Year: 2014
Type: dissertation
Abstract: __Abstract__ \n \nAcquired inflammatory demyelinating diseases of the central nervous system (CNS) cause \ndamage to myelin sheaths and typically result in white matter lesions due to inflammation, \nmyelin loss and axonal pathology. Clinically, this may result in transient, relapsing or progressive \nneurological dysfunction. Multiple sclerosis (MS) is the most common disease within this \nspectrum. It typically affects adults between 20 and 40 years old. MS is generally assumed to \nbe an autoimmune disease, of which the exact etiology remains unknown. Genetic, immunological \nand environmental factors each play a role in the pathophysiology of the disease.1 \nSeveral other immune-mediated demyelinating diseases of the CNS are known. These \ninclude Acquired Demyelinating Syndrome (ADS) of childhood, Neuromyelitis Optica \n(NMO), Acute Disseminated Encephalomyelitis (ADEM), Transverse Myelitis (TM) and Optic \nNeuritis (ON).2 3 \nAll these acquired demyelinating syndromes are rare. In general, clinicians encounter \nseveral problems when faced with rare diseases: \n- diagnosis is more complex or may be delayed, because a clinician rarely encounters \nthese diseases and thus may be inexperienced with or unaware of them \n- the disease course and future may be hard to predict because of lack of insight into \npathogenesis \n- treatment is often based on expert-opinion instead of large randomized controlled trials, \nmaking it difficult to establish the optimal treatment and timing of treatment \n- research is challenged by the low number of patients. \nFor patients, it is more challenging to find the right information or to find fellow sufferers of \nthe same disease.4 \nThis thesis focuses on pediatric ADS and MS, ADEM and NMO and describes the clinical \nfeatures of these diseases. The goal of these studies is to find disease-specific characteristics \nthat will improve early and accurate diagnosis.
Author: I. A. Ketelslegers
Language: English
Subfield: Clinical Neurology
Field: Medicine
Domain: Health Sciences
Open Access status: green
OpenAlex ID: W104373338