What is the definition of Amyotrophic Lateral Sclerosis?
ALS, also known as Lou Gehrig’s Disease, is a degenerative disease that affects both UMN’s and LMN’s and is the most common type of motor neuron disease (MND)
What is the genetic influence of ALS?
sporadic ALS 90-95%
familial ALS 5-10%
What is the gender influence of ALS?
Males 1.7:1
What is the average range of age of onset of ALS?
55-60 years
What is the prevalence rate of ALS?
30,000
What is the incidence rate of ALS?
5,000
What is the mortality rate of ALS?
3-5 years from onset
What is the cause of ALS?
Occur as the result of an intersection of a variety of cellular insults, such as superoxide dismutases, leading to neuron degeneration and death.
Research suggests an excess of glutamate in the synaptic cleft triggers a cascade of events that leads to cellular death.
What is the pathophysiology of ALS?
progressive motor neuron loss in spinal cord, brainstem, and cortex.
partial sparing of cranial nerves III, IV, and VI, sacral spinal level, spinocerebellar tract, smooth muscle, cardiac muscle, sensory tracts, and cognition
What is the progression of independence of ALS?
early stage: independent
middle stage: partially independent
late stage: dependent
What is the diagnosis criteria of ALS?
objective evidence of UMN and LMN damage
minimum of 3 regions affected
disease progression
What disease modifying drugs are used in ALS?
Rilutek
What symptomatic drugs are used in ALS?
Pain-Vicodin Fatigue- Amantadine Spasticity- Baclofen Sialorrhea- Artane Depression- Paxil