lateral sclerosis

2.  A-myo-trophic comes from Greek
› A = no/negative
› Myo = muscle
› Trophic = nourishment
› No Muscle Nourishment
 Lateral = defines location of the nerve cells that signal and
control the muscles
 Sclerosis = scarring and hardening in the degenerating
region

3.  Also known as:
› Motor neuron disease
› Charcot’s disease
› Lou Gehrig’s disease

4.  A progressive neurodegenerative disease.
 Cells of the brain and spinal cord are lost.
 Affects mostly motor neurons
› The cells that control needed voluntary muscle
activity such as:
 Speaking
 Skeletal muscle movement
 Breathing
 Swallowing

5.  When the motor neurons eventually die, the ability
of the brain to control muscle movement is lost.
 Causing paralysis of essential body systems.
 When muscles no longer receive the messages
from the motor neurons that they require to
function, the muscles begin to atrophy.

6.  According to the ALS CARE Database
› 60% of the people with ALS in the database are men
› 93% of patients in the database are caucasian
 Normally occurs in people between 40 to 70
years of age
› Also can occur in people in their 20’s and 30’s

7.  Copper/zinc Superoxide dismutase 1
(SOD1)
 Heavy neurofilament subunit (NEFH)
 Peripherin (PRPH)
 Dynactin (DCTN1)

8.  Due to a mutation in SOD1, the superoxide
radicals are not neutralized
 The radicals attack the motor neurons and
degrade them
 Muscles are not able to be stimulated

9.  Free radicals
› Inherited form of ALS often involves a mutation in a
gene responsible for producing a strong antioxidant
enzyme that protects cells from damage caused by
free radicals — the byproducts of oxygen
metabolism.
 Glutamate
› People who have ALS typically have higher than
normal levels of glutamate, a chemical messenger in
the brain, in their spinal fluid.
› Too much glutamate is known to be toxic to some
nerve cells.

10.  Autoimmune Response
› Immune system begins to attack normal cells
› Scientists have speculated that such antibodies
may trigger the process that results in ALS

11.  Two types of ALS:
› Sporadic – no family history
› Familial – family history/background
 Autosomal dominant
 Autosomal recessive
 X-linked dominant
 90% of the known cases are sporadic

13.  Early symptoms
› Increasing muscle weakness
 Especially in the arms and legs
› Difficulty speaking
› Trouble in swallowing
› Problems with breathing
› Twitching
› Cramping of muscles
 Mostly hands and feet

14.  Late Symptoms
› Extreme muscle atrophy
› Reflexes
 Slow to absent
› Excessive drooling
› Babinski's sign
 Big toe dorsiflexes and the other toes
fan out
› Increase spasticity
› Weight loss
› Choking
› Cardiac arrest due to respiratory arrest usually resulting
in death

16.  S/ S or patterns of symptoms are not the same for each ALS
individual
 Progressive muscle weakness and paralysis are universally
experienced
 Since ALS attacks only motor neurons, the sense of sight,
touch, hearing, taste, and smell are not affected
 Average life expectancy is 3 to 5 years after diagnosis

17.  MRI
 Muscle and nerve biopsy
 Electomyography (EMG)
 Nerve conduction velocity (NCV)
 Blood and urine studies
 Spinal tap
 Even with all this technology ALS is
extremely difficult to diagnose. This is
because many diseases mimic signs of ALS

23.  Progressive cervical extensor weakness:
› Cause the head to fall forward
› Resulting in overstretching of the posterior
musculature and soft tissues
 For mild to moderate cervical weakness a
soft foam collar may be worn during specific
activities
 For moderate to severe weakness, a semi
rigid or rigid collar is prescribed

26.  Shoulder Pain:
› May develop shoulder pain with capsular pattern
› Secondary to spasticity or weakness causing
imbalance that may lead to:
 Impingement
 Overuse of strong muscles
 Muscle strain
 Poor re sting position
 Glenohumeral subluxation secondary to weakness

27.  Breathing and positioning to optimize
ventilation
 Airway clearance techniques may be
necessary when conditions that cause
secretion retention arise

28.  Decrease the stress on remaining function
 Conserve energy
 Minimize local or general muscle fatigue
 The type of ambulatory assistive device
prescribed is dependent on:
› Proximal muscle strength or instability
› Function of the UEs
› The pattern , extent and rate of disease progression
› financial constraints