To evaluate prognostic factors for second attack and for disability in children presenting with an initial episode of central nervous system (CNS) demyelination.
A cohort of 296 children having a first episode of acute CNS inflammatory demyelination was studied by survival analysis.
The average follow-up was 2.9+/-3 years. At the end of the follow-up, 57% of patients had a diagnosis of multiple sclerosis (MS), 29% had a monophasic acute disseminated encephalomyelitis, and 14% had a single focal episode. The rate of a second attack was (1). higher in patients with age at onset >or=10 years (hazard ratio, 1.67; 95% CI, 1.04-2.67), MS-suggestive initial MRI (1.54; 1.02-2.33), or optic nerve lesion (2.59; 1.27-5.29); and (2). lower in patients with myelitis (0.23; 0.10-0.56) or mental status change (0.59; 0.33-1.07). Of patients with a second attack, 29% had an initial diagnosis of acute disseminated encephalomyelitis. At the end of the follow-up period, 90% of patients had no or minor disability. Occurrence of severe disability was associated with a polysymptomatic onset (3.25; 1.16-11.01), sequelae after the first attack (26.65; 9.42-75.35), further relapses (1.49; 1.16-1.92), and progressive MS (3.57; 1.21-8.72).
Risk of second attack of CNS demyelination is higher in older patients and lower in patients with mental status change. Risk of disability is higher in polysymptomatic and relapsing patients.
"Relapsing disease that follows ADEM beyond a second encephalopathic event currently suggests a chronic disorder that often predates the diagnosis of MS or NMO [53, 54]. Some studies have suggested that 18% to 29% of patients with ADEM as their first demyelinating attack progress to MS [47, 55]. However, in a recent prospective study following the definitions proposed by the International Pediatric Multiple Sclerosis Study Group (IPMSSG) on children with ADEM, only 6% developed MS in a 9-year followup . "
[Show abstract][Hide abstract] ABSTRACT: Multiple sclerosis (MS), a chronic inflammatory autoimmune disease of the central nervous system (CNS) commonly diagnosed in adults, is being recognized increasingly in children. An estimated 1.7%-5.6% of all patients with MS have clinical symptoms before reaching the age of 18 years. In comparison with adults, the diagnosis of MS in children can be more difficult, being dismissed or misdiagnosed as other clinical disorders. Although adults and children share basic aspects of the disorder, children have distinctive clinical features, neuroimaging, laboratory, and courses of the disease. The 2010 McDonald criteria have simplified the requirements for establishing the diagnosis of MS and have been proposed to be applicable for the diagnosis of pediatric MS, mainly in children 12 years and older. This paper describes the distinctive features of common pediatric demyelinating disorders, including MS, and summarizes the most recent advances based on the available literature.
"ON and ATM were seen as initial presentation, respectively, in 19.4% and 10.5% of our patients, that were similar to the literature. ON and ATM were reported to be the initial presentation of MS in 14% to 35% and up to 10% of children, respectively [3, 16, 17, 19–23]. "
[Show abstract][Hide abstract] ABSTRACT: In a retrospective review of patients with acquired demyelinating disorders of the central nervous system, 133 patients (5.6%) whose diseases started in childhood, were selected from 2369 patients, who had medical records in the Neurology Department of Dokuz Eylul University. Out of 133, 98 had relapsing remitting multiple sclerosis, 21 had secondary progressive multiple sclerosis, 8 had clinically isolated syndrome, 3 had neuromyelitis optica, 2 had Marburg disease, and 1 had radiologically isolated syndrome. In 55 patients (41.3%), disease onset was before age 16. Polysymptomatic presentation (22.6%) was the most common initial feature. The EDSS scores ranged from 0 to 9 with a median of 2.0 (2.22 ± 1.88) for 126 patients. MRI records of 111 patients were obtained. 97 patients had clinically definite multiple sclerosis. 11 MS patients (11.3%) did not initially present the diagnostic MRI features. All of the remaining multiple sclerosis patients fulfilled Barkhof-Tintore criteria (100%) and 88.7% fulfilled KIDMUS criteria. Cranial MRI of NMO patients was normal. Our findings demonstrate some important clinical and paraclinical features that can help the literature on acquired demyelinating disorders of childhood by utilizing data from Western Turkey.
"Familial MS was reported in only 3% of ADS patients, which is in agreement with previous reports of numbers varying between 3 and 8% [1, 3, 9]. This proportion is higher in retrospective studies on pediatric MS and in studies with longer follow-up durations [10, 11]. "
[Show abstract][Hide abstract] ABSTRACT: Acquired demyelinating syndromes (ADS) can be a first presentation of multiple sclerosis (MS) in children. The incidence of these disorders in Europe is currently unknown. Children (<18 years old) living in the Netherlands who presented with ADS were included from January 1, 2007 to December 31, 2010 by the Dutch pediatric MS study group and the Dutch surveillance of rare pediatric disorders. Demographic and clinical data were collected. Eighty-six patients were identified over 4 years, resulting in an incidence of 0.66/1,00,000 per year. Most patients presented with polyfocal ADS without encephalopathy (30%), followed by polyfocal ADS with encephalopathy (24%), optic neuritis (ON, 22%), monofocal ADS (16%), transverse myelitis (3%), and neuromyelitis optica (3%). Patients with polyfocal ADS with encephalopathy were younger (median 3.9 years) than patients with ON (median 14.6 years, p < 0.001) or monofocal ADS (median 16.0 years, p < 0.001). Patients with polyfocal ADS without encephalopathy (median 9.2 years) were also younger than monofocal ADS patients (median 16.0 years, p < 0.001). There was a slight female preponderance in all groups except the ON group, and a relatively large number of ADS patients (29%) reported a non-European ancestry. Familial autoimmune diseases were reported in 23%, more often in patients with relapsing disease than monophasic disease (46 vs. 15%, p = 0.002) and occurring most often in the maternal family (84%, p < 0.001). During the study period, 23% of patients were subsequently diagnosed with MS. The annual incidence of ADS in the Netherlands is 0.66/1,00,000 children/year. A polyfocal disease onset of ADS was most common.
Journal of Neurology 02/2012; 259(9):1929-35. DOI:10.1007/s00415-012-6441-6 · 3.38 Impact Factor
Ž. Krsmanović, M. Živković, T. Lepić, A. Stanković, D. Veljančić, R. Raičević, E. Dinčić
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