Year : 2014 | Volume : 2 | Issue : 1 | Page : 1 - 4  

Guillain Barre Syndrome – Effect of Plasma Pheresis: 6 months Study

Bheeshma1, V. Geeta2, Jijiya Bai3, G.Sreenivas4, O. Shravan Kumar5

1Associate professor of Pathology, 2Assistant professor of Pathology, 3Professor of Pathology & Principal, 4Associate Professor of Community Medicine, Government Medical College, Nizamabad; 5Professor and Head, Department of Pathology, Gandhi Medical College & Hospital, Hyderabad


Guillain Barre Syndrome is an acute poly neuropathy, a disorder affecting the peripheral nervous system. The typical symptoms are ascending paralysis, weakness starting at the feet and hands, migrating towards the trunk. Ours is a prospective study related to the effect of plasma pheresis in the treatment of Guillain Barre syndrome along with neurology & General Medicine departments. So far 10 patients were subjected to plasmapheresis along with the conventional treatment and physiotherapy. All the patients recovered completely.

Key words: Ascending Paralysis, Guillain Barre Syndrome, Plasma Pheresis.

Corresponding Author: Dr. Bheeshma Associate Professor of Pathology, Government Medical College, Nizamabad. Email:


[LANDRY S PARALYSIS] [GUILLAIN BARRE STROHL SYNDROME (GBS)]   is an acute poly neuropathy, a disorder affecting the peripheral nervous system. The typical symptoms are Ascending paralysis, weakness starting at the feet and hands migrating towards the trunk. [1]

Classification: 6 Sub types

  1. Acute Inflammatory Demyelinating Polyneuropathy [AIDP]

It is common form caused by an autoimmune response directed against Schwann cell   membrane.

  1. Miller Fischer Syndrome [MFS] [1, 2]

Rare variant constitute 5% of all cases and characterized by descending paralysis. The eye muscles are affected first next trunck accordingly. It manifests as the triad of ophthalomoplegia, ataxia, and areflexia. Limbs are spared. ANTI GQIb antibodies present in 90% of cases.

  1. Acute Motor Axonal Neauropathy [AMAN]

It is prevalent in China and Mexico, affects motor nodes of Ranvier may be due to an auto immune response directed against the axoplasm of peripheral nerves, ANTI –GD1a antibodies are present. ANTIGD3 antibodies are more common. The disease may be seasonal and recovery can be rapid.

  1. Acute Motor Sensory Axonal Neuropathy [AMSAN]

It is similar to AMAN but, it also affects sensory nerves with axonal damage, recovery is slow and incomplete.

  1. Acute Pan Automatic Neuropathy [APAN]

APAN is most rare variant, sometimes accompanied by encephalopathy. It is associated with high mortality rate due to cardiovascular involvement and dysarrythmias. Symptoms include impaired sweating, lack of tear formation, photophobia, and dryness of nasal oral mucosa itching and peeling of skin, nausea, dysphagia and constipation.

  1. Bicker Staffs Brainstem Encephalitis [BBE]      

This is characterized by acute onset of opthalomoplegia, ataxia, impaired consciousness, hyper reflexia or Babinski’s sign. Irregular hyper intense lesions located in brain stem - pons, midbrain and medulla. [2]

Signs and Symptoms:

The disorder is characterized by symmetrical weakness which affects the lower limbs first and rapidly progresses in an ascending fashion and the arms and facial muscles, lower cranial nerves are affected leading to bulbar weakness, oropharyngeal dysphagia and respiratory difficulties   Most patients require hospitalization and 30% require ventilator assistance. Loss of proprioception [position sense], areflexia, and pain in the weakened muscles are common. Severe cases manifest bladder dysfunction. Autonomic dysfunction resulting in increased blood pressure, orthostatic hypotension and cardiac arrhythmias. Acute paralysis may be related to Sodium channel blocking factor in the cerebrospinal fluid [CSF].

All forms of GBS are autoimmune diseases, due to an immune response to foreign antigens such as infectious agents [e.g. Campylobacter jejuni, [3, 4, 5] Cytomegalovirus, [6], Influenza virus, [7, 8, 9, 10] that is mis targeted at host nerve tissue Gangliosides instead, a phenomenon called molecular mimicry. [11]. 60% of cases do not have any cause. Autoimmune attack results in myelin damage which leads to nerve conduction block and muscle paralysis accompanied by sensory or autonomic disturbances.                      

Demyelination and degeneration of the myelin sheath and axon occur in the segmental peripheral nerves, and in the anterior and posterior spinal nerve roots. Antibodies directed against various constituents of peripheral myelin have been identified. Inflammation, edema, and damaged nerves result in both sensory and motor dysfunction (the muscles of the trunk and upper extremities). The diagnosis is supported by the existence of albuminocytologic dissociation in the cerebrospinal fluid.


The diagnosis of GBS usually depends on findings such as rapid development of muscle paralysis, areflexia, absence of fever, and a likely inciting event. Cerebrospinal fluid analysis (through a lumbar spinal puncture) and electro diagnostic tests of nerves and muscles (such as nerve conduction studies) are common tests ordered in the diagnosis of GBS.


In cerebrospinal fluid, characteristic findings include albumin-cytological dissociation. As opposed to infectious causes, there is elevated protein level (100-1000mg/dl), without an accompanying increased cell count (absence of pleocytosis). A sustained increased white blood cell count may indicate an alternative diagnosis such as infection.

Electromyography (EMG) and nerve conduction studies (NCS) may show prolonged distal latencies, conduction slowing, conduction block, and temporal dispersion of compound action potential in demyelinating cases. F-waves and H-reflexes may be prolonged or absent. Needle EMG is frequently normal in acute cases. Reduced, neuropathic recruitment in weak muscles can be seen. Fibrillations will be seen on needle EMG if there is some axonal injury after 3 to 4 weeks. In primary axonal damage, the findings include reduced amplitude of action potentials without conduction slowing.


Features required for diagnosis are progressive weakness in legs and often arms and areflexia (the absence of deep tendon reflexes).

Features that strongly support diagnosis are 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), autonomic dysfunction, pain (often present), high concentration of protein in CSF, and typical electro diagnostic features.

Features that should raise doubt about the diagnosis include severe pulmonary dysfunction with limited limb weakness at onset, severe sensory signs with limited weakness at onset, bladder or bowel dysfunction at onset, fever at onset, sharp sensory level, slow progression with limited weakness without respiratory involvement (sub acute inflammatory demyelinating poly neuropathy or chronic inflammatory demyelinating poly neuropathy [CIDP] is more likely), marked persistent asymmetry of weakness, persistent bladder or bowel dysfunction, increased number of mononuclear cells in CSF(>50x10/L), and polymorphonuclear cells in CSF.


Supportive care is the cornerstone of successful management in acute patients. Of greatest concern is respiratory failure due to paralysis of the diaphragm, the muscle most important for breathing-when the vital capacity (VC) is less than 20 ml/kg, the negative inspiratory force (NIF) is less negative (i.e., closer to zero) than -25 cmH2O, more than 30% decrease in either VC or NIF within 24 hours, rapid progression of disorder, or autonomic instability.

Subsequent treatment consists of attempting to reduce the body’s attack on the nervous system, either by plasmapheresis, filtering antibodies out of blood stream, or by speaking and swallowing ability, especially if the patient was intubated or received a tracheostomy.


Recovery usually starts after the fourth week from the onset of the disorder. Approximately 80% of patients have a complete recovery within a few months to year, although minor findings may persist, such as areflexia. About 5-10% recovers with severe disability, with most of such cases involving severe proximal motor and sensory axonal damage with inability of axonal regeneration. Despite all improvements in treatment and supportive care, [11, 12] the death rate is still about 2-3% even in the best intensive care units. Worldwide, the death rate runs slightly higher (4%), mostly from a lack of availability of life support equipment during lengthy plateau lasting four to six weeks, and in some cases up to one year, when a ventilator is needed in the worst cases. About 5-10% of patients have one or more late relapses, in which case they are then classified as having CIDP.

Poor prognostic factors include age over 40 years, history of preceding diarrheal illness, requiring ventilator support, high anti-GM1 titer and poor upper-limb muscle strength.

Therapeutic plasma Pheresis:

Therapeutic aphaeresis does not cure a disease but can be very effective in the alleviating symptoms of the underlying disease. The efficacy of the procedure is enhanced by concomitant drug therapy particularly immune suppressive therapy in immune mediated problems.

Plasma exchange:

It is not a cure for the underlying disease but rather a way to provide short term relief. During therapeutic plasma exchange, the pathological substances in the plasma are removed and replaced with a fluid. The replacement fluid may be plasma, albumin, saline or combination of albumin and saline. The plasma is constantly replaced with the fluid so patient’s blood volume does not change development of auto antibodies that is cause of disease in which IgM aphaeresis is effective as IgM is primarily intravascular. Whereas IgG is equally present in intravascular and extra vascular spaces. Plasma pheresis is indicated in the conditions medicated by plasma factors such as auto antibodies, immune complex, drugs, or toxins bound to problems high cholesterol or triglycerides. [12, 13, 14, 15]

Common conditions for therapeutic plasma pheresis and pathological substances:

  1. 1. Walden stroms macro globinaemia –immunoglobulin causing hyper viscosity
  2. Myasthenia gravis-auto antibodies
  3. Good pasture syndrome-auto antibodies
  4. Acute GBS-uncertain
  5. Lupus erythmatosus-immunocomplex

All aphaeresis procedures use anticoagulant to prevent blood from clotting as it enters the separation machine. Most commonly used anticoagulant is (ACD) normal saline is used to prime the system to keep line open and to help in maintaining the fluid volume and oncotic pressure.

Fresh frozen plasma (FFP):

FFP contains all constituents of the removed plasma and this is the optimal replacement fluid.FPP may transmit transfusion-transmitted diseases, can cause allergic reactions, ABO incompatibility or sensitization to plasma protein. FFP is recommended in plasma exchange in patients with TTP, HUS.

Case reports:

Total ten patients with GBS were subjected to plasmapheresis during six months periods. Number of plasma exchanged were ten in number in ten consecutive days.

Case number I:

72 years female complaining of weakness of both lower limbs followed by upper limbs of duration 10 days and was unable to get up from bed, not able to walk with support also.

Power:   Upper Limbs: 3/5   3/5, Lower Limbs: 2/5   2/5

Case number II: GBS (AMAN variant)

35 years male complained of sudden onset of pain in right lower limb since 10 days. Pain in the right calf progressed to involve right thigh. Two days after he developed pain in the left calf progressed to left thigh. He developed parenthesis and weakness of both lower limbs and developed difficulty in chewing hard objects 3 days later.

Case number III: [AIDP GBS]

This is about 36 years old male patient (known case of Retro Viral Disease since three years) on Anti Retro Viral Therapy. He is also on Anti Tuberculosis Treatment irregularly. He complained of tingling and paraesthesia of both feet of 25 days duration. Developed weakness of both lower limbs five days, later followed by upper limbs. EMNG report showed severe demyelonopathy, axonal motor sensory, neuropathy of all four limbs.

Case number IV:

A 23 years old male complaining of weakness of both lower limbs of 15 days duration followed by weakness of both upper limbs. He was diagnosed as having moderate to severe axonal and demyelinating motor sensory neuropathy (GBS).

Case number V:

A 25 years female, post Lower Segment Cesarean Section of ten days developed weakness of both lower limbs followed by upper limbs. She was unable to get up from bed and unable to walk associated with incontinence of urine. Foleys catheterization was done. She was diagnosed as GBS AMAN.

Case number VI:

A 24 years female presented with history of Intra Uterine Death of baby at seven months pregnancy. Two days after evacuation she developed paraesthesia and weakness of both lower limbs and progressed to upper limbs after 3 days. She was diagnosed as GBS.

Case number VII:

A 45 years male presented with history of fever 10 days back then developed parasthesias and weakness both lower limbs after 3 days progressed to upper limbs. He was diagnosed as: GBS AMSAN.

Case number VIII:

A 28 years male complained of weakness of both lower limbs three weeks back. After three days developed difficulty in walking and was not able to get up from squatting position. After one week developed weakness of both upper limbs. Patient was bedridden. He was diagnosed as: GBS AMSAN.

Case number IX:

A 27 years male complaining of weakness of both lower limbs of ten days duration progressed to upper limbs. He was diagnosed as: GBS AMAN.

Case number X:

A 48 years male presented with history of diarrhea 20 days back. Then he developed weakness of both lower limbs followed by upper limbs after seven days. He was diagnosed as: GBS AMAN.


All ten patients were subjected to plasmapheresis i.e ten cycles on ten consecutive days. On an average motor recovery was seen within 6 days after treatment. Patients were able to walk with assistance after the completion of 10 sessions i.e 10 days.


Plasma exchange dramatically improved GBS patients when compared to supportive care alone. The time to recover walking with aid, the time to recover walking unaided and the time to be improved by one or more disability grades were all shortened by plasma exchange. After four weeks, the combination of data from five studies accounting for a total of 604 patients showed that plasma exchange increased the number of patients being improved by one or more disability grade and the mean grade improvement. Plasma exchange shortened the time on ventilator, and the proportion of ventilator dependent patients was dramatically decreased. During the hospital stay, plasma exchange slightly decreased the risk of severe infections, cardiovascular instability and cardiac arrhythmias. Plasma exchange also had long-term benefits. It increased significantly the number of patients who had recovered full muscle strength after a year. Furthermore, plasma exchange decreased the proportion of patients with severe motor sequelae after a year. However, the relative risk of relapsing was slightly increased by plasma exchange. [16, 17, 18, 19]

Plasma exchange may be more efficacious when performed within seven days of onset of motor deficit. Nevertheless, patients with GBS may benefit from plasma exchange up to 30 days after disease onset.


Plasma pheresis is

  1. Cost effective when compared with IV immunoglobulin.
  2. Comparatively superior affect in mild GBS two sessions of plasma exchange are significantly superior to none. In moderate and severe GBS four sessions are significantly superior to two.
  3. Plasma exchange should preferably be started seven days or less after the onset of neuropathy. Nevertheless, patients with GBS may benefit from plasma exchange up to 30 days after disease onset. Since the risk of relapse is increase by plasma exchange, patients should be observed closely for the first few weeks after treatment.


  1. Fisher M. An unusual variant of acute idiopathic polyneuritis (syndrome of opthalmolplegia, ataxia and areflexia). N Engl J Med 1956;255(2):57-65.
  2. van Doorn PA, Ruts L, Jacobs BC. Clinical features, pathogenesis, and treatment of Gullian-Barre syndrome. Lancet Neurol. 2008 Oct;7(10):939-50.
  3. Ho TW, Mishu B, Li CY, Gao CY, Cornblath DR, Griffin JW et al. Gullian-barre syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies”. Brain. 1995 Jun;118 ( Pt 3):597-605.
  4. Yuki N. Campylobacter genes responsible for the development and determinant of clinical features of Guillain-Barre syndrome. Nihon Rinsho. 2008 Jun;66(6):1205-10.
  5. Kuwabara S, Ogawara K, Misawa S, Koga M, Mori M, Hiraga A et al. Does Campylobacter jejuni infection elicit "demyelinating" Guillain-Barre syndrome? Neurology. 2004 Aug 10;63(3):529-33.
  6. Orlikowski D, Porcher R, Sivadon-Tardy V, Quincampoix JC, Raphaël JC, Durand MC et al. Guillain-Barré syndrome following primary cytomegalovirus infection: A prospective cohort study. Clin Infect Dis. 2011 Apr 1;52(7):837-44.
  7. Sivadon-Tardy V, Orlikowski D, Porcher R, Sharshar T, Durand MC, Enouf V et al. Guillain-Barré syndrome and influenza virus infection. Clin Infect Dis. 2009 Jan 1;48(1):48-56.
  8. Haber P, DeStefano F, Angulo FJ, Iskander J, Shadomy SV, Weintraub E. Guillian-Barre syndrome following influenza vaccination. JAMA. 2004 Nov 24;292(20):2478-81.
  9. Lehmann HC, Hartung HP, Kieseier BC, Hughes RA. Guillain-Barré syndrome after exposure to influenza virus. Lancet Infect Dis. 2010 Sep;10(9):643-51.
  10. Liang XF, Li L, Liu DW, Li KL, Wu WD, Zhu BP et al. Safety of influenza A (H1N1) vaccine in postmarketing surveillance in China. N Engl J Med. 2011 Feb 17;364(7):638-47.
  11. Ang CW, Jacobs BC, Laman JD. Guillian-barre syndrome: a true case of molecular mimicry. Trends Immunol. 2004 Feb;25(2):61-6.
  12. Hughes RA, Wijdicks EF, Barohn R, Benson E, Cornblath DR, Hahn AF et al. Practice parameter: immunotherapy for Guillain-Barré syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2003 Sep 23;61(6):736-40.
  13. Davidson I, Wilson C, Walton T, Brissenden S. Physiotherapy and Guillain-Barré syndrome: results of a national survey. Physiotherapy. 2009 Sep;95(3):157-63.
  14. Karavatas SG. The role of neurodevelopmental sequencing in the physical therapy management of a geriatric patient with guillian-barre syndrome. Topics in Geriatric rehabilitation 2005; 21(2):133-5.
  15. Bril V, Ilse WK, Pearce R, Dhanani A, Sutton D, Kong K. Pilot trial of immunoglobulin versus plasma exchange in patients with Guillain-Barré syndrome. Neurology. 1996 Jan;46(1):100-3.
  16. Randomized trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Lancet. 1997 Jan 25;349(9047):225-30.
  17. Mori M, Kuwabara S, Fukutake T, Hattori T. Plasmapheresis and Miller Fisher syndrome: analysis of 50 consecutive cases. J Neurol Neurosurg Psychiatry. 2002 May;72(5):680.
  18. McKhann GM, Cornblath DR, Ho T, Li CY, Bai AY, Wu HS et al. Clinical and electrophysiological aspects of acute paralytic disease of children and young adults in northern China. Lancet. 1991 Sep 7;338(8767):593-7.
  19. Griffin JW, Li CY, Ho TW, Xue P, Macko C, Gao CY. Guillain-Barré syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases. Brain. 1995 Jun;118 ( Pt 3):577-95.

Acknowledgement: I sincerely thank the staff of blood bank and Department of Neurology, Gandhi Hospital, Hyderabad for their timely support.

Source of Support: Nil. Conflict of Interest: None.


Important links

adv apply rec

Open Access Journal

MRIMS Journal of Health Sciences is an open access journal which means that all content is freely available without charge to the user or his/her institution. Users are allowed to read, download, copy, distribute, print, search, or link to the full texts of the articles in this journal without asking prior permission from the publisher of the author. This is in accordance with the BOAI definition of open access.

Visitor Count

© 2020 Chandramma Education society . All Rights Reserved.