Teaching NeuroImages: Multifocal neurologic involvement as the only manifestation of IgG4-related disease
نویسندگان
چکیده
منابع مشابه
Teaching neuroimages: IgG4-related orbital disease and enlargement of the trigeminal nerve branches.
A 54-year-old man had a 5-year history of painless bilateral eyelid swelling, proptosis, and diplopia. MRI showed enlargement of extraocular muscles, lacrimal gland, and divisions of the trigeminal nerve (figure 1). Biopsy from the left infraorbital canal demonstrated lymphoplasmacytic infiltrate and fibrosis. Mean number of immunoglobulin G (IgG)41 cells was 155 per high-power field and ratio ...
متن کاملOtologic Manifestation in IgG4-Related Systemic Disease
IgG4-related systemic disease, including autoimmune pancreatitis, is a multi-organ disorder characterized by elevated serum immunoglobulin G4 (IgG4) concentration and IgG4-positive plasma cell infiltration. We report a case of a 66-year-old woman with IgG4-related hearing disorder, presenting with fluctuating mixed hearing loss with middle ear effusion. The serum IgG4 level was elevated and pat...
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A 69-year-old man had dyspnea followed by slowly progressive proximal leg weakness over 2 years. He had macroglossia (figure 1). Creatine kinase was 1,378 U/L. A deltoid biopsy revealed myopathy, denervation atrophy, and congophilic deposits around perimysial vessels, indicating amyloid (figure 2). Further workup revealed serum monoclonal lambda protein, bone marrow amyloid, and cardiomyopathy....
متن کاملIgG4-related disease presenting as panuveitis without scleral involvement
BACKGROUND The following case emphasizes the importance of including IgG4-related disease (RD) in the differential diagnosis of intraocular inflammation and multiple cranial nerve palsies. RESULTS A 33-year-old man, with a history of idiopathic bilateral panuveitis, presented with a new right pupillary-sparing partial third nerve palsy, which spontaneously resolved in 2 weeks, but was followe...
متن کاملPericardial Involvement in IgG4-related Disease.
We herein report the case of a 65-year-old man with pericardial involvement associated with autoimmune pancreatitis. Chest CT imaging showed pericardial thickening. The patient responded to corticosteroid therapy, and the pericardial thickening resolved. Multiple organs are involved in immunoglobulin G4 (IgG4)-related disease (IgG4-RD); however, only a few cases of IgG4-related chronic constric...
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ژورنال
عنوان ژورنال: Neurology
سال: 2013
ISSN: 0028-3878,1526-632X
DOI: 10.1212/wnl.0b013e31827f08ae