Guideline Treatment

8/10/2019 Guideline Treatment

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Anggi Dirgantara

Guideline treatment:Amyotrophic Lateral Sclerosis (ALS)

UPDATE


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Background

Amyotrophic lateral sclerosis (ALS)is the most common degenerativedisease of the motor neuron system.

ALS is a fatal disease, with amedian survival period of 3 yearsfrom onset of weakness

Really need to know how the ALSpatient management in order toimprove the quality of life


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Definition

Amyotrophy

referring to the atrophy of muscle fibers, whichare denervated as their corresponding anteriorhorn cells degenerate.

Lateral sclerosis

refers to the changes seen in the lateral

columns of the spinal cord as upper motorneuron (UMN) axons in these areas degenerateand are replaced by fibrous astrocytes (gliosis).


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Etiology

Excitotoxicity was suggested to occursecondary to overactivation of glutamatereceptor

Glutamate

All of the mutated genes found to giverise to familial ALS have also beenfound in patients with sporadic ALS

Gene 1 (SOD1)ALSare C9orf72, FUS(ALS6) and TARDBP

Gene

Free radical formation was also exploredas a cause of ALS

Discovery of mutations in the freeradicalscavenging enzyme superoxidedismutase 1 (SOD1)

Oxidativestress


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Diagnose criteria (WFN)

recognize the following 4 regions, or levels, of thebody

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 middleportion of the spinal muscles

Lumbosacral

Muscles of the lower back, groin, and lower extremities


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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 theLMN signs

Clinically probable, laboratory-supported ALS

UMN and LMN signs in 1 segment or UMN signs in 1 region coupled with LMN signs byelectromyography (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 insegments 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


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Differential diagnose chronically progressive

symmetric sensorimotor disorder

cytoalbuminologic dissociation

Interstitial and perivascularendoneurial infiltration bylymphocytes and macrophages.

Ex: SGB

ChronicInflamatory

demyelinatingpoliradiculopat

y Idiopathic inflammatory myopathy

that causes symmetrical, proximalmuscle weakness

Inclusion body

Dermatomiositis /

poliomyositis

immune-mediated inflammatorydisease that attacks myelinatedaxons in the central nervous system

producing significant physicaldisability, defisit sensomotor

Multipelsclerosis


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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 onlymedication that has shown efficacy in extending life in ALS.

Intake nutrition

Enteral/ parenteralPEG

Noninvasive ventilation

To treat respiratory insufficiency to prolong survival and slow thedecline of FVC

Mechanical insufflation/exsufflation

to clear secretions in patients with reduced peak cough flow


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Simptomatics Muscle relaxants to relieve spasticity

The combination of dextromethorphan and quinidine todecrease emotional lability of pseudobulbar affect

Anticholinergics and sympathomimetics for sialorrhea

Mucolytics for thickened secretions Lorazepam for anxiety

Selective serotonin reuptake inhibitors (SSRIs) fordepression

Nonsteroidal anti-inflammatory drugs (NSAIDs), tramadol(Ultram), ketorolac (Toradol), morphine (immediate orextended release), or transdermal fentanyl, for pain


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Conclusion

ALS

Atrophy of muscle fibous

Changes the lateral columns of the spinal cord as upper motorneuron (UMN) axons in these areas degenerate and arereplaced by fibrous astrocytes (gliosis)

American Academy of Neurologyrecommendation

Rhiluzole

Support management: intake nutrition, Noninvasive ventilation

Simptomatik management: antianxiety, antidepresant, NSAID


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References

Brooks BR, Miller RG, Swash M, Munsat TL. El Escorial revisited: revised criteria for thediagnosis of amyotrophic lateral sclerosis.Amyotroph Lateral Scler Other Motor NeuronDisord. Dec 2000;1(5):293-9.

[Guideline] Miller RG, Jackson CE, Kasarskis EJ et al. Practice Parameter update: The careof the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies(an evidence-based review). Report of the Quality Standards Subcommittee of the AmericanAcademy of Neurology. Neurology. 2009;73:1218-1226.

[Guideline] Miller RG, Jackson CE, Kasarskis EJ, et al. Practice parameter update: The care

of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptommanagement, and cognitive/behavioral impairment (an evidence-based review): report of theQuality Standards Subcommittee of the American Academy of Neurology. Neurology. Oct 132009;73(15):1227-1233.

Hardiman O, van den Berg LH, Kiernan MC. Clinical diagnosis and management of

amyotrophic lateral sclerosis. Nat Rev Neurol. Oct 11 2011;7(11):639-49.

King SJ, Duke MM, O'Connor BA. Living with amyotrophic lateral sclerosis/motor neuronedisease (ALS/MND): decision-making about 'ongoing change and adaptation'. J Clin Nurs.Mar 2009;18(5):745-54.

Eisen A. Amyotrophic lateral sclerosis: A 40-year personal perspective. J Clin Neurosci. Apr2009;16(4):505-12.


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Brooks BR. Managing amyotrophic lateral sclerosis: slowing disease progression and improving patient quality

of life.Ann Neurol. Jan 2009;65 Suppl 1:S17-23.

Al-Chalabi A, Fang F, Hanby MF, Leigh PN, Shaw CE, Ye W, et al. An estimate of amyotrophic lateral sclerosis

heritability using twin data. J Neurol Neurosurg Psychiatry. Dec 2010;81(12):1324-6.

Kanouchi T, Ohkubo T, Yokota T. Can regional spreading of amyotrophic lateral sclerosis motor symptoms be

explained by prion-like propagation?. J Neurol Neurosurg Psychiatry. Jul 2012;83(7):739-45.

Renton AE, Majounie E, Waite A, Simn-Snchez J, Rollinson S, Gibbs JR, et al. A hexanucleotide repeat

expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron. Oct 20 2011;72(2):257-68.

DeJesus-Hernandez M, Mackenzie IR, Boeve BF, Boxer AL, Baker M, Rutherford NJ, et al. Expanded

GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and

ALS. Neuron. Oct 20 2011;72(2):245-56

Seeley WW, Crawford RK, Zhou J, Miller BL, Greicius MD. Neurodegenerative diseases target large-scale

human brain networks. Neuron. Apr 16 2009;62(1):42-52.

Armon C. What is ALS?. In: Bedlack RS, Mitsumoto H, Eds.Amyotrophic Lateral Sclerosis: A Patient Care

Guide for Clinicians. New York: Demos Medical Publishing; 2012:1-23.

Pflumm, Michelle. 2014. AAN 2014: A Philadelphia Story. Diunduh dari
http://blogs.als.net/post/2014/05/30/AAN-2014-A-Philadelphia-Story.aspx%20pukul%2007.53http://blogs.als.net/post/2014/05/30/AAN-2014-A-Philadelphia-Story.aspx%20pukul%2007.53http://blogs.als.net/post/2014/05/30/AAN-2014-A-Philadelphia-Story.aspx%20pukul%2007.53http://blogs.als.net/post/2014/05/30/AAN-2014-A-Philadelphia-Story.aspx%20pukul%2007.53http://blogs.als.net/post/2014/05/30/AAN-2014-A-Philadelphia-Story.aspx%20pukul%2007.53http://blogs.als.net/post/2014/05/30/AAN-2014-A-Philadelphia-Story.aspx%20pukul%2007.53http://blogs.als.net/post/2014/05/30/AAN-2014-A-Philadelphia-Story.aspx%20pukul%2007.53http://blogs.als.net/post/2014/05/30/AAN-2014-A-Philadelphia-Story.aspx%20pukul%2007.53http://blogs.als.net/post/2014/05/30/AAN-2014-A-Philadelphia-Story.aspx%20pukul%2007.53http://blogs.als.net/post/2014/05/30/AAN-2014-A-Philadelphia-Story.aspx%20pukul%2007.53http://blogs.als.net/post/2014/05/30/AAN-2014-A-Philadelphia-Story.aspx%20pukul%2007.53http://blogs.als.net/post/2014/05/30/AAN-2014-A-Philadelphia-Story.aspx%20pukul%2007.53http://blogs.als.net/post/2014/05/30/AAN-2014-A-Philadelphia-Story.aspx%20pukul%2007.53

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