GUILLAIN BARRE SYNDROME

GUILLAIN BARRE SYNDROME

• It is the most common type of acute
polyneuropathy
• It is an acute diffuse post infective disease
involving spinal roots, peripheral nerves and
occasionally cranial nerves causing generalized
paralysis
Causes
• Viral infection – CMV, HIV, EBV, Chickenpox
• Bacterial – Campylobacter jejuni, Mycoplasma
Pneumonia
• Others
• Lymphoma (Hodgkin’s)
• Systemic Lupus Erythematosus
• Sarcoidosis
• Post vaccination – Older type of Rabies vaccine
Weakness
• Ascending type of progressive weakness (Acute
onset):The weakness usually starts in lower limbs, but
it may start in the upper limbs or facial muscles in
about 10% of patients.
• Weakness of proximal muscles > distal muscles
• Cranial nerves: Facial nerve weakness seen in >50%,
and oropharyngeal weakness seen in 50%.
Oculomotor weakness seen in 15 %.
• Severe respiratory muscle weakness necessitating
ventilatory support seen in 10 to 30%
Other features
• Absent deep tendon reflexes (in 90%)
• Paresthesias (No sensory impairment) Paresthesias in
the hands and feet accompany the weakness in more
than 80%, but sensory abnormalities on examination
are frequently mild or nil.
• Pain due to nerve root inflammation, mainly located
in the back and extremities, during the acute phase by
two-thirds of patients with all forms of GBS
• Dysautonomia occurs in around 70%. SIADH, which
may be due to autonomic involvement, is also seen in
GBS
DDs
• Periodic paralysis ( Variation in serum potassium , normal cerebrospinal fluid)
• Botulism (descending paralysis)
• Hypophosphatemia (irritable, apprehensive, hyperventilation, normal
cerebrospinal fluid)
• Myasthenia gravis (weakness and fatigue that improves with rest)
• Tick paralysis (sensory changes absent, normal cerebrospinal fluid)
• Transverse myelitis (abrupt bilateral leg weakness, ascending sensory)
• Vasculitic neuropathies (mononeuropathy)
• Basilar artery thrombosis (asymmetric limb paresis)
• Metabolic myopathies (cerebral and cerebellar symptoms)
• Poliomyelitis (purely motor disorder with meningitis)
• Polymyositis (chronic, affects proximal limb muscles)
 Diagnostic criteria
Required • Supportive
•Progressive weakness of 2 or more • Relatively symmetric weakness
limbs due to neuropathy • No sensory involvement
• Facial nerve or other cranial nerve
•Areflexia involvement
•Progression of disease less than 4 • Absence of fever
weeks • CSF study (Albumino-cytological
dissociation)
•Exclusion of other causes like
• Electrophysiological evidence of
• Vasculitis (PAN, SLE, CSS) demyelination
• Toxins (Organophosphorus poisoning,
Lead) • Features making the diagnosis
• Infections (Diphtheria, Botulism) doubtful
• Porphyria • Sensory loss
• Marked asymmetry or symptoms and
signs
• Severe bladder and bowel dysfunction
CSF study
• CSF – (Albumino-cytological dissociation - in up
to 66% of patients with GBS at one week after
onset of symptoms.)
• Protein – elevated
• Cells – Normal
• A CSF pleocytosis can be seen in GBS patients who
have HIV infection
NCV study
• Electrodiagnostic features(nerve conduction study)
• In demyelination prolonged distal latencies,
conduction velocity slowing, evidence of conduction
block, and temporal dispersion of compound action
potential are the usual features.
• In primary axonal pathology, the principal
electrodiagnostic finding is reduced amplitude of
compound action potentials without conduction
slowing or prolongation of distal latencies.
Management
• IV immunoglobulin (IVIG) - IVIG (400 mg/kg/d for 5
days)
• Plasmapheresis :
• Plasmapheresis is an extracorporeal blood purification
technique designed for the removal of large molecular
weight substances from the plasma.
• Patients with mild GBS on admission should receive 2 PEs.
Patients with moderate and severe forms should benefit
from 2 further exchanges , 1 to 1.5 plasma volume
exchanges per procedure
• Corticosteroids (oral or IV): not recommended