Guideline treatment:

Amyotrophic Lateral Sclerosis (ALS)

UPDATE
Anggi Dirgantara

Background
Amyotrophic lateral sclerosis (ALS)
is the most common degenerative
disease of the motor neuron system.
ALS is a fatal disease, with a
median survival period of 3 years
from onset of weakness
Really need to know how the ALS
patient management in order to
improve the quality of life

Definition
Amyotrophy
referring to the atrophy of muscle fibers, which
are denervated as their corresponding anterior
horn cells degenerate.

Lateral sclerosis
refers to the changes seen in the lateral
columns of the spinal cord as upper motor
neuron (UMN) axons in these areas degenerate
and are replaced by fibrous astrocytes (gliosis).

Etiology

Glutamate

Gene
Oxidative
stress

Excitotoxicity was suggested to occur

secondary to overactivation of glutamate
receptor
All of the mutated genes found to give
rise to familial ALS have also been
found in patients with sporadic ALS
Gene 1 (SOD1)ALS are C9orf72, FUS
(ALS6) and TARDBP
Free radical formation was also explored
as a cause of ALS
Discovery of mutations in the free
radicalscavenging enzyme superoxide
dismutase 1 (SOD1)

Diagnose criteria (WFN)

recognize the following 4 regions, or levels, of the

body
Bulbar
Muscles of the face, mouth, and throat

Cervical
Muscles of the back of the head and the neck,
shoulders, upper back, and upper extremities

Thoracic
Muscles of the chest and abdomen and the middle
portion of the spinal muscles

Lumbosacral
Muscles of the lower back, groin, and lower extremities

Category WFN
Clinically definite ALS
UMN and LMN signs in at least 3 body segments

Clinically probable ALS

UMN and LMN signs in at least 2 body segments with some UMN signs in a segment above the
LMN signs

Clinically probable, laboratory-supported ALS

UMN and LMN signs in 1 segment or UMN signs in 1 region coupled with LMN signs by
electromyography (EMG) in at least 2 limbs

Clinically possible ALS

UMN and LMN signs in 1 body segment, UMN signs alone in at least 2 segments, or LMN signs in
segments above UMN signs

Clinically suspected ALS (carried forward from the original El Escorial criteria)
Pure LMN syndrome with other causes of LMN disease adequately excluded

Differential diagnose
Chronic
Inflamatory
demyelinating
poliradiculopat
y
Dermatomiositi
s/
poliomyositis

Multipel
sclerosis

chronically progressive
symmetric sensorimotor disorder
cytoalbuminologic dissociation
Interstitial and perivascular
endoneurial infiltration by
lymphocytes and macrophages.
Ex: SGB
Idiopathic inflammatory myopathy
that causes symmetrical, proximal
muscle weakness
Inclusion body
immune-mediated inflammatory
disease that attacks myelinated
axons in the central nervous system
producing significant physical
disability, defisit sensomotor

Management recommendation
American Academy of Neurology Guideline (2009) and

Annual Meeting American Academy of Neurology 2014

Rhiluzole
The glutamate pathway antagonist riluzole (Rilutek) is the only
medication that has shown efficacy in extending life in ALS.

Intake nutrition
Enteral/ parenteral PEG

Noninvasive ventilation
To treat respiratory insufficiency to prolong survival and slow the
decline of FVC

Mechanical insufflation/exsufflation
to clear secretions in patients with reduced peak cough flow

Simptomatics
Muscle relaxants to relieve spasticity
The combination of dextromethorphan and quinidine to
decrease emotional lability of pseudobulbar affect
Anticholinergics and sympathomimetics for sialorrhea
Mucolytics for thickened secretions
Lorazepam for anxiety
Selective serotonin reuptake inhibitors (SSRIs) for
depression
Nonsteroidal anti-inflammatory drugs (NSAIDs), tramadol
(Ultram), ketorolac (Toradol), morphine (immediate or
extended release), or transdermal fentanyl, for pain

Conclusion
ALS
Atrophy of muscle fibous
Changes the lateral columns of the spinal cord as upper motor
neuron (UMN) axons in these areas degenerate and are
replaced by fibrous astrocytes (gliosis)

American Academy of Neurology recommendation

Rhiluzole
Support management: intake nutrition, Noninvasive ventilation
Simptomatik management: antianxiety, antidepresant, NSAID

References

Brooks BR, Miller RG, Swash M, Munsat TL. El Escorial revisited: revised criteria for the
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of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies
(an evidence-based review). Report of the Quality Standards Subcommittee of the American
Academy of Neurology. Neurology. 2009;73:1218-1226.

[Guideline] Miller RG, Jackson CE, Kasarskis EJ, et al. Practice parameter update: The care
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Renton AE, Majounie E, Waite A, Simn-Snchez J, Rollinson S, Gibbs JR, et al. A hexanucleotide repeat
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