Abstract
The 2015–2017 Zika Virus outbreak caused a high increase in patients with Guillain-Barré syndrome (GBS), a post infectious autoimmune disease of the peripheral nerves. The severity of GBS can range from mild impairment with fast recovery to complete paralysis including severe respiratory or autonomic failure. Recovery may take months and even years and may be incomplete despite disease modifying treatment with IVIG or plasma exchange. Therefore, optimal supportive care and effective rehabilitation remain crucial. Multidisciplinary rehabilitation is recommended but may be challenging in the acute phase because of limited patient participation due to profound muscle weakness and severe pain. Inactive denervated muscles will inevitably undergo rapid degeneration resulting in wasting, weakness, and contractures as major long-term complications in severely affected patients. In this chapter, the current evidence of rehabilitation on the short- and long-term motor function in GBS is reviewed, including newly obtained experiences with neuromuscular electrical stimulation (NMES). Rehabilitation remains an area lacking well designed and controlled clinical studies and thus a clear lack of evidence-based guidelines.
Keywords
- Guillain Barré syndrome
- prognosis
- chronic disability
- rehabilitation
- exercise
- neuromuscular electrical stimulation
1. Introduction
2. Treatment and rehabilitation in GBS
Despite several limitations, the authors of the review concluded that there is good evidence (Grade level II) to support ambulatory, outpatient multidisciplinary rehabilitation to obtain long-term improvements in levels of activity and participation in patients with GBS in the later stages of recovery. Further, the authors concluded that there is satisfactory (Grade level III) evidence to support (1) inpatient rehabilitation followed by outpatient rehabilitation thereby inducing functional recovery and (2) physical therapy and exercise to reduce joint contractures and muscle weakness. In another more recent case series of 51 patients with GBS, motor recovery following the acute pharmacological treatment response was assessed during the acute inpatient care as well as after outpatient and homebased rehabilitation [29]. A description of the intervention was not provided, but it included physical therapy for 61 ± 58 (mean ± SD) days for inpatients, 96 ± 70 days for outpatients, and 75 ± 15 days during home rehabilitation. Again, the natural history with spontaneous improvement after GBS and the lack of a control group impairs the possibility to draw any final conclusions based on this study regarding the effectiveness of rehabilitation. However, it was shown that muscle strength measured with a MRC sum score [30] and ambulation assessed with the GBS disability score [31] continue to improve beyond the first six months of rehabilitation.
3. Conclusions
Neuromuscular rehabilitation after GBS is important for the functional outcome of each individual patient. Studied rehabilitation interventions in the acute, subacute/intermediate, and chronic/long-term phase are summarized in Figure 1. However, the quality of the present evidence of rehabilitation efficacy is low, rehabilitation is both complex, time consuming and expensive, and there is currently no standardized care for patients with neuromuscular disabilities after GBS. Therefore, the rehabilitation effort may lack necessary resources and expertise. Because the monophasic course and spontaneous recovery in GBS challenge the interpretation of non-controlled studies, future large controlled studies and standardized sensitive efficacy outcome measures are needed to improve the interpretation of neuromuscular rehabilitation trials in GBS.
4. Policy and procedures
Stimulation of the quadriceps muscle was performed using a STIWELL med4 stimulation unit, https://www.ottobock.co.th/neurorehabilitation/solutions/solutions-with-functional-electrical-stimulation/stiwell-med-4/(Otto Bock, Konigsee, Germany) and two large stimulation pads (6 × 8 cm). The intensity of electrical stimulation was titrated individually at entry and weekly during the study to the point of maximal contraction or the highest tolerable intensity. During the first session of stimulation, the skin under the pads was inspected every five minutes for redness or other signs of tissue damage. Trained physical therapists attached the equipment and titrated the stimulation intensity, but after being attached to the patient the individualized stimulation protocol ran automatically.
The intention was to stimulate patients five to seven days a week including 20 minutes of MFS followed by 40 minutes of NMES. Also, the NMES was applied to patients where no visible contraction could be observed.
5. Mini-dictionary of terms
6. Key facts of neuromuscular rehabilitation in GBS
Neuromuscular rehabilitation in Guillain Barré Syndrome can include
Physical therapy to prevent muscle and joint shortening and contractures.
Multidisciplinary rehabilitation with two or more coordinated interventions for disabled patients to regain autonomy and functions of daily living.
Exercise and training to improve or maintain physical functioning.
Neuromuscular electrical stimulation to prevent muscle wasting.
The prognosis of Guillain Barré Syndrome
Guillain Barré Syndrome is a heterogenous disorder with a monophasic course.
Clinical severity ranges from mild impairment to complete paralysis combined with respiratory and autonomic failure.
In 20 to 30% of patients, mechanical ventilation is required at nadir of GBS.
The most severely affected patients have a long recovery phase and a poor prognosis.
More than half of all mechanically ventilated patients are unable to walk unassisted at one year follow up.
7. Summary points of neuromuscular rehabilitation in GBS
Most commonly, GBS is preceded by an infection, therefore, the incidence of GBS can increase during outbreaks of infectious diseases, which was most recently observed during the Zika Virus outbreak in the French Polynesia and Latin America with a high increase in the incidence of GBS in several countries.
Despite optimal evidence-based treatment with immunoglobulin and plasma exchange, a large proportion of patients with GBS will have substantial neuromuscular disabilities more than one year after disease onset. Among patients receiving mechanical ventilation, more than half will be able to walk unassisted.
In the acute phase of GBS, physical therapy is important to prevent muscle shortening and joint contractures.
Patients may still improve their physical function several years after onset of GBS.
There is evidence to support high intensity multidisciplinary rehabilitation and exercise which improves level of activity and participation in the late and chronic stages of GBS.
New approaches like Neuromuscular Electrical Stimulation and Virtual Motor Rehabilitation seem to be feasible methods in the acute and late stage recovery of GBS, but efficacy needs to be explored in future studies.
Abbreviations
FIM | functional independence measure |
GBS | Guillain Barré syndrome |
ICU | intensive care unit |
IVIG | intravenous immunoglobulin |
MFS | muscle fiber stimulation |
NMES | neuromuscular electrical stimulation |
PE | plasma exchange |
VMR | virtual motor rehabilitation |
References
- 1.
Leonhard SE, Conde RM, de Assis Aquino GF, Jacobs BC. Diagnosis and treatment of Guillain-Barre syndrome during the Zika virus epidemic in Brazil: A national survey study. J.Peripher.Nerv.Syst. 2019a; 24 (4):340-347 available from: PM:31746070 - 2.
Leonhard SE, Mandarakas MR, Gondim FAA, Bateman K, Ferreira MLB, Cornblath DR, et al. Diagnosis and management of Guillain-Barre syndrome in ten steps. Nat.Rev.Neurol. 2019b; 15 (11):671-683 available from: PM:31541214 - 3.
Sipila JO, Soilu-Hanninen M. The incidence and triggers of adult-onset Guillain-Barre syndrome in southwestern Finland 2004-2013. European Journal of Neurology. 2015; 22 (2):292-298 available from: PM:25196425 - 4.
Cao-Lormeau VM, Blake A, Mons S, Lastere S, Roche C, Vanhomwegen J, et al. Guillain-Barre syndrome outbreak associated with Zika virus infection in French Polynesia: A case-control study. Lancet. 2016; 387 (10027):1531-1539 available from: PM:26948433 - 5.
Cardona-Ospina, J.A., Henao-SanMartin, V., Acevedo-Mendoza, W.F., Nasner-Posso, K.M., Martinez-Pulgarin, D.F., Restrepo-Lopez, A., Valencia-Gallego, V., Collins, M.H., & Rodriguez-Morales, A.J. 2019. Fatal Zika virus infection in the Americas: A systematic review. Int.J.Infect.Dis., 88, 49-59 available from: PM:31499212 - 6.
Parra, B., Lizarazo, J., Jimenez-Arango, J.A., Zea-Vera, A.F., Gonzalez-Manrique, G., Vargas, J., Angarita, J.A., Zuniga, G., Lopez-Gonzalez, R., Beltran, C.L., Rizcala, K.H., Morales, M.T., Pacheco, O., Ospina, M.L., Kumar, A., Cornblath, D.R., Munoz, L.S., Osorio, L., Barreras, P., & Pardo, C.A. 2016. Guillain-Barre syndrome associated with Zika virus infection in Colombia. N.Engl.J.Med. , 375, (16) 1513-1523 available from: PM:27705091 - 7.
Rodriguez-Morales AJ, Failoc-Rojas VE, Diaz-Velez C. Gastrointestinal, respiratory and/or arboviral infections? What is the cause of the Guillain-Barre syndrome epidemics in Peru? Current status - 2019. Travel.Med.Infect.Dis. 2019; 30 :114-116 available from: PM:31265907 - 8.
Villamil-Gomez WE, Sanchez-Herrera AR, Hernandez H, Hernandez-Iriarte J, Diaz-Ricardo K, Castellanos J, et al. Guillain-Barre syndrome during the Zika virus outbreak in Sucre, Colombia, 2016. Travel.Med.Infect.Dis. 2017; 16 :62-63 available from: PM:28347781 - 9.
Zambrano LI, Fuentes-Barahona IC, Soto-Fernandez RJ, Zuniga C, da Silva JC, Rodriguez-Morales AJ. Guillain-Barre syndrome associated with Zika virus infection in Honduras, 2016-2017. Int.J.Infect.Dis. 2019; 84 :136-137 available from: PM:31096053 - 10.
Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barre syndrome. Lancet. 2016; 388 (10045):717-727 available from: PM:26948435 - 11.
Wakerley BR, Yuki N. Mimics and chameleons in Guillain-Barre and Miller fisher syndromes. Pract.Neurol. 2015; 15 (2):90-99 available from: PM:25239628 - 12.
Karam E, Giraldo J, Rodriguez F, Hernandez-Pereira CE, Rodriguez-Morales AJ, Blohm GM, et al. Ocular flutter following Zika virus infection. Journal of Neurovirology. 2017; 23 (6):932-934 available from: PM:29147884 - 13.
Hadden RD, Cornblath DR, Hughes RA, Zielasek J, Hartung HP, Toyka KV, et al. Electrophysiological classification of Guillain-Barre syndrome: Clinical associations and outcome. Plasma exchange/Sandoglobulin Guillain-Barre syndrome trial group. Annals of Neurology. 1998; 44 (5):780-788 available from: PM:9818934 - 14.
Al-Hakem H, Sindrup SH, Andersen H, de la Cour CD, Lassen LL, van den Berg B, et al. Guillain-Barre syndrome in Denmark: A population-based study on epidemiology, diagnosis and clinical severity. J.Neurol. 2019; 266 (2):440-449 available from: PM:30536111 - 15.
van den Berg B, Storm EF, Garssen MJP, Blomkwist-Markens PH, Jacobs BC. Clinical outcome of Guillain-Barre syndrome after prolonged mechanical ventilation. Journal of Neurology, Neurosurgery, and Psychiatry. 2018; 89 (9):949-954 available from: PM:29627773 - 16.
Hughes RA, Wijdicks EF, Benson E, Cornblath DR, Hahn AF, Meythaler JM, et al. Supportive care for patients with Guillain-Barre syndrome. Archives of Neurology. 2005; 62 (8):1194-1198 available from: PM:16087757 - 17.
Doets AY, Verboon C, van den Berg B, Harbo T, Cornblath DR, Willison HJ, et al. Regional variation of Guillain-Barre syndrome. Brain. 2018; 141 (10):2866-2877 available from: PM:30247567 - 18.
Chevret, S., Hughes, R.A., & Annane, D. 2017. Plasma exchange for Guillain-Barre syndrome. Cochrane.Database.Syst.Rev., 2, CD001798 available from: PM:28241090 - 19.
Hughes, R.A., Swan, A.V., & van Doorn, P.A. 2014. Intravenous immunoglobulin for Guillain-Barre syndrome. Cochrane.Database.Syst.Rev., 9, CD002063 available from: PM:25238327 - 20.
Davidson AI, Halstead SK, Goodfellow JA, Chavada G, Mallik A, Overell J, et al. Inhibition of complement in Guillain-Barre syndrome: The ICA-GBS study. J.Peripher.Nerv.Syst. 2017; 22 (1):4-12 available from: PM:27801990 - 21.
Misawa S, Kuwabara S, Sato Y, Yamaguchi N, Nagashima K, Katayama K, et al. Safety and efficacy of eculizumab in Guillain-Barre syndrome: A multicentre, double-blind, randomised phase 2 trial. Lancet Neurology. 2018; 17 (6):519-529 available from: PM:29685815 - 22.
Pritchard, J., Hughes, R.A., Hadden, R.D., & Brassington, R. 2016. Pharmacological treatment other than corticosteroids, intravenous immunoglobulin and plasma exchange for Guillain-Barre syndrome. Cochrane.Database.Syst.Rev., 11, CD008630 available from: PM:27846348 - 23.
Khan F, Amatya B. Rehabilitation interventions in patients with acute demyelinating inflammatory polyneuropathy: A systematic review. Eur.J.Phys.Rehabil.Med. 2012; 48 (3):507-522 available from: PM:22820829 - 24.
Khan F, Pallant JF, Amatya B, Ng L, Gorelik A, Brand C. Outcomes of high- and low-intensity rehabilitation programme for persons in chronic phase after Guillain-Barre syndrome: A randomized controlled trial. J.Rehabil.Med. 2011; 43 (7):638-646 available from: PM:21667009 - 25.
Demir SO, Koseoglu F. Factors associated with health-related quality of life in patients with severe Guillain-Barre syndrome. Disabil.Rehabil. 2008; 30 (8):593-599 available from: PM:17852306 - 26.
Gupta, A., Taly, A.B., Srivastava, A., & Murali, T. 2010. Guillain-Barre Syndrome–rehabilitation outcome, residual deficits and requirement of lower limb orthosis for locomotion at 1 year follow-up. Disabil.Rehabil., 32, (23) 1897-1902 available from: PM:20331413 - 27.
Meythaler JM, DeVivo MJ, Braswell WC. Rehabilitation outcomes of patients who have developed Guillain-Barre syndrome. American Journal of Physical Medicine & Rehabilitation. 1997; 76 (5):411-419 available from: PM:9354496 - 28.
Nicholas R, Playford ED, Thompson AJ. A retrospective analysis of outcome in severe Guillain-Barre syndrome following combined neurological and rehabilitation management. Disabil.Rehabil. 2000; 22 (10):451-455 available from: PM:10950498 - 29.
Prada V, Massa F, Salerno A, Fregosi D, Beronio A, Serrati C, et al. Importance of intensive and prolonged rehabilitative treatment on the Guillain-Barre syndrome long-term outcome: A retrospective study. Neurol.Sci. 2019 available from: PM:31586288 - 30.
Medical Research Council. Aids to examination of the peripheral nervous system. London: HMSO. Memorandum No. 1976; 45 - 31.
Hughes RA, Newsom-Davis JM, Perkin GD, Pierce JM. Controlled trial prednisolone in acute polyneuropathy. Lancet. 1978; 2 (8093):750-753 available from: PM:80682 - 32.
Simatos AN, Vincent PO, Yu BH, Bastien R, Sweeney A. Influence of exercise on patients with Guillain-Barre syndrome: A systematic review. Physiotherapy Canada. 2016; 68 (4):367-376 available from: PM:27904236 - 33.
Bussmann JB, Garssen MP, van Doorn PA, Stam HJ. Analysing the favourable effects of physical exercise: Relationships between physical fitness, fatigue and functioning in Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy. J.Rehabil.Med. 2007; 39 (2):121-125 available from: PM:17351693 - 34.
Garssen MP, Bussmann JB, Schmitz PI, Zandbergen A, Welter TG, Merkies IS, et al. Physical training and fatigue, fitness, and quality of life in Guillain-Barre syndrome and CIDP. Neurology. 2004; 63 (12):2393-2395 available from: PM:15623709 - 35.
Griffiths RD, Palmer TE, Helliwell T, MacLennan P, MacMillan RR. Effect of passive stretching on the wasting of muscle in the critically ill. Nutrition. 1995; 11 (5):428-432 available from: PM:8748193 - 36.
Reid CL, Campbell IT, Little RA. Muscle wasting and energy balance in critical illness. Clin.Nutr. 2004; 23 (2):273-280 available from: PM:15030968 - 37.
Maddocks, M., Gao, W., Higginson, I.J., & Wilcock, A. 2013. Neuromuscular electrical stimulation for muscle weakness in adults with advanced disease. Cochrane.Database.Syst.Rev. (1) CD009419 available from: PM:23440837 - 38.
Maffiuletti, N.A., Roig, M., Karatzanos, E., & Nanas, S. 2013. Neuromuscular electrical stimulation for preventing skeletal-muscle weakness and wasting in critically ill patients: A systematic review. BMC.Med., 11, 137 available from: PM:23701811 - 39.
Harbo T, Markvardsen LK, Hellfritzsch MB, Severinsen K, Nielsen JF, Andersen H. Neuromuscular electrical stimulation in early rehabilitation of Guillain-Barre syndrome: A pilot study. Muscle & Nerve. 2019; 59 (4):481-484 available from: PM:30549053 - 40.
El ML, Calmels P, Camdessanche JP, Gautheron V, Feasson L. Muscle strength recovery in treated Guillain-Barre syndrome: A prospective study for the first 18 months after onset. American Journal of Physical Medicine & Rehabilitation. 2007; 86 (9):716-724 available from: PM:17709995 - 41.
Albiol-Perez S, Forcano-Garcia M, Munoz-Tomas MT, Manzano-Fernandez P, Solsona-Hernandez S, Mashat MA, et al. A novel virtual motor rehabilitation system for Guillain-Barre syndrome. Two single case studies. Methods Inf.Med. 2015; 54 (2):127-134 available from: PM:25609504 - 42.
Harbo T, Brincks J, Andersen H. Maximal isokinetic and isometric muscle strength of major muscle groups related to age, body mass, height, and sex in 178 healthy subjects. Eur.J.Appl.Physiol. 2012; 112 (1):267-275 available from: PM:21537927