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العنوان
CXCL13 Level in Children with Acute and Relapsing Demyelinating Disorders of the Nervous system /
المؤلف
Habib, Habib Lahzi.
هيئة الاعداد
مشرف / حبيب لحظى حبيب
مشرف / إيمان على العجوزة
مشرف / دينا أحمد سليمان
مشرف / رغده محمد هشام زيتون
تاريخ النشر
2021.
عدد الصفحات
269 p. :
اللغة
الإنجليزية
الدرجة
ماجستير
التخصص
طب الأطفال ، الفترة المحيطة بالولادة وصحة الطفل
تاريخ الإجازة
1/1/2021
مكان الإجازة
جامعة عين شمس - كلية الطب - طب الأطفال
الفهرس
Only 14 pages are availabe for public view

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from 269

Abstract

This observational case-control study was conducted in the period from June 2019 to December 2020 in the Pediatric Out Patient Clinic, the Pediatric Neurology Clinic, the Emergency department and In-Patient wards at the Children Hospital, Ain Shams University.
The study was conducted on 20 patients diagnosed with acute acquired demyelinating disease (patient group), and 40 healthy age and gender matched children (control group).
The aim of this study was to measure the level of CXCL13 in blood and cerebrospinal fluid of patients diagnosed with acute central or peripheral demyelination and to correlate their levels with the patients’ clinical, radiological and laboratory profiles.
Known cases of collagen disorders, vasculitis, chronic illness or other condition with similar clinical presentation to autoimmune neurological diseases were excluded
Cases were subjected to full history taking and clinical examination. Lab tests included routine blood and CSF studies, with measurement of CSF and serum level of CXCL13. Further investigations, including MRI of brain and spine, Fundus examination, VEP, EEG and NCV studies, were tailored according to need based on patient’s presentation.
The mean age of cases was 7.08 ± 3.8 years, ranging from 0.83 to 14 years. 50% of patients were males. Subjects in the control group were age and gender matched. Only one case had consanguineous parents.
The commonest diagnosis within cases was acute disseminated encephalomyelitis (ADEM) (35%), followed by Guillain-Barré syndrome (GBS) (20%). Others diagnosis are acute necrotizing encephalopathy of childhood (ANEC), autoimmune encephalitis (AE) and transverse myelitis (TM) equally represent (15%).
As regards the clinical presentation, motor weakness was the most common clinical feature, seen in 90% of the cases, followed by altered mental status seen in 60% of the cases and bowel/bladder dysfunction in 30% of the cases. The least commonly observed clinical feature was movement disorder seen in 10% of the cases.
Motor weakness was seen in all cases with ADEM, ANEC, TM and GBS. Encephalopathy was seen in all cases of ADEM, ANEC and AE but not in the rest of the cases. Seizures were seen in 14% of cases of ADEM and in 66% of cases of AE. Sensory affection was seen in 14% of cases of ADEM, 25% of cases of GBS, and 33% of cases of TM, the latter demonstrating a clear sensory level. Cranial nerve affection was seen in 14% of cases of ADEM and 50% of cases of GBS but was not seen in other cases. Movement disorders were observed in 14% of the cases of ADEM, and one patient with GBS. Urine retention was seen in all patients with TM and in 50% of cases with GBS.
MRI of the brain was abnormal in 70% of all cases as opposed to only 25% for CT brain. 20% of the patients had an abnormal MRI of the spine. Whereas all cases of ADEM, ANEC and AE had abnormal MRI brain and all cases of TM had abnormal MRI spine.
As regards EEG findings, 50% of children had an abnormal EEG (4/7 (57%) ADEM, 3/3 (100%) ANEC and 3/3 (100%) AE). In all children where EEG was abnormal there was a slow background (4/7 (57%) ADEM, 3/3 (100%) ANEC and 3/3 AE (100%). Twenty percent of these children demonstrated epileptiform activity on EEG (1/7 (14%) ADEM and 1/3 (33%) AE).
Fundus examination was done for 18 patients and was abnormal in 3/18 (15%) from all the cases (1/7 (14%) ADEM, 1/3 (33%) AE, 1/4 (25%) GB), VEP was done for 19 patients and was abnormal in 3/19 (16%) of the cases (1/7 (14%) ADE, 1/3 (33%) AE), 1/4 (25%) GBS).
NCV was done for 7 patients (2/7 (28%) ADEM, 1/3 (33%) TM and 4/4 (100%) GBS) and was abnormal in all of them. All GBS cases had abnormal NCV.
Almost 60% of the children showed CSF hyperglycorrhachia, whereas none showed decreased CSF glucose level. 57.89% of the children showed increased CSF protein level, 61.11% showed increased IgG index and 11.76% had CSF lymphocytic pleocytosis. 15% of children showed leukocytosis on CBC and 5% of them had leucopenia. There was no statistically significant difference between the different diagnoses groups regarding CSF glucose, CSF proteins, IgG index, and CSF lymphocytic pleocytosis.
As regards treatment, 55% children received one treatment line, 40% of them received two treatment lines and 5% of them received three treatment lines before clinical improvement could be observed.
As regards residual deficits, 8/20 (40%) of children had residual deficit at 3 months after hospital discharge. Of children who had a deficit at 3 months: 6/20 (30%) had a motor deficit (2/7 (28.5%) of cases of ADEM, 1/3 (33%) of cases of AE, 1/3 (33%) of cases of TM and 2/4 (50%) GBS) of cases of GBS. Five out of 20 cases (25%) had a mental deficit: (3/7 (43%) of the cases of ADEM and 2/3 (67%) of cases of AE. Three out of 20 (15%) of the cases had remote symptomatic epilepsy: 1/7 (14%) of cases of ADEM and 2/3 (67%) of cases of AE.
The mean serum of CXCL13 was (341.75 ± 164.71 pg/ml) and for CSF, the mean CXCL13 level was (1458.21 ± 769.13 pg/ml). There was a significant statistical correlation between serum and CSF levels of CXCL13, though the serum levels were much lower than the CSF levels.
The lowest mean serum CXCL13 level was found among patients with ADEM (256.43 ± 57.57 pg/ml), whereas the highest level was found in patients with ANEC (593.33 ± 213.62 pg/ml). The lowest mean CSF CXCL13 level was found among patients with GBS (350 ± 0 pg/ml), whereas the highest level was found in patients with ANEC (2433.33 ± 444.64 pg/ml).
There was highly significant difference between the studied groups regarding serum CXCL13 level. Serum CXCL13 level status was significantly higher in cases compared to controls, where the mean serum CXCL13 level in cases was (341.75 ± 164.71 pg/ml) and in controls it was (51.5 ± 18.99).
The mean serum CXCL13 level was significantly higher in patients who needed 2 or more treatment lines before a clinical improvement could be observed. Its levels did not seem to differ significantly between patients who were encephalopathic at presentation and those who were not, or those who had residual deficits at 3 months versus those who did not, also it was not significantly associated with CSF lymphocytic pleocytosis, CSF glucose status, CSF proteins status, IgG Index status, total leucocytic count.
The mean CSF CXCL13 was significantly higher in patients with CSF lymphocytic pleocytosis and increased IgG index. It was also higher in patients who needed 2 or more treatment lines with the difference nearing statistical significance.
There was no statistically significant difference between mean CSF CXCL13 level regarding patients who were encephalopathic at presentation and those who were not, CSF glucose status, CSF proteins status, total leucocytic count and residual deficit at 3 months.