Pediatric multiple sclerosis

Multiple sclerosis (MS) occurs at all ages of the pédiatric population. Childhood MS may represent up to 10% of all MS cases. Establishing the diagnosis of MS in a child is complicated by the limited diagnostic criteria and the possibility of significant clinical and magnetic resonance imaging (MRI) overlap with acute disseminated encephalomyelitis and other pediatric diseases. Although the clinical profile of MS appears similar to that seen in adults, several features may differ and specific issues arise in children. Sex ratios are different between young children with MS and adolescents— implicating a role for sex hormones in disease pathogenesis and/or modification of disease expression. Younger patients with MS are more likely to have seizures, brainstem, and cerebellar symptoms than adults. Children with MS may have fewer T2 hyperintense areas on MRI scans, therefore not meeting MRI criteria established for adults. It is possible that the pediatric MS course is more indolent than in adult patients but the disease may lead to significant disability at a younger age, e.g., while patients are students, young professionals, or want to start a family. There has been no controlled clinical trial in children with disease modifying therapies approved for adult MS due to the limited number of patients under the age of 18 years compared with the adult contingent. As a result, children are receiving adult therapies in an arbitrary manner and our understanding of pediatric treatment effect and tolerability is limited. Available data on tolerability of approved drugs for adults is reviewed.     

Pediatric multiple sclerosis

Pediatric multiple sclerosis (MS) represents a particular MS subgroup with unique diagnostic challenges and many unanswered questions. Due to the narrow window of environmental exposures and clinical disease expression, children with MS may represent a particularly important group to study to gain a better understanding of MS pathogenesis. Acute disseminated encephalomyelitis (ADEM) is more common in children than in adults, often making the differential diagnosis of MS, particularly a clinically isolated syndrome, quite difficult. Although both disorders represent acute inflammatory disorders of the central nervous system and have overlapping symptoms, ADEM is typically (not always) self-limiting. The presence of encephalopathy is much more characteristic of ADEM and may help in distinguishing between the two. Young children (under ten years old) with MS differ the most from adults. They have a lower frequency of oligoclonal bands in their cerebrospinal fluid and are less likely to have discrete lesions on MRI. Problems of cognitive dysfunction and psychosocial adjustment have particularly serious implications in both children and teenagers with MS. Increased awareness of these difficulties and interventions are needed. While clinical research on therapies to alter the disease course is limited, the available data fortunately suggests that disease-modifying therapy is well tolerated and likely to be effective. Ultimately, multinational research studies are necessary to advance our knowledge of the causes, symptoms, and treatment of pediatric MS and such collaborations are currently underway.     

Pediatric multiple sclerosis

Diagnosing multiple sclerosis (MS) in a child is challenging because of the limited diagnostic criteria and their overlap with acute disseminated encephalomyelitis. Pediatric-onset MS patients are more likely to be male, have seizures, and have brainstem and cerebellar symptoms than adults, and are less likely to have spinal cord symptoms than adults. They mostly experience a relapsing-remitting course. Their initial brain MRI shows more frequent involvement of the posterior fossa, less well-defined ovoid lesions, and more confluent lesions that decrease over time in patients with prepubertal onset, making early diagnosis even more difficult. Although disability progression is slower than in adults, pediatric onset MS leads to significant disability at a younger age and may be worse in non-white patients (up to 50% in North America). The rareness of pediatric-onset MS has precluded enrollment in clinical trials. Thus, children are receiving off-label adult therapies without clear evidence of their effectiveness and limited knowledge of their tolerability.     

Pediatric multiple sclerosis

In the past 5 years, there has been an exponential growth in the knowledge about multiple sclerosis (MS) in children and adolescents. Recent publications have shed light on its diagnosis, pathogenesis, clinical course, and treatment. However, there remain several key areas that require further exploration. This article summarizes the current state of knowledge on pediatric MS and discusses future avenues of investigation.     

Pediatric onset multiple sclerosis

Multiple Sclerosis (MS) is the commonest among inflammatory demyelinating diseases. While the disease prevalence is high in adults, frequency of pediatric onset multiple sclerosis (POMS) is very low in children and particularities in this population have been identified. We will address in this review characteristics of POMS.     

晚发型多发性硬化

本文报告１５例晚发型多发性硬化（ＭＳ），首次发病年龄５０－６０岁，占同期住院ＭＳ患者的６．４％。通过对其临床表现、病变部位和预后等与同期成人ＭＳ对照分析，表现晚发型ＭＳ慢性起病多见，临床表现和受累部位与成人ＭＳ相似，但脊髓受累较成人ＭＳ更为常见，晚发型ＭＳ大多病情进展快，运动功能障碍严重，病情多不能彻底缓解，预后差。     

Astrocytoma-like multiple sclerosis

Multiple sclerosis (MS) may sometimes mimic clinically and radiologically a brain tumor. The initial recognition of such cases is essential as it might avoid a surgical intervention and supplementary treatment. However, even in patients who underwent surgery, the appropriate preparation of the specimen is of crucial importance for the correct pathological diagnosis since tumors and non-neoplastic demyelinating lesions share some common histopathological features. We present such a case of multiple sclerosis presenting with features of an astrocytoma and was treated with surgery and additional radiotherapy.     

Primary-progressive multiple sclerosis

About 10-15% of patients with multiple sclerosis (MS) present with gradually increasing neurological disability, a disorder known as primary-progressive multiple sclerosis (PPMS). Compared with relapse-onset multiple sclerosis, people with PPMS are older at onset and a higher proportion are men. Inflammatory white-matter lesions are less evident but diffuse axonal loss and microglial activation are seen in healthy-looking white matter, in addition to cortical demyelination, and quantitative MRI shows atrophy and intrinsic abnormalities in the grey matter and the white matter. Spinal cord atrophy corresponds to the usual clinical presentation of progressive spastic paraplegia. Although neuroaxonal degeneration seems to underlie PPMS, the pathogenesis and the extent to which immune-mediated mechanisms operate is unclear. MRI of the brain and spinal cord, and examination of the CSF, are important investigations for diagnosis; conventional immunomodulatory therapies, such as interferon beta and glatiramer acetate, are ineffective. Future research should focus on the clarification of the mechanisms of axonal loss, improvements to the design of clinical trials, and the development of effective neuroprotective treatments.     

Subclinical multiple sclerosis

Bei 2 Patienten wurde bei der Autopsie als Zufallsbefund das Vorliegen multipler demyelinisierender Läsionen, die für eine multiple Sklerose typisch waren, festgestellt. Bei keinem derselben war, trotz Vorliegen einer guten klinischen Anamnese und einer neurologischen Untersuchung, ein Hinweis für einen Schub einer multiplen Sklerose zu Lebzeiten erhältlich. Das Fehlen von klinischen Manifestationen der Krankheit kann mit folgenden Faktoren erklärt werden. Die geringe Ausdehnung und kleine Zahl der Herde, welche in klinisch stummen Regionen des Zentralnervensystems saßen, und das Erhaltenbleiben klinischer Funktionen wegen der nur partiellen Demyelinisation bzw. der guterhaltenen Axone in den Herden. Informationen über die tatsächliche Häufigkeit von klinisch stummen Fällen von multipler Sklerose sind wichtig, um die Epidemyologie bzw. die ätiologischen Faktoren der Erkrankung zu verstehen.