Guillain-Barré Syndrome (GBS)

Guillain-Barré Syndrome (GBS) is a rare neurological disorder, but one of the most commonly acquired polyneuropathies. The body’s immune system mistakenly attacks part of its peripheral nervous system (PNS) outside the brain and spinal cord. GBS symptoms can range from mild (brief weakness) to severe (paralysis). Most people with GBS recover, while some continue to have some degree of weakness.^1 2  Unlike other autoimmune diseases, GBS is typically monophasic and does not respond to immunosuppressive therapies such as corticosteroids.³ 

Etiology

In the US, an estimated 2 per 100,000 people acquire GBS.⁴ The exact cause of GBS is unknown. It can occur at any age with most at ages 30-50 being affected.⁵  Within a few days of onset, people may require intensive care ventilation and are unable to walk. Some infections associated with GBS include influenza, GI illnesses, pneumonia, HIV, herpes simplex, mononucleosis as well as lupius, Hodgkin disease, and after some surgeries. In contrast, other neuropathies progress slowly over a period of years and many cause no motor deficits. GBS often follows a diarrheal or respiratory illness (infection) and is characterized by ascending paralysis and hyporeflexia or areflexia with variable sensory and autonomic involvement.⁶  Besides infection, there may be immune stimulation that induces an autoimmune response that targets peripheral nerves and their spinal roots.⁷  In rare cases, vaccinations may increase the risk of GBS.²  This is a controversial topic that was first raised in 1976 with the swine flu vaccination. Data from two large US studies monitoring H1N1 vaccinations do not support an increased risk of GBS seen with other influenza vaccines. Other vaccines have been implicated as potential triggers, but the evidence is lacking.^8 9 

The current concern is whether COVID-19 or its vaccinations may increase the risk of GBS. No causal link was found with an increase in GBS during the first wave of COVID-19.¹⁰  “Generally, no contraindication to the vaccination of patients who previously have had Guillain-Barré syndrome seems to exist, except for patients who had had the disorder in the past 3 months or had vaccination-related Guillain-Barré syndrome, although risk and benefit might be discussed on a case-by-case basis.”¹¹ 

“Within a population of 1 billion people, one would expect about 17,000 cases of GBS to occur sporadically per annum, of which 1962 would occur in any 6-week period. When considering a more optimistic 4-billion-person immunization programme conducted over 1 year, 68 000 cases of GBS would be expected to occur naturally within this time period, irrespective of any vaccination programme. Of these GBS cases, 13 076 would occur in the 10-week window following double-dose vaccination with injections separated by 4 weeks. It is therefore inevitable that many thousands of sporadic cases of GBS caused by other factors will appear temporally associated with COVID-19 vaccination. But, as any statistician can confirm, this cannot be considered causal.”¹² 

Pathology

GBS often affects the myelin sheath (demyelination) as well as damage to other parts of the nerve (axons) which can cause it to not function altogether.⁵  Activated lymphocytes T and antibodies may contribute to the pathogenesis.¹  A few microorganisms have been identified to be associated with GBS: Campylobacter jejuni, Cytomegalovirus, Mycoplasma pneumoniae, Epstein-Barr virus, and Haemophilus influenza.¹³  GBS seems to be environmental and not associated with genetic inheritance. 30% of cases have no specific triggering factor.¹ 

GBS Subtypes

GBS is not a single disease state, but represents a spectrum of conditions that affect the peripheral nerves.⁶ 

Acute Inflammatory Demyelinating Polyradiculopathy (AIDP)

• The most common form of GBS accounts for 90% or more cases.
• Demyelination
• Prolonged distal motor latencies
• Conduction slowing or black
• Time dispersion of action potentials and F-wave latencies
• Abnormal sensory nerves.
• Inflammation¹⁴ 

Acute Motor Axonal Neuropathy (AMAN)

• Distinguished from AIDP for axonal pathology rather than demyelinating pathology
• Lack of sensory nerve involvement^15 16 
• Less common than AIDP
• Predominant form of GBS in Asia and Mexico (65% of patients) often in the summer.
• Associated with C. jejuni infection.
• Macrophages attack the nodes of Ranvier and invade periaxonally.^17 18 
• Patients usually recover as the damage to the axon is short-lived with a temporary blockage of conduction.
• Distal axonal degeneration may occur.
• Patients may be severely affected.¹⁷ 

Acute Motor and Sensory Axonal Neuropathy (AMSAN)

• Coexistent motor and sensory involvement.
• Often very severe.
• Similar to AMAN with ventral nerve root and peripheral motor axons targeted by macrophages.
• Dorsal nerve roots and sensory fibers are involved.
• Likely an antibody-mediated pathogenesis due to the presence of antibodies.¹⁹ 

Miller Fisher Syndrome (MFS)

• May be preceded by a viral infection.
• A unique antibody characterized the disorder
• Diminished sensory nerve conduction test; MRI and imagining otherwise normal
• Abnormal muscle coordination, balance, and gait (waddling, duck-like) ²⁰ 
• Paralysis of eye muscles
• Absent deep tendon reflexes, e.g., knee and ankle jerk
• Generalized muscle weakness
• Respiratory failure
• Treatment with intravenous immunoglobulin (IVIg) to remove antibodies from the blood
• Prognosis is good – most cases recover within 6 months
• Some individuals are left with residual deficits
• Relapses may occur (less than 3% of cases).²¹ 

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

• Characterized by symmetrical weakness and sensory changes.
• 1.5-3.6 cases per 1,000,000 annually.
• May persist for years
• In contrast to GBS, symptoms develop slowly over the course of months.
• Relapses and remissions of ascending weakness over the course of years.
• Breathing, swallowing, and speaking are spared.²² 
• Symptoms: gradual weakness and tingling in limbs, loss of reflexes, loss of balance and ambulation, loss of sensation.²³ 
• May be slow progressive deteriorating without improvement.
• Reinnervation and long-term disability are worse than GBS.
• Treatment: corticosteroids, plasmaphersis, IVIg.²⁴ 

Multifocal Motor Neuropathy (MMN)

• Rare chronic inflammatory neuropathy.
• Slow and variable presentation.
• Inflammation of motor nerves with slowed conduction.
• Diagnosed with nerve conduction tests and selective nerve mapping.
• Disorder may last as long as 20 years or more.
• Episodes of left- or right-sided asymmetric distal limb weakness.
• Muscle atrophy and fasciculations.
• Upper more than lower limbs are affected.
• Weakness may occur at the wrist, fingers, and/or ankles
• Treatment: IVig and immunosuppressive drugs.
• OT treatment of GBS and CIDP also applies to MMN with a customized plan of care.²⁴ 

Acute Pandysautonomia

• Sympathetic and parasympathetic failure
• Enteric (intestinal) functions affected
• Sparing of somatic nerve fibers – minimal to no somatosensory and motor impairment²⁵ 
• May be preceded by infection, vaccination, surgery, cancer
• Environmental exposures may play a role.
• Autoimmune leading to autonomic ganglionopathy
• Often a poor prognosis with chronic debilitation
• Symptoms: Syncope, Loss of accommodation, Pupillotonia, Lacrimation, Swallowing difficulties
• GI symptoms²⁶ 
• Postural orthostatic tachycardia syndrome (POTS) ²⁷ 
• Treatment involves immunomodulation (IVIg).²⁸ 

Prognosis

Most people recovery reasonably well, but recovery may take months or years. Those with respiratory muscle involvement are at a high risk of complications and mortality at 2.7%. Permanent impairment is seen in 15-20% of cases.⁶ 

Symptoms

• Nerve damage may cause tingling, muscle weakness, loss of balance, and paralysis.
• Sx depends on the subtype of GBS.

General Symptoms

• Muscle weakness
• Loss of muscle function
• Often symmetrical
• Starts in legs and spreads to the arms (ascending paralysis).
• May affect chest and diaphragm
• Loss of deep tendon reflexes in limbs
• Tingling or numbness
• Muscle tenderness
• Muscle pain
• Uncoordinated movement
• Low blood pressure
• Abnormal heart rate
• Blurred vision or diplopia
• Facial paralysis
• Palpitations

Emergency Symptoms

• Breathing temporarily stops
• Cannot take a deep breath
• Dyspnea
• Dysphagia
• Drooling
• Syncope
• Lightheaded when standing⁵ 

Psychosocial Symptoms

• Fear
• Anxiety
• Depression
• Guilt
• Low motivation²² 

Complications

• Respiratory failure
• Contractures and deformities
• Blood clots
• Infection
• Low or unstable blood pressure
• Permanent paralysis
• Pneumonia
• Skin ulcers
• Aspiration⁵ 

Treatment

• No cure – treatment is symptom-focused
• Apheresis or Plasmapheresis – removing/blocking antibodies that attack nerve cells
• Intravenous immunoglobulin (IVIg)
• Blood thinners for thrombosis prophylaxis
• Breathing support, e.g., ventilator
• Pain medications and interventions
• Body positioning
• Feeding tube
• Therapy⁵ 

Occupational Therapy

• Educate and provide helpful, clear, and comprehensive information on GBS to clients and their families.
• Provide clients and their families (each family member) with emotional support and encouragement. Strike a balance between giving time and space and knowing when to refer to other disciplines.
• Set realistic goals with an open discussion with client and family of prognosis, rehab goals, and possible functional outcomes.^29 30 
• Use faith to provide support, if appropriate.
• Recovery pattern is proximal to distal.
• Maintain joint and muscle function. Involve family members, e.g., PROM. Explain the importance but also caution clients that overstretching and overexerting may increase the rate of peripheral nerve demyelination.³¹ 
• Use intrinsic motivation to enhance performance and participation.^32 33 
• Use supportive equipment and functional adaptations to resume activities.
• Use a safety-first approach.²² 
• Splint paralyzed limbs to alleviate muscle and joint pain³⁴ ; maintain function, e.g., wrist cock-up splint, resting hand splint³⁵ 

Outcome Measures

• Barthel Index³⁶ 
• Rate of Perceived Exertion Borg Scale

Evaluation

• Patient and caregiver interview
• Sensory assessment: be sensitive of painful touch and causing pain; reassess frequently to track progress
• Skin inspection: check for lesions and pressure spots
• Joint ROM
• MMT: also assess grip and pinch strength
• Functional testing: ADLs, transfers, client’s work, and leisure
• Mobility
• Endurance and fatigue
• Autonomic dysfunction: dizziness, blood pressure, heart rate, orthostatic hypotension²² 

Acute Stage Treatment

• Provide patient and caregiver education and training.
• Prevent contractures, DVT, and bedsores.
• Ensure proper positioning – change Q2 with pressure relief when sitting.
• Avoid prolonged hip and knee flexion.
• Support weak upper extremities: armrests, wheelchair trays, pillows.
• Anticipate needs for assistive devices, other adaptive equipment and assistive technology – communication, function, ADLs, and comfort.
• Use gentle passive ROM.
• Introduce breathing and coughing exercises to maintain eupnea.²⁴ 
• Initiate ADLs if appropriate and safe; provide supervision and assistance when necessary.^37 29 
• Address intercostal muscle weakness as part of functional activities: controlled deep breathing, bridging, dissociated rolling exercise.³¹ 

Recovery Stage Treatment

• Target and improve sitting, standing balance, core muscle strength (abdominal muscles), and muscle endurance.³⁸ 
  • Progress from PROM to AAROM.
  • Low repetitions and resistance with frequent rest breaks.
  • Powder boards, slings, hydrotherapy with exercise.
  • PNF
  • Avoid exercising to fatigue which may delay recovery.
  • Educate on avoiding “no pain, no gain”²² 
• Practice in real-world environment and contexts with meaningful activities, e.g., instead of cones and peg boards, do bed mobility, transfers, gait, W/C mobility, sitting and standing balance while reaching, dressing, feeding, bathing, toileting, writing, typing, leisure, or reintegration into work.
• Pain and sensation: use PAMS – TENS, heat packs, sensory desensitization that is customized to each client and their preferences.
• Establish a home program along with the client and caregivers.
• Address the risk of falls due to decreased strength.
• Provide a variety of additional activities to promote gross-, fine-motor, and sensory stimulation or desensitization.²² 
• Address fatigue for ADLs and transfers.^38 39 40 
• Use energy conservation techniques during activities.
• Use graded activities with rest breaks to improve muscular and cardiovascular endurance.^{41 42 43 44 45 46} 
• Improve intrinsic hand muscles.

 

Bookmark

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