Encyclopedia of Clinical Neuropsychology

Living Edition
| Editors: Jeffrey Kreutzer, John DeLuca, Bruce Caplan

Guillain-Barré Syndrome

  • Khan Fary
  • Louisa Ng
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-56782-2_554-3

Synonyms

Short Description or Definition

Guillain-Barré syndrome (GBS) is a monophasic illness of immune etiology that presents as an evolving sensorimotor polyneuropathy of varying severity as a result of an inflammation of the peripheral nerves and nerve roots. GBS is a major cause of acute ascending neuromuscular paralysis and is often associated with autonomic dysfunction.

Categorization

GBS is recognized as a heterogeneous syndrome with several variant forms (see Table 1). The commonest type of GBS is AIDP. Axonal subtypes include acute motor axonal neuropathy and acute motor and sensory axonal neuropathy. Variants include Miller Fisher syndrome (cranial nerve involvement, ataxia) and acute pan-dysautonomia.
Table 1

Frequency of features and clinical variants of acute GBS (Reprinted with permission from Ropper (1992))

 

% Frequency

Condition

Initially

Fully developed illness

Features of syndrome

  

Paresthesia

70

85

Weakness

  

Arms

20

90

Legs

60

95

Face

35

60

Oropharynx

25

50

Opthalmoparesis

5

15

Sphincter dysfunction

15

5

Ataxia

10

15

Areflexia

75

90

Pain

25

30

Sensory loss

40

75

Respiratory failure

10

30

CSF protein >0.55 g/l

50

90

Abnormal electrophysiologic findings

95

99

Clinical variantsa

  

Fisher’s syndrome

 

5

Weakness without paresthesia or loss

 

3

Pharyngeal-cervical-brachial weakness

 

3

Paraparesis

 

2

Facial paresis with paresthesia

 

1

Pure ataxia

 

1

aVariants are associated with diminished reflexes, demyelinating features as detected on electrophysiologic studies, and elevated cerebrospinal concentrations of fluid protein. Frequencies shown are those found in fully developed illness

Epidemiology

GBS has an annual incidence of 1–2 per 100,000 worldwide with no geographical clustering. It affects both sexes equally and occurs at all ages, although most commonly between 30 and 50.

Natural History, Prognostic Factors, and Outcomes

The first clinical description of ascending paralytic illness was by Osler (1892), while Guillain et al. (1916) reported the syndrome of radiculoneuritis associated with elevated protein in the cerebrospinal fluid. The etiology of GBS is not fully understood. It is thought that an antecedent infection (most commonly Campylobacter jejuni) evokes an immune response (both cellular and humoral), resulting in demyelination. Within 2–3 weeks, the inflammation resolves and remyelination commences.

GBS generally has a favorable outcome. Eighty percent are ambulatory (ambulation without assistive devices) within 6 months of symptom onset. Fifty percent have minor residual neurological deficits, while 15% have residual functional deficits, and 5% may die of secondary systemic organ failure (Ropper 1992). In the acute phase of GBS, 5–10% may die and 25% require artificial ventilation owing to involvement of respiratory and bulbar muscles (Hughes et al. 2006). The onset, rate, and variability of recovery is unclear (Meythaler 1997), but 80% reach the nadir of the disease at 3 weeks after symptom onset (Pascuzzi and Fleck 1997) and subsequently gradually improve. GBS survivors can continue to improve for 10 years after onset, although slower recovery may occur in those who experienced ICU complications, prolonged mechanical ventilation, and early axonal abnormalities (Dhar et al. 2008).

Poorer prognostic factors are older age, need for respiratory support, rapid onset, progression to quadriplegia, severe disease at presentation, C. jejuni infection, and preceding diarrheal illness (Rees et al. 1995).

Although most patients make good physical recovery, the ongoing impact of GBS is significant. A longitudinal study (n = 76 patients) reported that despite good functional recovery up to 14 years post GBS (median 6 years, range 1–14), 16% of patients continued to report moderate to extreme impact on work, family, and social activities, and 22% reported ongoing substantial impact on mood, confidence, and ability to live independently (Khan et al. 2010a). Psychosocial performance does not necessarily correlate with the severity of impairment in GBS but may be explained by poor conditioning and fatigue (Forsberg et al. 2005).

Neuropsychology and Psychology of Guillain-Barré Syndrome

The psychological, social, and avocational outcomes of GBS are poorly studied. High levels of anxiety (82%), acute stress disorder, depressive episodes (67%), and brief reactive psychosis (25%) in an ICU setting have been reported (Weiss et al. 2002). Ongoing anxiety and fear is common due to sudden onset of symptoms and may accompany with depression in many (Hallum 2007). Psychotic symptoms (hallucinations, delusions, incoherence) have been reported in more severe patients (Hallum 2007) and is closely related to motor deprivation and loss of communication. Therapeutically, continuous psychosocial support and psychopharmacological measures may be valuable tools to ameliorate distress (Weiss et al. 2002).

Cognitive problems have been reported in pwGBS, especially in acute phase and in those with extended ICU stay (Hallum 2007; Khan and Ng 2009). Hydrocephalus and intracranial hypertension in acute stages of GBS are infrequent but well-recognized complications and often resolve spontaneously or after ventriculoperitoneal shunting. Brain imaging is necessary in GBS patients who deteriorate in cognitive function after disease onset, and neuropsychological evaluation should be considered (Liu et al. 2006). Early cognitive screening and implementation of psychological interventions such as good communication with patients and/or their family regarding prognosis and treatment plan, involvement in decision-making, and early counselling from experts (Hallum 2007; Khan and Ng 2009).

GBS has a significant impact on patients’ lives, which goes beyond their residual disability or impairment. Five years on, 27% continue to make substantial changes in their job, hobbies, or social activities despite near complete functional recovery (Bersano et al. 2006). In the transition period following discharge from hospital to the community, various adjustment issues may surface, such as perceptions of self-worth and self-image and role reversals within the family. Families may struggle to cope with new demands resulting from increased care needs, difficulties in returning to driving and work, financial worries, marital stress, and other limitations in societal participation. Ongoing monitoring, education, and counselling of the patient (and family) are important.

Carer support and respite care should be discussed. Referral to local GBS Society and support groups (GBS survivors) is also recommended, as these provide patients and families with ongoing support, resources, and equipment needs.

Evaluation

The features of GBS are shown in Table 1. Most patients present with symmetric limb weakness, absent (or diminished) reflexes, paresthesias and aching pain in large muscles of the back and legs, or sciatica (Ropper 1992). Thirty percent of patients run a fulminating course with rapid progression requiring ventilatory support within a couple of days (Ropper). Disease progression may be stuttering or insidious and generally continues over the next 2 weeks until it plateaus and is subsequently followed by a gradual recovery. The duration of illness in most patients is <12 weeks.

Weakness often commences distally and continues in an ascending fashion, resulting in variable weakness in the limbs, face, and oropharynx (Rees et al. 1995). Facial weakness and cranial nerve involvement occur in >50% (Pascuzzi and Fleck 1997) and 70% have autonomic dysfunction (sinus tachycardia or bradycardia, fluctuating hypertension or hypotension, flushing of the face, loss of or excessive sweating) (Zochodne 1994). Severe dysautonomia should be recognized as this is associated with sudden death (Zochodne). Unusual features of GBS include papilledema, facial myokymia, hearing loss, meningeal signs, vocal cord paralysis, and mental status changes.

The initial diagnosis of GBS is based on clinical presentation (see Table 2). Findings on cerebrospinal fluid examination and neurophysiology studies may then be used to confirm the diagnosis (see Tables 2 and 3).
Table 2

Diagnostic criteria for typical GBS (Adapted and reprinted with permission from Asbury and Cornblath (1990))

Features required for diagnosis

Progressive weakness in both arms and both legs

Areflexia

Features strongly supporting the diagnosis

Progression of symptoms over days to 4 weeks

Relative symmetry of symptoms

Mild sensory symptoms or signs

Cranial nerve involvement, especially bilateral weakness of facial muscles

Recovery beginning 2–4 weeks after progression ceases

Autonomic dysfunction

Absence of fever at the onset

Elevated concentration of protein in cerebrospinal fluid, with fewer than 10 cells/mm3

Typical electrodiagnostic features

Features making the diagnosis doubtful

Diagnosis of botulism, myasthenia, poliomyelitis, or toxic neuropathy

Abnormal porphyrin metabolism

Recent diphtheria

Purely sensory syndrome, without weakness

Table 3

Proposed electrodiagnostic criteria for demyelination of peripheral nerve (Reprinted with permission from Asbury and Cornblath (1990))

These criteria concern nerve conduction studies (including proximal nerve segments) in which the predominant process is demyelination

Must have three of the following four features:

1. Reduction in conduction velocity in two or more motor nerves

(a) <80% of lower limit of normal (LLN) if amplitude >80% of LLN

(b) <70% of LLN if amplitude <80% of LLN

2. Conduction block or abnormal temporal dispersion in one or more motor nerves: either peroneal nerve between the ankle and below fibular head, median nerve between the wrist and elbow, or ulnar nerve between the wrist and below elbow

Criteria for partial conduction block:

(a) <15% change in duration between proximal and distal sites and >20% drop in negative-peak area of peak-to-peak amplitude between proximal and distal sites

Criteria for abnormal temporal dispersion and possible conduction block:

(a) >15% change in duration between proximal and distal sites and >20% drop in negative-peak area or peak-to-peak amplitude between proximal and distal sites

3. Prolonged distal latencies in two or more nerves

(a) >125% of upper limit or normal (ULN) if amplitude >80% of LLN

(b) >150% of ULN if amplitude <80% of LLN

4. Absent F-waves or prolonged minimum F-wave latencies (10–15 trials) in two or more motor nerves

(a) >120% of ULN if amplitude >80% of LLN

(b) >150% of ULN if amplitude <80% of LLN

The differential diagnosis of GBS is shown in Table 4 CIDP and relapsing inflammatory polyneuropathy are distinct from GBS. These are important from the rehabilitation standpoint, as their diagnosis and prognosis is different (Meythaler 1997).
Table 4

Differential diagnoses of GBS

Acute polyneuropathy

 Vasculitis

 Lyme disease

 Porphyria

 Sarcoidosis

 Paraneoplastic disease

 Acute arsenic poisoning

 Critical illness

Diseases of the spinal cord

 Spinal cord compression

 Transverse myelitis

Diseases of the neuromuscular junction

 Botulism

 Myasthenia gravis

 Lambert-Eaton syndrome

Diseases of muscle

 Acute polymyositis

 Critical illness myopathy or neuropathy

Treatment

Specialist units have improved survival rates in patients with GBS and reduced mortality rate to less than 5% (Ropper 1992). Acute medical complications in GBS include development of sepsis, respiratory failure, aspiration pneumonia, deep vein thrombus, pulmonary embolism, and cardiac arrest (Dhar et al. 2008).

Plasmapheresis, Intravenous Immunoglobulin, and Steroids

Acute management of GBS includes intensive care and ventilatory support, and immune-modulating treatments (plasma exchange and intravenous immunoglobulin (IVIg)) to reduce nerve damage. Early treatment with either IVIg or plasma exchange (both are equally effective) within 2–4 weeks after symptom onset may decrease recovery time (Hughes et al. 2003), although there is a higher risk of relapse following treatment with IVIg.

Rehabilitation

Once medically stable, approximately 40% of patients with GBS need inpatient rehabilitation, particularly patients requiring ventilatory support, more severe disability, and medical complications (Meythaler 1997).

Medical complications from GBS such as dysautonomia, cranial nerve involvement, early de-afferent pain syndromes (Meythaler 1997), and complications resulting from immobility, pneumonia, deep vein thrombus, and musculoskeletal deconditioning (Dhar et al. 2008) can persist for some time and interfere with rehabilitation. Multidisciplinary rehabilitation programs in inpatient settings are effective in terms of improvements in activity (disability) and participation for up to 6 months (Khan et al. 2010b). A high-intensity (two to three times per week) outpatient rehabilitation program in the post-acute phase (median 6.5 years since diagnosis) is more effective than a low-intensity program in reducing motor disability (mobility, self-care, continence program) and improving participation (relationships) (Khan et al. 2011).

Supportive Ventilatory Care

Up to 30% of patients develop respiratory failure and pneumonia in the first 12 weeks of illness and many will recover adequately (Ropper 1992). Those requiring ventilatory support have longer hospital lengths of stay and increased rehabilitation costs (Meythaler 1997).

Patients are monitored closely in rehabilitation settings for signs of respiratory distress. Intubation is required if the vital capacity decreases to <18 ml/kg, maximum inspiratory and expiratory pressures are < than 30 cmH2O, and < than 40 cmH2O, respectively (Lawn et al. 2001). Weaning from ventilation depends on improvements in strength and serial lung function testing; tracheostomy may be required if lung function tests show no improvements from baseline at the 2–3-week mark (Hughes et al. 2003). Pulse oximetry and BiPAP may be indicated at nighttime for patients with hypoxia or hypercapnia. Physical therapy addresses the clearance of respiratory secretions, reduction in the work of breathing, breathing exercises, and resistive inspiratory training. Special weaning protocols are required to prevent over fatigue of respiratory muscles in tracheostomy patients (Meythaler 1997). Those with cranial nerve involvement are at risk of aspiration and respiratory complications.

Deep Venous Thrombus (DVT)

Pulmonary embolism can occur in up to a third of patients with GBS (Raman et al. 1971). The exact incidence or risk factors for DVT in GBS are unknown. Although prophylaxis (such as subcutaneous heparin and support stockings) is recommended, the type and length of prophylaxis is unclear. In rehabilitation settings, early mobilization using progressive mobilization strategies for improving bed mobility, practicing safe transfer techniques, and gait training is encouraged.

Dysautonomia

Dysautonomia can occur in up to 70% of patients with GBS and is associated with significant morbidity and mortality, need for mechanical ventilation (Dhar et al. 2008), and cardiac arrhythmias (Zochodne 1994). It extends duration of acute care (Zochodne) and rehabilitation (Meythaler 1997). Close monitoring of fluid balance, blood pressure, cardiac rhythm, and sensitivity to vasoactive medications is essential in managing these patients and should continue until adequate recovery is under way (Meythaler). Rehabilitation involves education of staff, patients, and their families, use of compression socks, adequate hydration, postural training, and use of tilt tables where appropriate, to allow for cardiovascular and autonomic adaptation.

Bladder (disturbed sensation and areflexia) (Sakakibara et al. 1997; Amatya et al. 2013) and bowel dysfunction (ileus) in GBS can occur early and usually resolves. Ileus can be treated with erythromycin or neostigmine, but promotility agents should be avoided (Hughes et al. 2003). Patients with GBS are initially catheterized to avoid bladder distension and to maintain urinary hygiene. Rehabilitation measures ensure social continence and avoidance of complications such as urinary infections, which occur in 30% of patients (Ropper 1992). Bladder dysfunction may include detrusor acontractility, disturbed bladder sensation, and non-relaxing urethral sphincter, causing symptoms causing symptoms such as voiding difficulty, urinary retention, frequency, and urge incontinence (Sakakibara et al. 2009). Individualized management programs include timed voiding, intermittent catheterization, and anticholinergic medication (Mullings et al. 2010). Pelvic floor muscle training, with or without biofeedback or electrical stimulation, is also commonly used in women with stress leakage and mixed urinary incontinence (Mullings et al. 2010). Similarly, effective bowel management program includes an appropriate diet, adequate fluids, scheduled bowel care, and laxatives for those with bowel dysfunction (Mullings et al. 2010).

Complications from Immobilization

Tilt-table training for immobilized patients can be effectively used in rehabilitation units, as prolonged immobilization leads to reduction of blood volume. Early mobilization of patients counters immobilization hypercalcemia (Meythaler 1997).

Graduated mobility programs include maintenance of posture and alignment, joint range of motion, provision of orthotics, endurance and muscle strengthening, flexibility, and progressive ambulation using adaptive gait aids. Strengthening programs should initially be non-fatiguing to avoid paradoxical weakness. Physical measures using neurodevelopmental sequencing, partial body weight support systems, and podiatrons have been reported.

Other complications from immobility such as compression nerve palsies and pressure sores can be avoided with appropriate positioning and skin and pressure care protocols. Heterotopic ossification is rare but more likely to occur in ventilated patients. Early recognition and modified therapy with joint ranging within the pain-free arc can be helpful.

Pain

Up to 70% of patients can develop musculoskeletal and neuropathic pain (Hughes et al. 2003). Effective medications include tricyclic antidepressants, tramadol, GABAmimetic agents (e.g., gabapentin), membrane-stabilizing agents (e.g., carbamazepine), and opiates. Treatment includes patient education and desensitization therapy using functional tasks of everyday life (grooming, dressing).

Fatigue

Severe fatigue can persist in 80% of patients and is unrelated to age, duration, or severity of initial illness, but probably partly related to muscle deconditioning and forced inactivity. Fatigue is associated with a reduced QoL despite good physical recovery, as many patients remain restricted in daily and social activities. Medications (such as amantadine) are not effective. Aerobic exercise improves functional outcome, QoL, and fatigue. Energy conservation and work simplification strategies also help manage fatigue and facilitate patient functional independence. The provision of adaptive equipment and aids facilitate personal and domestic activity, by decreasing task-associated energy expenditure.

Cross-References

References and Readings

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Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Department of MedicineUniversity of Melbourne and the Royal Melbourne HospitalParkvilleAustralia
  2. 2.Department of Rehabilitation MedicineRoyal Melbourne HospitalParkvilleAustralia