Amyotrophic Lateral Sclerosis

An Educational
Handout for
Health
Professionals
By Julianna Blischak

Pathway
Defined:

ALS is a rapidly progressing,

fatal motor neuron disease
that affects the nerve cells of
the brain and spinal chord.

Degeneration and death of

the upper and lower motor
neurons prevent impulses
from firing to the muscles to
signal contraction, resulting
in deterioration and
hardening of the muscles.

Origin and etiology:

ALS was discovered in 1869 by French neurologist, Jean-Martin Charcot. Only 5-10% of
all cases are inherited (familial). Otherwise, ALS is classified as sporadic/idiopathic.

Sx
v
v
v
v
v
v
v

Muscle weakness
Clumsiness
Fatigue of the arms and legs
Slurred speech
Dysphagia
Muscle cramps or twitches
Uncontrolled laughing or
crying
v Muscle atrophy
v Eventual paralysis
v Premature death

Dx
Very difficult to diagnose. A series
of tests (electrodiagnostic tests,
blood and urine studies, spinal taps,
X-rays, MRIs, myelograms of
cervical spine, muscle/nerve
biopsies, and neurological
examinations) are performed to rule
out diseases that behave like ALS.

MNT
Involves collaboration of speech
therapist and registered dietician

Labs
v Increased glutamate
v Increased creatine kinase

Medications & Tx
Riluzole (Rilutek) is the only FDA
approved medication currently on the
market. It slows the progression of the
disease by reducing levels of glutamate
in the brain.
Other forms of treatment include
respiratory, physical, occupational,
speech, and nutritional therapies.

v Risk of malnutrition is increased

due to declining ability to prepare meals
and feed oneself
v Numerous small meals may be
preferred, meals times will be extended
v Energy requirements must be
calculated and adjusted as mobility changes
v Foods may need to mechanical
soft or pureed based on chewing and
swallowing abilities
v Liquids may need to be thickened
v Special self-feeding devices should
be implemented
v May need help of caregiver to
feed
v PEG tube can be inserted for
extra support as the disease progresses

v Soft foods include: applesauce,

cooked/canned fruits and vegetables,
mashed potatoes, pudding, yogurt,
ground meats, etc.
Current Research: The cause and cure of ALS have not yet been discovered,
but progress is being made thanks to the ALS Association and other scientific bodies. In
1993, SOD1 was discovered, which is responsible for 20% of inherited cases. In 2009,
ALS6 was discovered, which causes 5% of inherited cases. In 2011, a genetic mutation
was discovered that is reported to account for approximately 50% of familial cases in
Finland.

For more information on ALS visit alsa.org