8 Essentials points for the Diagnosis and Treatment of Guillain-Barré Syndrome
Guillain-Barré Syndrome, also known as Acute Demyelinating Polyneuropathy or Landry’s Ascending Paralysis, is an inflammatory disorder of the Peripheral Nervous System. It is the most common cause of acute flaccid paralysis.
It is estimated that 1-2 people out of every 100,000 suffer from this syndrome annually.
This syndrome is characterised by a rapid onset of clinical manifestations with weakness and sensory signs in the legs, usually progressing upwards to the arms and trunk, although its presentation is heterogeneous and may vary.
Due to the sudden onset of the disease, it is essential to have tools that can be easily applied by clinicians to locate patients with an indication of this diagnosis, and to have guidelines for the treatment of Guillain-Barré Syndrome.
Thus, in 2019, the most important points for the diagnosis, treatment, diagnosis, prognosis and management of the disease were published in the prestigious journal Nature Reviews Neurology. This work was carried out thanks to the joint work of international review experts in this disease, together with the International Foundation for the support of patients with Guillain-Barré Syndrome or Chronic Inflammatory Demyelinating Demyelinating Polyneuropathy or similar, known as GBS – CIDP Foundation International.
1. When to suspect Guillain-Barré Syndrome
Generally speaking, we should consider its appearance in cases of patients who present bilateral weakness of rapid and progressive onset in legs and/or arms, in those circumstances in which there are no CNS-related causes (e.g.: stroke, concussion…) or other obvious causes.
The classic sensorimotor presentation usually presents with distal paraesthesias or sensory loss, accompanied or followed by weakness in the legs, progressing to the arms and cranial muscles. Other common signs and symptoms include decreased or absent reflexes, impaired autonomic system function (blood pressure instability, pupillary dysfunction, bladder disturbance, etc.), pain (muscular, radicular or neuropathic). Patients usually reach the maximum affectation of the syndrome within 2 weeks. In cases where this occurs in the first 24 hours or after 4 weeks, other diagnoses should be considered.
Atypical presentations of this syndrome may include asymmetric or proximal predominant onset of symptoms, or onset in all limbs at the same time. There is also a pure motor variant. In the following table, we summarise the variants of Guillain-Barré syndrome:
Guillain-Barré Syndrome Variants
| Variant | Clinical Characteristics | Frequency (% of GBS) |
|---|---|---|
| Classic sensorimotor GBS | Rapidly progressive symmetrical weakness and sensory signs with absent or reduced tendon reflexes, usually reaching nadir within 2 weeks | 30-85% |
| Pure motor | Motor weakness without sensory signs | 5-70% |
| Paraparetic | Paresis restricted to the legs | 5-10% |
| Pharyngeal-cervical-brachial | Weakness of pharyngeal, cervical and brachial muscles without lower limb weakness | <5% |
| Bilateral facial palsy with paraesthesias | Bilateral facial weakness, paraesthesias and reduced reflexes | <5% |
| Pure sensory | Acute or subacute sensory neuropathy without other deficits | <1% |
| Miller Fisher syndrome | Ophthalmoplegia, ataxia and areflexia. Incomplete forms with isolated ataxia (acute ataxic neuropathy) or ophthalmoplegia (acute ophthalmoplegia) can occur | 5-25% |
| Bickerstaff brainstem encephalitis | Ophthalmoplegia, ataxia, areflexia, pyramidal tract signs and impaired consciousness | <5% |
Pre-consultation events
It is important not to forget that about 2 out of 3 patients who develop Guillain-Barré syndrome have had an infection in the 6 weeks prior to the onset of the first symptoms. Among the pathogens studied in the literature: Campylobacter jejuni, cytomegalovirus, hepatitis E virus, Mycoplasma pneu- moniae, Epstein-Barr virus and Zika virus.
Zika Virus and Guillain-Barré Syndrome
In 2016, this work was published in the prestigious journal “The New England Journal of Medicine” where it was observed that in patients diagnosed with Guillain-Barré syndrome, there was clear evidence of having previously suffered from Zika virus infection in Colombia.
You can access the full article here.
2. How to diagnose Guillain-Barre Syndrome
Its diagnosis is based on the history and clinical findings found in the initial evaluation, although it can be complemented with Cerebrospinal Fluid analysis and electrodiagnostic studies. Some of the most commonly used diagnostic criterias iare that developed by the National Institute of Neurological Disorders and Stroke (NINDS) in 1978 or the Brighton Collaboration in 2011.
The authors of this 2019 paper (Leonhard SE et al 2019), recommend the NINDS criteria, as they are more suitable for the clinician, including the clinical features of classical and atypical onset of Guillain-Barré syndrome. Below are the NINDS points together with the modifications made in subsequent years:
In addition, we must always keep in mind the possible differential diagnoses that may resemble Guillain-Barré syndrome. There are symptoms that should make us suspect another pathology:
4. Medical Treatment in GBS
Treatment should begin with immunomodulatory therapy when the patient is unable to walk independently for 10 metres. Treatment should also be considered in patients who show rapidly progressive weakness or other severe symptoms such as autonomic dysfunction, bulbar or respiratory failure.
Intravenous immunoglobulin (IVIg) and plasma transfusions have been shown to be effective in the Guillain-Barré syndrome´s treatment, although IVIg is usually first-line selected because it is easier to administer and more readily available than plasma. No other drugs have been proven effective.
In those cases in which the evolution is imminently predisposing to respiratory failure, severe autonomic cardiovascular dysfunction (arrhythmias, marked changes in blood pressure…), severe swallowing dysfunction or decreased cough reflex, and/or rapid progression of weakness, the patient should be referred to the intensive care unit. The Erasmus GBS Respiratory Insuffiency Score (EGRIS) prognostic tool calculates the probability (1-90%) that the patient will require mechanical ventilation. We leave you a website to calculate it automatically!
5. Monitoring the GBS progression
It is essential to monitor respiratory function at all times, as the progression of the syndrome may lead to respiratory disorders that require direct referral to hospital. Among the evaluations we can include:
-Assessing the use of accessory respiratory musculature
-Assessment of full inspiratory capacity
– Forced vital capacity
– Peak inspiratory and expiratory pressures
The 20/30/40 rule is recommended: the patient is at risk of respiratory failure if forced vital capacity < 20 ml/kg, peak inspiratory pressure < 30 cmH2 or peak expiratory pressure < 40 cmH2.
The muscles strength of the neck, arms and legs (Scale 0-5) and the function of the patient with the specific disability scale “GBD Disability Scale” should also be used.
We should also monitor the ability to swallow or cough, and the presence of autonomic nervous system dysfunction by assessing heart rate, blood pressure, bladder and/or bowel function.
It is claimed that 2 out of 3 deaths caused by Guillain-Barré Syndrome are caused during the recovery phase, mainly due to cardiovascular and respiratory dysfunction, so every healthcare professional caring for the patient should be on the lookout for arrhythmias, BP changes or respiratory problems!
6. Expected results in Guillain-Barré patients
Despite these complications, most patients with Guillain-Barré syndrome (even more severe cases with mechanical ventilation) recover optimally, especially within the first year after the onset of the disease. Four out of five patients regain the ability to walk independently within 6 months of disease onset.
Only 3-10% of cases die as a result of cardiovascular or respiratory problems.
Recurrence data are rare (2-5% of patients).
7. Physiotherapy and Occupational Therapy in Guillain-Barré Syndrome
GBS patients usually present functional, motor and sensory limitations in the long term. This is why physiotherapists and occupational therapists have an important role in recovery process of their autonomy and functional ability.
Focusing on the upper limb, we must carry out an accurate assessment of the functional, motor and/or sensory status in order to select the most appropriate treatment for our patient.
Not sure where to start? Here is our blog post on the 12 questionnaires and measurement tools you MUST know about upper limb neurorehabilitation.
Exercise Therapy in Guillain-Barré Syndrome
Exercise Therapy has been described as one of the key therapeutic pillars in the Clinical Practice Guideline published in 2010 (Orsini M et al 2010).
Exercise programmes should include mobility, cardiorespiratory function (walking, cycling…) and strength modalities, which have been shown to be effective in improving patients’ physical capacities (Arsenault NS et al 2016) or perceived fatigue (Garssen MPJ et al 2004). It will be especially important to monitor patients’ fatigue during exercise.
Studies such as Prada V et al 2019 show the need for rehabilitation of Guillain-Barré Syndrome to be intense and prolonged in the long term (beyond the initial 6 months). Moreover, the results suggest that physiotherapy can enhance the effect of medical treatment in patients with Guillain-Barré syndrome!!
It is also important to assess pain, as it is stated that 1 in 3 patients with Guillain-Barré Syndrome with 1 year of evolution may suffer from chronic pain lasting more than 10 years. Among the possible causes described: joint immobilisation, neuropathic origin due to the regeneration of nerve fibres or nociceptive. For this, active work and patient education will be of vital importance.
An important point is to get the patient to adhere to our exercise programme. It is widely known that adherence rates in chronic pathology are especially low, and that they will condition the evolution of our patients. In our post The 14 keys to promote adherence to exercise therapy we propose some ideas.
Functional Training in Guillain-Barré Syndrome
Particularly important will be the transfers re-education such as sitting-standing, supine-sitting, etc. in a safe way, balance exercises and progressive walking. We may use assistive systems, supports or material to help the patient regain mobility.
Another important point will be the work of respiratory physiotherapy, in order to maintain airway hygiene or re-education techniques of the primary respiratory musculature. Remember the importance of this point, given the possible complications that the patient may suffer associated with respiratory dysfunction.
New Technologies in Neurorehabilitation of the Hand
We currently know thanks to a large amount of literature that motor relearning and functional recovery in neurological patients depends on individualised work adapted to the patient’s abilities and diagnosis, intensive and involving functional tasks. All these needs can be applied to our patient using digital tools such as the ReHand digital system.
ReHand is a prescription, treatment and monitoring system that enables patients to perform their hand exercises whenever and wherever they want, adapted to their abilities and state of recovery, and including fine functional gestures such as thumb-index pinch or wrist active movilization.
The reference center for Multiple Sclerosis treatment in Spain, the Multiple Sclerosis Association of Toledo (ADEMTO), already works with ReHand! Read the experience of their Occupational Therapist with these patients here.
A ReHand exercise example is the functional gesture of the pinch, which could be selected to restore hand function and dexterity. You can observe below a patient performing this exercise. The patient’s goal is to “pinch” the circle on the screen. In this way, and by progressively increasing the difficulty, we get the patient to work on this gesture with the visual feedback of the circle, and from his own tablet:
Another example can be proprioceptive exercises for the thumb, as you can observe in the following exercise. Proprioceptive exercise can help to recover the mobility of the finger, which consequently allows to gain function and dexterity in the hand.
The ReHand telerehabilitation system has been shown to be more effective in the functional recovery of the upper limb (Fugl-Meyer Assessment questionnaire) and manual dexterity (Nine Hole Peg Test) in neurological patients, as a home-based tool, complementary to face-to-face physiotherapy treatment in the ward (NCT05204225).
8. Psychological approach in Guillain-Barré Syndrome
The fact that the patient experiences a sudden and abrupt loss of functional and physical abilities, often in healthy subjects, can be a very traumatic event for any person, and can lead to depression and/or anxiety.
It is essential that these patients are referred early to a psychologist in order to begin his/her treatment as early as possible, since, as we know, the psychological state has an important influence on physical and functional recovery.
Among the first messages we should give to these patients is that recovery rate of GBS is usually high, and that there is a low risk of recurrence (2-5% of cases). This type of message can help to reduce their fear. Here is some material for you to give to your patients:
In addition to this video, the GBS | CIDP Foundation International has brochures that you can give to your patients:
It will also be interesting to connect patients who have previously experienced GBS, to help them in the recovery process and to share experiences. The GBS/CIDP Foundation International – the international association of GBS patients – and other national organisations can help to establish these networks.
ReHand in the approach to the neurological patient
ReHand is the first digital solution for the management of patients with neurological, trauma and orthopaedic conditions of the hand-wrist-finger segment.
This system enables the healthcare professional to 1) Prescribe exercise programmes personalised to the patient’s pathology and 2) Monitor the patient’s progress from their Dashboard, where adherence data, scales, validated questionnaires and progress reports are recorded. In turn, the patient 3) has his or her daily therapeutic exercise treatment system on his or her Tablet, with adjustment of the exercises according to his or her mobility and capacity prior to their start, and receives scales that allow his or her condition to be assessed.
ReHand’s scientific evidence
Clinical Effectiveness of the ReHand App in Hand Rehabilitation After Stroke . (in publication process)
Results: Compared with the control group, the experimental group patients had larger recoveries of hand dexterity and functional ability of the upper limb
Blanquero J, Cortés-Vega MD, Rodríguez-Sánchez-Laulhé P, Corrales-Serra BP, Gómez-Patricio E, Díaz-Matas N, Suero-Pineda A. Feedback-guided exercises performed on a tablet touchscreen improve return to work, function, strength and healthcare usage more than an exercise program prescribed on paper for people with wrist, hand or finger injuries: a randomised trial. J Physiother. 2020 Oct;66(4):236-242.
Conclusions: In people with bone and soft-tissue injuries of the wrist, hand and/or fingers, prescribing a feedback-guided home exercise program using a tablet-based application instead of a conventional program on paper hastened return to work and improved the short-term recovery of functional ability and pinch strength, while reducing the number of required healthcare appointments.
Blanquero J, Cortés-Vega MD, García-Frasquet MÁ, Sánchez-Laulhé PR, Nieto Díaz de Los Bernardos MI, Suero-Pineda A. Exercises using a touchscreen tablet application improved functional ability more than an exercise program prescribed on paper in people after surgical carpal tunnel release: a randomised trial. J Physiother. 2019 Apr;65(2):81-87. doi: 10.1016/j.jphys.2019.02.008.
Conclusion: Use of the ReHand tablet application for early rehabilitation after carpal tunnel release is more effective in the recovery of functional ability than a conventional home exercise program. It remains unclear whether there are any benefits in grip strength, pain or dexterity.
Are you interested?
Pablo Rodríguez Sánchez-Laulhé
PhD Candidate | PT & eHealth Researcher
References
Leonhard SE, Mandarakas MR, Gondim FAA, Bateman K, Ferreira MLB, Cornblath DR, et al. Diagnosis and management of Guillain–Barré syndrome in ten steps. Nat Rev Neurol [Internet]. 2019;15(11):671–83. Available from: http://dx.doi.org/10.1038/s41582-019-0250-9
Prada V, Massa F, Salerno A, Fregosi D, Beronio A, Serrati C, et al. Importance of intensive and prolonged rehabilitative treatment on the Guillain-Barrè syndrome long-term outcome: a retrospective study. Neurol Sci. 2020;41(2):321–7.
Orsini M, De Freitas MRG, Presto B, Mello MP, Reis CHM, Silveira V, et al. Guideline for neuromuscular rehabilitation in Guillain-Barré syndrome: What can we do? Rev Neurociencias. 2010;18(4):572–80.
