Guillain-Barre Syndrome, MS, ALS Flashcards
What is Guillain-Barre Syndrome
- Autoimmune disorder of the peripheral nervous system causing progressive weakness with diminished/absent tendon reflexes
- Acute, inflammatory, demyelinating, polyradiculoneuropathy presenting as rapid loss of myelin.
Etiology of GBS
Unknown
Associated with an autoimmune attack - occurs after recovery from an infectious illness.
can recover but can also kill you
What is pathophys of GBS
Inflammatory process affects Schwann cells
Macrophages attach Schwann cells — primary demyelination of the axon but leaves axon intact usually — affects sensory, motor, and autonomic systems.
remyelination occurs rapidly
Patients might experience secondary …
Axon al damage due to lymphocytic infiltration and cyto-destructive events in the axon causing wallerian denervation of the distal axon.
Signs and symptoms of GBS
- Acute demyelination of both cranial and peripheral nerves/nerve roots
- Sensory loss, paresthesias (tingling, burning), pain. Sensory loss usually not as significant as motor loss.
- Motor paresis or paralysis relative symmetrical distribution of weakness, may produce full quadriplegia with respiratory failure if not treated which is how most people die. motor loss is ascending in nature
- Dysarthria, dysphasia, diplopia, and facial weakness may develop in more severe cases.
Describe GBS progression
- Progression of symptoms usually over a few days or weeks, usually ascending symptoms.
- Recovery is slow — months to 2 years but most regain function
- 10-20% have severe disability.
- 5% die
Describe management for GBS
- Medical management - plasmapheresis, IVG
- Interventions in the acute phase include: respiratory care, passive movements, positioning, splinting, gentle progressive strengthening exercises.
*in acute phase, more intensive strengthening and functional activities.
What are some features associated with poorer outcome?
- Requirement for respiratory support
- Abnormal peripheral nerve function - before or months after GBS
- No plasmapheresis or intravenous immune globulin performed
- Subgroup of GBS with primary atonal degeneration
- Patients with rapid onset
- Progression to quadriplegia
- Respiratory dependence
- Severe disease at presentation
- Patients showing no improvement at 3 weeks of plateau of disease.
What is one big thing we need to be aware of with treatment of GBS
- Fatigue like really severe fatigue. (Defined as a score of 5+ on the fatigue severity scale.
- Central fatigue in patients with GBS related to the decreased number of remaining motor units.
- Management of excessive fatigue includes use of energy conservation techniques, lifestyle changes, pacing, regular periods of naps during the day and improvement of sleep
- Exercise programs to reduce fatigue.
How would we want to schedule GBS in inpatient rehab
1.5 hours 2x/day — less fatigue this way getting up and going.
Once they improve and don’t have as much fatigue then you can do 3 30 minutes.
Treatment for sensory loss
Education of the patient and preventative program — positioning and moving off pressure points.
Treatment for autonomic dysfunction and pain
Symptoms include decreased sweating, OH, gastro paresis, constipation, vomiting diarrhea, impotence, and flaccid urinary bladder.
Treatment for neurogenic bladder and bowel
Pelvic floor training.
Treatment for physical deconditioning
Avoidance of overwork
because of transmission problems — fewer numbers of successfully recruited muscle fibers available to produce significant forces for function — increase force production in a muscle.
- There are newly re-myelinated nerves and less motor units so really don’t want to overwork them!!
How might excess muscle activity happen in people with GBS
- Spotty/patchy demyelination
- Abnormal remyelination
- Partial denervation
What are overworking symptoms
- DOMS — peaking between day 1 and 5 post activity
- Reduction in maximum force production that gradually recovers.
all caused by an inflammatory process resulting in a secondary muscle injury
What else should you avoid with treatment of muscles in GBS
- Avoidance of eccentric contractions
— probability of injury is greatest for exercise with eccentric contractions compared with isometric and concentric contractions
— Eccentric contractions are necessary for daily activities. Eccentrically train to resist muscle damage.
When can you progress to antigravity movement?
Wait until patients can move the weight of their limb against gravity, then external stress can be applied.
When can you start to practice gait with GBS patients
Until patient has at least a fair grade in the lower extremity musculature — have to be able to move the weight of their own leg against gravity.
Assessment of overall weakness
- Rest intervals, objective testing of strengths and evaluating complaints of muscle soreness are required.
- PT should at minimum assess the patient’s strength and muscle soreness complaints from one day to the next or evaluate the patient after a weekend of no therapy.
Submax strength training
Minimal number of repetitions should be performed.
Aerobic training
Carefully monitor for appropriate physiological responses.
- Patients may experience autonomic involvement
- Work the patient to a moderate exercise intensity level using the borg scale of perceived exertion
- Frequent rest periods with gradual increases in continuous exercise time.
Fast twitch fibers
Recruit/train fast twitch muscle fibers
EX: push for speed or rapid rises and falls in muscle force production such as fast walking, jumping, and quick changes in direction during walking and lunges.
not doing this week 2 — more of an outpatient thing
Multi-joint work
PT should encourage patients to move in a multi joint pattern from the beginning of the disease process.
- By linking multiple joints together for a movement, the patient reinforces muscle synergies where prime movers and stabilizers are active and coordination between joints with the patient’s new neuro system will be encouraged to develop from the onset.
Functional activity practice
Practice eccentric contractions with functional activities
Respiratory function in GBS
Concern for aspiration, weakness of muscles of inspiration, weakness of muscles expiration, pulmonary complications (embolism and pneumonia)
What is ALS
Rapidly progressive neuro degenerative disease of middle life that is rarely seen prior to age 40.
Disease of UMN and LMNs — loss of anterior horn cells in SC and the motor cranial nuclei in brainstem (LMN). Demyelination and gliosis of CST and CBT in motor cortex (UMN)
Major signs of ALS
LMN - muscle weakness, atrophy, fasciculations, hyporeflexia, muscle cramps
UMN - spasticity, hyperreflexia, pathological reflexes
Bulbar signs - nocturnal respiratory difficulty, exertional dyspnea, accessory muscle use, paradoxical breathing (usually what kills them)
Others - fatigue, weight loss, cachexia, tendon shortening, contractures, frozen shoulder