Guillain-Barré syndrome
(GBS)
Prepared by
Dr. Madiha Anees PT
Asst. Prof/VP RCRS
MS-PT, BS-PT
Content
Introduction
Incidence
Classification
Pathophysiology
Causes
Risk factors
Symptoms
Diagnosis
Managment
Introduction
Guillain Barre Syndrome (GBS) or Landry's paralysis is a serious disorder that occurs
when the body’s defense (immune) system mistakenly attacks part of the peripheral
nervous system.
It is an acute, progressive, autoimmune, inflammatory demyelination of polyneuropathy of
the peripheral sensory and motor nerves and nerve roots.
The syndrome is named after the French physicians Georges Guillain and Jean Alexandre
Barré and strohl. who described it in 1916.
INCIDENCE
1–2 cases per 100,000 people annually
Men are one and a half times more likely to be affected than women
It is the most common cause of acute non- trauma-related paralysis in the world.
Classification
There are 6 different subtypes of GBS and they are:
Acute Inflammatory demyelinating polyneuropathy
Miller Fisher syndrome
Acute motor axonal neuropathy
Acute motor sensory axonal neuropathy
Acute panautonomic neuropathy
Bickerstaff’s brainstem encephalitis
1) Acute Inflammatory Demyelinating
Polyneuropathy (AIDP)
Most common
Auto immune response against schwann
cell.
2) Miller Fisher Syndrome (MFS)
Rare variant
Manifest as a descending paralysis.
Usually affects the eye muscles first and
presents with the triad of ophthalmoplegia,
ataxia, and areflexia.
3) Acute Motor Axonal Neuropathy (AMAN)
Also known as Chinese paralytic
syndrome.
Attacks motor nodes of Ranvier and is
prevalent in China and Mexico.
4) Acute Motor Sensory Axonal Neuropathy
(AMSAN)
Similar to AMAN
Affect the sensory with several axonal damage.
5) Acute Panautonomic Neuropathy
Is the most rare variant of GBS, sometimes accompanied by encephalopathy.
Frequently occurring symptoms include
Impaired sweating
Lack of tear formation
Photophobia
Dryness of nasal and oral mucosa
Itching and peeling of skin
Nausea
Dysphagia
Constipation unrelieved by laxatives or alternating with diarrhea.
Contt.
Initial nonspecific symptoms of
Lethargy
Fatigue
Headache
decreased initiative
followed by autonomic symptoms including
Orthostatic lightheadedness
Blurring of vision
Abdominal pain
Diarrhea
Dryness of eyes
Disturbed micturition.
6) Bickerstaff's brainstem encephalitis (BBE)
It is characterized by acute onset of
Ophthalmoplegia
Ataxia
Disturbance of consciousness
Hyperreflexia.
Large, irregular hyper intense lesions located mainly in the brainstem, especially in the
pons, midbrain and medulla, are described in the literature.
Pathophysiology
GBS is a rare and severe disease.
It occurs after an acute infectious procedure.
Normally it is acute form of paralysis in lower body area that moves towards upper limb
and face.
Gradually patient loses all his/her reflexes and goes through a complete body paralysis.
GBS is a life threatening disorder and needs timely treatment and supportive care.
Contt..
GBS is considered to be an autoimmune disease triggered by a preceding bacterial or viral
infection.
Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus and Mycoplasma pneumoniae are
commonly identified antecedent pathogens.
They have specific antigens in their capsule that they share with nerves.
The immune system usually then response to these components in the capsule by producing
antibodies that cross-react wit the myelin in the peripheral nervous system, causing
demyelination and then damage to the peripheral nervous system.
In GBS there is infiltration of the spinal roots and peripheral nerves via the lymphatic system,
causing stripping of the myelin.
This will lead to a defect in the transmission of electrical nerve impulses which will then lead to
flaccid paralysis.
Causes
Nobody knows precisely what the exact cause is.
It is due to an immune response to foreign antigens, such as infectious agents (bacteria,
etc.) that the body's immune system mistargets - attacks good tissue by mistake.
Experts believe our immune system mistakenly attacks gangliosides - compounds which
are naturally present in nerve tissues.
The most common infection which precedes the development of GBS is Campylobacter
jejuni - one of the most widespread causes of human gastroenteritis.
Even so, in over half of all cases no previous infection was present; in other words, there
was nothing to which the doctor could link the syndrome.
It is believed that influenza virus may also trigger an autoimmune response which causes
the syndrome.
Contt…
Experts believe the foreign agent (bacterium/virus) causes the body's immune system to
attack the myelin sheath of the peripheral nerves.
The sheath becomes damaged, causing nerve damage, resulting in faulty sending of signals
between nerves and muscles.
This faulty wiring causes muscle weakness, numbness and tingling, and eventually
paralysis.
Risk factors
Age (15-35) and (60-75)
In men more likely than woman
Recent gastrointestinal or respiratory infection by viruses or bacteria
Recent vaccination (especially influenza and meningococcal)
Recent surgery
History of lymphoma, Systemic lupus erythromatosus, or HIV and AIDS
Signs and Symptoms
Pain
Progressive Muscle weakness
Numbness
Loss of reflexes in arms and legs
Low blood pressure
Uncoordinated movement
Facial weakness
Clumsiness and falling
Severe pain in the lower back
Sensation changes
Tenderness or muscle pain
Blurred vision
Respiratory problems
DIAGNOSTIC EVALUATION
Medical history
Physical examination
Tests -
Nerve conduction studies (NCS)
CSF examination
Electromyography (EMG)
Electrocardiogram (ECG)
Pulmonary Function Test (PFT)
Medical management
Treatment is non specific and symptomatic.
Observe continuously for adequacy of respiratory effort.
Patients with GBS will be hospitalized initially (medical emergency).
It is important to monitor the individual's respiration carefully.
If breathing problems are severe he/she may be placed in an ICU and put on a ventilator.
According to the National Health Service, the two main initial treatment options for GBS
are intravenous immunoglobulin, which is safer and easier to give, or plasmapheres
(plasma exchange).
After the acute phase of the syndrome, the patient may need rehabilitation to regain
functions that were lost.
Treatment concentrates on improving activities for daily living, such as brushing teeth,
washing, getting dressed and performing some other everyday tasks.
Physiotherapeutic problems
Acute Respiratory failure due to paralysis of diaphram and secretions
Decreased A/E due to paralysis of diaphram and secretions
Decreased chest expansion due to decreased A/E
Increased secretions due to pneumonia, poor cough attempt and weakness of respiratory
muscles
Post acute Decreased Fx w.r.t. ADL due to muscle weakness
Poor proximal and distal control due to weakness
Decreased Fx due to immobility
Associated problems
Muscle atrophy due to weakness and inactivity
Poor sensation due to nerve damage
Contractures due to immobility
Decreased exercise tolerance due to inactivity
Decreased circulation due to inactivity
Secondary lung infection due to secretions
Pressure sores and DVT due to immobility
Weakness due to inactivity
Short term Goals
Improve/maintain respiratory function
Prevention of pressure sores and DVT
Prevention of muscle atrophy
Improve/maintain muscle strength
Improve/maintain function
Long term Goals
Maintain exercise tolerance
Maintain function
Wheelchair handling
Support groups
Retrain proximal control
Maintain muscle strength
Retraining of gait
That’s All Folks…..