Figure - available from: European Journal of Medical Research
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Macroscopic, magnetic resonance imaging and histologic imaging of the CNS space-occupying lesions. Panel A: macroscopic appearance of a lesion enucleated during neurosurgical procedure. Panel B: MRI shows multiple intraparenchymal subcortical cystic lesions of varying sizes in the right frontal and left temporal lobes. On T2 weighted images these lesions are characterized by a thin rim of intermediate signal intensity and are surrounded by a small area of perifocal high intensity zone most consistent with vasogenic edema. High central intensity on DWI with corresponding low signal on ADC maps reflects restricted diffusion within the lesion. There is a peripheral ring of enhancement on T1 weighted sequences obtained after gadolinium-based contrast agent injection. Panel C: histologic analysis of the lesion shown in panel A. Figure (A): Haematoxylin–Eosin, 40×: (a) a diffuse inflammatory infiltrate composed of histiocytes and less numerous plasma cells, lymphocytes and granulocytes can be seen in a fibrotic tissue, also with incremented vascularization; (b) Immunohistochemistry CD138, 40×: this staining highlights the plasma-cellular infiltrate; (c) Immunohistochemistry IgG, 40×: shows a high number of IgG-positive cells amidst the inflammatory infiltrate; (d) Immunohistochemistry IgG4, 40×: high number of IgG4-positive cells: in a hot-spot of 470 plasma cells more than 10 IgG4 + per HPF can be seen; IgG4/IgG ratio is also increased (45%)
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Background
Cerebral intraparenchymal masses represent usually a neoplastic, or infectious differential diagnostic workup in neurology or infectious disease units.
Case presentation
Our patient was an 82-year-old male presenting with seizures, cerebral masses and a history of past treated pulmonary tuberculosis. Initial workup included a differenti...
Citations
... More recently, De Maria and colleagues reported a case presenting with seizures and cerebral masses related to IgG4-RD. 8 The patient showed favorable Complete remission of central nervous system manifestations of IgG4-related disease with rituximab -a case report Aleksi J. Sihvonen , Sini M. Laakso, Olli Tynninen, Heikki Saaren-Seppälä and Mervi Löfberg Abstract: IgG4-related disease (IgG4-RD) is an emerging immune-mediated chronic fibrotic disease characterized by tumour-like mass formation. Reports of brain parenchymal involvement in IgG4-RD are rare and complete treatment-related remission of lesions has never been reported. ...
... pachymeningoencephalitis) even more so. 7,8,11 The disease often responds well to corticosteroids, also when there are meningeal manifestations. 12 However, in severe, refractory, and steroid-dependent cases inclusion of rituximab in the treatment regime has shown promise. ...
... 7,13 In parenchymal infiltrates, such reports are scarce. 7,8,11 To our knowledge, this is the first report of complete clinical and radiological remission of probable IgG4-RD pachymeningoencephalitis after rituximab treatment. Infliximab has also been reported beneficial in treating IgG4-RD 14 but our patient had long-lasting infliximab-therapy with confirmed high blood concentration and no autoantibody production against the medication at admittance, thus suggesting suboptimal effect against preventing manifestations of the central nervous system. ...
IgG4-related disease (IgG4-RD) is an emerging immune-mediated chronic fibrotic disease characterized by tumour-like mass formation. Reports of brain parenchymal involvement in IgG4-RD are rare and complete treatment-related remission of lesions has never been reported. Here, we present a woman in her mid-50s who developed headache and seizures. Brain magnetic resonance imaging revealed frontal bilateral pachymeningitis and a left frontal lobe parenchymal lesion, and pathologic findings were consistent with an IgG4-RD central nervous system manifestation. She had a history of tumour-like growth around the right optic nerve, orbital and maxillary cavities treated successfully with corticosteroids 28 years ago, and was receiving infliximab as a maintenance therapy for uveitis for the last 14 years. After initial high-dose corticosteroid treatment, the patient was treated with rituximab, and after three months, the patient presented with complete remission of IgG4-RD lesions and associated symptoms. This case illustrates the chronic, decades-spanning nature of IgG4-RD, and a complete response to rituximab even with intracerebral mass lesions that had emerged despite the use of infliximab, a therapy previously reported successful in IgG4-RD.
