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Multiple Sclerosis and Related Disorders Feb 2020
Topics: Demyelinating Diseases; Diagnosis, Differential; Humans; Vascular Malformations
PubMed: 31711000
DOI: 10.1016/j.msard.2019.101410 -
Journal of Neurology Oct 2023
Topics: Humans; Immune Checkpoint Inhibitors; Neoplasms; Demyelinating Diseases
PubMed: 37597071
DOI: 10.1007/s00415-023-11933-6 -
Bailliere's Clinical Neurology Oct 1997In multiple sclerosis, partial remyelination is conspicuous in many lesions, and is thought to contribute significantly to lasting recovery from acute relapse. However,... (Review)
Review
In multiple sclerosis, partial remyelination is conspicuous in many lesions, and is thought to contribute significantly to lasting recovery from acute relapse. However, myelin repair ultimately fails during progression of the disease, as disability and handicap accumulate. In this chapter we explore the biological background to myelin repair in CNS demyelinating disease, and the reasons underlying the failure of more widespread and lasting remyelination in multiple sclerosis. Experimental studies provide clear evidence that therapies promoting myelin repair can be highly successful in the CNS, and we discuss the clinical approaches which might allow the translation of these laboratory studies to neurological practice, together with some of the potential hazards and pitfalls likely to arise.
Topics: Animals; Brain Tissue Transplantation; Cell Transplantation; Demyelinating Diseases; Disease Models, Animal; Disease Progression; Humans; Immunoglobulins; Multiple Sclerosis; Myelin Sheath; Nerve Growth Factors; Nerve Regeneration; Oligodendroglia; Remission, Spontaneous; Time Factors
PubMed: 10101588
DOI: No ID Found -
Continuum (Minneapolis, Minn.) Aug 2013In the past decade, the number of studies related to demyelinating diseases in children has exponentially increased. Demyelinating disease in children may be monophasic... (Review)
Review
PURPOSE OF REVIEW
In the past decade, the number of studies related to demyelinating diseases in children has exponentially increased. Demyelinating disease in children may be monophasic or chronic. Typical monophasic disorders in children are acute disseminated encephalomyelitis and clinically isolated syndromes, including optic neuritis and transverse myelitis. However, some cases of acute disseminated encephalomyelitis or clinically isolated syndrome progress to become chronic disorders, including multiple sclerosis and neuromyelitis optica. This review summarizes the current knowledge on monophasic and chronic demyelinating disorders in children, focusing on an approach to diagnosis and management.
RECENT FINDINGS
Improved diagnostic definitions for pediatric demyelinating diseases have led to enhanced recognition of these disorders. Additionally, increased awareness and focused national and international efforts continue to inform about the clinical course, response to treatment, and disease pathogenesis.
SUMMARY
Significant advances have been made in the recognition, diagnosis, and management of pediatric demyelinating disorders over the past 10 years. This review summarizes these advances and provides an updated approach to the diagnosis and management of pediatric demyelinating disorders.
Topics: Adolescent; Age of Onset; Child; Cognition Disorders; Diagnosis, Differential; Encephalomyelitis, Acute Disseminated; Female; Humans; Leukoencephalitis, Acute Hemorrhagic; Male; Multiple Sclerosis; Muscle Weakness; Neurologic Examination; Neuromyelitis Optica; Optic Neuritis
PubMed: 23917099
DOI: 10.1212/01.CON.0000433285.84973.43 -
Journal of Neurology, Neurosurgery, and... Aug 1974A 17 year old girl died after an illness characterized by progressive mental deterioration and severe myoclonic jerks. Extensive pathological, virological, and...
A 17 year old girl died after an illness characterized by progressive mental deterioration and severe myoclonic jerks. Extensive pathological, virological, and immunological studies failed to support the diagnosis of subacute sclerosing panencephalitis but were compatible with disseminated demyelinating disease. The spinal fluid was positive for measles virus antibodies when examined by the fluorescent antibody technique, by complement fixation, and by haemagglutination inhibition tests, but the antibody titres were not high. The concentration of vaccinia antibody in the serum was consistent with that found in the general population and none was detected in the spinal fluid. Animal and tissue culture studies failed to disclose a viral agent, but pathological sections revealed perivascular cellular infiltration, demyelination, rare inclusion bodies, and multinucleated giant cells. Although these studies were not definitive, a record of procedures used and results obtained, both positive and negative, may be helpful to other investigators interested in defining more accurately the clinical features and the pathogenesis of these diseases.
Topics: Adolescent; Antibodies, Viral; Astrocytes; Chickenpox; Complement Fixation Tests; Culture Techniques; Demyelinating Diseases; Female; Fluorescent Antibody Technique; Frontal Lobe; Hemagglutination Inhibition Tests; Herpes Simplex; Humans; Inclusion Bodies; Microscopy, Electron; Paramyxoviridae Infections; Vaccinia; Virus Cultivation; Viruses
PubMed: 4371078
DOI: 10.1136/jnnp.37.8.874 -
Neuroimaging Clinics of North America May 2013
Topics: Brain; Child; Demyelinating Diseases; Humans; Magnetic Resonance Imaging; Nerve Fibers, Myelinated; Neuroimaging
PubMed: 23608696
DOI: 10.1016/j.nic.2012.11.002 -
Neuroradiology Aug 1996We studied 21 cases of pathologically confirmed tumefactive demyelinating lesions and reviewed the spectrum of tumefactive demyelinating lesions in the literature.... (Review)
Review
We studied 21 cases of pathologically confirmed tumefactive demyelinating lesions and reviewed the spectrum of tumefactive demyelinating lesions in the literature. Radiological features and clinical data were reviewed to characterize the lesions as consistent with a known demyelinating disease, most notably multiple sclerosis. Atypical clinical or radiological features (other than tumefaction) were noted. Most lesions were part of a clinical and/or radiological picture consistent with multiple sclerosis. No case strongly suggestive of variants or related diseases, such as Schilder's disease or Balo's concentric sclerosis, were found. There was one case suggestive of acute disseminated encephalomyelitis. Features which help distinguish the lesions from tumour are discussed.
Topics: Adult; Biopsy; Brain; Brain Diseases; Brain Neoplasms; Child; Demyelinating Diseases; Diagnosis, Differential; Diffuse Cerebral Sclerosis of Schilder; Encephalomyelitis, Acute Disseminated; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Multiple Sclerosis; Neurologic Examination
PubMed: 8880719
DOI: 10.1007/BF00626098 -
Trends in Neurosciences May 1991Cellular and humoral immune mechanisms have been implicated in the pathogenesis of human and experimental demyelinating diseases of the CNS. How these interact in the... (Review)
Review
Cellular and humoral immune mechanisms have been implicated in the pathogenesis of human and experimental demyelinating diseases of the CNS. How these interact in the complex sequence of events that culminates in phagocytosis of myelin by macrophages has yet to be resolved. The relationship between leakage of the blood-brain barrier and demyelination, the reason why recurrent inflammatory demyelination occurs--seemingly in the absence of an antigen-specific immune response--and the lack of effective remyelination all require explanation if a coherent account of immunologically mediated demyelination is to be achieved. One approach to these problems is to study in vitro the developmental and cellular biology of oligodendrocytes--the glial cells responsible for the synthesis and maintenance of CNS myelin. This provides experimental opportunities not offered by more direct investigation of the intact nervous system, but carries the clear disadvantage that observations made in vitro cannot necessarily be extrapolated to humans.
Topics: Animals; Demyelinating Diseases; Humans; Oligodendroglia
PubMed: 1713717
DOI: 10.1016/0166-2236(91)90099-g -
Trends in Microbiology Jan 1997A variety of neurological diseases in humans, including multiple sclerosis (MS), have been postulated to have a viral etiology. The use of animal models provides... (Review)
Review
A variety of neurological diseases in humans, including multiple sclerosis (MS), have been postulated to have a viral etiology. The use of animal models provides insights into potential mechanism(s) involved in the disease process. The murine coronavirus-induced demyelinating disease in rodents is one such model for demyelinating disease in humans.
Topics: Animals; Chronic Disease; Coronavirus Infections; Demyelinating Diseases; Disease Models, Animal; Humans; Mice; Molecular Biology; Multiple Sclerosis; Murine hepatitis virus; Rats; Virus Diseases; Virus Latency
PubMed: 9025229
DOI: 10.1016/S0966-842X(97)81768-4 -
Annals of Diagnostic Pathology Oct 2002Demyelinating disease presenting as a solitary contrast-enhancing mass poses a diagnostic challenge for both radiologists and surgical pathologists. We report the cases...
Demyelinating disease presenting as a solitary contrast-enhancing mass poses a diagnostic challenge for both radiologists and surgical pathologists. We report the cases of two female patients, aged 23 and 37 years, who exhibited the clinical and radiologic features of a space-occupying mass strongly suggestive of neoplasia. In both patients, magnetic resonance imaging showed a ring-enhancing parietal lesion. Intraoperative frozen sections in both patients displayed histologic features strongly suggestive of a glial neoplasm, including marked hypercellularity, a prominent astrocytic component, and easily identifiable mitotic figures. However, permanent sections showed additional and helpful histologic findings that included Creutzfeldt astrocytes and granular mitoses. Subsequent immunostaining showed that the hypercellularity was principally caused by macrophage infiltration (HAM-56 and CD68) and an associated reactive astrocytosis (glial fibrillary acidic protein). Additional confirmatory tests included special stains for myelin (Luxol-fast-blue), which demonstrated focal, sharply marginated loss of myelin, and for axons (silver stain for axons and neurofilament protein immunohistochemistry), which showed relative preservation of axons in areas of myelin loss. Together, the special stains confirmed the demyelinating nature of the lesions. The keys to avoiding misdiagnosing a demyelinating pseudotumor as a diffuse glioma include a general awareness of this potential pitfall, including the radiologic appearance of demyelinating pseudotumors as contrast-enhancing solitary masses that mimic tumor; knowledge of the characteristic histologic features, including Creutzfeldt astrocytes and granular mitoses; and a high index of suspicion for macrophage infiltration combined with a willingness to use appropriate confirmatory immunohistochemical studies in suspicious or uncertain cases. This approach will minimize the chance of misdiagnosis and subsequent use of inappropriate and deleterious therapies.
Topics: Adult; Astrocytes; Brain Neoplasms; Demyelinating Diseases; Diagnosis, Differential; Female; Glioma; Humans; Immunohistochemistry; Magnetic Resonance Imaging; Radiography
PubMed: 12376918
DOI: 10.1053/adpa.2002.35737