History

Fact Explanation
Weakness of the legs and arms Guillain-Barré syndrome (GBS) is a autoimmune disease affecting the peripheral nervous system (AIDP - acute inflammatory demyelinating polyradiculoneuropathy). It usually follows an infection. That infection/antigens trigger the production of antibodies. The cross reactivity between neural antigens and those antiganglioside antibodies due to molecular mimicry causes neuronal damage. The primary target is Schwann cell surface causing widespread myelin damage. Macrophage activation creates a variable degree of secondary axonal damage. Pathologically both humoral and cellular immune mechanisms are involved. When the peripheral nervous system is affected, It produces a symmetrical motor weakness which usually starts from the lower limbs and ascends upwards. (Ascending flaccid paralysis) [1,2,3,4] Proximal muscles will involve earlier than the more distal ones. Patient may complain of difficulty in rising up from sitting position or climbing stairs. The symptoms are bilateral and reach their peak by the second week. Though the weakness usually affect the lower limbs, it progresses rapidly (usually over periods of hours to days) to affect trunk and bilateral upper limbs too. Therefore severity may range from mild weakness to complete tetraplegia. Patient may complain this as a generalized fatigue. Acute inflammatory demyelinating polyradiculoneuropathy is the most common subtype of GBS.(90%) So, this symptom is the most common one among all others. Descending weakness is a rare but occurs in some sub types such as Miller Fisher syndrome and pharyngeal-cervical-brachial sub type. defined by rapidly progressive oropharyngeal and cervicobrachial weakness associated with areflexia in the upper limbs. Serial nerve conduction studies suggest that PCB represents a localised subtype of Guillain-Barré syndrome characterised by axonal rather than demyelinating neuropathy. This variant is often associated with IgG anti GT1a. [1,2,3,4,5,6]
Numbness or tingling Sensory symptoms often precede the weakness. Most patients with AIDP complains of mild sensory involvement. But in acute motor sensory axonal neuropathy (AMSAN), another subtype of GBS, sensory axons are mainly involved, making sensory involvements such as numbness, tingling and sensory impairments predominant in toes and fingertips. These symptoms are generally benign, and tend to progress upward, although they usually do not extend beyond the wrists or ankles. Acute motor sensory axonal neuropathy (AMSAN) is a sub type characterized by acute onset of distal weakness, loss of deep tendon reflexes and sensory symptoms. Mildly reduced nerve conduction velocities combined with a marked reduction of muscle action and sensory nerve action potentials are the nerve conduction test results. In severe forms, respiratory and bulbar symptoms will occur. Primary axonal degeneration takes place in this subtype too. But it has a poorer prognosis. [1,2,3]
Pain in affected lims Pain usually proceeds the weakness in some patients and raised with movements. It is severe, deep, aching or cramping in nature and involves the affected muscles or back. It gets worsen at night. The origin of the pain is either nociceptive or neuropathic. [1,2]
Muscle pain Cramping muscle pain and local muscle tenderness are uncommon findings of GBS. They are thought to be caused by inflammation of surrounding nerves. [1,2,7,8]
Difficulty in swallowing The ascending disease progression eventually affects nuclei of cranial nerves IX, X, XI and XII or below (lower motor neuron lesion). Oropharyngeal dysphagia arises from abnormalities of muscles, nerves or structures of the oral cavity, pharynx, and upper esophageal sphincter which are supplied by the cranial nerves mentioned above. [1,2,3,4,9,10]
Drooling of saliva This is due to difficulty in swallowing (Oropharyngeal dysphagia). Facial, oropharyngeal and oculomotor muscles are affected as well which may result in a drooling. This mimics Bell's palsy. [1,2,3,11]
Shortness of breath Shortness of breath or shortness of breath on exertion occurs late in the course. This suggests diaphragmatic and intercostal muscle weakness due to phrenic nerve and intercostal nerves involvement respectively. Shortness of breath is also attributed to the bulbar weakness and Oropharyngeal dysphagia. In acute motor axonal neuropathy, early and severe respiratory involvements are common. It is a subtype that cause motor symptoms only. There is no sensory involvement. Pathology is primary axonal degeneration. This subtype commonly affect children and young adults. Campylobacter jejuni serology is positive up to 75% of cases. anti-GM and anti GD1a antibodies are often positive.[1,2,3,7,9,10]
Double vision It is usually the result of impaired function of the extraocular muscles due to affected cranial nerves (III, IV, and VI) which innervate them. This can be accompanied with ataxia, areflexia and bilateral ophthalmoplegia which are collectively called as Miller Fisher syndrome. Miller Fisher syndrome is a rare sub type. Accounting for about 5% of GBS cases, it manifests as a descending paralysis. 96% of patients with Miller Fisher syndrome are positive for anti-GQ1b antibodies. [1,2,3,11,12]
Faintishness/ collapse Sympathetic and parasympathetic system dysfunction is a common finding in GBS. Wide fluctuations in blood pressure, orthostatic hypotension and cardiac arrhythmias can cause sudden collapse or faintishness. Acute autonomic neuropathy is the rarest form of GBS in which the autonomic symptoms specially cardiovascular are predominant. The mechanism fo this sub type has not been clarified.Autoantibodies to nicotine ganglionic acetylcholine receptors likely have a pathogenic role. The recovery is slow and may be incomplete. [1,2,4,5,6,12]
Urinary symptoms Urinary retention may occur in up to one third of patients. Bladder dysfunction is particularly common in GBS secondary to sacral parasympathetic nerve and pudendal motor nerve dysfunction. They are consisted of nocturnal or diurnal urinary frequency, sensation of urgency, urinary incontinence and enuresis and difficulty in voiding including hesitation and prolongation. [1,7,8]
Proceeding events Acute infectious illnesses are well-known antecedent events in two thirds of patients who have GBS. Respiratory tract infection or gastroenteritis are the commonest. So,the most common symptoms reported before the onset of GBS are fever, caugh, soar throat, abdominal pain or loose stools. Campylobacteriosis is the most common precipitant in GBS. Other antecedent infections include cytomegalovirus, HIV, Epstein-Barr virus, and varicellazoster virus. Vaccinations, surgical procedures, and trauma are the other common triggering conditions. This particular illness or event usually occur 1-3 weeks prior to the onset of weakness. [1,2,3,4,5,6]
References
  1. WALLING AD, DICKSON G. Guillain-Barré syndrome. Am Fam Physician [online] 2013 Feb 1, 87(3):191-7 [viewed 06 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23418763
  2. PITHADIA AB, KAKADIA N. Guillain-Barré syndrome (GBS). Pharmacol Rep [online] 2010 Mar-Apr, 62(2):220-32 [viewed 06 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20508277
  3. WINER J. B.. An Update in Guillain-Barré Syndrome. Autoimmune Diseases [online] 2014 December, 2014:1-6 [viewed 06 June 2014] Available from: doi:10.1155/2014/793024
  4. AKBAYRAM SINAN, AKGüN CIHANGIR, SAYIN REFAH, BEKTAS MEHMET-SELçUK, DOGAN MURAT, PEKER ERDAL, AKTAR FESIH, ÇAKSEN HüSEYIN. Clinical features and prognosis with Guillain-Barré syndrome. Ann Indian Acad Neurol [online] 2011 December [viewed 06 June 2014] Available from: doi:10.4103/0972-2327.82793
  5. LIN KP. Clinical manifestations, serology and epidemiology of Guillain-Barré syndrome. Acta Neurol Taiwan [online] 2012 Jun, 21(2):51-3 [viewed 06 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/22879112
  6. BURNS TM. Guillain-Barré syndrome. Semin Neurol [online] 2008 Apr, 28(2):152-67 [viewed 06 June 2014] Available from: doi:10.1055/s-2008-1062261
  7. SAKAKIBARA R, HATTORI T, KUWABARA S, YAMANISHI T, YASUDA K. Micturitional disturbance in patients with Guillain-Barr? syndrome J Neurol Neurosurg Psychiatry [online] 1997 Nov, 63(5):649-653 [viewed 06 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2169821
  8. HARMS M.. Inpatient Management of Guillain-Barre Syndrome. The Neurohospitalist [online] December, 1(2):78-84 [viewed 06 June 2014] Available from: doi:10.1177/1941875210396379
  9. NYATI KISHAN KUMAR, NYATI ROOPANSHI. Role of Campylobacter jejuni Infection in the Pathogenesis of Guillain-Barré Syndrome: An Update. BioMed Research International [online] 2013 December, 2013:1-13 [viewed 06 June 2014] Available from: doi:10.1155/2013/852195
  10. DIMACHKIE MAZEN M., BAROHN RICHARD J.. Guillain-Barré Syndrome and Variants. Neurologic Clinics [online] 2013 May, 31(2):491-510 [viewed 06 June 2014] Available from: doi:10.1016/j.ncl.2013.01.005
  11. CINGOZ F., TAVLASOGLU M., KURKLUOGLU M., SAHIN M. A.. Guillain-Barre syndrome after coronary artery bypass surgery. Interactive CardioVascular and Thoracic Surgery [online] December, 15(5):918-919 [viewed 06 June 2014] Available from: doi:10.1093/icvts/ivs367
  12. MEENA AK, MURTHY J. M. K., KHADILKAR SV. Treatment guidelines for Guillain-Barré Syndrome. Ann Indian Acad Neurol [online] 2011 December [viewed 06 June 2014] Available from: doi:10.4103/0972-2327.83087

Examination

Fact Explanation
Signs of autonomic dysfunction Autonomic disturbance is seen in more than 50%. It usually manifests as tachycardia but more serious autonomic nervous system dysfunction may occur, including life-threatening arrhythmias, hypotension, hypertension, and gastrointestinal dysmotility. [1,2,3,4,5]
Facial weakness Sudden weakness or paralysis on face that causes it to droop. Ptosis and drooling are also accompanied. This is due to facial nerve paralysis along with oropharyngeal and oculomotor nerves. Facial weakness is usually unilateral and rarely bilateral. [6,7,8]
Opthalmoplegia Oculomotor nerve involvement in GBS causes weakness in eye movements due to extra occular muscle paresis. Bilateral ophthalmoplegia is common in miller fisher sub type of GBS. [1,2,8]
Dysarthria Slurring of speech can be observed due to bulbar palsy. Paralysis of oro pharyngeal musculature causes deficiency in articulation and slow rate of speech. [1,2,4,9]
Slurring of speech Slurring of speech can be observed due to bulbar palsy. Paralysis of oro pharyngeal musculature causes deficiency in articulation and slow rate of speech. [1,2,4,9]
Poor respiratory effort When the ascending paralysis affects the phrenic and intercostal nerves, diaphragmatic and intercostal muscle weakness take place. This weakness along with bulbar weakness and oropharyngeal dysphagia results in poor respiratory effort. [1,2,5,6,7]
Single Breath count The patients is asked to count in normal speaking voice following a maximum effort of inspiration. Studies have suggested that SBC correlates with standard measures of pulmonary function. If the patient can count to "10" on one breath they likely have a forced vital capacity of about 1000 ml, if they can count to "25" then the vital capacity can be estimated at about 2000 ml.
Limb weakness Weakness or reduced power of the distal limb muscles is the striking feature of GBS. This weakness initially affect the lower limb before rapidly progress to the trunk and upper limb within hours to days due to ascending acute inflammatory demyelinating polyradiculoneuropathy (AIDP). AIDP usually affects proximal muscles. Patient may show difficulty when he/she is asked to stand from a sitting position. [1,2,3,4]
Areflexia Complete areflexia often occurs in affected limbs. Reflexes sometimes may be reduced. Areflexia peaks within 4 weeks. Areflexia is manifested in an ascending pattern. First it affects lower limb deep tendon reflexes (Ankle and knee jerks) and then with the progression it may affect the upperlimb reflexes such as biceps and triceps jerks. It is common to several subtypes such as acute inflammatory demyelinating polyradiculoneuropathy and Miller fisher syndrome. Deep tendon reflexes may be preserved in acute motor axonal neuropathy. [1,2,3,4]
Dysesthesia Mild sensory impairment, tingling or numbness are not uncommon in acute inflammatory demyelinating polyradiculoneuropathy. These signs usually do not go above wrist of ankle. These sensory symptoms are more predominant in acute motor sensory axonal neuropathy. [1,2,5,6]
References
  1. WALLING AD, DICKSON G. Guillain-Barré syndrome. Am Fam Physician [online] 2013 Feb 1, 87(3):191-7 [viewed 06 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23418763
  2. BURNS TM. Guillain-Barré syndrome. Semin Neurol [online] 2008 Apr, 28(2):152-67 [viewed 06 June 2014] Available from: doi:10.1055/s-2008-162261
  3. DIMACHKIE MAZEN M., BAROHN RICHARD J.. Guillain-Barré Syndrome and Variants. Neurologic Clinics [online] 2013 May, 31(2):491-510 [viewed 06 June 2014] Available from: doi:10.1016/j.ncl.2013.01.005
  4. PITHADIA AB, KAKADIA N. Guillain-Barré syndrome (GBS). Pharmacol Rep [online] 2010 Mar-Apr, 62(2):220-32 [viewed 06 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20508277
  5. LIN KP. Clinical manifestations, serology and epidemiology of Guillain-Barré syndrome. Acta Neurol Taiwan [online] 2012 Jun, 21(2):51-3 [viewed 06 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/22879112
  6. NARAYANAN RP, JAMES N, RAMACHANDRAN K, JARAMILLO MJ. Guillain-Barr? Syndrome presenting with bilateral facial nerve paralysis: a case report Cases J [online] :379 [viewed 06 June 2014] Available from: doi:10.1186/1757-1626-1-379
  7. HARMS M.. Inpatient Management of Guillain-Barre Syndrome. The Neurohospitalist [online] December, 1(2):78-84 [viewed 06 June 2014] Available from: doi:10.1177/1941875210396379
  8. WINER J. B.. An Update in Guillain-Barré Syndrome. Autoimmune Diseases [online] 2014 December, 2014:1-6 [viewed 06 June 2014] Available from: doi:10.1155/2014/793024
  9. AKBAYRAM SINAN, AKGüN CIHANGIR, SAYIN REFAH, BEKTAS MEHMET-SELçUK, DOGAN MURAT, PEKER ERDAL, AKTAR FESIH, ÇAKSEN HüSEYIN. Clinical features and prognosis with Guillain-Barré syndrome. Ann Indian Acad Neurol [online] 2011 December [viewed 06 June 2014] Available from: doi:10.4103/0972-2327.82793

Differential Diagnoses

Fact Explanation
Critical illness polyneuropathy It is characterized by widespread muscle weakness and neurological dysfunction that can develop in critically ill patients receiving intensive care. The neuromuscular syndrome of acute limb and respiratory weakness that commonly accompanies patients with multi-organ failure and sepsis. [1,2]
Acute myelopathies Acute myopathy may be traumatic, inflammatory or vascular in origin. (eg -transverse myelitis, cord compression, infarct) Usually there is no proceeding illness. Upper motor neuron signs such as weakness, spasticity, clonus, hyperreflexia and extensor plantar responses are the common signs. [2,3,4]
Diphtheria Diphtheria is an upper respiratory tract illness caused by Corynebacterium diphtheriae. Fever, sore throat, difficult and painful swallowing and difficulty in breathing is followed by multiple cranial neuropathies (eg - diplopia, ptosis, dysarthria, numb tongue, gingivae and face). CSF analysis resembles GBS with cytoalbuminological dissociation. [2,5]
Myasthenia gravis Myasthenia gravis is an autoimmune neuromuscular disease leading to fluctuating muscle weakness and fatigue. Diplopia, ptosis and bulbar symptoms like dysarthria are other common signs. There is no sensory involvement or proceeding illness. Weakness progress in a descending manner. CSF analysis is normal. [2,6]
Lyme disease Lyme disease is an infectious disease caused by at least three species of bacteria belonging to the genus Borrelia. It is characterized by meningitis, fever, myalgias, arthralgias, facial weakness, tick bite rash. CSF analysis shows pleocytosis. Lyme disease is a common tick-borne disease which is endemic in Northern Hemisphere temperate regions. [7]
Poliomyelitis Poliomyelitis is caused by poliovirus. It is an acute, viral, infectious disease spreading primarily via the fecal-oral route Non specific symptoms such as sore throat, fever, nausea, vomiting, headache are followed by acute-onset lower motor neuronopathy with myalgias and fasciculations. CSF analysis shows pleocytosis. [8]
Botulism Botulism is a rare and potentially fatal paralytic illness caused by a toxin produced by Clostridium botulinum. Bilateral symmetrical & descending flaccid paralysis occurs after 12-36 hours of ingestion contaminated food. The classic symptoms of botulism include double vision, blurred vision, drooping eyelids, slurred speech, difficulty swallowing and dry mouth. CSF analysis is normal. [9]
Hypokalaemic periodic paralysis Hypokalemic periodic paralysis is a rare, autosomal dominant channelopathy characterized by muscle weakness or paralysis. Symptoms typically begin in the first or second decade, attacks of flaccid paralysis usually occurring on awakening in the night or in the early morning. Weakness may be focal or generalized, usually sparing facial and respiratory muscles. Symptoms lasts for hours to days with gradual resolution. [2,10]
Acute intermittent porphyria A diseases involving heme metabolism defects which results in excessive porphyrins secretion and porphyrin precursors. The resultant axonal polyradiculoneuropathy or neuronopathy is characterized by abdominal pain, psychiatric symptoms, such as hysteria, and peripheral neuropathies. These are accompanied by autonomic symptoms such as tachycardia, hypertension, constipation and urinary retention. Patient usually has a history of prior suggestive attacks. [11]
References
  1. KUKRETI V, SHAMIM M, KHILNANI P. Intensive care unit acquired weakness in children: Critical illness polyneuropathy and myopathy. Indian J Crit Care Med [online] 2014 Feb, 18(2):95-101 [viewed 07 June 2014] Available from: doi:10.4103/0972-5229.126079
  2. BURNS TM. Guillain-Barré syndrome. Semin Neurol [online] 2008 Apr, 28(2):152-67 [viewed 06 June 2014] Available from: https://www.orpha.net/data/patho/Pro/en/GuillainBarre-FRenPro834v01.pdf
  3. HAGEN EM, REKAND T, GRøNNING M, FæRESTRAND S. Cardiovascular complications of spinal cord injury. Tidsskr Nor Laegeforen [online] 2012 May 15, 132(9):1115-20 [viewed 07 June 2014] Available from: doi:10.4045/tidsskr.11.0551
  4. HOLLAND NR. Acute myelopathy with normal imaging. J Child Neurol [online] 2013 May, 28(5):648-50 [viewed 07 June 2014] Available from: doi:10.1177/0883073812448438
  5. BESA NC, COLDIRON ME, BAKRI A, RAJI A, NSUAMI MJ, ROUSSEAU C, HURTADO N, PORTEN K. Diphtheria outbreak with high mortality in northeastern Nigeria. Epidemiol Infect [online] 2014 Apr, 142(4):797-802 [viewed 07 June 2014] Available from: doi:10.1017/S0950268813001696
  6. SUH J, GOLDSTEIN JM, NOWAK RJ. Clinical characteristics of refractory myasthenia gravis patients. Yale J Biol Med [online] 2013 Jun, 86(2):255-60 [viewed 07 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23766745
  7. WRIGHT WF, RIEDEL DJ, TALWANI R, GILLIAM BL. Diagnosis and management of Lyme disease. Am Fam Physician [online] 2012 Jun 1, 85(11):1086-93 [viewed 07 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/22962880
  8. DESAI S, PELLETIER L, GARNER M, SPIKA J. Increase in poliomyelitis cases in Nigeria. CMAJ [online] 2008 Oct 21, 179(9):930 [viewed 07 June 2014] Available from: doi:10.1503/cmaj.081265
  9. THANONGSAKSRIKUL J, CHAICUMPA W. Botulinum neurotoxins and botulism: a novel therapeutic approach. Toxins (Basel) [online] 2011 May, 3(5):469-88 [viewed 07 June 2014] Available from: doi:10.3390/toxins3050469
  10. ABBAS H, KOTHARI N, BOGRA J. Hypokalemic periodic paralysis. Natl J Maxillofac Surg [online] 2012 Jul, 3(2):220-1 [viewed 07 June 2014] Available from: doi:10.4103/0975-5950.111391
  11. CARVALHO AA, ARçARI DD. Acute intermittent porphyria after gastroplasty. Arq Neuropsiquiatr [online] 2011 Dec, 69(6):992 [viewed 07 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/22297895

Investigations - for Diagnosis

Fact Explanation
Full blood count Has a less value in diagnosis. But it is used to exclude competing diagnoses and assess functional status and prognosis. [1,2]
Creatine phosphokinase (CPK) Creatine phosphokinase is done to exclude several differential diagnoses such as myopathies or systemic inflammatory conditions. [1,2]
Nerve conduction tests Nerve conduction studies helps to establish the diagnosis of GBS and to identify the GBS subtype. Nerve conduction signs of GBS can include nerve conduction slowing, prolongation of the distal latencies and prolongation or absence of the F-waves of the motor nerves. Sensory nerve conduction studies help to differentiate sub types. [1,2,3,4]
Electromyography (EMG) EMG has a limited value. Reduction in motor unit recruitment and absence of denervation help to support the suggestion of a demyelination though they can not be used to differentiate GBS from wallerian degeneration. [1,2]
Cerebrospinal fluid (CSF) analysis Elevated CSF protein (>400 mg/L) with normal cell count (albumino cytological dissociation) is characteristic for GBS. Mononuclear cell count may be either normal or less than 50 cells/mm. The CSF is normal in the first week of the illness but protein level rises significantly during the second week. Around 10% will not have a protein elevation and this includes patients with the Miller-Fisher variant. [1,2,3,4]
MRI scan This is not usually done. But contrast-enhanced spinal MR imaging can be used as a supplementary diagnostic modality in the diagnosis specially when the clinical and electrophysiologic findings are equivocal. Marked enhancement of nerve roots of the conus medullaris and cauda equina have been reported may play a role in diagnosing GBS. [5]
References
  1. WALLING AD, DICKSON G. Guillain-Barré syndrome. Am Fam Physician [online] 2013 Feb 1, 87(3):191-7 [viewed 06 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23418763
  2. BURNS TM. Guillain-Barré syndrome. Semin Neurol [online] 2008 Apr, 28(2):152-67 [viewed 06 June 2014] Available from: doi:10.1055/s-2008-162261
  3. PITHADIA AB, KAKADIA N. Guillain-Barré syndrome (GBS). Pharmacol Rep [online] 2010 Mar-Apr, 62(2):220-32 [viewed 06 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20508277
  4. WINER J. B.. An Update in Guillain-Barré Syndrome. Autoimmune Diseases [online] 2014 December, 2014:1-6 [viewed 06 June 2014] Available from: doi:10.1155/2014/793024
  5. ALKAN OZLEM, YILDIRIM TULIN, TOKMAK NAIME, TAN MELIHA. Spinal MRI Findings of Guillain-Barré Syndrome. Radiology Case [online] 2009 March [viewed 06 June 2014] Available from: doi:10.3941/jrcr.v3i3.153

Investigations - Fitness for Management

Fact Explanation
Peak flow meter Peak flow meter measures peak expiratory flow rate (PEFR) which is used in assessing the respiratory function. It is readily available and reproducible. Peak expiratory flow (PEF) is the maximal flow achieved during the maximally forced expiration initiated at full inspiration, measured in liters per minute or in liters per second. A fall in the peak expiratory flow rate to one quarter the predicted value but record normal Paco2 values imply that it's safe to withhold assisted ventilation. So, serial measurement of PEFR in Guillain Barre syndrome to predict the involvement of respiratory muscle is clinically important to give warning of the hypoventilation and need for ventilator support. [1,2]
Spirometry FEV1/FVC ratio represents the proportion of a person's vital capacity that they are able to expire in the first second of expiration. That value is below normal/ reduces if respiratory function is getting compromised. [1,2]
References
  1. GRANT I., MAWDSLEY C, CROMPTON G K, JELLINEK E., WILLEY R., ASHWORTH B. Ventilatory failure in Guillain-Barre syndrome: indications for assisted ventilation in neurological diseases. Thorax [online] 1981 February, 36(2):159-160 [viewed 09 June 2014] Available from: doi:10.1136/thx.36.2.159
  2. WALLING AD, DICKSON G. Guillain-Barré syndrome. Am Fam Physician [online] 2013 Feb 1, 87(3):191-7 [viewed 06 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23418763

Management - General Measures

Fact Explanation
Deep vein thrombosis prophylaxis Deep vein thrombosis prophylaxis should be given to all patients due to increased risk. Subcutaneous anticoagulation with fractionated or unfractionated heparin and graduated compression stockings should be instituted until the patient is able to walk independently. [1,2,3,4]
Analgesia As the origin of the pain is either nociceptive or neuropathic, it is difficult to control it with simple analgesics or nonsteroidal anti-inflammatory drugs. Sometimes even opioids may not provide adequate pain relief. Sometimes opioids may worsen some autonomic symptoms instead. Some studies shows that gabapentin and carbamazepine are beneficial for some patients with GBS. If this pain tends to continue as a chronic pain, Tricyclic antidepressants and tramadol may be added for the chronic/ long term management. [1,2]
Respiratory assistance Patient should be closely monitored regarding the respiration. This includes maximal inspiratory pressures and vital capacities. If vital capacity is less than 20 mL/kg or maximal inspiratory pressure is worse than -30 cmH2O, he/ she must be electively intubated ideally in an intensive care setting. The duration of ventilation may lengthy. If it exceeds 2 weeks, tracheostomy should be considered. Weaning should be guided by serial lung function monitoring and assessment of strength. [1,2,4,5]
Haemodynamic monitoring This should be started as early as possible. Pulse and blood pressure which tends to fluctuate should be monitored frequently or with a multi-monitor. This should be ideally continued until the patient is off ventilator support and have begun to recover. Prolonged hypotensive state should be managed with fluid bolus, whereas hypertension should be treated with short acting antihypertensives such as labetalol. [1,2,5,6]
Nutrition Nasogastric or gastric tube feeding should be considered early. High energy and high protein diet is helpful to prevent muscle wasting and to assist respiratory weaning. Continuous enteral feeding is better tolerated than bolus feeding. [1,2,6,7]
Positioning Patient's position on bed should be changed time to time in order to prevent pressure sores with prolonged bedridden state. [1,2,5]
Rehabilitation Following the acute state, patient should be managed in a multidisciplinary approach to focus on improving independence including training on activities of daily living. This includes physiotherapy, occupational therapy and speech therapy. [5,6,7]
Physical therapy Patient should be involved in daily range of movement exercises. This helps to prevent disuse atrophy and contractures. This should be followed by active muscle strengthening exercises. Physiotherapists assist to correct functional movements. physiotherapy is useful in regaining strength, endurance, and gait quality. Ankle-foot orthosis can be used for lasting foot drop. A wheelchair should be supplied as an early aid to improve activities of daily living. [5,6,7]
Speech and language therapy Speech and language therapists help regain speaking and swallowing abilities, This is particularly useful in patients who were intubated. These patients are those who were suffering from severe oropharyngeal weakness in acute stage. [5,6,7]
Education and counselling of the patient and family Even on discharge patient may suffer several disabilities. Long term disability brings some psychological issues such as mild depression and mental fatigue. Caregivers may anxious or worried about the special care they should give, inability to return to driving and work, financial constraints and issues regarding marital stress. So, both parties should be thoroughly educated regarding the disease, home care and prognosis. Counselling should be applied if needed. [5,7]
References
  1. WALLING AD, DICKSON G. Guillain-Barré syndrome. Am Fam Physician [online] 2013 Feb 1, 87(3):191-7 [viewed 06 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23418763
  2. PITHADIA AB, KAKADIA N. Guillain-Barré syndrome (GBS). Pharmacol Rep [online] 2010 Mar-Apr, 62(2):220-32 [viewed 06 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20508277
  3. BURNS TM. Guillain-Barré syndrome. Semin Neurol [online] 2008 Apr, 28(2):152-67 [viewed 06 June 2014] Available from: doi:10.1055/s-2008-1062261
  4. WINER J. B.. An Update in Guillain-Barré Syndrome. Autoimmune Diseases [online] 2014 December, 2014:1-6 [viewed 06 June 2014] Available from: doi:10.1155/2014/793024
  5. KHAN F. Rehabilitation in Guillian Barre syndrome. Aust Fam Physician [online] 2004 Dec, 33(12):1013-7 [viewed 07 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/15630924
  6. MEENA AK, MURTHY J. M. K., KHADILKAR SV. Treatment guidelines for Guillain-Barré Syndrome. Ann Indian Acad Neurol [online] 2011 December [viewed 06 June 2014] Available from: doi:10.4103/0972-2327.83087
  7. HARMS M.. Inpatient Management of Guillain-Barre Syndrome. The Neurohospitalist [online] December, 1(2):78-84 [viewed 06 June 2014] Available from: doi:10.1177/1941875210396379

Management - Specific Treatments

Fact Explanation
Immunotherapy Immunotherapy comprises intravenous immunoglobulins or plasma exchange. Both have been shown to be equally efficacious. [1,2,3]
Plasmapheresis Plasmapheresis removes circulating antigen -antibody complexes which initiate the disease progression. It has been proven to reduce the time for recovery, and the need and duration of ventilation. Plasmapheresis is more effective in severe disease when is used within the first week of symptom onset. Usually four plasmapheresis sessions are needed for severe disease. Two sessions will be sufficient for mild disease. [1,2,4]
Intravenous immunoglobulin (IV Ig) IV Ig is a blood product which contains the pooled, polyvalent, IgG antibodies extracted from the plasma of donors. In GBS,it is recommended to start IV Ig within 2 weeks of symptom onset. The usual dose is 0.4 g per kg per day for 5 days. IG blocks Fc receptors on macrophages preventing antibody targeted attachment on Schwann cell membrane and myelin or on axolemma. The usage of it hastens recovery and reduces long-term morbidity. The complications of immunoglobulin therapy is low than plasmapheresis. IV Ig is contraindicated in IgA deficiency and renal failure. [1,2,4]
Corticosteroids Though oral steroids are recommended in some studies, some studies say they are not beneficial and may even be harmful. It may cause inhibition of the macrophage repair process. Intravenous corticosteroids also do not have any significant short- or long-term benefits. [1,2,5]
Treatment setting Diagnosed GBS patients should be admitted to a hospital for close monitoring. Patients with respiratory insufficiency or autonomic dysfunction with cardiovascular complications should be continuously monitored in an emergency care setting. If ventilatory support is required, patient should be sent to intensive care unit. [1,2]
References
  1. WALLING AD, DICKSON G. Guillain-Barré syndrome. Am Fam Physician [online] 2013 Feb 1, 87(3):191-7 [viewed 06 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23418763
  2. PITHADIA AB, KAKADIA N. Guillain-Barré syndrome (GBS). Pharmacol Rep [online] 2010 Mar-Apr, 62(2):220-32 [viewed 06 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20508277
  3. WINER J. B.. An Update in Guillain-Barré Syndrome. Autoimmune Diseases [online] 2014 December, 2014:1-6 [viewed 06 June 2014] Available from: doi:10.1155/2014/793024
  4. HUGHES RA. Give or take? Intravenous immunoglobulin or plasma exchange for Guillain-Barré syndrome. Crit Care [online] 2011 Jul 28, 15(4):174 [viewed 07 June 2014] Available from: doi:10.1186/cc10312
  5. GRAVESON GS. The use of steroids in the treatment of the Guillain-Barre syndrome. Proc R Soc Med [online] 1961 Jul:575-6 [viewed 07 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/13708018