What is ALS?
Amyotrophic Lateral Sclerosis, also known as Lou Gehrig’s disease in the United States and Charcot’s disease in Europe, is the most common motor neuron disease (MND). It is a progressive degenerative disease involving both the upper and lower motor neurons of the brain and the spinal cord in adults (Yorkston, 2010). Diagnosis of ALS is based on a clinical profile and electrophysiologic confirmation. Clinical criteria for diagnosing ALS must include both UMN and LMN involvement and affect two or more body regions (body extremities, bulbar, or respiratory). Additionally, electromyographic findings of fibrillations help to establish the presence of LMN disease (Duffy, 2013). Other varieties of MND include Spinal Muscle Atrophy (SMA), Progressive Bulbar Palsy (PBP), and Primary Lateral Sclerosis (PLS).
ALS is associated with a number of common problems including respiratory, cognitive and language function, swallowing, and speech. Weakening of the respiratory muscles results in a number of complications including respiratory muscle fatigue, respiratory failure, ineffective cough, and risks of aspiration. Making decisions about ventilation should also include a discussion about the decline of speech and the use of augmentative communication. Some patients may demonstrate cognitive problems that interfere with communication and the learning of new communication techniques (Yorkston, 2010). In the early stages, patients may exhibit mild cognitive impairments and progress to Frontotemporal Lobar Dementia (FTLD) in the later stages. The incidence of FTLD is higher in patients with bulbar onset. Patients with ALS experience difficulties with maintaining adequate nutrition, saliva management, and respiratory interruption during swallowing. Additionally, since ALS is a mixed UMN and LMN disease that often affects bulbar muscles, it is frequently associated with mixed spastic-flaccid dysarthria (Duffy, 2013).
The incidence of ALS is about 1 to 5 per 100, 000 population, affecting more men than women (Duffy, 2013). ALS usually occurs sporadically in 90-95% of cases, but about 5-10% are caused by a genetic or familial link. Although 70-80% of patients have onset in limbs (corticospinal tract), about 20-30% of patients have initial onset in the bulbar muscles (corticobulbar tract), which affects the speech and swallowing structures. The onset of the disease usually occurs during the fifth or sixth decade with an average survival rate of 3-5 years. Patients with limb onset tend to live longer and have a better prognosis than those with bulbar presentation, whose respiratory problems result in a rapid course.
References:
Duffy, J. R. (2013). Motor Speech Disorders . St. Louis: Elsevier Mosby.
Yorkston, K. M., Beukelman, D. R., Strand, E. A., & Hakel, S. (2010). Management of motor speech disorders in children and adults. Austin: Pro-Ed.
ALS is associated with a number of common problems including respiratory, cognitive and language function, swallowing, and speech. Weakening of the respiratory muscles results in a number of complications including respiratory muscle fatigue, respiratory failure, ineffective cough, and risks of aspiration. Making decisions about ventilation should also include a discussion about the decline of speech and the use of augmentative communication. Some patients may demonstrate cognitive problems that interfere with communication and the learning of new communication techniques (Yorkston, 2010). In the early stages, patients may exhibit mild cognitive impairments and progress to Frontotemporal Lobar Dementia (FTLD) in the later stages. The incidence of FTLD is higher in patients with bulbar onset. Patients with ALS experience difficulties with maintaining adequate nutrition, saliva management, and respiratory interruption during swallowing. Additionally, since ALS is a mixed UMN and LMN disease that often affects bulbar muscles, it is frequently associated with mixed spastic-flaccid dysarthria (Duffy, 2013).
The incidence of ALS is about 1 to 5 per 100, 000 population, affecting more men than women (Duffy, 2013). ALS usually occurs sporadically in 90-95% of cases, but about 5-10% are caused by a genetic or familial link. Although 70-80% of patients have onset in limbs (corticospinal tract), about 20-30% of patients have initial onset in the bulbar muscles (corticobulbar tract), which affects the speech and swallowing structures. The onset of the disease usually occurs during the fifth or sixth decade with an average survival rate of 3-5 years. Patients with limb onset tend to live longer and have a better prognosis than those with bulbar presentation, whose respiratory problems result in a rapid course.
References:
Duffy, J. R. (2013). Motor Speech Disorders . St. Louis: Elsevier Mosby.
Yorkston, K. M., Beukelman, D. R., Strand, E. A., & Hakel, S. (2010). Management of motor speech disorders in children and adults. Austin: Pro-Ed.