Abstract Details

Title Inflammatory Demyelinating Central Nervous System Diseases in Childhood: Clinical and Paraclinical Profiles in 133 Patients

Topic MS and Related Diseases

Presentation(s) P02 - (-)

Poster/Presentation
Number 123

Objective

Background BACKGROUND: The term "Acquired demyelinating diseases (ADD)" depict as a first acute[frasl]sub-acute clinical event with presumed inflammatory[frasl]demyelinating cause [monophasic acute disseminated encephalomyelitis (ADEM) and clinically isolated syndrome (CIS)], and also diseases composed of multiple episodes of central nervous system (CNS) demyelination [recurrent ADEM, multiple sclerosis (MS), and neuromyelitis optica (NMO)]. To describe the features in the spectrum in pediatric patients is very important. Early diagnosis and immunosuppresive treatment may help slow the accumulation of severe disability.

Design/Methods DESIGN/METHODS: 133 patients (5.6%) whose diseases started before age 18, were selected from 2369 patients. 63.2% of the patients were women. 98 patients had relapsing remitting MS, 21 secondary progressive MS, 8 CIS, 3 NMO, 2 Marburg disease, and 1 had radiologically isolated syndrome.

Results RESULTS: The mean age at onset was 15.07±2.72. In 55 patients (41.3%), disease onset was before age 16, and in 78 patients it was after age 16 (58.7%). The female/male ratio was 1.3:1 for ages below 16. Polysymptomatic presentation (22.6%) was the most common initial feature. The mean disease duration was 9.48±8.43 years. 51.1% of the patients received disease-modifying and immunossuppresant drugs. The median EDSS score was 2.0 for 126 patients. MRI records of 111 patients were obtained. 11 MS patients did not initially present the diagnostic MRI features. All of the remaining MS patients fulfilled Barkhof-Tintore criteria (100%) and 88.7% the fulfilled KIDMUS criteria. Cranial MRI of NMO patients was normal. Out of 133 patients, OCB results were obtained from only 35 patients and were positive in 26 patients. 4.6% of the patients had positive family history of MS.

Conclusions CONCLUSIONS: Children with ADD should be of particular interest as they require accurate evaluation aiming to decrease the burden of the disease when they reach adulthood.

Authors/Disclosures
Derya Kaya PRESENTER	No disclosure on file
Egemen Idiman, MD	No disclosure on file
Serkan Ozakbas, MD (Department of Neurology Izmir University of Economics Medical Point Hospital)	Dr. Ozakbas has nothing to disclose.
Jeffrey A. Cohen, MD (Cleveland Clinic)	Dr. Cohen has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Convelo. Dr. Cohen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astoria. Dr. Cohen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Bristol Myers Squibb. Dr. Cohen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Viatris. Dr. Cohen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for PSI. Dr. Cohen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Shionogi. Dr. Cohen has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Celltrion.

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