• 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
International League Against Epilepsy Classification and Definition of Epilepsy Syndromes With Onset in Childhood Position Paper by The ILAE Task Force On Nosology and Definitions