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Nigerian Journal of Clinical Practice Jun 2024Demyelinating disorders of the central nervous system (CNS) are rare disorders characterized by inflammation and the selective destruction of CNS myelin. The incidence...
BACKGROUND
Demyelinating disorders of the central nervous system (CNS) are rare disorders characterized by inflammation and the selective destruction of CNS myelin. The incidence of this disorder is increasing in developed countries. Nigerian studies on the pediatric population on the subject are very scarce.
AIMS
The aim of the study was to document the epidemiology, clinical profile, and impact of late presentation on the treatment outcome of demyelinating diseases of the CNS in pediatric patients.
METHODS
The retrospective review of patients aged 1-15 years admitted in a tertiary hospital from January 2018 to December 2022 with various symptoms suggestive of demyelinating CNS disorders. The diagnosis was clinically and radiologically confirmed. Information retrieved from the case notes included patients' demographics, clinical symptoms and signs, number of days with symptoms to presentation in the hospital, results of the magnetic resonance imaging (MRI), treatment, and treatment outcomes. Data were entered in Excel sheet and results were presented in tables and percentages.
RESULTS
The incidence of demyelinating disorders over the period was 0.013% (10 out of 769 patients admitted over the period). Acute demyelinating encephalomyelitis (ADEM) was the most common disorder seen in the study population (60%, n = 6), followed by transverse myelitis and two (20%) had optic neuritis (ON). Most of the patients with ADEM were in the 1-5-year age group. The female-to-male ratio was 2.3:1. Paraplegia, visual impairment, and ataxia were the most common clinical presentations in the study population. One of the patients met the criteria for the diagnosis of multiple sclerosis during follow-up. Human immunodeficiency virus (HIV) was identified as the cause of demyelination in one case. Most of the patients improved with steroids.
CONCLUSION
ADEM was the most common clinical phenotype seen in this study. Patients with ADEM and ON had a better prognosis than transverse myelitis. Late presentation was also identified as a poor prognostic factor. Follow-up of cases is very important to monitor disease progression to multiple sclerosis.
Topics: Humans; Nigeria; Child; Female; Male; Adolescent; Child, Preschool; Retrospective Studies; Infant; Demyelinating Diseases; Magnetic Resonance Imaging; Incidence; Treatment Outcome; Myelitis, Transverse; Optic Neuritis
PubMed: 38943292
DOI: 10.4103/njcp.njcp_155_23 -
BMC Neurology Jun 2024Neuropsychological symptoms in the Cognitive, Energetic, Behavioural, and Affective (CEBA) domains are common in people with multiple sclerosis (PwMS) and can negatively... (Observational Study)
Observational Study
Protocol for the MS-CEBA study: an observational, prospective cohort study identifying Cognitive, Energetic, Behavioural and Affective (CEBA) profiles in Multiple Sclerosis to guide neuropsychological treatment choice.
BACKGROUND
Neuropsychological symptoms in the Cognitive, Energetic, Behavioural, and Affective (CEBA) domains are common in people with multiple sclerosis (PwMS) and can negatively affect societal participation. The current study aims to investigate whether there are combinations of symptoms in the different CEBA domains that consistently occur together, that is, if there are CEBA profiles that can be identified. If so, this study aims to develop a screening instrument identifying CEBA profiles in PwMS to select the most suitable neuropsychological rehabilitation treatment for a given CEBA profile and consequently improve the societal participation of PwMS.
METHODS
This study is an observational, prospective cohort study consisting of 3 phases. Phase 1 focuses on the identification of CEBA profiles in a large sample of PwMS (n = 300). Phase 2 focuses on validating these CEBA profiles through replication of results in a new sample (n = 100) and on the development of the screening instrument. Phase 3 focuses on qualitatively evaluating in a small group of PwMS whether the selected treatment is suitable for the given CEBA profile or whether existing neuropsychological treatments should be adapted to meet the needs of PwMS suffering from symptoms in multiple CEBA domains simultaneously. Primary outcome is the CEBA profile, which will be derived from performance on neuropsychological assessment consisting of tests and questionnaires regarding the CEBA domains using a latent profile analysis. Inclusion criteria include MS diagnosis, sufficient ability in the Dutch language, and an age between 18 and 70 years.
DISCUSSION
The results of the current study will contribute to a more comprehensive understanding of the entire spectrum of neuropsychological symptoms in PwMS. Identification of possible CEBA profiles, and accordingly, the development of a screening instrument determining the CEBA profile of PwMS in clinical practice, contributes to the timely referral of PwMS to the most suitable neuropsychological rehabilitation treatment. If necessary, adjustments to existing treatments will be suggested in order to sufficiently meet the needs of PwMS. All of this with the ultimate aim to improve societal participation, and thereby quality of life, of PwMS.
TRIAL REGISTRATION
Dutch Central Committee on Research Involving Human Subjects (CCMO) NL83954.042.23; ClinicalTrials.gov NCT06016309.
Topics: Humans; Multiple Sclerosis; Prospective Studies; Neuropsychological Tests; Male; Female; Adult; Middle Aged; Cohort Studies
PubMed: 38943063
DOI: 10.1186/s12883-024-03737-6 -
Neuroimaging Clinics of North America Aug 2024
Topics: Humans; Multiple Sclerosis; Neuroimaging; Demyelinating Diseases; Brain; Magnetic Resonance Imaging
PubMed: 38942530
DOI: 10.1016/j.nic.2024.04.001 -
Neuroimaging Clinics of North America Aug 2024
Topics: Humans; Multiple Sclerosis; Demyelinating Diseases; Neuroimaging; Brain
PubMed: 38942529
DOI: 10.1016/j.nic.2024.04.002 -
Neuroimaging Clinics of North America Aug 2024Cognitive impairment in multiple sclerosis (MS) is common and can have negative effects on quality of life. The clinical presentation can be more subtle and insidious.... (Review)
Review
Cognitive impairment in multiple sclerosis (MS) is common and can have negative effects on quality of life. The clinical presentation can be more subtle and insidious. Thus, cognitive impairment is often underrecognized by both persons with MS (PwMS) and clinicians, leading to underestimation disability due to MS. Recent evidence supports that relapses affect cognition in a similar pattern to other physical relapse symptoms and may be the only symptom of a relapse. Regular screening using validated tests for PwMS will improve the care provided and quality of life of PwMS.
Topics: Humans; Multiple Sclerosis; Cognitive Dysfunction
PubMed: 38942528
DOI: 10.1016/j.nic.2024.03.010 -
Neuroimaging Clinics of North America Aug 2024Multiple sclerosis (MS) is increasingly understood not only as a white matter disease but also involving both the deep and cortical gray matter (GM). GM pathology in... (Review)
Review
Multiple sclerosis (MS) is increasingly understood not only as a white matter disease but also involving both the deep and cortical gray matter (GM). GM pathology in people with MS (pwMS) includes the presence of lesions, leptomeningeal inflammation, atrophy, altered iron concentration, and microstructural changes. Studies using 7T and 3T MR imaging with optimized protocols established that GM damage is a principal driver of disease progression in pwMS. Future work is needed to incorporate the assessment of these GM imaging biomarkers into the clinical workup of pwMS and the assessment of treatment efficacy.
Topics: Humans; Multiple Sclerosis; Gray Matter; Magnetic Resonance Imaging; Neuroimaging; Brain
PubMed: 38942527
DOI: 10.1016/j.nic.2024.03.007 -
Neuroimaging Clinics of North America Aug 2024Magnetic resonance imaging is the most sensitive method for detecting inflammatory activity in multiple sclerosis, particularly in the brain where it reveals subclinical... (Review)
Review
Magnetic resonance imaging is the most sensitive method for detecting inflammatory activity in multiple sclerosis, particularly in the brain where it reveals subclinical inflammation. Established MRI markers include contrast-enhancing lesions and active T2 lesions. Recent promising markers like slowly expanding lesions and phase rim lesions are being explored for monitoring chronic inflammation, but require further validation for clinical use. Volumetric and quantitative MRI techniques are currently limited to clinical trials and are not yet recommended for routine clinical use. Additionally, MRI is crucial for detecting complications from disease-modifying treatments and for implementing MRI-based pharmacovigilance strategies, such as in patients treated with natalizumab.
Topics: Humans; Multiple Sclerosis; Magnetic Resonance Imaging; Brain; Treatment Outcome; Neuroimaging
PubMed: 38942526
DOI: 10.1016/j.nic.2024.03.009 -
Neuroimaging Clinics of North America Aug 2024Atypical demyelinating lesions (ADLs) can be idiopathic, occurring as isolated and self-limited events, or can appear in different stages of relapsing demyelinating... (Review)
Review
Atypical demyelinating lesions (ADLs) can be idiopathic, occurring as isolated and self-limited events, or can appear in different stages of relapsing demyelinating diseases. Not infrequently, ADLs occur in inflammatory syndromes associated with exogenous or endogenous toxic factors, metabolic imbalance, or infectious agents. It is important to recognize imaging patterns that indicate an inflammatory/demyelinating substrate in central nervous system lesions and to investigate potential triggers or complicating factors that might be associated. The prognostic and treatment strategies of ADLs are influenced by the underlying etiopathogenesis.
Topics: Humans; Demyelinating Diseases; Magnetic Resonance Imaging; Brain
PubMed: 38942525
DOI: 10.1016/j.nic.2024.03.006 -
Neuroimaging Clinics of North America Aug 2024Optic neuritis is a common feature in multiple sclerosis and in 2 other autoimmune demyelinating disorders such as aquaporin-4 IgG antibody-associated neuromyelitis... (Review)
Review
Optic neuritis is a common feature in multiple sclerosis and in 2 other autoimmune demyelinating disorders such as aquaporin-4 IgG antibody-associated neuromyelitis optica spectrum disorder and myelin oligodendrocyte glycoprotein antibody-associated disease. Although serologic testing is critical for differentiating these different autoimmune-mediated disorders, MR imaging, which is the preferred imaging modality for assessing the optic nerve, can provide valuable information, suggesting a specific diagnosis and guiding the appropriate serologic testing.
Topics: Humans; Multiple Sclerosis; Optic Nerve; Magnetic Resonance Imaging; Optic Neuritis; Neuromyelitis Optica; Neuroimaging
PubMed: 38942524
DOI: 10.1016/j.nic.2024.03.005 -
Neuroimaging Clinics of North America Aug 2024Spinal cord MRI plays an important role in the diagnosis and prognosis of multiple sclerosis (MS) and related disorders. The ANATOMICAL, pathologic, imaging and... (Review)
Review
Spinal cord MRI plays an important role in the diagnosis and prognosis of multiple sclerosis (MS) and related disorders. The ANATOMICAL, pathologic, imaging and prognostic consideriations for the spinal cord for MS and the most important other demyelinating disorders, neuromyelitis optica spectrum disorder and myelin oligodendrocyte glycoprotein-associated disease, are reviewed. Finally, differential diagnostic considerations of spinal cord MRI in MS and related disorders are discussed.
Topics: Humans; Multiple Sclerosis; Magnetic Resonance Imaging; Spinal Cord; Neuromyelitis Optica; Diagnosis, Differential
PubMed: 38942523
DOI: 10.1016/j.nic.2024.03.011