acute inflammatory demyelinating polyneuropathy (AIDP) no lay-term
Conditions
Interventions
None listed
Sponsors
Erasmus MC, Universitair Medisch Centrum Rotterdam
Eligibility
Age
2 Years to 99 Years
Inclusion criteria
Inclusion criteria: Inclusion criteria: · Fulfilling the diagnostic criteria for GBS [Asbury 1990] · Age of diagnosis of GBS 18 years or less · Diagnosis of GBS was made in 1987 or thereafter · Patients were either treated at the ward or outpatient clinic of the department of Paediatric Neurology of the Sophia Children*s Hospital, Erasmus MC, or were included in the earlier clinical trials of the GBS study group of the Erasmus MC · Written informed consent given by the patient and/or parents/care takers
Exclusion criteria
Exclusion criteria: · Additional diseases or disorders at the time of diagnosis that may influence the endpoints · Diagnosis of GBS less than 6 month ago
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Main study parameter/endpoint Residual effects on long-term outcome, with respect to nerve physiology dysfunction, neurological impairment, restrictions in daily activity and participation, and quality of life. Long-term outcome will be defined at least six months from diagnosis, the period in which most recovery occurs. The outcome levels will be determined by using various questionnaires and physical tests that are validated for the appropriate age categories. By using these tests, preferably the results obtained in patients from various ages can be combined. | — |
Secondary
| Measure | Time frame |
|---|---|
| Secondary study parameters/endpoints Demographic and clinical features available in the acute phase of disease will be related to these outcome endpoints. This information is usually available in the patient record files and includes age, sex, type of preceding clinical infection, cranial and sensory nerve involvement, severity of weakness and disability, and routine neurophysiology. All this information should be available preferably within two weeks of diagnosis, the period in which additional treatment in thought to be still effective. Other study parameters · To further define outcome, the residual damage of nerve physiology caused by GBS will be determined by a compound muscle action potential (CMAP) scan [Blok 2008]. In this routine 10 minutes non-invasive neurophysiological examination, the ulnar nerve of the non-dominant arm will be tested by a series of non-painful electrical stimuli in the wrist. This test will provide information on the extent and the mechanism of nerve dysfunction, especially on the recruitment of motor units. · To further define the factors that influence the extent of residual defects, genetic polymorphisms will be determined. Previous studies identified a relation with polymorphisms in the mannose binding lectin (MBL) gene and subsequent levels of MBL in serum and poor outcome in adult patients [Geleijns 2007]. Since these genetic polymorphisms remain unchanged trough life, this information is also available in the acute phase of disease and potentially can be used to predict outcome. | — |
Countries
Netherlands
Outcome results
None listed