1/315. Cortical dysplasia with angiodysgenesis and chronic inflammation in multifocal partial epilepsy.A 25-year-old man with a long history of temporal lobe epilepsy developed right occipital lobe seizures and a progressive right homonymous hemianopia. MRI showed diffuse enhancement of the left temporoparieto-occipital white matter and cortical thickening of the left medial temporal lobe. The resected temporal lobe revealed cortical dysplasia and angiodysplasia with foci of more recent ischemic necrosis and chronic inflammation as an explanation for the clinical deterioration.- - - - - - - - - - ranking = 1keywords = inflammation (Clic here for more details about this article) |
2/315. Autoaggressive inflammation of the myenteric plexus resulting in intestinal pseudoobstruction.After a 3-year history of severe constipation, a 16-year-old girl required surgery to be relieved of impacted stools. Histologic examination showed ganglionitis in the myenteric plexus of the large bowel and ileum, whereas the submucosal plexus was spared. At this time, antineuronal nuclear antibodies (ANNA-1, anti-Hu) were found at high titer in the serum of the patient. One and a half years earlier, a paravertebral ganglioneuroblastoma had been removed. Histologic examination had shown undifferentiated neuroblasts and morphologically mature ganglion cells with both cell types embedded in an inflammatory infiltrate morphologically similar to the lymphoplasmocytic infiltration seen in the myenteric plexus. The patient's serum was found to bind to nuclei of mouse intestinal tract neurons, thus fulfilling defining criteria for ANNA-1. The serum also reacted with antigens of defined molecular weight in a Western blot, thus fulfilling defining criteria for anti-Hu. Expression of the Huantigen could be visualized in the nuclei of the patient's tumor cells by immunohistochemistry. These tests showed that an antitumor inflammatory response was the cause of the bowel disease. This is the first report of a tumor from the neuroblastoma group that caused paraneoplastic intestinal pseudoobstruction. Ganglionitis and subsequent aganglionosis are the hallmark of the morphologic diagnosis which cannot be obtained by suction biopsy in patients with intact submucosal plexus. Instead, serum testing for autoantibodies can reveal the etiology.- - - - - - - - - - ranking = 0.8keywords = inflammation (Clic here for more details about this article) |
3/315. Conjunctival melanocytic nevi of childhood.Two young patients with conjunctival compound nevi are presented to illustrate two types of abnormalities that lead to difficulty in distinction of these nevi from invasive melanomas. In Case 1, inflammation is associated with disruption of the nevus cell architecture and cytologic atypia. In Case 2, the occurrence of a combined nevus (compound and blue nevus types) in the conjunctiva leads to diagnostic problems. Circumscription of the lesions, lack of mitoses in the substantia propria, and lack of pagetoid spread of atypical cells in the adjacent conjunctival epithelium support benign diagnoses in both cases.- - - - - - - - - - ranking = 0.2keywords = inflammation (Clic here for more details about this article) |
4/315. Lesion development in Marburg's type of acute multiple sclerosis: from inflammation to demyelination.We report a patient who suffered from acute inflammatory CNS demyelination and underwent two consecutive diagnostic stereotactic brain biopsies during the early disease course. The first lesion was drawn 33 days after the onset of disseminated neurological symptoms. macrophages and T lymphocytes diffusely infiltrated small vessel walls and the white matter. mRNA for tumor necrosis factor alpha (TNFalpha) and inducible nitric oxide synthase (iNOS) was abundantly expressed. Myelin sheaths were entirely preserved. The second biopsy 76 days later showed confluent demyelinating lesions with a diffuse infiltration of macrophages that were positive for myelin debris, activation markers and TNFalpha and iNOS mRNA. IgG and C9neo deposits were found along myelin sheaths. The patient had received intravenous immunoglobulins (IVIG) prior to biopsy. Findings from this single patient affirm that demyelination follows the migration of inflammatory cells from the circulation into the white matter with subsequent inflammation and demyelination. inflammation alone may be sufficient to cause significant clinical deficits without demyelination. Inflammatory mediators such as TNFalpha and NO are involved at very early stages in the pathogenetic process. IVIG treatment may lead to the deposition of immunoglobulins and to the activation of the complement cascade, but the clinical relevance of this particular finding remains uncertain.- - - - - - - - - - ranking = 1keywords = inflammation (Clic here for more details about this article) |
5/315. Loss of endothelial surface expression of e-selectin in a patient with recurrent infections.Neutrophil accumulation at sites of inflammation is mediated by specific groups of cell adhesion molecules including the beta2 (CD18) integrins on leukocytes and the selectins (P- and e-selectin on the endothelium and l-selectin on the leukocyte). This is supported by studies of patients with leukocyte adhesion deficiency syndromes whose leukocytes are genetically deficient in the expression of beta2 integrins or selectin carbohydrate ligands (eg, sialyl-Lewis(x)). However, inherited deficiency or dysfunction of endothelial cell adhesion molecules involved in leukocyte recruitment has not been previously described. In this report we describe a child with recurrent infections and clinical evidence of impaired pus formation reminiscent of a leukocyte adhesion deficiency syndrome, but whose neutrophils were functionally normal and expressed normal levels of CD18, l-selectin, and sialyl-Lewis(x). In contrast, immunohistochemical staining of inflamed tissue from the patient showed the absence of e-selectin from the endothelium, although e-selectin mRNA was present. However, e-selectin protein was expressed as significantly elevated levels of circulating soluble e-selectin were detected, the molecular size of which was consistent with a proteolytically cleaved form of e-selectin. Gene sequencing failed to show evidence of a secreted mutant variant. These data represent, to our knowledge, the first description of a potentially inherited dysfunction of an endothelial cell adhesion molecule involved in leukocyte recruitment and provide additional human evidence of the importance of endothelial selectins in the inflammatory response.- - - - - - - - - - ranking = 0.2keywords = inflammation (Clic here for more details about this article) |
6/315. Painful swelling of the thigh in a diabetic patient: diabetic muscle infarction.A 44-year-old woman with a 5-year history of poorly controlled Type 1 diabetes mellitus presented with a painful, firm and warm swelling in her right thigh. pain was severe but the patient was not febrile, and had no history of trauma or abnormal exercise. Laboratory tests showed ketoacidosis, major inflammation (erythrocyte sedimentation rate (ESR) = 83 mm/h), normal white blood cell count and normal creatine kinase level. Plain radiographs were normal, and there were no signs of thrombophlebitis at Doppler ultrasound. magnetic resonance imaging (MRI) showed diffuse enlargement and an oedematous pattern of the adductors, vastus medialis, vastus intermedius and sartorius of the right thigh. The patient's symptoms improved dramatically, making biopsy unnecessary, and a diagnosis of diabetic muscular infarction was reached. Idiopathic muscular infarction is a rare and specific complication of diabetes mellitus, typically presenting as a severely painful mass in a lower limb, with high ESR. The diabetes involved is generally poorly controlled longstanding Type 1 diabetes with established microangiopathy. Differential diagnoses include deep vein thrombosis, acute exertional compartment syndrome, muscle rupture, soft tissue abscess, haematoma, sarcoma, inflammatory or calcifying myositis and pyomyositis. In fact, physician awareness should allow early diagnosis on the basis of clinical presentation, routine laboratory tests and MRI, thereby avoiding biopsy and its potential complications as well as unnecessary investigations. rest, symptomatic pain relief and adequate control of diabetes usually ensure progressive total recovery within a few weeks. Recurrences may occur in the same or contralateral limb.- - - - - - - - - - ranking = 0.2keywords = inflammation (Clic here for more details about this article) |
7/315. Acute self-limiting jejunitis as the first manifestation of microscopic polyangiitis associated with Sjogren's disease: report of one case and review of the literature.We report a case of acute self-limiting ulcerative jejunitis of unknown aetiology in a 72-year-old female patient in which a subsequent diagnosis of microscopic polyangiitis and sjogren's syndrome was made. All known causes of jejunal ulceration and inflammation were excluded. Previously reported cases of acute self-limiting jejunitis are reviewed and the possibility that acute jejunitis in this patient had been the first manifestation of systemic vasculitis is discussed.- - - - - - - - - - ranking = 0.2keywords = inflammation (Clic here for more details about this article) |
8/315. Anti-adhesion molecule therapy as an interventional strategy for autoimmune inflammation.Functional inactivation of leukocyte adhesion molecules has been used to intervene in the development of tissue injury in experimental models of postperfusion infarction as well as autoimmune inflammation. We investigated the use of humanized monoclonal antibodies (mAb) against CD18 in the treatment of five patients with vasculitic tissue injury sufficient to threaten infarction or gangrene. The treatment was monitored in three ways: (i) whole-body gamma camera scintiscanning of autologous indium-labeled PMN, (ii) an index of the therapeutic inhibition of adhesion derived from comparison pre, during, and post mAb treatment of the ability of patients' PMN to be aggregated after activation by fMLP, and (iii) flow cytometric analysis of PMN CD18 expression. Four of five patients given anti-CD18 at 20 mg/day for up to 3 weeks showed prompt clinical improvement, with healing of the ulceration and restoration of limb function within 4 weeks, which was sustained. The fifth patient, who was not doing well clinically, decided to withdraw from all active treatment: at autopsy there was no evidence of the underlying vasculitis evident pretreatment. Our findings suggest that anti-adhesion molecule treatment might be an effective immediate treatment in severe vasculitis especially when tissue viability is threatened by progressive infarction and/or development of gangrene.- - - - - - - - - - ranking = 1keywords = inflammation (Clic here for more details about this article) |
9/315. Pseudotumor of the orbit in early childhood.orbital pseudotumor, also known as idiopathic orbital inflammation, is defined as a nonspecific, nonneoplastic inflammatory process of the orbit without identifiable local or systemic causes. The disorder, first described by Birch-Hirschfield in 1905, is more prevalent in the adult population than in the pediatric population. In our study we discuss two cases of pseudotumor of the orbit in children less than 18 months old. This report will highlight the evaluation and management of pediatric orbital pseudotumor and the importance of its inclusion in the differential diagnosis of orbital disorders in young children.- - - - - - - - - - ranking = 0.2keywords = inflammation (Clic here for more details about this article) |
10/315. Recurrent steroid-responsive trismus and painful ophthalmoplegia.A 63-year-old woman experienced two episodes of trismus and painful ophthalmoplegia at an interval of six years. She suffered left visual loss, and enhanced CT scan and MR imaging revealed heterogeneous enlargement of the left extraocular muscles extending to the orbital apex. In addition, the left pterygopalatine fossa was filled with a mass isointense with muscle without evidence of surrounding tissue invasion; 67Ga scintigraphy showed high uptake in this lesion. Steroid administration dramatically resolved the trismus, and the mass in the orbit and extraorbit vanished completely. orbital pseudotumor is characterized by self-limited, relapsing, steroid-responsive painful ophthalmoplegia, and this case could be a variant of this entity with inflammation extending into the extraorbital area.- - - - - - - - - - ranking = 0.2keywords = inflammation (Clic here for more details about this article) |
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