ALS Amyotrophic Lateral Sclerosis

Question 1

Aka Lou Gehrig’s Disease

Answer 1

Amyotrophic Lateral Sclerosis

Question 2

Characterized by

Answer 2

degeneration and scarring of the motor neurons in the lateral aspect of the spinal cord, brainstem and cerebral cortex
Peripheral nerve changes result in muscle fibre atrophy or amyotrophy

Question 3

Affects

Answer 3

upper motor neurons of cerebral cortex descending via corticospinal and corticobulbar tracts to synapse with LMNs
It can also directly affect the LMN with disease of the anterior horn cells in the spinal cord and brainstem
The areas of the brain controlling coordinated movements (cerebellum) and cognition(frontal cortex) are not affected

Question 4

Age, Gender, Cause

Answer 4

usually between the ages of 40 and 70 (average of 55)
20% more common in men than women
The cause of ALS is unknown; there is no cure
The following have been implicated in its cause:
Mutations of a gene that produce SOD1 enzyme; this enzyme is a powerful antioxidant that protects the body from damage caused by free radicals
Higher levels of glutamate
Autoimmune responses
Environmental factors
Dietary deficiency or trauma

Question 5

Symptoms

Answer 5

Muscle weakness and atrophy become widespread as the disease progresses
There is no sensory deficit
Increasing problems with moving, swallowing and speaking or forming words
Upper motor neuron:
Tight and stiff muscles (spasticity)
Hyperreflexia
Positive babinski’s sign
Lower motor neuron:
Muscle weakness and atrophy
Muscle cramps
Fleeting twitches of muscles that can be seen under the skin (fasciculations)
Anxiety and depression
Later stages, difficulty breathing as the muscles of respiratory system weaken

Question 6

Diagnosis

Answer 6

Primarily based on the signs and symptoms the physician observes in the patient
EMG (recording technique that detects electrical activity in muscles
Nerve conduction velocity
MRI
Blood and urine tests

Question 7

Treatment

Answer 7

Treatments are designed to relieve symptoms and improve the quality of life for patients:
Riluzole – first FDA approved drug that is believed to reduce damage to motor neurons by decreasing the release of glutamate
As well, drugs to control drooling (anticholinergic) and spasticity (baclofen, diazepam)
Maintenance of nutrition
Prevent respiratory complications; respiratory failure and inability to eat are final stages of ALS
Spasticity contributes to weakness – consistent slow stretching that decrease tone may benefit
Cramps respond to daily stretching
Ankle dorsiflexion is lost before loss of strength in plantarflexion
Hamstring strength appears to correlate with walking and the decrease is parallel with the loss of walking ability
Contractures should be routinely stretched taking care to support the joints as there is minimal control of muscle activity in late stages
Abdominal muscles to help increase inspiration when diaphragm and intercostal muscles become weak