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Guillain-Barre Syndrome Epidemiology, prognosis, medical and rehabilitation treatment !
!Laura Stigler !
Bellarmine University !2014 !
!!
https://www.youtube.com/watch?v=zLs0MLxfgYU
Prevalence Likelihood of acquiring GBS is 1: 1,000. 1!
North America and Europe: Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)- 90% of cases. 1!
China and Japan: Acute Motor Axonal Neuropathy (AMAN) most common. 1!
Peak incidence between June-July and September-October.1!
Occurs in all ages: Most common in young adults and white males. 2
EpidemiologyCorrelation between recent infection and onset of GBS: infection-induced aberrant immune response that damages peripheral nerves. 3!
1/4 of GBS patients have gad a recent C. Jejuni infection-AMAN form most common. 1!
About two-thirds of patients have symptoms of an infection in the 3 weeks before the onset of weakness. 3!
Up to 60% have a preceding upper respiratory illness. 4!
27% have no preceding illness. 4!
Infection with cytomegalic virus (CMV) and Campulobacter jejuni- associated with axonal form of GBS. 4!
Associated with: diabetes, alcohol abuse, exposure to heavy metals, epidural aesthetic, and drug abuse. 4
Disease Process Immune response attacks myelin-producing Schwann cells which are destroyed by macrophages- AIDP!
Underlying axon left intact !
Affects both motor and sensory peripheral
Diagnosis Form of GBS Characteristics
Acute inflammatory demyelinating
polyradiculoneuropathy
Motor, bilateral facial and pharyngeal, occasional sensory,
and autonomic disturbances.
Acute motor axonal neuropathy Only motor
Miller fisher syndrome Ophthalmoplegia, ataxia, areflexia.
1
Clinical Features Ascending motor symmetrical, flaccid areflexia paralysis!
Parathesias and hypersthesias!
Cranial nerve involvement !
Autonomic dysfunction: sinus tachycardia or less often bradycardia, fluctuating blood pressure, loss of sweating!
History of flu-like illness!
Recovery after 2-4 weeks of plateau of the disease process.
https://indianclinicalknowledgedotnet1.wordpress.com/tag/gbs/
Frequency of features in acute GBS
Features Frequency in fully developed cases 4Weakness in legs 95%
Weakness in arms 90%Areflexia 90%
Parasthaesia 85%Sensory loss 75%
Oropharyngeal weakness 50%Pain 30%
Respiratory failure 30%Opthalmoparesis 15%
Ataxia 15%Sphincter involvement 5%
Differential Diagnosis • Brain stem encephalitis!• Meningitis
carcinomatosis !• Vitamin B1 deficiency !• Botulism !• Polymytosis!• Dermatomyotosis!• Acute rhabdomyolysis 3!• Spinal cord compression !• Transverse myelitis!• Myasthenia gravis!• Vasculitis neuropathy !• Paraneoplastic
neuropathy 4
Differential Diagnosis Red Flags Raising Other
Diagnostic Possibilities - Fever at onset - Severe pulmonary dysfunction
with limited weakness at onset - Severe sensory signs with
limited weakness at onset - Persistent bladder or bowel
dysfunction, or dysfunction at onset.
- Sharp sensory level - Marked persistent asymmetry
of weakness - Increased number of
mononuclear cells in CSF
Tests and Measures
Electrodiagnosis: demonstrates demyelination. 1 !
Sural sparing: normal Sural sensory nerve response !
CSF analysis: Elevated CSF protein, fever than 10 cells/3mm. 4!
CSF examination helps to rule out other causes of weakness- lyme disease, HIV related radiculitis. 3
http://intensivecarehotline.com/clinical-pictures/guillain-barre-syndrome/
Medical Treatment Clinical Sign Management
Respiratory Failure Ventilator (2-6 weeks)
Dysautonomia Possible pacemaker placement for bradycardia
Hyperoxygenation during suction Short lived antihypertensives
Treatment of dysmotility
Hyponatremia Electrolyte replacement
Deep Vein Thrombosis Prophylaxis
Nutrition Nasogastric or gastric tube
High energy and high protein- prevent muscle wasting and promote ventilator weaning
Immunity Plasmapheresis Immunotherapy (IVIg)
Plasmapheresis vs Intravenous Immunoglobulins
Plasmapheresis (PE) and Intravenous Immunoglobulin (IVIg) therapy are both utilized !
IVIg more convenient- greater availability !
Neither treatment found to be more effective 1!
Higher incidence of relapse after IVIg treatment 4!
Few cost analyses though PE may be more cost effective than IVIg!
Not more effective when combined. 1
Plasmapheresis
Meta-analysis showed patients treated with plasmapheresis at onset were at a reduced risk for developing respiratory failure. 1!
Patients treated with PE have better secondary outcomes including: !
Decreased time to recover walking without an AD!
Reduced necessity and duration of ventilator use!
Full muscle strength recover after 1 year. 1
Plasmapheresis Adverse effects of PE include: !
hypotension!
septicemia!
pneumonia!
abnormal clotting!
hypocalcemia. 1!
Contraindications to using PE: !
hemostatic disorder, unstable cardiovascular state, active infection, and pregnancy. 1
http://www.giveplasma.ca/plasmapheresis
Intravenous Immunoglobulins Side effects of IVIg include: !
CHF!
Stroke!
Myocardial ischemia!
Renal failure!
Thrombocytopenia!
Hemolysis!
Anaphylatic shock. 5
Prognosis
Rehabiliation can take up to 1 year, though residual effects can be present 3-6 years. !
Patient disability is seen in 20%-30%. 1
Clinical features associated with poorer outcomes
• Older age • Requirements for respiratory
support • Abnormal nerve function • No plasmapheresis is performed • Primary axonal degeneration • Patients with rapid onset • Progression to quadriplegia • Respiratory dependence • Severe disease at presentation • Campylobacter jeuni infection • Patients showing no
improvement at 3 weeks of plateau of disease
Clinical Picture Progression: from distal to proximal 1!
Recovery: from proximal to distal 1!
Where do we start???!
Proximally- train muscles as they recover!
AAROM arms, sitting balance, core strengthening. !
Progress to sliding board transfers- once there is recovery of UE strength.
https://www.youtube.com/watch?v=VwQzjj9aQnQ
Rehab- Acute Care
What is the patient experiencing?!
Fear and anxiety !
Depression and guilt !
Pain
http://www.oregonlive.com/health/index.ssf/2010/12/a_mysterious_disease_sends_por.html
Rehab: Acute Care- Evaluation
Patient/caregiver interview!
Sensory assessment !
Skin inspection !
Joint ROM !
Muscle Testing
Functional testing !
Mobility !
Respiration!
Activity tolerance
Rehab: Acute Care Prevention of contractures and maintenance of posture and joint alignment 4,8!
Family education: transfers, positioning, PROM 4,8 !
Reducing effects of prolonged immobilization: tilt tables for weight-bearing and positioning to prevent skin care issues and nerve palsies. 4!
Densentitisation therapy for pain and depression. 4!
Introduce breathing and coughing techniques 8!
Anticipate rehab and AD needs 8
Acute Care- Rehabilitation !
Provide gentle stretching and active or active assistive exercise at a level consistent with patient’s strength 2, 8!
Don’t exercise to muscle fatigue- overuse or overstretch can impede recovery 2!
Emphasize patient communication about fatigue and pain. Educate on signs of fatigue. 2!
Teach energy conservation techniques 2,8!
Introduce coughing and breathing exercises to insure good air exchange 2,8!
Increase repetitions before resistance 2!
Use PNF 2
Rehab: Sub-Acute
Progress strengthening !
Progress to ambulation- decrease assistance as patient progresses strength, balance and coordination!
Emphasize proper mechanics and avoid substitutions!
Continue to educate family on rehabilitation progress!
HEP for patient and family members !
Rehab similar to incomplete spinal cord injured patient !
Partial body weight support treadmill training 9
Rehab- Chronic 12-week bicycle extensive training program has positive effects on fatigue, anxiety, depression, and functional outcome. 1!
Pool therapy!
Functional training !
Dynamic balance and strength training!
Make it fun yet challenging.
Outcome Measures
Modifed Barthel Index,!
Functional Independence Measure, !
Environmental Status Scale !
Handicap Assessment Scale 7
Resources 1.Meena AK, Khadilkar SV, Murthy JM. Treatment guidelines for Guillain-Barré Syndrome. Ann Indian Acad Neurol. 2011;14. 73-81.!
2. Lesch, D. Gullaine-Barre Syndrome. [PowerPoint]. Louisville, KY: Bellarmine University Doctor of Physical Therapy Program; 2014. !
3. Van doorn PA, Ruts L, Jacobs BC. Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome. Lancet Neurol. 2008;7(10):939-50.!
4.Khan F. Rehabilitation in Guillian Barre syndrome. Aust Fam Physician. 2004;33(12):1013-7.!
5. Harms M. Inpatient management of guillain-barré syndrome. Neurohospitalist. 2011;1(2):78-84.!
6. Ranjani P, Khanna M, Gupta A, Nagappa M, Taly A, Haldar P. Prevalence of fatigue in Guillain-Barre syndrome in neurological rehabilitation setting. Annals Of Indian Academy Of Neurology [serial online]. July 2014;17(3):331-335. !
7. Nicholas R, Playford E, Thompson A. A retrospective analysis of outcome in severe Guillain-Barre syndrome following combined neurological and rehabilitation management. Disability & Rehabilitation [serial online]. July 10, 2000;22(10):451-455.!
8. Guillain-Barré Syndrome, CIDP and Variants: Guidelines for Physical and Occupational Therapy.GBS/CIDP Foundation International.!
9. Tuckey J, Greenwood R. Rehabilitation after severe Guillain-Barré syndrome: the use of partial body weight support. Physiotherapy Research International [serial online]. June 2004;9(2):96-103.
