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Multiple Sclerosis and Related Disorders Nov 2014To evaluate the prevalence of and associated factors impacting vitamin D insufficiency and deficiency in childhood versus adult-onset demyelinating disease.
OBJECTIVE
To evaluate the prevalence of and associated factors impacting vitamin D insufficiency and deficiency in childhood versus adult-onset demyelinating disease.
METHODS
We conducted a retrospective, cross-sectional, chart-review, cohort study on geographically-similar pediatric, young adult, and adult patients with a diagnosis of demyelinating disease identified at the University of Virginia from 2008 to 2013. Group prevalence of vitamin D insufficiency and deficiency as well as relevant factors associated with vitamin D status was analyzed and compared.
RESULTS
We identified 24 childhood-onset (CO), 33 young adult-onset (Y-AO), and 59 adult-onset (AO) cases. There was no difference in the prevalence of vitamin D insufficiency or deficiency between the cohorts. Non-Caucasian race and elevated body mass index were significantly associated with low vitamin D levels, regardless of age of onset. In regression models, race and obesity were independent predictors of vitamin D status. The prevalence of obesity was significantly higher in the childhood-onset cohort (CO=58.5%; Y-AO=31%; AO=34%; p=0.02).
CONCLUSIONS
Our findings demonstrate no difference in the prevalence of vitamin D insufficiency/deficiency between childhood and adult-onset demyelinating disease, suggesting age at disease onset is irrelevant to vitamin D status in demyelinating disease. Both race and obesity are independent factors associated with vitamin D insufficiency/deficiency, regardless of age of disease onset. Obesity, independent of gender, is significantly higher in children compared to adult patients diagnosed with multiple sclerosis and may have a role in the development of childhood-onset demyelinating disease.
Topics: Adolescent; Adult; Age of Onset; Child; Cross-Sectional Studies; Demyelinating Diseases; Female; Humans; Male; Middle Aged; Obesity; Prevalence; Retrospective Studies; Risk Factors; Vitamin D; Vitamin D Deficiency; Young Adult
PubMed: 25891547
DOI: 10.1016/j.msard.2014.07.004 -
Current Neuropharmacology 2019Demyelinating diseases of the central nervous system (CNS) comprise a group of neurological disorders characterized by progressive (and eventually irreversible) loss of... (Review)
Review
INTRODUCTION
Demyelinating diseases of the central nervous system (CNS) comprise a group of neurological disorders characterized by progressive (and eventually irreversible) loss of oligodendrocytes and myelin sheaths in the white matter tracts. Some of myelin disorders include: Multiple sclerosis, Guillain-Barré syndrome, peripheral nerve polyneuropathy and others. To date, the etiology of these disorders is not well known and no effective treatments are currently available against them. Therefore, further research is needed to gain a better understand and treat these patients. To accomplish this goal, it is necessary to have appropriate animal models that closely resemble the pathophysiology and clinical signs of these diseases. Herein, we describe the model of toxic demyelination induced by cuprizone (CPZ), a copper chelator that reduces the cytochrome and monoamine oxidase activity into the brain, produces mitochondrial stress and triggers the local immune response. These biochemical and cellular responses ultimately result in selective loss of oligodendrocytes and microglia accumulation, which conveys to extensive areas of demyelination and gliosis in corpus callosum, superior cerebellar peduncles and cerebral cortex. Remarkably, some aspects of the histological pattern induced by CPZ are similar to those found in multiple sclerosis. CPZ exposure provokes behavioral changes, impairs motor skills and affects mood as that observed in several demyelinating diseases. Upon CPZ removal, the pathological and histological changes gradually revert. Therefore, some authors have postulated that the CPZ model allows to partially mimic the disease relapses observed in some demyelinating diseases.
CONCLUSION
for five decades, the model of CPZ-induced demyelination is a good experimental approach to study demyelinating diseases that has maintained its validity, and is a suitable pharmacological model for reproducing some key features of demyelinating diseases, including multiple sclerosis.
Topics: Animals; Brain; Cuprizone; Demyelinating Diseases; Disease Models, Animal; Oligodendroglia; Reproducibility of Results
PubMed: 28714395
DOI: 10.2174/1570159X15666170717120343 -
Multiple Sclerosis and Related Disorders Aug 2019MOG-antibody associated demyelinating disease is a new emerging diagnostic entity. Recently, international recommendations for testing of anti-MOG antibodies were...
MOG-antibody associated demyelinating disease is a new emerging diagnostic entity. Recently, international recommendations for testing of anti-MOG antibodies were published. Herein, we describe a case of anti-MOG antibody-associated demyelinating disease initially diagnosed as typical MS, and, at presentation, not fulfilling the proposed recommendations. This case highlights the expanding spectrum of anti-MOG antibody-associated demyelinating disease, illustrating the distinct and overlapping features of MS and MOG-antibody associated demyelinating disease, providing evidence that on rare occasions these recommendations may prove too restrictive.
Topics: Adult; Autoantibodies; Autoantigens; Demyelinating Autoimmune Diseases, CNS; Diagnosis, Differential; Humans; Male; Multiple Sclerosis; Myelin-Oligodendrocyte Glycoprotein
PubMed: 31158804
DOI: 10.1016/j.msard.2019.05.021 -
Annals of Neurology Mar 1978Painful bilateral orbicularis clonus on eccentric gaze developed in 2 patients with demyelinating disease. This unusual phenomenon was of variable intensity but...
Painful bilateral orbicularis clonus on eccentric gaze developed in 2 patients with demyelinating disease. This unusual phenomenon was of variable intensity but persisted for years and did not respond to phenytoin or carbamazepine therapy.
Topics: Adult; Blepharospasm; Demyelinating Diseases; Eyelid Diseases; Fixation, Ocular; Humans; Male
PubMed: 666264
DOI: 10.1002/ana.410030310 -
Progress in Medical Virology.... 1969
Review
Topics: Adult; Aged; Autopsy; Brain; Brain Diseases; Cell Membrane; Cell Nucleus; Cytoplasm; Demyelinating Diseases; Female; Humans; Kidney; Lung; Male; Microscopy; Microscopy, Electron; Middle Aged; Oncogenic Viruses; Papillomaviridae; Polyomaviridae
PubMed: 4906870
DOI: No ID Found -
AJR. American Journal of Roentgenology Jan 2014The purpose of this article is to discuss classic applications in diffusion-weighted imaging (DWI) in demyelinating disease and progression of DWI in the near future. (Review)
Review
OBJECTIVE
The purpose of this article is to discuss classic applications in diffusion-weighted imaging (DWI) in demyelinating disease and progression of DWI in the near future.
CONCLUSION
DWI is an advanced technique used in the follow-up of demyelinating disease patients, focusing on the diagnosis of a new lesion before contrast enhancement. With technical advances, diffusion-tensor imaging; new postprocessing techniques, such as tract-based spatial statistics; new ways of calculating diffusion, such as kurtosis; and new applications for DWI and its spectrum are about to arise.
Topics: Contrast Media; Demyelinating Diseases; Diagnosis, Differential; Diffusion Magnetic Resonance Imaging; Disease Progression; Humans; Image Enhancement; Image Interpretation, Computer-Assisted
PubMed: 24370163
DOI: 10.2214/AJR.13.11400 -
Journal of Neurology, Neurosurgery, and... Sep 2008The diversity of multiple sclerosis (MS) and the nosology of the conventional form of MS (CMS), optic-spinal MS (OSMS) and neuromyelitis optica (NMO) have been subject...
BACKGROUND
The diversity of multiple sclerosis (MS) and the nosology of the conventional form of MS (CMS), optic-spinal MS (OSMS) and neuromyelitis optica (NMO) have been subject to controversy.
AIMS
The purpose of this study was to investigate whether the current Asian optic-spinal multiple sclerosis (OSMS) criteria could also apply in Western countries, and whether or not cerebrospinal fluid (CSF) and imaging features in the Western Australian patient population of demyelinating disease was similar to that found in Asia.
METHODS
This study retrospectively reviewed 915 individual case notes with central nervous system demyelinating disease seen by two neurologists in Western Australia (WA). 842 cases had sufficient data to be included in the analysis. The patient population was predominantly Caucasian, representing approximately two-thirds of MS cases in WA. The mean duration of follow-up for the whole studied cohort was 12.5 years, with 136 patients (16.2%) being followed-up for more than 20 years.
RESULTS
The study confirmed the relatively low frequency of OSMS as a proportion of total demyelinating disease occurring in western countries, with 31 OSMS (3.7%) cases in contrast to 703 CMS cases (83.5%). It is likely, however, that our retrospective classification significantly underestimated the proportion of OSMS cases when compared with prospectively classified Asian cohorts. There were 11 OSMS cases that could also be classified as NMO according to published diagnostic criteria. The remainder of the spectrum comprised clinically isolated syndromes such as 50 acute myelitis (AM, 5.9%), 42 optic neuritis (ON, 5%) and 16 "atypical" cases such as tumefactive MS and acute disseminated encephalomyelitis (1.9%). The clinical characteristics of OSMS in our study were compatible with so-called Asian MS in many respects: oligoclonal bands (OCBs) were less frequent in OSMS (29.4%) than in CMS (66.4%, p = 0.003); visual evoked potentials and spinal MRI abnormalities were more prevalent in OSMS (85% and 92.6%) than in CMS (71.4% and 85%); as were long spinal cord lesions in OSMS (22.2%) versus CMS (3.4%, p,0.001). Brain abnormalities were seen in 48.4% of OSMS patients and 96.2% of CMS patients (p = 0.001). OCBs were identified in 7% of acute myelitis, 14.3% of optic neuritis and 73.4% of primary progressive MS patients.
CONCLUSIONS
This cross-sectional study presents the full spectrum of demyelinating disease in WA, which has a stable population representing 10% of the total Australian population and suggests that the current classifications of MS, OSMS or NMO, ON and AM share many clinical and laboratory features, such as female predominance, age at onset, duration of disease and CSF investigations (including OCBs and MRI). Moreover, characteristics of the WA population were similar to those reported in Asian patients.
Topics: Adult; Australia; Catchment Area, Health; Cross-Sectional Studies; Demography; Demyelinating Diseases; Diagnosis, Differential; Evoked Potentials, Visual; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Male; Multiple Sclerosis; Neuromyelitis Optica; Prevalence; Retrospective Studies
PubMed: 18356258
DOI: 10.1136/jnnp.2007.131177 -
Journal of the Neurological Sciences May 2013Multiple sclerosis is an autoimmune inflammatory demyelinating disease of the central nervous system characterized by dissemination of the lesions in time and space.... (Review)
Review
Multiple sclerosis is an autoimmune inflammatory demyelinating disease of the central nervous system characterized by dissemination of the lesions in time and space. While tremor is frequently seen in patients with multiple sclerosis, other movement disorders such as parkinsonism, dystonia, chorea, ballism, paroxysmal dystonia, paroxysmal chorea, myoclonus, tourettism, restless leg syndrome and hemifacial spasm are less frequently reported. In this systematic review of the literature, we describe the different movement disorders reported in patients with multiple sclerosis and attempt to characterize their relation with the underlying demyelinating process. We also summarize the reports of movement disorders described in other demyelinating diseases such as neuromyelitis optica, acute disseminated encephalomyelitis and central pontine myelinolysis.
Topics: Demyelinating Diseases; Humans; Movement Disorders; Multiple Sclerosis
PubMed: 23522528
DOI: 10.1016/j.jns.2013.02.007 -
Current Opinion in Neurobiology Oct 1991The pace of research on the pathogenesis and treatment of multiple sclerosis, the principal human demyelinating disease of the central nervous system, has intensified in... (Review)
Review
The pace of research on the pathogenesis and treatment of multiple sclerosis, the principal human demyelinating disease of the central nervous system, has intensified in the past 3 years, due in part, to the application of advances in molecular and cellular immunology. Many lessons that have been learned in an animal model of central nervous system demyelinating disease, experimental allergic encephalomyelitis, also apply to multiple sclerosis and certain successful approaches for the treatment of this disease are now being attempted in humans.
Topics: Animals; Demyelinating Diseases; Humans; Immunosuppression Therapy; Immunotherapy
PubMed: 1821688
DOI: 10.1016/0959-4388(91)90066-g -
Turkish Neurosurgery 2017Demyelinating pseudotumor is a rare inflammatory demyelinating disease of the central nervous system (CNS) that has a similar clinical presentation and computed... (Review)
Review
Demyelinating pseudotumor is a rare inflammatory demyelinating disease of the central nervous system (CNS) that has a similar clinical presentation and computed tomography (CT) and magnetic resonance imaging (MRI) imaging findings as brain tumors or abscesses. Unlike brain tumors, demyelinating pseudotumors respond well to steroid hormones. There are only a few reported cases of intracranial demyelinating pseudotumors in the literature. In this case report, we present the diagnosis and treatment of demyelinating pseudotumor in a patient whose condition was initially misdiagnosed as an astrocytoma. Based on the literature and our case, we formulated an outline for the differential diagnosis of demyelinating pseudotumor and astrocytoma. A timely and correct diagnosis of demyelinating pseudotumor would avoid blind surgery, radiotherapy and chemotherapy, which are used to treat brain tumors.
Topics: Adult; Astrocytoma; Brain Neoplasms; Demyelinating Diseases; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Neuroimaging; Tomography, X-Ray Computed
PubMed: 27349392
DOI: 10.5137/1019-5149.JTN.10920-14.0