Prognostic Indicators of Gullian-Barre Syndrome

Guillain-Barre Syndrome Clinical Trial

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

Status Not yet recruiting

Clinical Trial Summary

Prognostic indicators of Gullian-Barre syndrome and the predictive factors associated with worse prognosis in the Guillain-Barré syndrome (GBS), which can be helpful to fully evaluate the disease progression and provide proper treatments.

Clinical Trial Description

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.

Acute motor axon neuropathy, diabetes, high blood pressure, uroschesis, high body temperature, ventilator support,consciousness disorder, absence of upper respiratory tract preceding infection, hyperglycemia, hyponatremia,hypoalbuminemia, high leukocyte count, hyperfibrinogenemia, abnormal hepatic and renal function were demonstrated as poor prognostic factors.

Dysautonomic syndrome, cardiac arrest and respiratory failure were the leading causes of death.

Paralysis of respiratory muscles is a dreaded complication that occurs in one-fourth of the subjects. Respiratory weakness occurs due to the involvement of both inspiratory and expiratory muscles. Diaphragmatic weakness occurs due to phrenic nerve demyelination. Careful and close monitoring is mandatory to identify patients at high risk for respiratory failure, and triage them to the Intensive Care Unit (ICU) and mechanical ventilation.

The diagnosis was based mostly on clinical judgement and was confirmed by autopsy only in a few instances. In the presence of suggestive findings the complementary test as demyelinising changes in the nerve conduction studies (NCS) or albuminocytological dissociation in the cerebrospinal fluid (CSF), help to confirm the diagnosis.

• Consider specific treatment with IVIg or PE:

• IVIg is usually the first- line therapy for children with GBS. Although some paediatric centres administer IVIg as 2 g/kg (body weight) over 2 days, rather than the standard adult regimen of 2 g/kg (body weight) over 5 days.

• plasma exchange can be difficult to perform in young children, and care should be taken in patients with autonomic cardiovascular instability because of the large volume shifts involved in the plasma exchange procedure.

1. Indications to start IVIg or PE:

Severely affected patients (inability to walk unaided, GBS disability scale ≥3).

Unknown whether IVIg is effective:

Mildly affected patients (GBS disability scale ≤2) or MFS patients .

2. Indications for re-treatment with IVIg:

• Secondary deterioration after initial improvement or stabilisation (treatment-related fluctuation): treat with 0·4 g/kg for 5 days.

• No proven effect of re-treatment with IVIg in patients who continue to worsen.

• Steroids :

Oral steroids or intravenous methylprednisolone (500 mg daily for 5 consecutive days) alone are not beneficial in GBS. The combination of IVIg and intravenous methylprednisolone was not more effective than IVIg alone.

-Give good general care: Monitor progression and prevent and manage potentially fatal complications.

• Indication for admission to an intensive care unit:

• Rapid progressive severe weakness often with impaired respiration (vital capacity <20 mL/kg).

• Need for artifi cial ventilation Insufficient.

• swallowing with high chance of pulmonary infection.

• Severe autonomic dysfunction.

• Physiotherapy and Rehabilitation. ;

Related Conditions & MeSH terms

• Guillain-Barre Syndrome
• Syndrome

• NCT number: NCT06300359
• Study type: Observational
• Source: Assiut University
• Contact: Amira A Sayed, Resident
• Phone: +201118695191
• Email: amira.abdelhakem97@gmail.com
• Status: Not yet recruiting
• Start date: March 2024
• Completion date: June 2025