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Flashcards in ALS Deck (54):
1

ALS- Definition

"a degenerative disease of the neuromuscular system involving both upper and lower motor neurons, resulting in weakness of the limbs, trunk, bulbar, and respiratory muscles"

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ALS
- neuropathology
- signs/symptoms

neuropathology: lateral, corticospinal tracts, anterior horn cells, anterior roots and peripheral nerves, CN nuclei, corticobulbar trances
Signs and Sx: spasticity and weakness in bulbar and spinal system

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Pseudo-Bulbar Palsy (PBP)
- neuropathology
- signs/symptoms

Neuropathology: bulbar nuclei
Signs and Sx: flaccid weakness of bulbar muscles (swallowing and speech)

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Progressive Muscular Atrophy (PMA)
- neuropathology
- signs/symptoms

Neuropathology: Ventral horn cells of spinal cord (LMN)
Signs and Sx:weakness and wasting of extremities

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Primary Lateral Sclerosis (PLS)
- neuropathology
- signs/symptoms

Neuropathology: motor cells of the cortex and corticospinal tracts (UMN)
Signs and Sx: spastic weakness of trunk and extremities

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Spinal Muscular Atrophy (SMA)
- neuropathology
- signs/symptoms

Neuropathology: primary motor eurons in anterior horn of SC and brainstem motor nuclei
Signs and Sx: weakness and wasting of extremities and bulbar muscles

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UMN Syndrome signs

Loss of dexterity
loss of muscle strength
spasticity
hyperreflexia
pathological reflex
flexor spasms
involuntary emotional expressive disorder (IEED)- inappropriate laughing and crying

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LMN Syndrome signs

1. loss of muscle strength
2. muscle atrophy
3. muscle hypotonia
4. hyporeflexia
5. fasciculations
6. muscle cramps

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Uncommon manifestations of ALS

1. sensory impairments (if sensory impairment, not thinking ALS)
2. ocular palsy (muscle of the eyes are typically spared)
3. Bladder and bowel dysfunction
4. Dementia

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Fronto-Temporal Dementia (FTD)

occurs in 15% of ALS patients, involves cortical atrophy
symptoms:
- word finding difficulties
- behavioral changes
- executive dysfunction
- poor judgement

* memory is not usually impaired, screening exam with MMSE

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Pathogenesis: 3 hypotheses

1. oxidative stress and mitochondrial dysfunction causing inflammation
2. excitotoxicity: increased levels of glutamate in CNS (kills neurons)
3. Cell death and apoptosis (via MAP kinase activtation)

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Diagnostic Testing: 3 factors

1. Lower motor neuron involvement (EMG)
2. Upper motor neuron involvement (MRI, MR Spec, TMS, clinical exam)
3. Additional Tests to rule out everything else (lumbar puncture, muscle and nerve biopsies, blood tests)

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Lower Motor Neurons Testing: Expected presentation/occurance on EMG

1. Denervation
2. decreased motor units
3. fibrillations

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Lower Motor Neurons Testing: Procedure of EMG

Needles are inserted into motor points.
Performed in the limb, tongue, paraspinals
Uncomfortable!

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Upper Motor Neuron Testing: Clinical exam findings

*clinical exam extremely important in UMN diagnosis
- hyperreflexia/abnormal reflexes (babinski, hoffman)

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Differential Diagnoses of ALS

1. Degenerative arthritis
2. motor neuropathy
3. inflammatory muscle disease
4. metabolic. infectious, endocrine and electrolyte imbalance

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ALS: sex ratio, age of onset, racial predisposition

sex ratio: 1.2-1.6:1 (M:F)
age of onset: 60s
Racial: none

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Prognosis: mean survival time, 5yr survival, 10yr survival

Mean survival time: 27-43months (2-4yrs)
5-year survival time: 9-40% (25% mean)
10-year survival time: 8-16%

*UMN lesions tend to live longer

19

AAN Practice parameters: guidelines for practice in treating ALS pt

1. Breaking the news
2. symptom management
3. nutrition management
4. respiratory management
5. Palliative care

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Only drug that is FDA approved as treatment in ALS

Riluzole- extends life by 3 months

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Symptomatic Treatment: what symptoms get treated

1. Muscle cramps
2. Spasticity
3. Sialorrhea Management (uncontrolled drooling/management of secretions)
4. Depression
5. Involuntary Expressive Disorder (IEED)

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Muscle Cramp Treatment

1. Quinine sulfate (perscription)
2. Tonic water
3. stretching profalactively
*more common in LMN syndrome

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Spasticity treatment

1. oral baclofen or tizanidine
2. intrathecal baclofen pump
3. stretching
4. heat
5. repetitive, light resistance exercise
*sometimes spasticity helps with function

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Sialorrhea Management

1. suction machine
2. drugs to dry secretions (atrophine, scopolamine, cold/allergy meds, amitriptyline-antidepressant)
3. Botox to parotid glands
4. radiation to parotid and submandibular glands
5. litigation of ducts of salivary glands

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Depression management

1. antidepressants (Celexa, paxil, remeron)
2. psychotherapy
3. support groups

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Involuntary Emotional Expressive Disorder (IEED) Management

Nuedexta

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Nutritional Management

1. maintain weight (difficult- pts have high caloric demands)
2. high calorie diet
3. Nutritional supplements (Ensure)
4. Early PEG (sweet spot with timing- risky if pulmonary function is too low)

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Respiratory Treatment

1. Non-invasive ventilation (NIV)
2. Cough Assist (if weak cough)
3. Tracheostomy- ONLY lift sustaining measure

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Non-Invasive Ventilation (NIV)
- what does it do
- type of ventilators
- when is it used

- decreases the work of breathing
- volume ventilators (BiPAP- pressure controlled)
- Used when FVC

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Tracheostomy
- purpose
- how many pts choose it

- only life sustaining measure (but disease continues to progress)
- long term mechanical ventilaiton
- 2-5% choose LTMR, even though it is reversible and life sustaining

31

ALS Functional Rating Scale Revised (ALSFRS-R)
- purpose
- predicatbility
- administration/items
- subcategories
- Pro

- disease specific measure of global function; correlates with changes in strength measures over time; compares function today with time prior to ALS
- predicts survival!!!!
- 12 item scale, questions asked by assessor
- 4 categories: bulbar, arm, leg, respiratory function
- easily administered
* used in stage 2 and 3 clinical trials

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MMT v MVIC in strength assessment

MMT: not functionally meaninful, burdensome for pt
MVIC: used as marker for disease, but not functionally meaningful and burdensome, requires elaborate equipment and specialized training

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Timed Up and Go in ALS population
- purpose
- predictability
- con

Tests for balance and mobility
Pedictable of falls (

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Forced Vital Capacity in ALS population
- Purpose
- predictability

- assesses diaphragmatic strength, aids in determining timing or nutritional, respiratory, and palliative treatments
- predicts survival

*useful in clinical trials

35

Rehab goal in the ALS population

to maintain the highest quality of life and function

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Goals of Exercise in ALS

1. improve function
2. avoid complication of weakness
**NOT to rebuild muscle- maintenance is more important

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Types of Exercise for ALS rehab

1. Functional (walking, transfers, balance)
2. Aerobics (walking, bike/elliptical, aquatics)
3. ROM

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Van der Kooi EL et. al: Exercise trials in muscle diseases from 1966-2002

Moderate intensity exercise shows no benefit (disease modifying) or harm

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Cup EH et al: Systematic review of exercise and/or physcial therapy intervention in neuromuscular disease

No adverse effects of individualized progressive strengthening protocols. Combination of strengthening and aerobic exercises is likely to be effective (the best approach)

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Lui 2009: A systematic review of exercise studies in ALS

Small-moderate effect sizes supporting moderate exercise in early-stage ALS. No adverse effects

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Animal model- SOD1 mice

moderate intensity exercise extended survival; high intensity exercise hastened onset of symptoms and decreased survival time

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Dal Bello-Haas 2007:

- 5 reps at full 6RM weight, 5 reps at 75%RM weight, 5 reps at 50% of 6RM weight
- higher ALSFRS-R and leg strength MVIC scores in exercise group at 6 months

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Kilmer 2001: Eccentric exercise

showed similar responses to high intensity eccentric loads in persons with slowly progressive muscular dystrophies and controls

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Aerobic/cardiovascular recommendations in ALS

60-80% HRmax
11-14 on BORG
30mins 5x/wk

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Pinto: studied TM exercise with NIV to assist tissue oxygenation in ALS

demonstrated improved respiratory function in treated group at 1 year
*note: consider inclusion (NVIC, FVC at 70%); pts more able to perform this activity

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Sanjak 2012: BWS TM training in ALS

improvement in 6MWT distance, ALSFRS-R, and stride length

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General guidelines for exercise

1. avoid fatigue (recovery within about 1hr)
2. avoid overworking muscles (DOMS)
3. exercise in moderation- aerobics (Borg)
4. low resistance strengthening (multiple reps)
5. Stretching
6. personalize (consider exercise hx)
7. revisable (not static)

48

Splinting and Bracing- when do we consider this?

When function deteriorates, assist with orthosis
i.e.- when footdrop, give them a brace. Don't wait

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Braces of the Upper limb

1. Finger extensor splint (extensors weak, prevent contractures)
2. Hemi Shoulder Sling (straps work as deltoid, doesn't immobilize, provides/promotes distal function)

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Neck Braces

*useful due to weak neck extensors
- soft collar
- head master collar
- philadelphia collar

51

Considerations with DMA

Rollators- great to prevent falls (has a seat), not great with spasticity (slippery)

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Bulbar symptoms

1. Dysarthria (difficulty swallowing)
2. Dysphonia (can't phonate)
3. Dysphagia (can't speak)
4. Sialorrhea (uncontrolled drooling)
5. Aspiration (liquid in lungs)
6. Pseudobulbar symptoms/IEED

53

Cranial nerves involved in speech and swallowing

CN V, VII, IX, X, XII

54

Locked in syndrome

no muscle function (not even eye function) for communication
ultimately ALS pts all progress to being locked in