Guideline Treatment
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Transcript of 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
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