Stanbridge - T6 - Neuro2 - W8 - GDS, CIDP, & PPS

• Neurological Interventions II, PTA 1015

Neurological Disorders Covered

• Guillain Barre Syndrome
• Chronic Inflammatory Demyelinating Polyneuropathy
• Post-polio Syndrome

Learning Objectives

• Describe the incidence, etiology, and clinical manifestations of persons with Guillain-Barre Syndrome (GBS), Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), and Post-polio syndrome (PPS).
• Understand the typical medical and surgical management for GBS, CIDP, and PPS.
• Identify specific treatment interventions relative to the stage or degree of progression, activity limitations, and participation restrictions for GBS, CIDP, and PPS.
• Discuss strategies for patient/family education to address the functional limitations in persons with GBS, CIDP, and PPS.

Guillain-Barre Syndrome (GBS)

• An acute inflammatory demyelinating polyradiculoneuropathy disorder involving progressive muscle weakness or paralysis.
• This is a type of lower motor neuron disease.

GBS - Epidemiology

• Most individuals have had a respiratory or GI illness prior to onset.
• Incidence is 1.2 - 2.3 per 100,000 people.
• It is not gender or age specific.
• Multiple potential causal agents have been linked to viruses, vaccinations, bacterium, and surgery.
• Has a good overall prognosis and recovery.

GBS - Pathophysiology

• Myelin is destroyed accompanied with inflammation.
• Lesions are present within several days of the onset of symptoms.
• Nerve conduction slowed and may be blocked completely
• Schwann cells are destroyed but axons are left intact.
• In most cases, 2-3 weeks after onset, Schwann cells proliferate, and re-myelination begins.

GBS Medical Management Includes

• Plasmapheresis is performed by removing blood, separating RBCs/WBCs from the plasma, and returning the blood cells to the body.
• The theory is that this process eliminates immune factors allowing disease progression.
• Infusion of Intravenous Immunoglobulins (IVGs) are both performed within the first 2 weeks of the disease course to shorten the progression.

GBS – Clinical Presentation

• Involves symmetrical ascending progressive loss of motor function.
• The condition begins distally and progresses proximally.
• Distal paresthesias (burning/tingling) or hypesthesias (abnormal sensitivity to touch) are symptoms.
• Motor is more affected than sensory functions.
• If the diaphragm is involved, the patient will need mechanical ventilation.
• 50% of patients experience autonomic impairment i.e. fluctuating BP, poor venous return, tachycardia, and arrhythmias are symptoms.
• Other common clinical presentations include:
• Tachycardia, arrhythmias
• Myalgia (pain in muscles)
• Dysarthria and dysphagia
• Facial weakness (CN VII)
• Diplopia (CN III, IV, VI)
• Absent DTR's due to demyelination of peripheral nerves = areflexia
• GBS has three phases:
• Acute phase: lasts 1-4 weeks - where symptoms appear and worsen.
• Plateau phase: lasts 4 weeks - once symptoms stabilization occurs.
• Recovery phase: lasts up to 1 year - symptoms improve.
• Patients who need ventilatory support, have rapid demyelination, and low distal motor amplitudes on EMG tend to have a poorer outcome.

GBS – Physical Therapy Management - Acute Phase

• Acute phase lasts 1-4 weeks.
• Patients are hospitalized during this phase.
• PT goals include:
• Minimize acute signs/ symptoms
• Support pulmonary function
• Prevent skin breakdown and contracture formation.
• Manage pain
• Interventions during the acute phase include:
• PROM at least 2x/day
• Positioning, postural drainage and percussion.
• STM and TENS for pain control

GBS – Physical Therapy Management - Plateau Phase

• Plateau phase lasts 3-4 weeks after onset.
• Stabilization of respiratory and autonomic functions occurs.
• PT Goals include:
• Acclimation to upright positions
• Maintenance of ROM
• Improve pulmonary function
• Prevent fatigue and overexertion
• Interventions include:
• Functional training to acclimate to the upright posture
• PROM
• Positioning to decrease contractures

GBS – Physical Therapy Management - Recovery Phase

• Recovery phase can last up to one year.
• PT Goals include:
• Strengthening (using the non-fatiguing protocol)
• Maximizing functional abilities
• Carry over of goals from previous phases
• Interventions include:
• Short periods of non-fatiguing exercises matched to patient's strength
• Exercises should be matched at increasing function rather than solely on strength
• Perform low repetitions and short, frequent bouts of exercise
• Involve gravity eliminated exercises

Non-fatiguing Exercise Principles

• Short periods of non-fatiguing exercise matched to patient's strength
• Exercises should be matched at increasing function rather than solely on strength
• Low repetitions and short, frequent bouts of exercise
• Gravity eliminated exercises
• Additional interventions that include gait training and ambulation once muscle grade is 3/5 in LEs, adaptive equipment, lightweight orthotics and endurance training
• Signs of overwork weakness:
• Delayed onset of muscle soreness, which gets worse one to five days after exercising
• Reduction in the max amount of force the muscle is able to generate
• Additional Recommendations:
• Avoid overworking the patient.
• Return the patient to bed if function or strength declines.
• Note that residual weakness in distal muscles may be present such as the ankle/foot, or wrist/hand.
• Return of muscle strength occurs in a descending order

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

• A rare neurological disorder in which there is inflammation of nerve roots and peripheral nerves.
• There is a destruction of the fatty protective covering (myelin sheath) of the nerve fibers.
• The etiology is mostly idiopathic but there are indications it is an autoimmune disorder.
• There are some cases in which a respiratory or GI infection may have precipitated the condition.
• Generally considered to be the chronic counterpart of GBS.
• Incidence:
• Number of new cases per year is 1-8 per 100,000 people.
• The disease may be present in a person for years prior to diagnosis.
• Actual prevalence of disease may be higher.
• Peak incidence occurs between 40-60 years of age.
• Occurs more in males than females (2:1).
• Typical symptoms:
• Progressive and gradual onset of symmetric motor and sensory disorder that has proximal and distal weakness.
• Notice the DTRs are absent.
• Atypical variants include:
• Multifocal: deficits are asymmetrical and may involve individual nerves.
• Pure sensory variants: imbalance and incoordination, but no muscle weakness.
• Distal variant: sensory and motor only affect the hands and feet.
• Pure motor variant: no sensory abnormality is found.

CIDP - Diagnosis

• This is a condition that can be difficult to diagnose.
• Symptoms must be present for at least 2 months for a diagnosis.
• Proximal and distal weakness that is symmetrical with hyporeflexia or areflexia is highly suggestive of CIDP.
• Diagnostic tests can include:
• Blood and urine tests
• Nerve conduction velocity testing
• Electromyography (EMG)
• Lumbar puncture and MRI of spine
• Nerve biopsy (rarely done)

CIDP Medical Treatments

• Glucocorticoids (steroids) such as Prednisone
• Some patients with CIDP may respond positively to this medication alone.
• It maybe used in conjunction with immunosuppressive drugs.
• Intravenous immunoglobulin (IVIg), in high doses, is prescribed for initial treatment.
• Patients will require ongoing intermittent treatments.
• Plasma exchange (PLEX) removes immunoglobulins and other components of immune response from the blood.
• Like IVIg, PLEx is effective for a few weeks and may require ongoing intermittent treatments.
• Immunosuppressive agents such as: azathioprine, mycophenolate, methotrexate, cyclosporine and cyclophosphamide.

CIDP - Physical Therapy Management

• Unlike GBS, patients will begin PT in an outpatient clinic or home-health setting.
• Patients will only be admitted to a hospital if they experience a relapse, or exacerbation of their symptoms.
• PT Goals:
• Facilitate optimal use of muscles and prevent disuse atrophy.
• Improve fitness and energy levels.
• Prevent secondary complications such as muscle contractures and chest infections.
• PT Interventions:
• Therapeutic exercises and activities to promote strengthening (follow non-fatiguing protocol).
• Aerobic conditioning (follow non-fatiguing protocol).
• Balance and gait training
• Patient education on proper and safe use of assistive devices.
• Manual therapy may be indicated to prevent joint contracture and maintain available ROM
• Modalities for pain management (e.g. TENS).

Managing a CIDP Relapse

• During a relapse or exacerbation of CIDP, the patient may not tolerate or be able to participate in active movement.
• PT goals and intervention will be the same as GBS - Acute stage.
• Which include:
• Minimize acute signs/symptoms; support pulmonary function; prevent skin breakdown/contracture formation; and manage pain.
• During a CIDP relapse, perform PROM at least 2x/day, positioning, postural drainage/percussion; and STM/TENS for pain control.
• Physical Therapy - Additional Recommendations:
• Like GBS, it is important that patients with CIDP also do not over exert themselves with exercise.
• DOMS is expected, but if soreness or pain that persists longer than 12-48 hours, with or without a loss of strength, may be a sign of overexertion.

Post-polio Syndrome (PPS)

• Denotes the late effects of poliomyelitis.
• Polio is a viral infection that attacks the anterior horn cells in the spinal cord and results in muscular paralysis.

PPS – Epidemiology

• Polio was an epidemic in the US from 1910 to 1959.
• The viral spinal cord infection caused paralysis in the body.
• PPS affects between 25-40 out of every 100 polio survivors (as per the CDC).

PPS – Etiology

• Theory is that PSS is caused by decades of increased metabolic demand made on the body by giant motor units .
• These giant motor units were formed in the recovery process from the original infection.
• As the body ages, it is unable to keep up with the repair process of the large motor units and they begin to fail.

PPS – Diagnosis

• Polio DX is used to diagnose.
• Positive neurologic exam or electromyogram (EMG).
• There must be a period of relative stability lasting 15 years.
• New onset of neurologic weakness and fatigue must last for at least a year unexplained by any other pathology.

Common PPS Clinical Features

• Common signs and symptoms include:
• Fatigue, new weakness and pain.
• Losing muscle tissue (atrophy)
• Other Symptoms include:
• Cold Intolerance
• Decreased Function

PPS – Medical Management

• No medications have proven effective.
• Medical management is indicated to focus on managing signs and symptoms through:
• Lifestyle changes: healthy diet, exercise in moderation, staying warm and treatment for sleep apnea.

PPS – Physical Therapy Management

• Goals for PT:
• Decrease workload on muscles.
• Avoid fatigue and help the patient ambulate safely.
• Achieve optimal level of function.
• Patient/Family Education.
• From the American Academy of Neurologic Physical Therapy (DDSIG):
• Exercise should not cause muscle soreness or pain.
• Exercise shouldn't lead to fatigue that prevents participation in other activities that day or days following
• Strengthening exercises should only be attempted with muscles that can move through their full ROM.
• Minimal to moderate intensity exercise is recommended and the progress of exercise should be slow.
• Aerobic Exercise
• Should not cause muscle fatigue, generalized fatigue, muscle soreness or pain
• Recommended duration:
• 3-4 times per week building up to a total of 30 minutes each session
• Mode of exercise:
• Walking over ground or on a TM for individuals who do not have symptoms of leg weakness or pain.
• UBE or arm bike for individuals whose arms are strong but have symptoms of leg weakness.
• Stationary bike if UE weakness is primary problem, or balance problems limit safe ambulation.
• Intensity:
• Light to moderate intensity
• Stretching:
• May be used to combat pain or cramping from overuse.
• Increase ROM that must be supported by strengthening for carry over.
• Exercise
• Strengthen but not induce muscle fatigue
• Aquatics, short Intervals with rest breaks between, every other day and conditioning/endurance.
• Use low intensity.

PPS – Physical Therapy Management - Energy Conservation

• Modify lifestyle to prevent fatigue.
• Pacing activities.
• Incorporate more frequent rest breaks.
• Note that modifications will be different for each patient.

PPS – Physical Therapy Management - Pain Management

• Cramping - Gentle stretching following the application of heat is recommended.
• Musculoskeletal issues - Structures must be identified first: muscle, tendon, bursa, and/or fascia; treat accordingly once identified.
• Biomechanical issues can result from degenerative joint changes, LBP and nerve compression.
• Postural adjustments should be made and recommendations of ADs to Orthotics to improve alignment can also be useful.
• • Note: a Patient may have a combination of any of these contributing factors.
• Orthotics can also be helpful.
• Ankle Foot Orthoses
• A solid polypropylene AFO controls genu recurvatum associated with weakness
• Hinged AFO with 90-degree posterior stop and free DF controls genu recurvatum associated with weakness.
• Floor reaction AFO with DF stop prevents tibial collapse during the mid and terminal stance.
• KAFO with drop ring lock in stance phase prevents genu recurvatum but allows for free knee flexion during swing phases of gait
• The use of Loft strand crutches and front wheeled walkers are assistive device options.

PPS – Physical Therapy Management

• Lifestyle Modification Recommendations:
• Reduce physical and emotional stress.
• Joint protection.
• Modification of work/home environments.
• Use of mobility aids to reduce and preserve function.
• Energy conservation.

PPS – Complications to be Aware Of

• Falls – Weakness in the lower extremity increases the risk for falls and potential injury
• Chronic respiratory failure – Weakness in diaphragm and chest muscles will make it harder to take deep breaths and cough
• Osteoporosis – Prolonged inactivity and immobility are often associated with loss of bone density.
• Sleep disorders – Sleep apnea and Restless Leg Syndrome common in people with PPS.
• Malnutrition, dehydration and pneumonia – Individuals who had bulbar polio may experience difficulty with chewing and swallowing