الفهرس 
Inflammatory Demyelinating Disorders of the nervous systemOnly 14 pages are availabe for public view
 |  | from | 223 |  |  |
| | | from | 223 | | |
Abstract
V
itamin D performs various immunomodulatory, anti-inflammatory, antioxidant and anti-fibrotic actions. Autoimmune diseases result from an aberrant activation of the immune system, whereby the immune response is directed against harmless self-antigens. Over the past years, researchers’ interest in vitamin D and its correlations with autoimmune diseases has considerably increased.
The aim of this study was to measure the level of 25(OH) vitamin D3 in the serum of patients diagnosed with acute nervous system demyelination, comparing it to levels found in healthy controls, and to correlate the patient’ vitamin D levels with their clinical, laboratory and radiological profiles.
This observational case-control study was conducted in the period from June 2018 till December 2020 at Pediatric Out Patient Clinic, Pediatric Neurology Clinic, the Emergency department and In-Patient wards at the Children Hospital, Ain Shams University. On 20 patients diagnosed with acute acquired demyelinating disease (patient group), and 40 healthy age and gender matched children (control group) were enrolled in the study.
Cases were subjected to full history taking, examination, MRI of the brain and spinal cord as needed, EEG in case of encephalopathy and/or seizures, Lumbar puncture for CSF cytology, chemistry and culture if indicated, NCV in patients with peripheral nervous system demyelination and Serum level of vitamin D (25(OH)2 D3).
The mean age of cases was 7.08 ± 3.8 years (range 0.83 to 14 years). 50% of the patients were males. Subjects in the control group were age and gender matched. Acute disseminated encephalomyelitis was the most common diagnosis among cases in 35%, followed by Guillain-Barré syndrome (GBS) (20%). Acute necrotizing encephalopathy of childhood (ANEC), autoimmune encephalitis (AE) and transverse myelitis (TM) were each observed in 15% of the cases.
Collectively, 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 Bladder and/or bowel incontinence seen in 30% of the cases. The least commonly observed clinical feature was movement disorder seen in ten percent of the cases.
In patients with ADEM and ANEC, motor weakness and encephalopathy were present in all cases. In AE patients, 66% had both seizures and encephalopathy. In all patients with TM had motor weakness as well as urine retention. In patients with GBS, motor weakness in 100% of cases. urine retention and cranial nerve palsy in 50% of cases.
MRI of the brain was abnormal in 70% of all cases Whereas all cases of ADEM, ANEC and A.E had abnormal MRI brain. 20% of the patients had an abnormal MRI of the spine, all cases of T.M had abnormal MRI spine.
50% of children had an abnormal EEG. In all children where EEG was abnormal there was a slow background and 20% demonstrated epileptiform activity on EEG.
Fundus examination and VEP was abnormal in three cases, and NCV was done for seven patients and was abnormal in all of them. All G.B cases had abnormal NCV.
55% children received one treatment line, mostly PMP, 40% of them received two treatment lines PMP plus IVIG and five percent of them received three treatment lines added PLEX, before clinical improvement was observed.
The mean of Vitamin D level among cases is (14.4 ± 11.14 ng/ml), was deficient in 75% of the cases and insufficient in 15% of cases, which is significantly lower than that for controls (27.18 ± 7.8 ng/ml).
The lowest mean level of vitamin D was found among patients with AE (4 ± 1 ng/dl) and ANEC (6.33 ± 1.15 ng/ml), whereas the highest level was found in patients with GBS (27.75 ± 8.81 ng/ml).
The mean vitamin D level did not differ significantly even with or without encephalopathy, Also with CSF parameters, total leucocytic count, number of treatment lines and residual deficit at three months.
As regards CSF analysis, none of the patients had low CSF glucose while it was elevated as well as CSF proteins in 57.89%. and increased IgG index in 61.11%. CSF lymphocytic pleocytosis was observed in 11.76% of the cases. Blood leukocytosis was present in 15% of cases. With no significant difference between the different patient diagnoses regarding CSF parameters.
40% of children had a residual deficit at three months. Motor deficits were slightly more common than mental deficits (75% vs 62.5%). Remote symptomatic epilepsy was seen in 37.5% of cases with deficits.