Objective: To evaluate the clinical course of MS in individuals with onset of MS before age 16.
Methods: Patients with onset of MS before age 16 (n = 116) with complete clinical information on the clinical course from the MS Clinic at The University of British Columbia (UBC) Site Hospital computerized database (MS-COSTAR) were included in this study. The data were compared to those from the Canadian natural history study for MS clinic attendees, regardless of age at onset.
Results: The mean duration of observation was 19.76 +/- 0.90 years; the mean age at MS onset was 12.73 +/- 0.25 years. Only three cases (2.6%) had a primary progressive (PP) MS course. To date, 60 (53.1%) of 113 subjects have developed secondary progressive (SP) MS. The 50% probability for SPMS was reached 23 years after onset. For patients with relapsing remitting (RR) or SPMS the mean disease duration from onset to the time of confirmed Expanded Disability Status Scale (EDSS) 3.0 was 16.03 +/- 1.17 years (at mean age 28.47 +/- 1.14); mean duration from onset to the time of EDSS 6.0 was 19.39 +/- 1.43 years (at mean age 32.32 +/- 1.44). Annual relapse rate was 0.54 +/- 0.05 per year. The correlation between the number of relapses during the first year of disease and the course of the disease was also significant.
Conclusions: The prevalence of early onset MS (3.6%) in our study confirms the previous findings on early onset MS. A RR course was seen in the majority of cases of early onset MS. A high frequency of relapses, early age at permanent disability, and the presence of malignant cases raise the question of possible early use of disease-modifying therapy in patients with early onset MS.
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http://dx.doi.org/10.1212/wnl.59.7.1006 | DOI Listing |
Acta Radiol
January 2025
R Madhavan Nayar Center for Comprehensive Epilepsy Care, Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.
Background: The role of imaging in autoimmune encephalitis (AIE) remains unclear, and there are limited data on the utility of magnetic resonance imaging (MRI) to diagnose, treat, or prognosticate AIE.
Purpose: To evaluate whether MRI is a diagnostic and prognostic marker for AIE and assess its efficacy in distinguishing between various AIE subtypes.
Material And Methods: We analyzed data from 96 AIE patients from our prospective autoimmune registry.
Front Genet
December 2024
Department of Pediatrics, West China Second University Hospital, Chengdu, Sichuan, China.
Background: Autosomal recessive cutis laxa type 1B (ARCL1B) is an extremely rare disease characterized by severe systemic connective tissue abnormalities, including cutis laxa, aneurysm and fragility of blood vessels, birth fractures and emphysema. The severity of this disease ranges from perinatal death to manifestations compatible with survival. To date, no cases have been reported in the Chinese population.
View Article and Find Full Text PDFCureus
December 2024
Department of Internal Medicine, Division of Hematology and Oncology, University of Texas Southwestern Medical Center, Dallas, USA.
Disseminated intravascular coagulation (DIC) is a hematological disorder characterized by the abnormal activation of the coagulation system, which leads to widespread clotting and subsequent consumption coagulopathy. DIC is often associated with the progression of prostate cancer and can be a life-threatening condition. In this case report, we present a patient with recurrent DIC in the setting of advanced prostate cancer.
View Article and Find Full Text PDF: Persistence of childhood adiposity is known to be associated with long-term adverse cardiometabolic risks. Yet, cross-sectional body mass index (BMI) is often used to classify obesity in clinical care and research. This study aimed to develop and validate a childhood obesity classification system using longitudinal clinical data.
View Article and Find Full Text PDFTo determine the frequency of confirmed Lyme neuroborreliosis (LNB) cases in adult patients with three different clinical presentations consistent with early LNB. Data were obtained through routine health care at the UMC Ljubljana, Slovenia from 2005-2022, using clinical pathways. The patients were classified into three groups: i) radicular pain of new onset (N = 332); or ii) involvement of cranial nerve(s) but without radicular pain (N = 997); or iii) erythema migrans (EM) skin lesion(s) in conjunction with symptoms suggestive of nervous system involvement but without either cranial nerve palsy or radicular pain (N = 240).
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