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Flashcards in ALS [Guest Lecture} Deck (24)
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1
Q

Describe ALS

A

ALS is a family of disorders affects upper and/or lower motor neurons

It can be inheritied or sporadics

All forms are progressive and spare sensory neurons

Includes: ALS, PMa, PLS, PBP (IBALS), and SMA

2
Q

List the areas of the nervous system affected by ALS

A

BOTH UMN and LMN

  • Motor cortex
  • Corticospinal/bulbar stracts
  • Brainstem
  • Anterior horn spinal neurons
3
Q

List the sx of ALS

A
  • Weakness: hands, arms, leg, mm of speech/swallowing/breathing
  • Mm fasciculation and cramping
  • Dysarthria and dysphagia
  • Respiratory compromise
4
Q

Differentiate between UMN and LMN signs

A

UMN = spasticity, hyperreflexia, pseudobulbar sx

LMN = weakness, atrophy, fasciculation

5
Q

List the negative sx (rule out) for ALS

A
  • Lack of progression
  • Sensory impairment
  • Visual decline
  • B/B dysfunction
  • Imaging, EMG, or other evidence of alternative disease
6
Q

List the DDx for ALS

A
  • HIV
  • Lyme disease
  • Syphilis
  • MS
  • Post-polio syndrome
  • Spinal mm atrophy
  • Kennedy’s disease
7
Q

Describe the pathophysiology of Sporadic ALS (sALS)

A

No linked to any specific factors

Pathophysiology is complex, likely interaction of multiple environmental and genetic factors

8
Q

Describe the pathophysiology of familial ALS (fALS)

A

Autosomal dominant, mutation in superoxide dismutase type 1 (SOD1), TDP41, ubiquitin-2, and/or C9ORF72 genes

5-10% of ALS cases

9
Q

Describe the cellular cascade of ALS

A

Once intiated…

Oxidative stress > glutamate induced excitotoxicity > intracellular protein aggration > mitochondiral dysfunction > growth factor deficiency > axonal transport failure > capsase enzyme activity

10
Q

Condition:

  • LMN degeneration with UMN sparing
  • Onset 40-60
  • Often converts to ALS
  • 5 year survival rate of 33%
A

Progressive Muscular Atrophy

11
Q

Condition:

  • UMN degeneration with LMN sparing
  • Onset 40-60 yo
  • Clinical course over 2-3 yrs confirms dx
  • May convert to ALS
  • NOT considered fatal
  • 16 year survival rate 77%
A

Primary Lateral Sclerosis

12
Q

Condition:

  • Degerenation of lower CN (IX, X, XI, XII)
  • Rarely occurs w/o some involvement of extremities
  • Pseudobulblar affect
  • Frontotemporal dementia
A

Progressive bulbar palsy

13
Q

Condition:

  • Family of hereditary MN disorders
  • Infantile onset
  • 2 year lifespan
A

Spinal Muscular Atrophy

Type 1 or Werdnig-Hoffman

14
Q

Condition:

  • Family of hereditary MN disorders
  • Onset 6-18 mo
  • Survive into adulthood
  • Significant motor impairment
A

Spinal Muscular Atrophy

Type 2 or Juvenile/Chronic

15
Q

Condition

  • Family of hereditary MN disorders
  • Onset in toddlerhood/adolescence
  • Usually remain ambulatory
  • Increased risk for respiratory compromise
A

Spinal Muscular Atrophy

Type 3 or Wolhlfart-Kugelberg-Welander

16
Q

List the prognostics factors for ALS

A
  • Age
  • Limb vs. bulbar onset
  • Rate of progression
  • Onset of respiratory dysfunction
  • Psychological well-being
  • Time to dx (longer = better, less aggressive)
  • Time to cognitive dysfunction
17
Q

Describe the 1 approved pharmacological management for ALS

A

Rilutek - glutamate inhibitory

Prolongs survival by 2-3 mo

18
Q

Describe the 2 drugs in phase 2 of trials

A

NPOO1 = reduces macrophage activity

Tirasemtiv = modulates calcium/troponin interaction

19
Q

Describe the respiratory management options for ALS

A

BiPAP - provides additional pressure at inspiration, used when PVC < 50%

Tracheostomy - may extend life 5-15 yrs, potential for locked in syndrome

Supplemental O2/CPAP generally NOT appropriate

20
Q

Describe ways to approach dysarthira in those with ALS

A

Use Augmentative or Alternative Communication (AAC)

pen/paper, writing boards

electronics: (non)dedicated, access via type/switch/eye gaze, covered by insurance w/20% copay, know to medicare as speech generatving devices

21
Q

Describe what can be done for MSK pain in ALS

A

Common areas = back, legs, arms, shld, neck

May be due to atrophy, afferent disruption, MU changes

Can be addressed with modalities, stretching, ROM, positioning, etc

22
Q

Describe what can be done for spasticity and mm cramping in ALS

A

Spasticity can be tx with baclofen

Mm cramping can be address with stretching, diet changes; in severe cases can be tx with Valium, Kolonpin or Ativan

23
Q

Describe what can be done for fasciculations in ALS

A

Typically addressed with caffeine restriction

With severe sx can be tx with ativan or morphine

24
Q

Describe the role of PT for those with ALS

A

ANTICIPATION of NEEDS

Adaptive equipment

Moderal aerobic/resistance training may improve QoL w/low risk

EDUCATION and ADVOCACY