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Flashcards in ALS and GBS Deck (32):
1

ALS:

Destruction of UMN and LMN (degeneration of anterior horn cells and descending corticobulbar and corticospinal)

2

Etiology of ALS:

unknown (viral/autoimmune/toxic), 5-10% genetic (autosomal dominant)

3

Signs and Symptoms of ALS:

Causes weakness and atrophy to the body (limbs progressing to whole body, atrophy, cramping)
Spasticity, hyperreflexia
Dysarthria, dysphagia, dysphonia secondary to pseudobulbar palsy and progressive bulbar palsy
Usually absence of sensory changes; small number (20%) may show deficits
Loss of voluntary control movement
ANS Dysfunction in 1/3 patients

4

Bulbar onset:

progressive bulbar palsy

5

Spinal cord onset:

progressive muscle atrophy

6

Stage I ALS:

early disease, mild focal weakness, asymmetrical distribution, symptoms of hand cramping and fasiculations

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Stage II ALS:

moderate weakness in groups of muscles, some wasting (atrophy) of muscles; modified independence with assistive devices

8

Stage III ALS

severe weakness of specific muscles, increasing fatigue, mild to moderate functional limitations, ambulatory

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Stage IV ALS

severe weakness and wasting of Les, mild weakness of Ues; moderate assistive and assistive devices, wheelchair user

10

Stage V ALS

progressive weakness with deterioration of mobility and endurance, increased fatigue, moderate to severe weakness of whole limbs and trunk, spasticity, hyperreflexia, moss of head control, maximal assist

11

Stage VI ALS:

bedridden, dependent ADLs, FMS; progressive respiratory distress

12

ALS Functional Rating Scale (ALSFRS)

Assess disease progression and function across 10 functional categories
Scored 0 (loss of function) to 4 ( normal function)
40 maximal score

13

Stage I Motor Learning and Recovering of Function

independent of mobility and ADL’s
Active ROM
Continue normal activities
Stretching of affected joints
Resistive exercises to unaffected mm
Aerobic activities

14

Stage II Motor Learning and Recovering of Function

moderate weakness in groups of mm
Assess for use of assistive devices
Continue c stage 1 activities
Increased need for caregiver assistance
Exercise 2-3 x per day with rest in between

15

Stage III Motor Learning and Recovering of Function

Continued ambulation but severe weakness in mm groups
Foot drop or hand weakness
Goal is to keep pt physically independent
Use of splints, orthotic

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Stage IV Motor Learning and Recovering of Function

severe weakness of legs, involvement of arms WC
Continue AROM and PROM to prevent contractures
Strengthening there ex
Integumentary

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Stage V Motor Learning and Recovering of Function

Progressive weakness of mobility and endurance
Inability to transfer
Pain due to cramping and contracture
Stretching, splinting, STM, orthotic

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Stage VI Motor Learning and Recovering of Function

Bed ridden, max assist, hospital bed, frequent repositioning, pain management, postural drainage, coughing techniques, airway clearance

19

Main forms of Guillain-Barre:

Demyelinating Polyradiculoneuropathy (AIDP)
Acute Axonal Neuropathy (AMAN)

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Guillain-Barre: UMN or LMN

LMN disorder

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Causes of Guillain-Barre:

Campylobacter jejuni (gasteroenteritis cause)
Viruses
Bacterium
Surgery
Vaccinations

22

Pathophysiology of Guillain-Barre:

Autoimmune reaction – cross reaction with neural tissue
When myelin is destroyed – destruction – inflammation
Acute inflammatory lesions are present within several days of onset of symptoms
Nerve conduction is slowed or blocked
Schwann cells that produce myelin in the PNS are destroyed, axons are left intact in all but the most severe cases

23

Recovery of Guillain-Barre:

from proximal to distal

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Presentation of Guillain-Barre:

•50% of cases, progression stops by 2 weeks
•80-90% of cases, progression stops by 4 weeks
•Static phase of 2-4 weeks

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Nadir:

Point of greatest severity

26

Clinical Features of Guillain-Barre:

Ascending progressive loss of motor function
Begins distally & progresses proximally
Distal sensory impairments vary:
Diaphragm & CN can also be affected

27

Which cranial nerves of affected with Guillain-Barre:

Facial nerve (CN VII)
Diplopia (double vision) – from eye mm weakness (CN III, IV, VI)

28

Phases of GBS

Acute (lasts about 4 weeks)
Plateau (lasts about 4 weeks) = stabilization of symptoms
Recovery – (last a few months to several years) = Patient begins to improve but may have neurological sequel or residual effects

29

Complications of GBS:

Respiratory impairment and failure
Autonomic instability
Pain; myalgia
Risk of pneumonia
Prolonged hospitalizations and immobility

30

GBS PT Acute Phase:

Positioning to ↓ potential contractures & skin breakdown – hand & foot splints may be used
Postural drainage with percussion
Gentle stretching of chest wall – Be sure patient is in subtalar neutral when stretching Achilles
Trunk rotation

31

GBS PT Plateau Phase:

Increase tolerance to upright – Initiate gradually (may still be on a ventilator)
Acclimation to upright – may need support of trunk d/t demyelination
Maintain ROM
Improve pulmonary function
Avoid fatigue & overexertion

32

GBS PT Recovery Phase:

Muscle strength usually recovered 2-4 weeks after reaching plateau
Muscles return in reverse order or descending pattern
Tilt table – acclimation to upright & WB
Positioning splints for LEs & Thrombolytic stockings to ↓venous pooling
Maximize functional abilities but strengthening is a challenge & can be harmful or beneficial!