Guillain–Barre syndrome

Presented by
Dr.Ruma Dey
Dept. of Kayachikitsa

Guillain–Barre syndrome
(GBS), also known as Landry's
paralysis , is an immune-mediated
disorder of nervous system of
acute or subacute onset
characterized commonly by
generalized progressive
weakness of arms and legs, limb
paraesthesias and relative or
complete areflexia
• GBS patients often develop cranial nerve weakness, usually in the form
of facial or pharyngeal weakness.
• The usual pattern follows the flaccid paralysis typically ascending in
nature evolving over a few days to a few weeks.
• Autonomic dysfunctions are common with usual manifestations as
loss of vasomotor control with wide fluctuation in blood pressure,
postural hypotension and cardiac arrhythmias.
• Respiratory failure and oropharyngeal weakness may require
ventilatory assistance in about one-third of hospitalized patients
making it a disease of vital importance for early management
• It is believed that GBS may not be a single disease, but a variety of
acute neuropathies with a number of related immune-mediated
pathogenic mechanisms most common being endoneurial
inflammation of spinal nerve roots, distal nerve segments and
potential nerve entrapment sites
Guillain-Barré syndrome time course
EPIDEMIOLOGY
• The reported incidence for GBS is 1-2/100,000 population and
increases linearly with age, and men are about 1.5 times more
affected than women.
• Prior infection such as upper respiratory tract infection is well-
established predating event in the development of GBS by 10-14
days.
• Many antecedent illnesses associated with GBS have been
identified including Campylobacter jejuni gastroenteritis,
cytomegalovirus, Mycoplasma pneumoniae, Epstein-Barr virus and
influenza virus infections.
Etiology and Pathogenesis
Antecedent events are implicated in the pathogenesis of GBS. TABLE 1 lists some of
the identified events that lead to GBS.
More than 60% of patients who are diagnosed with GBS have been diagnosed with
an infection in the 3 weeks prior to the onset of weakness. The most common
antecedent symptoms have been fever, cough, sore throat, and nasal discharge.

Pathophysiology

Pathophysiology Cont.
• In the acute motor axonal neuropathy (AMAN) form of GBS, the
infecting organisms probably share homologous epitopes to a
component of the peripheral nerves (molecular mimicry) and,
therefore, the immune responses cross-react with the nerves
causing axonal degeneration; the target molecules in AMAN are
likely to be gangliosides GM1, GM1b, GD1a and GalNAc-GD1a
expressed on the motor axolemma. In the acute inflammatory
demyelinating polyneuropathy (AIDP) form, immune system
reactions against target epitopes in Schwann cells or myelin result
in demyelination; however, the exact target molecules in the case
of AIDP have not yet been identified.


• The body's immune system begins to attack the body
itself. The immune responses causes a cross-reaction
with the neural tissue. When myelin is destroyed,
destruction is accompanied by inflammation. These
acute inflammatory lesions are present within several
days of the onset of symptoms. Nerve conduction is
slowed and may be blocked completely. Even though the
Schwann cells that produce myelin in the peripheral
nervous system are destroyed, the axons are left intact
in all but the most severe cases. After 2-3 weeks of
demyelination, the Schwann cells begin to proliferate,
inflammation subsides, and re-myelination begins.
Subtypes of Guillain-Barre Syndrome
Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
• Autoimmune disorder, antibody mediated
• Is triggered by antecedent viral or bacterial infection
• Electrophysiologic findings demonstrate demyelination.
• Inflammatory demyelination may be accompanied by axonal nerve loss.
• Remyelination occurs after the immune reaction stops.
Acute motor axonal neuropathy (AMAN)
• Pure motor axonal form of neuropathy
• Sixty-seven percent of patients are seropositive for campylobacteriosis.
• Electrophysiologic studies are normal in sensory nerves, reduced or absent in motor
nerves.
• Recovery is typically more rapid.
• High proportion of pediatric patients
Acute motor sensory axonal neuropathy (AMSAN)
• Wallerian-like degeneration of myelinated motor and sensory fibers
• Minimal inflammation and demyelination
• Similar to AMAN except AMSAN affects sensory nerves and roots
• Typically affects adults
Miller Fisher syndrome
• Rare disorder
• Rapidly evolving ataxia, areflexia, mild limb weakness, and ophthalmoplegia
• Sensory loss unusual, but proprioception may be impaired.
• Demyelination and inflammation of cranial nerve III and VI, spinal ganglia, and
peripheral nerves
• Reduced or absent sensory nerve action potentials, tibial H reflex is usually absent.
• Resolution occurs in one to three months.
Acute panautonomic neuropathy
• Rarest of all the variants
• Sympathetic, parasympathetic nervous systems are involved.
• Cardiovascular involvement is common (postural hypotension, tachycardia,
hypertension, dysrhythmias).
• Blurry vision, dry eyes, and anhydrosis
• Recovery is gradual and often incomplete.
• Often combined with sensory features
Diagnostic criteria for Guillain-Barré syndrome
Features needed for diagnosis of Guillain-Barré syndrome in
clinical practice
• Progressive weakness in legs and arms (sometimes initially only
in legs).
• Areflexia (or decreased tendon reflexes) in weak limbs.
Additional symptoms
• Progressive phase lasts days to 4 weeks (often 2 weeks).
• Relative symmetry.
• Mild sensory symptoms or signs (not present in acute motor
axonal neuropathy).
• Cranial nerve involvement, especially bilateral weakness of facial
muscles.
• Autonomic dysfunction.
• Pain (common).
Diagnostic Procedures:
• Cerebrospinal fluid investigation: It will elevated at some stage of the illness but remains normal
during the first 10 days. There may be lymphocytosis (> 50000000 cells/L).
• Electrophysiological studies: it includes nerve conduction studies and electromyography. They are
normal in the early stages but show typical changes after a week or so with conduction block and
multifocal motor slowing, sometimes most evident proximally as delayed F-waves.
The only way to classify a patient as having the axonal or nonaxonal type is electrodiagnostically.
• Chest X-ray
• Stool culture
• Immunological tests to rule out the presence of cytomegalovirus or mycoplasma
• Antibodies to the ganglioside GQ1b for Miller Fisher Variant.
• MRI
• Lumbar Puncture: Most, but not all, patients with GBS have an elevated CSF protein level (>400
mg/L), with normal CSF cell counts. Elevated or rising protein levels on serial lumbar punctures and
10 or fewer mononuclear cells/mm3 strongly support the diagnosis.
Features that should raise doubt about the diagnosis of Guillain-Barré
syndrome
• CSF: increased number of mononuclear cells or polymorphonuclear
cells (>50 cells per μL).
• Severe pulmonary dysfunction with little or no limb weakness at onset.
• Severe sensory signs with little or no weakness at onset.
• Bladder or bowel dysfunction at onset.
• Fever at onset.
• Sharp spinal cord sensory level.
• Marked, persistent asymmetry of weakness.
• Persistent bladder or bowel dysfunction.
• Slow progression of weakness and without respiratory involvement
(consider
subacute inflammatory demyelinating polyneuropathy or acute onset
chronic
inflammatory demyelinating polyneuropathy).
Differential diagnosis of rapidly progressive limb
weakness (with or without respiratory failure)
CNS
Encephalitis, acute disseminated encephalomyelitis, transverse
myelitis, brainstem or myelum compression, leptomeningeal
malignancy
Motor neurons
Poliomyelitis, West Nile virus anterior myelitis, amyotrophic
lateral sclerosis, progressive spinal muscular atrophy
Plexus
Neuralgic amyotrophia, diabetes mellitus

Nerve

roots
Guillain-Barré syndrome, acute onset chronic infl ammatory
demyelinating neuropathy,Lyme disease, cytomegalovirus-related
radiculitis, HIV-related radiculitis, leptomeningeal malignancy
Peripheral nerves
Guillain-Barré syndrome, acute onset chronic inflammatory
demyelinating neuropathy,iatrogenic, toxic, critical illness myopathy-
neuropathy, vasculitis, diphtheria, porphyria,thiamine deficiency,
porphyria, Lyme disease, metabolic or electrolyte
disorders(hypokalaemia, phosphataemia or magnesaemia,
hypoglycaemia)
Neuromuscular junction
Myasthenia gravis, botulism, intoxication Muscles Critical illness
myopathy-neuropathy, mitochondrial disease, acute rhabdomyolysis,
polymyositis, dermatomyositis
 Diagnosis and treatment of Guillain-Barré syndrome

Admission to ICU, high-care, or general neurology ward

(consider using EGRIS prognostic model)
Regularly check (initially
every 1–3 h):
Unable to walk unaided (Guillain-Barré syndrome • Respiration
disability score ≥3), especially when less than 2 weeks • Progression of
Reconside from onset of weakness? weakness
r • Treatment indication with IVIg (0·4 g/kg daily for • Swallowing difficulties
ICU 5 days) or plasma exchange • Autonomic dysfunction
• Mildly affected patients (Guillain-Barré syndrome • Pain
admission disability score 1–2): check for further deterioration
or treatment indication
Complications:
TRF after IVIg or plasma exchange? • Venous thrombosis
• Deterioration after initial stabilisation or improvement: • Pressure ulcer
re-treatment with IVIg (0·4 g/kg daily for 5 days) or • Pulmonary infections
plasma exchange


Acute onset chronic inflammatory demyelinating
polyneuropathy? Start or strongly consider:
• Three or more deteriorations (TRFs) or any • Physiotherapy
deterioration after 8 weeks requires consideration of • Rehabilitation
re-treatment with IVIg or with steroids (as for chronic • Logopaedic help
inflammatory demyelinating polyneuropathy • Psychological help
• Patient support group
Further improvement?
• Consider discharge to neurology ward, rehabilitation
centre or home
Management
There is no known cure 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.
Medical Management
The mainstay of medical management of patients with GBS is
Immunotherapy:
Immunotherapy has been shown to accelerate recovery in patients with GBS,
particularly when initiated early after motor symptoms appear. There are two forms
of immunotherapy indicated for specific therapy of GBS, plasma exchange and
intravenous immunoglobulin G (IVIG).
• Plasma exchange is a process that removes or dilutes the circulating
immune factors implicated in the pathogenesis of GBS. This procedure has been
shown to reduce the need for mechanical ventilation and hospitalization time by
hastening recovery in non ambulant patients who seek treatment within 4 weeks
of the onset of neuropathic symptoms.

The maximal benefit therapy is seen if it is initiated within the first
2 weeks of onset. The usual regimen of plasma exchange is 5
times over 2 weeks, with a total exchange of about 5 plasma
volumes.
• IVIG is recommended for patients who cannot ambulate without
assistance within 2 or 4 weeks of neuropathic symptom onset.
The recommended dose is 0.4 g/kg per body weight daily for 5
consecutive days. As an added advantage, the patient’s CD8+ T-
cell function is enhanced by an unknown function by the use of
IVIG.

Corticosteroids: While oral corticosteroids and IV
methylprednisolone were once believed to be useful in the
treatment of GBS due to their immune-mediated inflammatory
mechanism, they are no longer used because they do not seem to
• Mechanical Ventilation
Even though immunotherapy has almost halved the duration of mechanical
ventilation, about 25% of all patients with GBS demonstrate respiratory failure
requiring ICU admission and invasive mechanical ventilation. Respiratory failure
tends to be more likely in cases with rapid progression, bulbar palsy, upper limb
involvement, and autonomic dysfunction. Endotracheal intubation and mechanical
ventilation should be initiated within 24 hours of symptom onset in an ICU together
with regular monitoring and measurement of vital capacity.
• Pain Management
Pain remains an undertreated but important aspect of GBS. While opioids and
NSAIDs have traditionally been used in pain management plans, their host of side
effects has prompted the development of safer therapeutic modalities. Opioids,
while providing effective pain relief, have been linked to tolerance, dependence,
respiratory depression, sedation, and constipation. On the other hand, NSAIDs can
lead to ulceration, bleeding, platelet dysfunction, and renal and hepatic failure
• Physical therapy involving gentle massage, passive
range of motion exercises, and frequent position changes
may provide adjuvant relief in some patients. This may be
integrated together with a rehabilitation program including
occupational and physical therapy to overcome the persistent
fatigue that is experienced due to the loss of axons.
Diagnosis and management of Guillain-Barré syndrome in ten
steps"
Ayurveda treatment
Bāhya snehan:Around 50 ml of indirectly heated candanabalalākṣāditailam was applied
in anuloma gati (downward) for 15 min
Nāḍīsvedana :By nirguṇḍī (vitex nigundo) and dashamūla siddha kvātha (decoction) for
a period of 15 minutes.
Pinda Sweda:15 g of bala mūla (root of Sida cordifolia) 15 g of aśvagandhā (Withania
somnifera) cūrṇa and 15 g śatāvarī (Asparagus racemosus) was processed with 500 ml
of kṣīra (milk) wherein milk was boiled to reduce the quantity to half with 25 g of
ṣaṣṭikaśāli (processed ṣaṣṭika rice) was cooked very soft and made like paste with
above filtrate of kṣīra. This paste was applied with gentle circular movements for 20
min in anuloma gati. Patient was treated for a total of 36 days.
Śirodhārā : Using tila tailam (lukewarm sesame oil) for a period of 15-20 min for 16
days.
Basti: Kṣīra processed with pittahara dravya in the form of basti was used and tila taila
basti (sesame oil enema) given on alternate days,for a duration of 36 days .
Medicine:Bṛhatvātachitamani kalpa which is composed of bṛhatvātachitamani, 1 g;
guḍūci (Tinospora cordifolia) sattva, 30 g; rajata bhasma 5 g and sūtaśekhara rasa 30
tab each of 250 mg powdered together and divided into 60 divided doses BD was given