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GUILLAIN-BARRÉ SYNDROME (GBS) – AN

ORPHAN DISEASE

Article in World Journal of Pharmaceutical Research · May 2017

DOI: 10.20959/wjpr20175-8325

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 World Journal of Pharmaceutical Research
Mishra et al. World Journal of Pharmaceutical Research
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Volume 6, Issue 5, 393-400. Review Article ISSN 2277–7105

GUILLAIN-BARRÉ SYNDROME (GBS) – AN ORPHAN DISEASE

Ambed Mishra1*, Sai Krishna G.1 and Komal Krishna T.2

1
Department of Pharmacy Practice, JSS College of Pharmacy, Jagadguru Sri
Shivarathreeshwara University, Mysore 570 015, India.
2
Department of Physiotherapy, JSS Hospital, Mysore 570 004, India.

ABSTRACT
Article Received on
25 Feb. 2017, Guillain-Barré syndrome (GBS) is an unusual illness of the nervous
Revised on 17 March 2017, system in which a person’s own immune system damages the nerve
Accepted on 05 April 2017
DOI: 10.20959/wjpr20175-8325
cells, causing muscle weakness, and sometimes, paralysis. This
condition has an expected incidence of 1-2 cases per 100,000
individuals. The mortality rate due to misdiagnosis is very high.
*Corresponding Author
Though the expected number of cases to that are reported vary from
Dr. Ambed Mishra
Department of Pharmacy underdeveloped and developing countries, and are very less. The actual
Practice, JSS College of reported cases of occurrence of GBS is nearly negligible from these
Pharmacy, Jagadguru Sri countries. This may be attributed to the disease misdiagnosis.
Shivarathreeshwara
Misdiagnosis occurs due to lack of knowledge of GBS in many
University, Mysore 570
practitioners, where they misdiagnose GBS to be a case of paralysis.
015, India.
This becomes a serious issue as GBS may cause death when untreated
within few hours to days. This review highlights the major symptoms including causes of
misdiagnosis due to similar presenting signs and symptoms to other diseases and treatment of
this rarely reported disease. This review aims to pass a safety message to all the healthcare
practitioners regarding GBS and to aid them in recognizing the disease better. Hence,
decreasing the misdiagnosis and mortality.

KEYWORDS: Guillain-Barré syndrome, GBS, increased mortality, Nervous system.

INTRODUCTION
Guillain-Barré syndrome (GBS) is an uncommon disorder in which a person’s self-immune
system attacks part of the peripheral nervous system and causes damage to the nerve cells,
thereby resulting in muscle weakness and paralysis. It can cause symptoms which usually last

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Mishra et al. World Journal of Pharmaceutical Research

for a few weeks.[1, 2] It is often triggered by bacterial or viral infection and characterized by
acute symmetric ascending flaccid paralysis with or without sensory changes in the body.[3]
GBS is a very rare but serious disorder. It can cause lethal complications if autonomic
nervous system is involved or if the respiratory muscles are affected. The weakness reaches
its peak in 2-4 weeks.[4] It is named after the French physicians Georges Guillain and Jean
Alexandre Barré, who described it in 1916. The best description of 'ascending paralysis' was
made by a Frenchman named Jean Baptiste Octave Landry de Thézillat in 1859.[5, 6]

SYNONYMS[1, 3, 6]
Acute Idiopathic Polyradiculoneuritis / Acute Inflammatory Polyneuropathy / Demyelinating
Polyradiculoneuropathy / Acute Idiopathic Polyneuritis / French Polio / Landry’s Ascending
Paralysis and Landry Guillain-Barré Syndrome.

EPIDEMIOLOGY
The annual incidence of GBS is nearly 1-2/100,000. Men are affected approximately 1.5
times more than women.[7] There are no incidence studies of GBS in Indian population, but
some case based studies have been reported. It occurs evenly throughout the western
hemisphere, without geographical clustering.[8-10] It can occur in any individual irrespective
of age. A mortality rate of 2% to 6% is seen in patients with GBS increasing to between 15%
and 30% in patients requiring mechanical ventilation. Death due to acute cardiovascular
collapse (in the setting of autonomic dysfunction) has been found to occur in up to 14% of
patients.[11]

PATHOPHYSIOLOGY
GBS is triggered by immunologic mechanisms, although exact pathogenesis remains
incompletely defined. GBS is viewed as an autoimmune disease often triggered by an
antecedent bacterial or viral infection, most commonly an upper respiratory tract infection or
gastroenteritis.[3, 4] Molecular mimicry is postulated to be responsible for inducing GBS. It is
likely that immune responses directed toward the infecting organisms cross react with neural
tissues, targeting epitopes in cranial and spinal nerves and roots. Types, symptoms and
pathophysiology of the disease is depicted in the table 1.

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Table 1: Types, symptoms and pathophysiology of Guillain-Barré syndrome[1, 4, 5]

TYPE SYMPTOMS PATHOPHYSIOLOGY
Motor inflammatory demyelination, Macrophages invade intact myelin sheaths and
AIDP
Axonal damage. denude the axons
Macrophages invade the nodes of Ranvier
Motor respiratory involvement, where they insert between the axon and
AMAN
primary axonal degeneration. surrounding Schwann-cell axolemma, leaving
the myelin sheath intact
Motor and sensory involvement
with respiratory dysfunction, Similar to AMAN with the involvement of
AMSAN
primary axonal degeneration with ventral and dorsal roots
prognosis.
Opthalmoplegia, sensory ataxia, Abnormality in sensory conduction, although
MFS
areflexia. the underlying pathology is not clear
Most rare form accompanied by Widespread sympathetic and parasympathetic
APN
encephalopathy. failure occurs
AIDP- Acute inflammatory demyelinating polyradiculoneuropathy; AMAN- Acute motor axonal
neuropathy; AMSAN- Acute motor and sensory axonal neuropathy; MFS- Miller Fisher
Syndrome; APN- Acute Pandysautonomic Neuropathy.

Other environmental toxins related disease mimicking GBS symptoms: Toxins such as
puffer fish toxin or shellfish poisoning, should be considered unlike GBS, toxic neuropathies
generally are slowly progressive, with sensory loss far exceeding weakness and motor loss.
However, the neuropathy seen with exposure to toxins such as organophosphates is more
subacute and is predominately motor. The key to diagnosis of a toxic neuropathy is exposure
history.[12]

ETIOLOGY[4, 5]

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RISKS ASSOCIATED WITH OCCURRENCE

Patients often complain of a preceding bacterial or viral infection. However, there are other
acute neuropathies associated with infection such as diphtheria or botulism that must be
considered.[13, 14]
Appropriate diagnostic labs should be ordered based on the history. The
medical history should also focus on factors that might point to a diagnosis other than GBS
such as a previous toxin or heavy metal exposure (arsenic, organophosphates), or systemic
symptoms of conditions that might produce acute weakness. Other important historical
information includes a social history for risks such as HIV, a family history indicating a
condition such as periodic paralysis, or a medical history of carcinoma. In the most severe
cases, paralysis may involve the trunk and cranial nerves, leading to respiratory failure and
death within several days. The system-wise risks are: Cardiovascular: Labile blood pressure,
Hypertension, Hypotension, Tachyarrhythmia; Gastrointestinal: Dysphagia; Musculoskeletal:
Myalgia, Skeletal muscle tenderness; Neurologic: Paralysis, Sensory deprivation isolation,
Neurological muscle weakness, Cranial nerve disorder, Ataxia, Paraesthesia, Hyporeflexia;
Ophthalmic: Optic disc oedema. Bacterial infectious disease suck as infection with
Campylobacter jejuni is the most frequent pathogenic antecedent to be associated with GBS.
Infection with Mycoplasma pneumoniae has also been implicated. Numerous viruses have
been implicated in GBS, but convincing data are available only for cytomegalovirus, Epstein
Barr virus, and varicella zoster virus. CMV infections (upper respiratory tract infection,
pneumonia, nonspecific flulike illness) comprise the most common viral triggers of GBS
(10% to 20%).

DIAGNOSIS[4, 15]
I. Lumbar Puncture (LP): Cerebrospinal fluid with elevated protein level. A lumbar
puncture is an important diagnostic aid in the diagnosis of GBS. The cerebrospinal fluid
typically demonstrates a normal WBC count and an elevated protein level, though the
protein level may initially be normal, then rise to a peak in 4 to 6 weeks.
II. Nerve Conduction Velocity (NCV) & Electromyogram (EMG): Performed with EMG,
and together, they are often referred to as EMG/NCV studies. NCV analyses the speed at
which signals pass along the nerves. Electromyogram (EMG) records muscle activity
which can display the loss of reflexes is disease characteristic lagging of nerve responses.
Electro-diagnostic studies usually reveal reduced motor nerve conduction velocities or
block, prolonged distal latencies, and an abnormal F response.

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III. Other tests

Electrolytes measurement; serum Arterial blood gas analysis.

White blood cell count with differential: A moderate leucocytosis is present and the
lymphocyte count is decreased, although B cells are increased.

Pulmonary function test: Forced vital capacity <20 mL/kg, maximal inspiratory pressure
<30 cm H2O, or maximal expiratory pressure <40 cm H2O may predict respiratory failure.

MANAGEMENT
There is no specific treatment for Guillain-Barré syndrome. However, there are therapies that
lessen the severity of the illness and accelerate the recovery in most patients. There are also a
number of ways to treat the complications of the disease.

I. Plasma exchange (Plasmapheresis):[16] Plasma exchange has proven to be helpful in the

management of GBS. It is still unknown exactly how Plasmapheresis is helpful in GBS, but it
seems to reduce the severity of the disease episode.[17] This may be due to the fact that plasma
exchange can clear away antibodies and other immune cell-derived factors that could
contribute to nerve damage.
II. Immunoglobulin therapy:[18-20] In high-dose immunoglobulin therapy, intravenous
injections of the proteins are administered in small quantities which the immune system uses
naturally to fight against invading organisms. Studies suggests that high doses of these
immunoglobulins, derived from a pool of thousands of normal donors, to GBS patients can
diminish the immune attacks on the nervous system.
III. Use of steroid hormones: It has also been tested as a way to decrease the severity of
Guillain-Barré, but controlled clinical trials have demonstrated that this treatment not only is
not effective but may even have a deleterious effect on the disease. Usually dosing is 0.4
g/kg/day IV for 5 doses.

DISSCUSSION
An orphan disease is defined as a condition that affects fewer people worldwide. This
includes diseases as familiar as Melioidosis, Tired All The Time Syndrome (Chronic Fatigue
Syndrome), Beauty Parlour Syndrome, Fat Wallet Syndrome, Male Breast Cancer, Selfie
syndrome and many more are of increased concern and clinicians must be made aware of
these Orphan diseases for better diagnosis and management.[21-26]

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There is a deficit of medical and scientific knowledge related to these diseases. Doctors,
researchers and policy makers were unaware of most of the rare diseases and until very
recently there was no real research concerning issues related to the field. There is no cure for
most rare diseases, but the appropriate treatment and medical care can improve the quality of
life of those affected.

CONCLUSION
Guillain-Barré Syndrome can be a devastating disorder because of its sudden and unexpected
onset. In addition, recovery is not necessarily quick. As discussed above, patients usually
reach the point of considerable weakness or paralysis within few days or weeks after the first
symptoms occur. Patients face not only physical difficulties, but emotionally painful periods
as well. It is often awfully difficult for patients to accustom to sudden paralysis and depend
on others for help with routine daily activities. Hence, if there is awareness among patients
and clinicians related to this disease will definitely help improve the current status of care.

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