Epidemiology of multiple sclerosis in the pediatric population of Sardinia, Italy

In this review the most important aspects of pediatric multiple sclerosis are presented and compared with the adult form. Some findings appear peculiar of pediatric MS: a. Clinical manifestations are similar in adolescents and adults with MS, however in very young subjects MS frequency at onset is similar in males and females, with a higher frequency of brainstem/cerebellar involvement and acute polysymptomatic/ADEM like onset; b. The course is relapsing-remitting in the large majority of patients, with a high relapse rate; c. Mild or severe disability is reached after a longer interval, but at a lower age compared to adult MS; d. The frequency of cognitive dysfunction is relatively high, with a quick deterioration but also the capability to partially recover with time; e. MRI is fundamental for diagnosis and prognosis: the pattern of MRI has some peculiar aspects in pediatric MS patients: i) the classic diagnostic criteria cannot be fulfilled, ii) lesion load is more relevant, iii) lesions are less destructive, have a more pronounced inflammatory pattern and have enhanced capability to recover with time; f. Diagnostic criteria of adults can be applied to patients with less than 18 years, but with limitations for subjects with less than 12 years and ADEM-like onset; g. The approach in differential diagnosis is particular complex and many disorders with clinical manifestations in the pediatric age must be considered: in this context it is important to pay attention to clinical, MRI and CSF red flags. Anti-MOG abs syndrome has been recently identified and should be carefully considered in patients with Acquired Demyelinating Syndrome; h. CSF oligoclonal bands are less frequent in pediatric MS patients, their presence in patients with Acquired Demyelinating Syndrome is strongly correlated to the risk of MS; i. The interplay between genetic and environmental factors determine the risk of developing MS; j. MS has a more pronounced inflammatory pattern (as suggested by the high relapse rate and the most relevant inflammatory pattern at brain MRI); k. The treatment is based on the approach used for adults: as MS has a strong impact on patients and their family the model of care should involve a team with specialized neurologists, pediatricians and neuro-pediatricians, nurses, psychologists, social workers and specialists of rehabilitative medicine.

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“…Some recent studies [7,8] have reported a higher incidence, up to 2.85/ 100,000 for pediatric-MS (ped-MS) cases in Sardinia (Italy) [7].…”

Section: Introductionmentioning

confidence: 99%

Pediatric versus adult MS: similar or different?

Ghezzi

Baroncini

Zaffaroni

et al. 2017

Mult Scler Demyelinating Disord

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“…Only one of these studies estimated the incidence of pediatric MS in the USA, reporting 0.57 cases per 100,000 children and adolescents in the state of Utah (Table 2)(7). Eight studies reported incidence rates for specific countries within Europe (8)(9)(10)(11)(12)(13)(14)(15). In the most commonly reported age group, 0-to 15year-olds, incidence rates ranged from 0.3 (16) to 0.64 (17) per 100,000 person-years.…”

Section: Incidencementioning

confidence: 99%

A systematic literature review of the burden of pediatric multiple sclerosis in North America and Europe

Lb¹,

Goble²,

Kroes³

et al. 2019

Preprint

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Background: Multiple sclerosis (MS) is a chronic neurodegenerative condition that severely impacts patient functioning and quality of life (QoL). Onset of MS typically occurs in adults and evidence is growing for onset in children and adolescents. Several studies describe the clinical burden of pediatric MS; however, to date, no systematic literature review summarizing the clinical burden of pediatric MS has been published. Objective: To conduct a systematic literature review to identify published data on the epidemiology, disease characteristics, QoL, and economic burden of pediatric MS in North America and Europe. Methods: Systematic searches were conducted in Ovid MEDLINE®, Embase, and Cochrane library to identify studies that enrolled patients with pediatric MS and reported at least one outcome related to the epidemiological, humanistic, and economic burden of pediatric MS. Only English-language studies were included. Results: In total, 7,447 unique records were identified, of which 103 met the study’s inclusion criteria. Of the included articles, 9 reported incidence and prevalence, 39 reported relapse rates, 15 reported QoL, 22 reported fatigue, 37 reported cognitive function, and 17 reported school performance. Incidence of pediatric MS ranged from 0.30 to 2.64 per 100,000 person-years, with few studies suggesting that incidence may increase with age. Prevalence was reported as high as 14.6 cases per 100,000 persons in the USA and 9.6% of all MS patients in European countries. The range of annualized relapse rates was similar across countries, at 0.36-2.76 in North America and 0.65-1.11 in European countries. Patients with pediatric MS experienced reduced QoL compared to healthy controls. Patients experienced reduced diminished cognitive functioning, worse school performance and increased fatigue. Limited information was identified for the mortality and economic burden of pediatric MS. Conclusions: Several studies describe the clinical burden of pediatric MS. Although incidence and prevalence rates for the disease are relatively low, the rate of relapse and burden to patients is considerable. Further studies are required to address gaps in the understanding of prognostic factors for relapse and the impact of QoL, fatigue, and cognitive function. Additional research on mortality and economic outcomes will be important to guide treatment pathways and reimbursement decisions.

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“…Studies have shown that there are areas with higher prevalence of MS in the world, such as North America and certain countries in northern Europe (17,18). The Orkney Islands represent an area that has the highest prevalence of MS, with 300 patients per 100,000 citizens (19), but some studies have also pointed out that Sardinia has the highest prevalence of pediatric MS (20). If we look at the American continent, the rise of African Americans with pediatric MS is noticeable, but still MS is most commonly seen in non-Hispanic white individuals (21).…”

Section: Introductionmentioning

confidence: 99%

Multiple Sclerosis Therapies in Pediatric Patients: Challenges and Opportunities

Jančić

Nikolić

Ivančević

et al. 2017

Multiple Sclerosis: Perspectives in Treatment and Pathogenesis

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Multiple sclerosis (MS) is an autoimmune, chronic, inflammatory, and demyelinating disease of the central nervous system (CNS). The etiology of MS is most likely multifactorial; it is dependent on genetic, autoimmune, and environmental factors, with a variable course among patients. The two main clinical events that characterize MS are relapses and progression. In recent years, diagnosis and treatment of pediatric MS has drawn attention of the scientific community. Management of pediatric MS focuses on reducing relapses and symptoms via administration of disease-modifying drugs (DMDs) and specific symptomatic treatment. A multidisciplinary approach to pediatric MS treatment is preferred, which aims at alleviating and preventing the accumulation of neurological deficits. MS therapy should be based on DMDs, that is, immunomodulatory drugs. These drugs, which sequester immune system activity, are further subdivided into two categories: first-line and second-line immunomodulatory therapy. First-line immunomodulatory therapy (interferon beta-1a, interferon beta-1b, Multiple Sclerosis in Pediatric Patients 40and glatiramer acetate) is ineffective (either no response or partial response) in roughly 30% of patients. Patients with a poor response to first-line therapy require second-line immunomodulatory therapy (natalizumab, mitoxantrone, fingolimod, teriflunomide, azathioprine, rituximab, dimethyl fumarate, daclizumab, alemtuzumab, and ocrelizumab). In addition to immunomodulatory drugs, treatment of relapses also involves the use of high intravenous doses of corticosteroids, administration of intravenous immunoglobulins, and plasmapheresis.

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Epidemiology of multiple sclerosis in the pediatric population of Sardinia, Italy

Abstract: The pediatric MS incidence in Sardinia is estimated to be significantly higher than those reported elsewhere. The pediatric MS prevalence in Sardinia is among the highest values worldwide.

Cited by 42 publications

References 35 publications

Pediatric versus adult MS: similar or different?

Pediatric versus adult MS: similar or different?

A systematic literature review of the burden of pediatric multiple sclerosis in North America and Europe

Multiple Sclerosis Therapies in Pediatric Patients: Challenges and Opportunities

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