Prognostic Indicators of Gullian-Barre Syndrome at Assiut University Children Hospital

Guillain Barre Syndrome (GBS) is an autoimmune disorder with post-infectious polyneuropathy involving motor, sensory and sometimes the autonomic nerves. With the eradication of wild polio virus infection, GBS has become the most common cause of acute flaccid paralysis in both the developed and developing countries.GBS occurs throughout the world with a median annual incidence of 1.3 cases per population of 100 000.

GBS is characterized by ascending paralysis (lower limb numbness, paraesthesia or pain followed by weakness which ascends symmetrically and gradually progresses over a period of 1 to 28 days with maximum severity of weakness by four weeks after the onset). Bulbar involvement is seen in 50% cases and autonomic disturbances are seen in about 20%. Since there may be mortality related to acute complications of respiratory muscle paralysis and morbidity related to the long duration of the paralysis.

It can be classified electrophysiologically into demyelinating and axonal subtypes. Acute inflammatory demyelinating polyneuropathy (AIDP) is the demyelinating subtype, while acute motor axonal (AMAN) and acute motor-sensory axonal neuropathy (AMSAN) are the axonal subtypes. It is difficult to differentiate between them clinically and nerve conduction studies (NCS) can play an important role. Determining the electrophysiological subtype is useful as it can give insights into the underlying pathophysiology. This has both therapeutic and prognostic significance. Various electrodiagnostic criteria sets have been proposed to differentiate between the demyelinating and axonal subtypes.

GBS typically occurs after an infectious disease (Two-thirds of patients report symptoms of a respiratory or gastrointestinal tract infection before the onset of GBS. In about half of patients with GBS, a specific type of preceding infection can be identified and Campylobacter jejuni is responsible for at least one-third of these infections. Other pathogens that cause antecedent infections related to GBS are cytomegalovirus, Epstein- Barr virus, Mycoplasma pneumonia, Haemophilus influenzae, and influenza A virus) in which the immune response generates antibodies that crossreact with gangliosides at nerve membranes. This autoimmune response results in nerve damage or functional blockade of nerve conduction. The type of preceding infection and the specificity of the antiganglioside antibodies largely determine the subtype and clinical course of GBS.

The chance of recovery was significantly affected by age, antecedent gastroenteritis, disability, electrophysiological signs of axonopathy, latency to nadir and duration of active disease.The main treatments did not seem to affect the chance of recovery.