scholarly journals Paraneoplastic Diseases of the Central Nervous System

F1000Research ◽  
2020 ◽  
Vol 9 ◽  
pp. 167 ◽  
Author(s):  
Jonathan Galli ◽  
John Greenlee

Paraneoplastic neurological syndromes are nonmetastatic complications of malignancy secondary to immune-mediated neuronal dysfunction or death. Pathogenesis may occur from cell surface binding of antineuronal antibodies leading to dysfunction of the target protein, or from antibodies binding against intracellular antigens which ultimately leads to cell death. There are several classical neurological paraneoplastic phenotypes including subacute cerebellar degeneration, limbic encephalitis, encephalomyelitis, and dorsal sensory neuropathy. The patient’s clinical presentations may be suggestive to the treating clinician as to the specific underlying paraneoplastic antibody. Specific antibodies often correlate with the specific underlying tumor type, and malignancy screening is essential in all patients with paraneoplastic neurological disease. Prompt initiation of immunotherapy is essential in the treatment of patients with paraneoplastic neurological disease, often more effective in cell surface antibodies in comparison to intracellular antibodies, as is removal of the underlying tumor.

2019 ◽  
Vol 6 ◽  
pp. 2329048X1983047 ◽  
Author(s):  
Carlos A. Pérez ◽  
Stephanie Garcia-Tarodo ◽  
Regina Troxell

Myelin oligodendrocyte glycoprotein is expressed in the central nervous system on the surface of oligodendrocytes and is associated with a broad range of adult and pediatric demyelinating phenotypes. The entire spectrum of clinical and radiologic features of myelin oligodendrocyte glycoprotein antibody spectrum disorder remains to be fully elucidated. We describe the case of a 9-year-old boy with immune-mediated myelitis undetectable by conventional magnetic resonance imaging in the context of relapsing anti-myelin oligodendrocyte glycoprotein spectrum disorder. Despite the severe clinical presentation, his symptoms improved significantly following treatment with corticosteroids. Because timely diagnosis and treatment is imperative to prevent disease recurrence and reduce long-term morbidity, serum anti-myelin oligodendrocyte glycoprotein antibody testing should be considered in all children with acute demyelinating syndromes and unusual clinical presentations—including seizures—both at presentation and at follow-up.


2012 ◽  
Vol 2012 ◽  
pp. 1-12 ◽  
Author(s):  
Bharath Wootla ◽  
Makoto Eriguchi ◽  
Moses Rodriguez

Multiple sclerosis (MS) is an inflammatory demyelinating disease of the central nervous system (CNS) with varied clinical presentations and heterogeneous histopathological features. The underlying immunological abnormalities in MS lead to various neurological and autoimmune manifestations. There is strong evidence that MS is, at least in part, an immune-mediated disease. There is less evidence that MS is a classical autoimmune disease, even though many authors state this in the description of the disease. We show the evidence that both supports and refutes the autoimmune hypothesis. In addition, we present an alternate hypothesis based on virus infection to explain the pathogenesis of MS.


2018 ◽  
Vol 89 (10) ◽  
pp. A19.3-A19
Author(s):  
White Daniel ◽  
Patel Markand ◽  
Muhammad Wasim ◽  
Hayton Tom ◽  
Ramalingam Satheesh ◽  
...  

BackgroundAutoimmune Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy is a relatively new category of immune-mediated disease involving the central nervous system that demonstrates a widely variable spectrum of clinical presentations, ranging from the relatively mild or subacute onset of cognitive impairment, seizures, encephalopathy, meningeal symptoms to more complex forms of encephalomyelitis.Materials and MethodsWe present a radiological review of two cases which were recently diagnosed in our institution. They presented with fever, meningoencephalitis and bilateral papilloedema. CSF antibody analysis (GFAPα-IgG) from Mayo Clinic confirmed the diagnosis of GFAP in both cases.ResultsThe typical radiological findings in both of the cases were a radial pattern of enhancement in brain and longitudinally extensive myelitic lesions in the spinal cord. The first patient improved with immunosupression treatment. The second patient had a significantly more severe clinical presentation with drug-refractory progression, who later died.ConclusionPatients presenting with subacute onset of cognitive impairment, meningoencephalomyelitis and papilloedema should raise the suspicion of autoimmune GFAP astrocytopathy. Though it is a relatively new disease entity, the radial pattern of enhancement and long spinal cord lesions on imaging are striking and CSF and serum antibodies are highly specific.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Aulia Rahmi Pawestri ◽  
Niracha Arjkongharn ◽  
Ragkit Suvannaboon ◽  
Aekkachai Tuekprakhon ◽  
Vichien Srimuninnimit ◽  
...  

AbstractAutoimmune retinopathy (AIR) is a rare immune-mediated inflammation of the retina. The autoantibodies against retinal proteins and glycolytic enzymes were reported to be involved in the pathogenesis. This retrospective cohort study assessed the antiretinal autoantibody profiles and their association with clinical outcomes of AIR patients in Thailand. We included 44 patients, 75% were females, with the overall median age of onset of 48 (17–74, IQR 40–55.5) years. Common clinical presentations were nyctalopia (65.9%), blurred vision (52.3%), constricted visual field (43.2%), and nonrecordable electroretinography (65.9%). Underlying malignancy and autoimmune diseases were found in 2 and 12 female patients, respectively. We found 41 autoantibodies, with anti-α-enolase (65.9%) showing the highest prevalence, followed by anti-CAII (43.2%), anti-aldolase (40.9%), and anti-GAPDH (36.4%). Anti-aldolase was associated with male gender (P = 0.012, OR 7.11, 95% CI 1.54–32.91). Anti-CAII showed significant association with age of onset (P = 0.025, 95% CI − 17.28 to − 1.24), while anti-α-enolase (P = 0.002, OR 4.37, 95% CI 1.83–10.37) and anti-GAPDH (P = 0.001, OR 1.87, 95% CI 1.32–2.64) were significantly associated with nonrecordable electroretinography. Association between the antibody profiles and clinical outcomes may be used to direct and adjust the treatment plans and provide insights in the pathogenesis of AIR.


1993 ◽  
Vol 268 (7) ◽  
pp. 5279-5284
Author(s):  
B.S. Weeks ◽  
K. Desai ◽  
P.M. Loewenstein ◽  
M.E. Klotman ◽  
P.E. Klotman ◽  
...  

2021 ◽  
pp. 106689692199356
Author(s):  
Fleur Cordier ◽  
Lars Velthof ◽  
David Creytens ◽  
Jo Van Dorpe

Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated inflammatory and demyelinating disorder of the central nervous system. Its characteristic perivenular demyelination and inflammation aid in the differential diagnosis with other inflammatory demyelinating diseases. Here, we present a clinical case of ADEM, summarize its histological hallmarks, and discuss pitfalls concerning the most important neuropathological differential diagnoses.


1991 ◽  
Vol 266 (28) ◽  
pp. 18655-18659 ◽  
Author(s):  
P.F. Blackmore ◽  
J. Neulen ◽  
F. Lattanzio ◽  
S.J. Beebe

2021 ◽  
Vol 84 (2) ◽  
pp. 71-84
Author(s):  
Mohsen Ali Alhomoud ◽  
Abdul Sattar Khan ◽  
Iftetah Alhomoud

<b><i>Background:</i></b> Multiple sclerosis (MS) is an inflammatory demyelinating chronic neurological disease that affects the central nervous system of young adults and their quality of life. Several studies have investigated the effects of pregnancy and breastfeeding on MS. However, the evidence regarding the influence of pregnancy and breastfeeding on MS is still accumulating. This review aimed to summarize the current evidence regarding the effects of pregnancy and breastfeeding on MS. <b><i>Summary:</i></b> A systematic electronic literature search of the PubMed and Embase databases was conducted to determine relevant published articles. The eligible studies were summarized and evaluated in tables. <b><i>Key Messages:</i></b> The majority of the studies indicated that pregnancy appears to lower the rate of MS relapses, particularly in the third trimester. The evidence regarding the effect of breastfeeding on MS remains inconsistent. Despite reports of negative obstetric outcomes in some pregnant women with MS, pregnancies in women with MS should not be categorized as high-risk pregnancies.


2004 ◽  
Vol 287 (1) ◽  
pp. L210-L216 ◽  
Author(s):  
Dirk Haufe ◽  
Thomas Luther ◽  
Matthias Kotzsch ◽  
Lilla Knels ◽  
Thea Koch

Intrapulmonary application of perfluorocarbons (PFC) in acute lung injury is associated with anti-inflammatory effects. A direct impact on leukocytic function may be involved. To further elucidate PFC effects on cellular activation, we compared in an in vitro model the response of concanavalin A (ConA)-stimulated lymphocytes and monocytes exposed to perfluorohexane. We hypothesized that perfluorohexane attenuates the action of the lectin ConA by altering stimulant-receptor interaction on the cell surface. Mononuclear blood cells were stimulated by incubation with ConA in the presence of different amounts of perfluorohexane. The response of lymphocytes and monocytes was determined by means of IL-2 secretion and tissue factor (TF) expression, respectively. The influence of perfluorohexane on cell-surface binding of fluorescence-labeled ConA was studied using flow cytofluorometry and fluorescence microscopy. Perfluorohexane itself did not induce a cellular activation but significantly inhibited both monocytic TF expression and, to a far greater extent, IL-2 secretion of ConA-stimulated mononuclear blood cells. The effect of perfluorohexane was due neither to an alteration of cell viability nor to a binding of the stimulant. The amount of cell surface-bound ConA was not altered by perfluorohexane, and the overall pattern of ConA receptor rearrangement did not differ between controls and treated cells. In the present study, we provide further evidence for an anti-inflammatory effect of PFC that might be beneficial in states of pulmonary hyperinflammation. A PFC-induced alteration of stimulant-receptor interaction on the surface membrane does not seem to be the cause of attenuated cell activation.


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