A case of MuSK myasthenia gravis associated with solitary lymphadenopathy and insidious autoimmune‐like diffuse encephalopathy

Published on Jul 1, 2019in Neurology and Clinical Neuroscience
· DOI :10.1111/ncn3.12283
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#1Maartje G. Huijbers (LEI: Leiden University)H-Index: 12
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Last. Jan J.G.M. Verschuuren (LEI: Leiden University)H-Index: 56
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#1Izabela Rozmilowska (Medical University of Silesia)H-Index: 2
#2Monika Adamczyk-Sowa (Medical University of Silesia)H-Index: 11
Abstract Myasthenia gravis (MG) is a disease with autoimmune etiology. The main pathomechanism is related to the production of antibodies against nicotinic acetylcholine receptor (AChR) located in the postsynaptic membrane. The present study is set to compare the serum level of interleukin-33 (IL-33)/receptor ST2 in patients with myasthenia gravis and controls; and to study the relation with the severity of disease. Our observations confirm that a significant increase in serum concentrations of ...
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#1Mahmoud A. AbdelRazek (Harvard University)H-Index: 1
#2Nagagopal Venna (Harvard University)H-Index: 16
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Summary IgG4-related disease can involve nearly any organ system, including the central and peripheral nervous systems. The pathology findings are consistent from organ to organ, but careful clinicopathological correlation is necessary to establish the diagnosis. Many non-neurological and neurological inflammatory conditions, previously regarded as idiopathic in nature, are now recognised to fall within the spectrum of IgG4-related disease. The condition is highly treatable, but probably remains...
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#1Haruko Tanji (YU: Yamagata University)H-Index: 5
#2Hiroaki Okada (YU: Yamagata University)H-Index: 2
Last. Takeo Kato (YU: Yamagata University)H-Index: 43
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A 58-year-old woman with a 1-month history of right hand clumsiness and speaking difficulty was admitted to our hospital. A neurological examination revealed sensory aphasia and right hemiparesis. Her laboratory tests showed elevated serum levels of IgG and IgG4, pancytopenia, and liver dysfunction. The results of the imaging studies of her abdomen were compatible with sclerosing cholangitis. Brain MRI showed extensive signal abnormalities in the left hemisphere on T2-weighted and fluid-attenuat...
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#1D Joshi (UCL: University College London)H-Index: 2
#2Rolf Jager (UCL: University College London)H-Index: 3
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© 2015 Royal College of Physicians.IgG4-related disease is a recently recognised multi-system disease. Common organ involvement includes the pancreas, biliary tree and salivary glands. Central nervous system involvement has been infrequently reported. In a single-centre cohort of 84 patients, we report cerebral involvement in three (4%) patients. Details of cerebral involvement in these patients are outlined, including pituitary involvement in two patients and a diffuse autoimmune-like encephalo...
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#1L.L. Martinez (Radboud University Nijmegen Medical Centre)H-Index: 1
#2E. Friedlander (Radboud University Nijmegen Medical Centre)H-Index: 1
Last. Konnie M. Hebeda (Radboud University Nijmegen Medical Centre)H-Index: 22
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OBJECTIVES: IgG4-related disease is a recently recognized condition that can be associated with lymphadenopathy, with several histologic patterns and increased absolute number and ratio of immunoglobulin G4 (IgG4)-positive plasma cells. However, these findings are considered to be not exclusively specific for IgG4-related disease. METHODS: The occurrence of the histologic patterns reported in patients with isolated lymphadenopathy was studied and correlated with the clinical presentation to dete...
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#1Keren Regev (Tel Aviv Sourasky Medical Center)H-Index: 13
#2Tami Nussbaum (Tel Aviv Sourasky Medical Center)H-Index: 1
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Importance IgG4-related disease (IgG4-RD) is characterized by an inflammatory reaction rich in IgG4-positive plasma cells. Head and brain involvement is rare in IgG4-RD, and brain parenchyma involvement has never been reported. Observation A man in his mid-50s with multiorgan IgG4-RD developed progressive spastic hemiparesis and dementia. Magnetic resonance imaging of the brain revealed several cortical and subcortical lesions. Pathologic findings in the brain were consistent with IgG4-related c...
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#1Ruka Nakata (Nagasaki University)H-Index: 4
#2Masakatsu Motomura (Nagasaki University)H-Index: 22
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Background and purpose The differences in the characteristics of thymus histology, coexisting autoimmune diseases and related autoantibodies between anti-muscle-specific receptor tyrosine kinase (MuSK)-antibody (Ab)-positive myasthenia gravis (MG) patients, and anti-acetylcholine receptor (AChR)-Ab-positive MG patients are not clearly defined. Methods The types of thymus histology, coexisting autoimmune diseases and associated Abs in 83 MuSK-Ab-positive patients nationwide were investigated and ...
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