ALS and GBS Flashcards
ALS:
Destruction of UMN and LMN (degeneration of anterior horn cells and descending corticobulbar and corticospinal)
Etiology of ALS:
unknown (viral/autoimmune/toxic), 5-10% genetic (autosomal dominant)
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
Bulbar onset:
progressive bulbar palsy
Spinal cord onset:
progressive muscle atrophy
Stage I ALS:
early disease, mild focal weakness, asymmetrical distribution, symptoms of hand cramping and fasiculations
Stage II ALS:
moderate weakness in groups of muscles, some wasting (atrophy) of muscles; modified independence with assistive devices
Stage III ALS
severe weakness of specific muscles, increasing fatigue, mild to moderate functional limitations, ambulatory
Stage IV ALS
severe weakness and wasting of Les, mild weakness of Ues; moderate assistive and assistive devices, wheelchair user
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
Stage VI ALS:
bedridden, dependent ADLs, FMS; progressive respiratory distress
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
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
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
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
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
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
Stage VI Motor Learning and Recovering of Function
Bed ridden, max assist, hospital bed, frequent repositioning, pain management, postural drainage, coughing techniques, airway clearance
Main forms of Guillain-Barre:
Demyelinating Polyradiculoneuropathy (AIDP) Acute Axonal Neuropathy (AMAN)
Guillain-Barre: UMN or LMN
LMN disorder
Causes of Guillain-Barre:
Campylobacter jejuni (gasteroenteritis cause) Viruses Bacterium Surgery Vaccinations
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
Recovery of Guillain-Barre:
from proximal to distal
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
Nadir:
Point of greatest severity
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
Which cranial nerves of affected with Guillain-Barre:
Facial nerve (CN VII) Diplopia (double vision) – from eye mm weakness (CN III, IV, VI)
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
Complications of GBS:
Respiratory impairment and failure Autonomic instability Pain; myalgia Risk of pneumonia Prolonged hospitalizations and immobility
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
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
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!
