Introduction
Amyotrophic lateral sclerosis is a terminal neurological disorder characterized by progressive degeneration of nerve cells in the spinal cord and brain. It is a rapidly progressive degeneration of upper and lower motor neurons, which results in weakness and wasting of muscle in the arms, leg, trunk and bulbar region. There is associated spasticity in the arms, legs and bulbar regions.
Clinical symptoms of Amyotrophic lateral sclerosis
Clinical symptoms of amyotrophic lateral sclerosis include:
- loss of ambulation
- loss of arm and hand function
- difficulty with speech and swallowing and
- breathlessness. Aspirations
- pneumonia and respiratory insufficiency are common terminal events.
The characteristic onset is from symptoms and signs at a single site, progressing to contiguous regions, often leading to death within 3–5 years of symptom onset .
Pharmacological Intervention (Treatment)
There is no truly effective treatment for amyotrophic lateral sclerosis though several therapies have shown promise in preclinical models of motor neuron disease; however, most of them failed in human studies.
The objective of pharmacological intervention in amyotrophic lateral sclerosis (ALS) is to prevent the deterioration of motor neuron function and thereby, slow down or even arrest the currently inexorable course of the condition (‘disease modification’, ‘neuroprotection’).
Riluzole, a putative blocker of glutamate release is the only drug established as the first disease-modifying treatment of ALS by improving survival of the patients. Riluzole reduces damage to motor neurons by decreasing the release of glutamate, which reduces glutamate receptor stimulation and helps to regulate intracellular calcium concentrations in the neuron. This prevents the initiation of apoptosis by mitochondria sensitive to high calcium levels. Riluzole does not reverse damage already done to motor neurons.