Guillain-Barre Syndrome
What is Guillain- Barre syndrome (GBS)?
GBS is a rare but serious autoimmune disorder that can affect any part of the nervous system outside of the brain and spinal cord. It is a collection of clinical syndromes that manifests as an acute inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes.
It is an autoimmune disorder such that the immune system attacks and destroys the body's healthy cells. In the case of GBS, the immune system attacks the myelin sheaths of peripheral nerves. The myelin sheaths are the coatings on the axons of nerves, and myelin is essential for the speedy carrying of axonal nerve impulses. When the myelin is damaged, the nerves can no longer send information to the spinal cord and brain, such as touch sensations. This causes the sensation of numbness and muscle weakness. (Demyelinating neuropathy with ascending weakness)
The disease often begins with tingling sensations and weakness in the feet and legs. It then slowly spreads and ascends upward until a large portion of the body is affected. This condition is considered a medical emergency, and an individual should receive medical attention as soon as possible.
GBS can affect people of any age or either sex, although it is slightly more common in older people and males. The annual US incidence of GBS is 1.2-3 per 100,000 inhabitants. The condition usually begins following an infectious disease. The exact causes are still unknown. Most people fully recover within 12 months, but full recovery may take up to 3 years in some cases.
GBS is a rare but serious autoimmune disorder that can affect any part of the nervous system outside of the brain and spinal cord. It is a collection of clinical syndromes that manifests as an acute inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes.
It is an autoimmune disorder such that the immune system attacks and destroys the body's healthy cells. In the case of GBS, the immune system attacks the myelin sheaths of peripheral nerves. The myelin sheaths are the coatings on the axons of nerves, and myelin is essential for the speedy carrying of axonal nerve impulses. When the myelin is damaged, the nerves can no longer send information to the spinal cord and brain, such as touch sensations. This causes the sensation of numbness and muscle weakness. (Demyelinating neuropathy with ascending weakness)
The disease often begins with tingling sensations and weakness in the feet and legs. It then slowly spreads and ascends upward until a large portion of the body is affected. This condition is considered a medical emergency, and an individual should receive medical attention as soon as possible.
GBS can affect people of any age or either sex, although it is slightly more common in older people and males. The annual US incidence of GBS is 1.2-3 per 100,000 inhabitants. The condition usually begins following an infectious disease. The exact causes are still unknown. Most people fully recover within 12 months, but full recovery may take up to 3 years in some cases.
Subtypes of Guillain-Barré syndrome
- Acute motor axonal neuropathy (AMAN) AMAN is a purely motor disorder that is more prevalent in pediatric age groups. It is generally characterized by rapidly progressive symmetrical weakness and ensuing respiratory failure.About 70% of patients with AMAN are seropositive for Campylobacter, with the most cases being associated with preceding C jejuni diarrhea. Prognosis is often quite favorable.It is more common in Japan, China, and Mexico.
- Acute motor-sensory axonal neuropathy (AMSAN): a severe acute illness differing from AMAN in that it also affects sensory nerves and roots. Patients are typically adults. It presents as rapid and severe motor and sensory dysfunction with characteristic marked muscle wasting and recovery is poorer than AMAN. It is also often associated with preceding C jejunidiarrhea. It is more common in Japan, China, and Mexico.
- Acute inflammatory demyelinating polyradiculoneuropathy (AIDP): This is the most common type in the U.S. Typically, the weakness begins in the lower part of the body and gradually ascends to the other body parts. It is generally preceded by a bacterial or viral infection. Nearly 40% of patients are seropositive for C jejuni.
- Miller Fisher syndrome (MFS): MFS occurs in around 5-10% of GBS cases in the U.S. However, this form of GBS is more prevalent in Asia. It classically presents as a triad of ataxia, areflexia, and ophthalmoplegia. Patients may also have mild limb weakness, ptosis, facial palsy, or bulbar palsy. Anti-GQ1b antibodies are prominent. Recovery generally occurs within 1-3 months.
- Acute panautonomic neuropathy: rarest GBS variant, involving the sympathetic and parasympathetic nervous systems. Patients have severe postural hypotension, bowel and bladder retention, anhidrosis, decreased salivation and lacrimation, and pupillary abnormalities. Cardiovascular involvement is common, and dysrhythmias are a significant source of mortality. Recovery is gradual and often incomplete.
- Pure sensory variant of GBS: is characterized a rapid onset of sensory loss, sensory ataxia, and areflexia in a symmetrical and widespread pattern.The prognosis is generally good.
Causes of Guillain-Barre syndrome
The exact causes of GBS are still not known. GBS is considered to be a postinfectious, immune-mediated disease targeting peripheral nerves.
The condition often develops a 1-3 weeks after an infection of the digestive or respiratory tract. In several studies, C jejuni was the most commonly isolated pathogen in GBS. Cytomegalovirus (CMV) infections are the second most commonly reported infections. Other significant infectious agents include Epstein-Barr virus (EBV), Mycoplasma pneumoniae, zika virus, varicella-zoster virus and human immunodeficiency virus.
In rarer cases, the syndrome can occur following surgery, other infections, or immunization. The adjusted relative risk is 1.7 cases per 1 million influenza vaccinations which is very rare thus the benefit of influenza vaccination still outweighs this very rare risk of GBS. According to the Centers for Disease Control and Prevention (CDC):"It is important to keep in mind that severe illness and death are associated with influenza, and vaccination is the best way to prevent influenza infection and its complications." Thus it is generally regarded as safe to continue receiving flu vaccinations.
Risk factors include:
The exact causes of GBS are still not known. GBS is considered to be a postinfectious, immune-mediated disease targeting peripheral nerves.
The condition often develops a 1-3 weeks after an infection of the digestive or respiratory tract. In several studies, C jejuni was the most commonly isolated pathogen in GBS. Cytomegalovirus (CMV) infections are the second most commonly reported infections. Other significant infectious agents include Epstein-Barr virus (EBV), Mycoplasma pneumoniae, zika virus, varicella-zoster virus and human immunodeficiency virus.
In rarer cases, the syndrome can occur following surgery, other infections, or immunization. The adjusted relative risk is 1.7 cases per 1 million influenza vaccinations which is very rare thus the benefit of influenza vaccination still outweighs this very rare risk of GBS. According to the Centers for Disease Control and Prevention (CDC):"It is important to keep in mind that severe illness and death are associated with influenza, and vaccination is the best way to prevent influenza infection and its complications." Thus it is generally regarded as safe to continue receiving flu vaccinations.
Risk factors include:
- Sex: Males are slightly more likely to get GBS.
- Age: Risk increases with age.
- Campylobacter jejuni bacterial infection: A common cause of food poisoning (gastrointestinal infection) prior to onset of GBS
- Influenza virus, HIV, or Epstein-Barr virus (EBV): respiratory infection prior to onset of GBS
- Mycoplasma pneumonia: This is a bacterial infection of the lungs.
- Surgery: Some surgeries can trigger GBS.
- Hodgkin's lymphoma
- Influenza vaccination or childhood vaccinations: These have also been linked to GBS in very rare cases.
Symptoms of Guillain-Barre syndrome (GBS)
Up to two thirds of patients with GBS report an antecedent illness usually upper respiratory of gastrointestinal illnesses or event 1-3 weeks prior to the onset of weakness.
It often begins with tingling and weakness starting in your feet and legs and ascends upwards spreading to your upper body and arms. But in half of the patients, symptoms begin in the arms or face. As Guillain-Barre syndrome progresses, muscle weakness can evolve into paralysis. Patients usually experience their most significant weakness within two to four weeks after symptoms begin.
Signs and symptoms and complications of Guillain-Barre syndrome may include:
Up to two thirds of patients with GBS report an antecedent illness usually upper respiratory of gastrointestinal illnesses or event 1-3 weeks prior to the onset of weakness.
It often begins with tingling and weakness starting in your feet and legs and ascends upwards spreading to your upper body and arms. But in half of the patients, symptoms begin in the arms or face. As Guillain-Barre syndrome progresses, muscle weakness can evolve into paralysis. Patients usually experience their most significant weakness within two to four weeks after symptoms begin.
Signs and symptoms and complications of Guillain-Barre syndrome may include:
- Sensory changes: Prickling, pins and needles sensations in your fingers, toes, ankles or wrists . Sensory symptoms often precede the weakness. Loss of vibration, proprioception, touch, and pain distally may be present.
- Weakness in your legs that spreads to your upper body. Proximal muscles may be involved earlier than the more distal ones.
- Unsteady walking or inability to walk or climb stairs
- Ophthalmoplegia: Difficulty with eye or facial movements, diplopia and pupillary disturbances
- Facial and oropharyngeal weakness: Facial droop (may mimic Bell palsy), difficulty in speaking (dysarthria), chewing or swallowing (dysphagia)
- Severe pain that may feel achy or throbbing in nature and which may be worse at night. Dysesthesias frequently are described as burning, tingling, or shocklike sensations and are often more prevalent in the lower extremities than in the upper extremities. Dysesthesias may persist indefinitely in 5-10% of patients.
- Autonomic changes: Autonomic nervous system involvement with dysfunction in the sympathetic and parasympathetic systems. It causes Rapid heart rate (tachycardia), low heart rate (bradycardia), Low or high blood pressure, orthostatic hypotension, Anhidrosis and/or diaphoresis. Difficulty with bladder control or bowel function
- Respiratory involvement : Difficulty breathing, Dyspnea on exertion, Difficulty swallowing, Slurred speech. In rare cases, Ventilatory failure with required respiratory support may be required in very severe cases.
Diagnosis and investigations for Guillain-Barre Syndrome
Guillain-Barre syndrome can be difficult to diagnose in its earliest phases as its signs and symptoms are similar to other neurological disorders and may vary from individual to individual. A feature of GBS is when the weakness occurs on both sides of the body and onset is rapid. Whereas neurological conditions are much slower than GBS.
Taking a thorough medical history especially with regards to any recent infections and vaccinations followed by a thorough physical examination with focus on neurological system is important. Guillain-Barré syndrome (GBS) is generally diagnosed on clinical grounds.
Investigations:
Guillain-Barre syndrome can be difficult to diagnose in its earliest phases as its signs and symptoms are similar to other neurological disorders and may vary from individual to individual. A feature of GBS is when the weakness occurs on both sides of the body and onset is rapid. Whereas neurological conditions are much slower than GBS.
Taking a thorough medical history especially with regards to any recent infections and vaccinations followed by a thorough physical examination with focus on neurological system is important. Guillain-Barré syndrome (GBS) is generally diagnosed on clinical grounds.
Investigations:
- Bloods: Full blood count, and metabolic panels are usually normal. They are often done to exclude other diagnoses. Viral and bacteria serology are sometimes done to determine preceding infection.
- Spinal tap (lumbar puncture): A small amount of fluid is withdrawn from the spinal canal in your lower back. During the acute phase of GBS, characteristic findings on CSF analysis include albuminocytologic dissociation, which is an elevation in CSF protein (>0.55 g/L) without an elevation in white blood cells. The increase in CSF protein is thought to reflect the widespread inflammation of the nerve roots.
- Electromyography: Thin-needle electrodes are inserted into the muscles your doctor wants to study. The electrodes measure nerve activity in the muscles. In the acute phase, the only needle EMG abnormality may be abnormal motor recruitment, with decreased recruitment and rapid firing motor units in weak muscles.
- Nerve conduction studies (NCS): Electrodes are taped to the skin above your nerves. A small shock is passed through the nerve to measure the speed of nerve signals. Abnormalities in NCS that are consistent with demyelination are sensitive and represent specific findings for classic GBS.
- Imaging like MRI and CT scan: usually only done to exclude other neurological conditions
- Pulmonary function parameters: should be performed frequently to monitor respiratory status and the need for ventilatory assistance.
Treatment
There is currently no cure for GBS, but medications (e.g. analgesics for pain and heparin to prevent DVT) are available to relieve symptoms. Early recognition and treatment of GBS also may be important in the long-term prognosis, especially in the patient with poor clinical prognostic signs, such as older age, a rapidly progressing course, and antecedent diarrhea.
There are two main types of treatment that can reduce the severity of symptoms and improve the rate of recovery:
Occupational and Physiotherapy
- As the sensory and motor nerves are affected, patients may require physiotherapy exercise to rehabilitate and regain muscle strengths.
- Training with adaptive devices, such as a wheelchair or braces, to give you mobility and self-care skills
- Speech therapy is also necessary for those patients whose speech is also affected.
There is currently no cure for GBS, but medications (e.g. analgesics for pain and heparin to prevent DVT) are available to relieve symptoms. Early recognition and treatment of GBS also may be important in the long-term prognosis, especially in the patient with poor clinical prognostic signs, such as older age, a rapidly progressing course, and antecedent diarrhea.
There are two main types of treatment that can reduce the severity of symptoms and improve the rate of recovery:
- Immunoglobulin therapy: Immunoglobulin containing healthy antibodies from healthy donors are given intravenously (IV). High doses of immunoglobulin can block the damaging antibodies that may contribute to Guillain-Barre syndrome and reduce the autoimmune response that occurs.
- Plasma exchange (plasmapheresis): Blood is taken from the body and blood plasma is then separated from the blood cells. The blood cells are returned, and the body regenerates plasma. This process helps to remove the antibodies that attacks healthy peripheral nerve cells.
Occupational and Physiotherapy
- As the sensory and motor nerves are affected, patients may require physiotherapy exercise to rehabilitate and regain muscle strengths.
- Training with adaptive devices, such as a wheelchair or braces, to give you mobility and self-care skills
- Speech therapy is also necessary for those patients whose speech is also affected.
Prognosis and recovery
In general for most patients, the nerve damage worsens quickly for around a couple of weeks and stops deteriorating by around 4 weeks.. Symptoms reach a plateau within four weeks. The average recovery time is 6 to 12 months. Recovery can be very slow, it can be as long as 3 years. Patients need a lot of emotional support, physiotherapy, counseling, and occupational therapy during recovery.
Patients recover at different rates, and some experience incomplete or delayed recovery. About 80% can walk independently six months after diagnosis and about 60 %fully recover motor strength one year after diagnosis. About 5 to 10 percent have very delayed and incomplete recovery. However, the majority of patients make a full recovery.
In general for most patients, the nerve damage worsens quickly for around a couple of weeks and stops deteriorating by around 4 weeks.. Symptoms reach a plateau within four weeks. The average recovery time is 6 to 12 months. Recovery can be very slow, it can be as long as 3 years. Patients need a lot of emotional support, physiotherapy, counseling, and occupational therapy during recovery.
Patients recover at different rates, and some experience incomplete or delayed recovery. About 80% can walk independently six months after diagnosis and about 60 %fully recover motor strength one year after diagnosis. About 5 to 10 percent have very delayed and incomplete recovery. However, the majority of patients make a full recovery.