ALS and GBS

Question 1

ALS:

Answer 1

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

Question 2

Etiology of ALS:

Answer 2

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

Question 3

Signs and Symptoms of ALS:

Answer 3

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

Question 4

Bulbar onset:

Answer 4

progressive bulbar palsy

Question 5

Spinal cord onset:

Answer 5

progressive muscle atrophy

Question 6

Stage I ALS:

Answer 6

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

Question 7

Stage II ALS:

Answer 7

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

Question 8

Stage III ALS

Answer 8

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

Question 9

Stage IV ALS

Answer 9

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

Question 10

Stage V ALS

Answer 10

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

Question 11

Stage VI ALS:

Answer 11

bedridden, dependent ADLs, FMS; progressive respiratory distress

Question 12

ALS Functional Rating Scale (ALSFRS)

Answer 12

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

Question 13

Stage I Motor Learning and Recovering of Function

Answer 13

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

Question 14

Stage II Motor Learning and Recovering of Function

Answer 14

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

Question 15

Stage III Motor Learning and Recovering of Function

Answer 15

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

Question 16

Stage IV Motor Learning and Recovering of Function

Answer 16

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

Question 17

Stage V Motor Learning and Recovering of Function

Answer 17

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

Question 18

Stage VI Motor Learning and Recovering of Function

Answer 18

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

Question 19

Main forms of Guillain-Barre:

Answer 19

Demyelinating Polyradiculoneuropathy (AIDP)
Acute Axonal Neuropathy (AMAN)

Question 20

Guillain-Barre: UMN or LMN

Answer 20

LMN disorder

Question 21

Causes of Guillain-Barre:

Answer 21

Campylobacter jejuni (gasteroenteritis cause)
Viruses
Bacterium
Surgery 
Vaccinations

Question 22

Pathophysiology of Guillain-Barre:

Answer 22

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

Question 23

Recovery of Guillain-Barre:

Answer 23

from proximal to distal

Question 24

Presentation of Guillain-Barre:

Answer 24

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

Question 25

Nadir:

Answer 25

Point of greatest severity

Question 26

Clinical Features of Guillain-Barre:

Answer 26

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

Question 27

Which cranial nerves of affected with Guillain-Barre:

Answer 27

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

Question 28

Phases of GBS

Answer 28

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

Question 29

Complications of GBS:

Answer 29

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

Question 30

GBS PT Acute Phase:

Answer 30

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

Question 31

GBS PT Plateau Phase:

Answer 31

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

Question 32

GBS PT Recovery Phase:

Answer 32

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!