALS is supportive, palliative, and multidisciplinary. Non-invasive ventilation prolongs survival and improves quality of life. Riluzole is the only drug that has been shown to extend survival and improves quality of life. Riluzole is the only drug that has been shown to extend survival.
Autors: Wijesekara, Lokesh; Leigh, P Nigel.
Orphanet Journal of rare diseases 2009 4:3.