The Clinical and Epidemiological Profile of Paediatric-Onset Multiple Sclerosis in Poland.

Background. Paediatric-onset MS (POMS) has a unique clinical profile compared to the more prevalent adult-onset MS. For this study, we aimed to determine the demographic and clinical characteristics of POMS in Poland as well as addressing some of its epidemiological aspects. Methods. A retrospective study was conducted based on the Polish Multiple Sclerosis Registry, considering a population of children and adolescents with MS (age ≤ 18 years). Data were collected by all 13 centres across Poland specializing in diagnosing and treating POMS. The actual course of the disease and its clinical properties were compared between child (≤12 years) and juvenile (>12 years) patients. MS onset and its prevalence were assessed at the end of 2019, stratified by age range. Results. A total of 329 paediatric or juvenile patients (228 girls, 101 boys) with a clinically definite diagnosis of MS, in conformity with the 2017 McDonald Criteria, were enrolled. For 71 children (21.6%), the first symptoms appeared before the age of 12. The female: male ratio increased with age, amounting to 1:1 in the ≤12 years group and to 2.9:1 in the >12 years group. In most cases, the disease had multi-symptomatic onset (31.3%), and its course was mostly of a relapsing−remitting character (95.7%). The initial Expanded Disability Status Score for both groups was 1.63 ± 1.1, whereas the annual relapse rate was 0.84 during the first 2 years. The time between the onset of symptoms and diagnosis was longer in the younger patients (8.2 ± 4.2 vs. 4.6 ± 3.6 months; p < 0.005). On 31 December 2019, the age-adjusted prevalence standardized to the European standard population was 5.19/100,000 (95% CI, 4.64−5.78). Significantly higher prevalence was noted in the 13−18 years group (7.12; 95% CI, 6.64−7.86) than in the 9−12 years group (3.41; 95% CI, 2.98−3.86) and the <9 years group (0.56; 95% CI, 0.46−0.64; p < 0.001). Conclusion. POMS commencing at the age of ≤12 years is rare, differing significantly from the juvenile-onset and adult MS in terms of clinical characteristics, course, and incidence, as stratified by gender.

Pediatric-onset multiple sclerosis in Poland: A registry-based retrospective cohort study.

BACKGROUND: Epidemiologic data on pediatric-onset multiple sclerosis (POMS) in Central and Eastern Europe are limited. The aim of this study was to determine the incidence, prevalence and the clinical features of POMS in Poland. METHODS: Registry-based retrospective study was conducted among Polish children population (age ≤ 18 years), between 1 January 2010 and 31 December 2019. A total of 329 pediatric or juvenile patients fulfilled the International Pediatric MS Study Group (IPMSSG) criteria for MS, reported to the Polish Multiple Sclerosis Registry, were considered for estimation of age- and sex-specific prevalence (per 100,000 persons), and incidence rates (per 100,000 person-years). The demographic data, clinical presentation and treatment strategies also were investigated. RESULTS: On December 31, 2019 in the database were collected data of 329 patients up to 18 years with POMS diagnosis (101 boys and 228 girls; mean age 15.3 ± 3.8 years). The age-adjusted prevalence standardized to the European Standard Population was 5.19/100,000 (95% confidence interval (CI), 4.64-5.78). A significantly higher prevalence was recorded in girls (7.41; 95% CI, 6.48-8.44) than in boys (3.08; 95% CI, 2.50-3.74; P<0.001). The mean annual standardized incidence in Poland between 2015 - 2019 was 0.77 (95%CI, 0.45-1.02) per 100,000 person-years. The highest overall standardized incidence 1.06 (95%CI, 0.82-1.34) was noted in 2018. Most of patients (95.7%) had relapsing-remitting disease with polysymptomatic onset in one-thirds of the cases, and 82.3% were treated with disease-modifying drugs. Family history of MS was reported in 26 cases (7.9%). CONCLUSION: In this first report of registry-based study from Poland an increasing prevalence and incidence of POMS was found during the last years. This temporal trend corroborate the findings of studies conducted elsewhere.

Thrombomodulin and antibeta2-glycoprotein I in stroke in children.

BACKGROUND: A quantitative systematic review recently discussed the role of thrombomodulin (Thm) and antibeta2-glycoprotein I (beta2-GPI) in cerebral strokes in adults. Little is known about the problem in children. The aim of the study was to see if there is a difference in the values of Thm and beta2-GPI in children with ischemic stroke. MATERIAL/METHODS: Seventy patients were included, comprising 40 children who had had ischemic stroke of unknown etiology hospitalized from January 1995 to December 2005 at the Department of Developmental Neurology, Chair of Neurology Medical University of Gdansk, and 30 healthy volunteers (no autoimmunologic disease or headache in interview). The concentrations of thrombomodulin (Thm) and antibeta2-glycoprotein I (beta2-GPI) in A, M, and G immunoglobulins were determined according to an immunoenzyme method (ELISA). RESULTS: None of the investigated subjects had elevated levels of beta2-GPI. The patients with stroke had significantly higher Thm values than the healthy group. CONCLUSIONS: This finding of elevated levels of thrombomodulin in cases of pediatric cerebral stroke could help in measuring the extent or duration of parenchymal brain injury, or even perhaps response to future therapeutic maneuvers. All these implications may aid not only in the diagnosis and management of acute ischemic stroke, but encourage prophylactic action to prevent probable stroke relapse.

[Sporadic hemiplegic migraine in a 14-year-old boy--a case report]. / Sporadyczna migrena polowiczoporazna u 14-letniego chlopca--opis przypadku.

Hemiplegic migraine is a specific form of migraine with aura including hemiparesis. It is a rare condition of unclear aetiology. The authors present a case of a 14-year-old boy with persistent foramen ovale, suffering from sporadic hemiplegic migraine. The authors broadly describe the symptoms and types of hemiplegic migraine as well as underlining diagnostic and therapeutic difficulties of the attacks. The patient suffered from recurrent severe headaches, vomiting and co-existing focal neurological symptoms, including alternant hemiparesis. On the basis of the history, thorough clinical observation and numerous accessory investigations a diagnosis of sporadic hemiplegic migraine was established. However, the last attack, with prolonged neurological deficits, was treated as a complication of migraine and defined as persistent aura without infarction. The authors also broadly discuss a differential diagnosis, including other stroke-like incidents, vascular diseases, immunological and mitochondrial disorders that mimic the symptoms and clinical course of sporadic hemiplegic migraine.

[Antiphospholipid antibodies in children with migraine]. / Przeciwciala antyfosfolipidowe w migrenie u dzieci.

BACKGROUND AND PURPOSE: There are only a few investigations in the literature, that address the occurrence and the role of anticardiolipin antibodies (aCL) in children with migraine. The results of those studies are often contradictory. The aim of the study was to determine if the values of aCL in children with migraine differ from the control group. We tried to assess whether the type of migraine (with aura or without aura) had the influence on those values. MATERIAL AND METHODS: Sixty patients (mean age: 10.9+/-3.3 years), including 30 children with migraine hospitalized from January 2000 to December 2003 in the Department of Developmental Neurology Medical University of Gdansk and 30 healthy children, were studied. The values of aCL in class IgA, IgM and IgG were assessed by the immunoenzymatic method (ELISA test). RESULTS: The values aCL in IgA and IgG class were significantly different between the migraineurs and control group. The mean value of aCL in patients with migraine was 8.7+/-1.27 U/ml, while in the control group--3.81+/-1.74 U/ml. The positive values of aCL in class IgG were found in 11 (37%) children with migraine, and positive values of aCL in class IgM were noted in 6 (20%) cases in the same group. The type of migraine had no influence on the values of aCL. CONCLUSIONS: Children with migraine present with the higher values of aCL than the control group. The mean values of aCL were within the normal range, therefore their role in pathogenesis of migraine remains unclear. The further observation is needed to assess the reliable role of higher values of aCL in pathophysiology of vascular disorders.

[Epileptic seizures in the vascular malformations in children]. / Napady padaczkowe w wadach naczyniowych mózgu u dzieci.

OBJECTIVES: Cerebral vascular anomalies have been rare pathology of the brain in childhood. The most frequent clinical symptoms of them are: intracerebral hemorrhage, subarachnoid hemorrhage, headache, neurological deficits and epilepsy. AIM: Because of very few reports about epilepsy in cerebral vascular anomalies in children the authors continued studies in this subject. MATERIAL AND METHODS: The subject of the study were 20 patients in the age between 5 to 15 years with cerebral vascular anomalies hospitalized in the Department of Developmental Neurology Medical University of Gdansk in the years 1980-2001. The clinical state and CT, MRI, angiography and EEG recordings were evaluated in all the patients. Arteriovenous malformations (AVM) were diagnosed in 13 (65%), in 5--angioma cavernous (25%), in 2--aneurysms (10%). RESULTS: In 4 children epilepsy was the first symptom of cerebrovascular anomalies. Epileptic seizures were observed in 4 patients in the acute stage of the disease. In 6 children epilepsy was the consequence of arteriovenous malformation. CONCLUSION: Epilepsy occurred in 50% of children with vascular malformations. Among cerebrovascular anomalies, arteriovenous malformations are the most frequent cause of epilepsy in the group of examined children.

[Headache occurrence and assessment of regional blood flow after brain concussion in children]. / Wystepowanie bólów glowy oraz zmian regionalnego przeplywu mózgowego krwi po wstrzasnieniu mózgu u dzieci.

BACKGROUND AND PURPOSE: Headaches which can last for many months and even years belong to the most frequent consequences of closed head injuries in children. The method which makes it possible to demonstrate the presence and extent of the nervous tissue damage caused by trauma is the regional cerebral blood flow (rCBF) evaluation with single photon emission computerized tomography (SPECT). In the present study the regional cerebral blood flow (rCBF) was assessed 10-15 days after trauma, 3 months and one year in cases of brain concussion. We tried to establish whether there was a correlation between changes in rCBF and the occurrence of posttraumatic headache. MATERIAL AND METHODS: SPECT was applied in 32 children, aged 6-16, 10-15 days, and then 3 and 12 months after brain concussion. In all children no changes were found in CT and MRI examinations. RESULTS: In the studied group in the early period after trauma, blood flow impairment was found in 21 children, mostly in frontal areas. One year after trauma the rCBF improved in 11 children, in 10 cases the pattern was normal. In a group of 4 children with headache one year after brain concussion, three of them still presented the impairment of blood flow. CONCLUSIONS: SPECT shows a great sensitivity and usefulness in the assessment of consequences of head trauma and it can explain some posttraumatic complaints in children.