Ignite Creation Date:
2024-05-06 @ 8:14 PM
Last Modification Date:
2024-10-26 @ 3:23 PM
Study NCT ID:
NCT06300359
Status:
NOT_YET_RECRUITING
Last Update Posted:
2024-03-15
First Post:
2024-02-21
Brief Title:
Prognostic Indicators of Gullian-Barre Syndrome
Sponsor:
Assiut University
Organization:
Assiut University
Study Overview
Official Title:
Prognostic Indicators of Gullian-Barre Syndrome at Assiut University Children Hospital
Status:
NOT_YET_RECRUITING
Status Verified Date:
2024-03
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
None
Brief 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
Detailed 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 countriesGBS occurs throughout the world with a median annual incidence of 13 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 diseaseThe main treatments did not seem to affect the chance of recovery
Acute motor axon neuropathy diabetes high blood pressure uroschesis high body temperature ventilator supportconsciousness disorder absence of upper respiratory tract preceding infection hyperglycemia hyponatremiahypoalbuminemia 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 gkg body weight over 2 days rather than the standard adult regimen of 2 gkg 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 04 gkg 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 mLkg
Need for artifi cial ventilation Insufficient
swallowing with high chance of pulmonary infection
Severe autonomic dysfunction
Physiotherapy and Rehabilitation
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 diseaseThe main treatments did not seem to affect the chance of recovery
Acute motor axon neuropathy diabetes high blood pressure uroschesis high body temperature ventilator supportconsciousness disorder absence of upper respiratory tract preceding infection hyperglycemia hyponatremiahypoalbuminemia 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 gkg body weight over 2 days rather than the standard adult regimen of 2 gkg 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 04 gkg 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 mLkg
Need for artifi cial ventilation Insufficient
swallowing with high chance of pulmonary infection
Severe autonomic dysfunction
Physiotherapy and Rehabilitation
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
False
Is a FDA Regulated Device?:
False
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None