ALS fasciculations (Amyotrophic lateral sclerosis)(Motor neuron disease)
ALS-AMYOTROPHIC LATERAL SCLEROSIS IS A NEURODEGERERATIVE DISEASE THE PRIMARILY AFFECTS THE MOTOR NEURON CELL POPULATION IN THE MOTOR CORTEX,BRAIN STEM AND SPINAL CORD.IT IS A PROGRESSIVE DISEASE AND MOST PATIENTS SUCCUMB TO RESPIRATORY FAILURE.
STEPHEN HAWKING had this rare early-onset slow-progressing form of motor neurone disease (also known as amyotrophic lateral sclerosis, "ALS", or Lou Gehrig's disease) that gradually paralysed him over the decades] Even after the loss of his speech, he was still able to communicate through a speech-generating device, initially through use of a hand-held switch, and eventually by using a single cheek muscle. He died on 14 March 2018 at the age of 76.
Fasciculation represents a brief spontaneous contraction that affects a small number of muscle fibres, causing a flicker of movement under the skin. While an understanding of the role of fasciculation in ALS remains incomplete, fasciculations derive from ectopic activity generated in the motor system. A proximal origin seems likely to contribute to the generation of fasciculation in the early stages of ALS, while distal sites of origin become more prominent later in the disease, associated with distal motor axonal sprouting as part of the reinnervation response that develops secondary to loss of motor neurons. Fasciculations are distinct from the recurrent trains of axonal firing described in neuromyotonia. Fasciculation without weakness, muscle atrophy or increased tendon reflexes suggests a benign fasciculation syndrome, even when of sudden onset. Regardless of origin, fasciculations often present as the initial abnormality in ALS, an early harbinger of dysfunction and aberrant firing of motor neurons.
