1/28. Distensible venous malformations of the orbit: clinical and hemodynamic features and a new technique of management.OBJECTIVE: To investigate distensible venous malformations of the orbit (DVMO) as part of a spectrum of orbital vascular malformations, including some that involved periorbital skin, extraorbital sites (central nervous system or nasal sinuses), or combinations of these. The authors also investigated the effectiveness of a new technique of management for selected cases. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Thirty patients had distensible venous anomalies, of which four were combined distensible venous-lymphatic vascular malformations. Distensible lesions were defined as those showing clinical or radiographic expansion with valsalva maneuver or when the head was placed in a dependent position. These lesions were then classified as superficial (anterior to the equator of the globe), deep (posterior to the globe's equator), combined (deep and superficial), or complex (with intracranial or major extraorbital involvement). INTERVENTION: Surgery was performed on 15 patients (50%), mainly for pain or for cosmetic indications. Six patients underwent this new technique, which involved intraoperative direct venography with control of outflow via pressure at the superior or inferior orbital fissure. The venous malformation was then embolized (by use of cyanoacrylate glue) and excised. RESULTS: The mean age at presentation was 28.2 years (range, 8 months to 75 years). Sixty-six percent of cases involved the left orbit. Superior and medial orbital involvement was most common. Three cases (10%) were classified as superficial, and 13 (43%) as deep. Six patients (20%) had combined superficial and deep components. Eight (27%) had major extraorbital involvement (4 intracranial, 2 facial, and 2 paranasal sinus). Direct venography demonstrated complex multichannel anomalies draining to various sites, including the face and pterygopalatine fossa, without necessarily having a direct connection to the major orbital venous circulation. CONCLUSIONS: Distensible venous malformations of the orbit are part of a spectrum of developmental venous malformations that may be localized to the orbit or involve it as part of a more extensive lesion. The authors describe their clinical and radiologic features and report a new technique of management for selected cases. This method of vascular isolation and embolization of lesions may greatly facilitate excision.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/28. Primary sjogren's syndrome with severe central nervous system disease.OBJECTIVE: central nervous system (CNS) involvement in primary sjogren's syndrome (pSS) is controversial with regard to frequency, significance, and etiology. methods: We describe a young woman with pSS and severe CNS disease and review the literature on the pathophysiology, clinical significance, symptoms, diagnostic examinations, and treatment of CNS disease with concomitant pSS (CNS-SS). RESULTS: Our patient with pSS had a 5-month history of benign lymphadenopathy and myositis, after which she developed severe CNS disease, vasculitic lesions on her hands, and a neurogenic bladder attributable to spinal cord involvement. The diagnosis was based on the clinical picture and the results of a brain magnetic resonance imaging (MRI) scan, electroencephalography (EEG), and cerebrospinal fluid (CSF) analysis. The disease did not respond to corticosteroids, but the administration of cyclophosphamide resulted in recovery. In the literature, the incidence of CNS-SS varies widely, from rare to incidence rates of 20% to 25%. The clinical picture is diverse, ranging from mild cognitive symptoms to fatal cerebrovascular accidents. The pathophysiology of CNS-SS is unclear, specific diagnostic methods are not available, and diagnosis is based on the clinical picture and a combination of examinations. MRI is the most sensitive test and cerebral angiography the most specific. CSF reflects involvement of the leptomeninges, and EEG is nonspecific. There are no controlled studies of the treatment of CNS-SS. Regimens for vasculitis are commonly used. CONCLUSIONS: CNS-SS is uncommonly recognized and difficult to diagnose. Increasingly accurate and available diagnostic examinations will yield more information about the association of CNS disease with pSS.- - - - - - - - - - ranking = 4.3114041750498keywords = central nervous system, nervous system, cerebral (Clic here for more details about this article) |
3/28. Cerebral tuberculomas in northern ireland.Two children presenting with very different clinical pictures were both found to have intracranial tuberculomas. This condition, although rare in developed countries, should be suspected in any child with enhancing cerebral lesions or cranial computed tomography.- - - - - - - - - - ranking = 0.081788553503265keywords = cerebral (Clic here for more details about this article) |
4/28. Langerhans cell histiocytosis infiltration in cerebrospinal fluid: a case report.Langerhans cell histiocytosis (LCH) is a disease of unknown etiology characterized by a proliferation of histiocytic cells resembling the integumentary cells bearing the name of langerhans cells. LCH can be unifocal or multifocal, with one- or many-organ involvement. We present a case of LCH diagnosed in the cerebrospinal fluid of a patient with generalized lymphadenopathy and central nervous system involvement.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/28. Massive acute thymic haemorrhage and cerebral haemorrhage in an intrauterine fetal death.Massive acute thymic haemorrhage in the neonate occurs extremely rarely and is associated with haemorrhagic disease of the newborn. A 30 year old woman with an unremarkable previous obstetric history presenting at 37 weeks and 4 days gestation with the complaint of loss of fetal movement was found to have a male fetus with findings at necropsy of massive acute thymic haemorrhage, acute intracranial haemorrhage, and hydrops fetalis. This is the first report of massive acute thymic haemorrhage in utero. Massive thymic haemorrhage should be added to the reported causes of lethal non-immunological hydrops.- - - - - - - - - - ranking = 0.32715421401306keywords = cerebral (Clic here for more details about this article) |
6/28. Primary diagnosis of whipple disease manifesting as lymphadenopathy: use of polymerase chain reaction for detection of tropheryma whippelii.whipple disease is a rare, chronic multisystem disease associated with the recently characterized organism tropheryma whippelii. Extraintestinal manifestation involving the central nervous system, heart, and joints occasionally occurs. Involvement of the abdominal lymph nodes, especially the mesenteric and periaortic nodes, is not uncommon. However, peripheral lymphadenopathy as the sole clinical manifestation of whipple disease is rare. We describe 2 patients with whipple disease whose initial manifestation was lymphadenopathy. lymph nodes from both patients showed infiltration of the sinuses by macrophages containing periodic acid-Schiff-positive, diastase-resistant, sickle-like structures. Electron microscopic evaluation confirmed the presence of rod-like organisms. dna from each sample was amplified by the polymerase chain reaction using a specific set of oligonucleotide primers developed against the 16S ribosomal rna coding sequence of T. whippelii. The histopathologic features and differential diagnosis of lipogranulomatous lymphadenopathy secondary to whipple disease, as well as use of molecular-based assays, are discussed.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/28. Chronic lymphadenopathic toxoplasmosis. A case with marked hyperglobulinemia and impaired delayed hypersensitivity responses during active infection.A patient with lymphadenopathic toxoplasmosis characterized by prolonged symptoms and repeated relapses with isolation of toxoplasma from lymph nodes is described. As the disease persisted and progressed, striking immunologic changes occurred that ultimately resulted in a state of extreme hyperglobulinemia associated with impaired delayed hypersensitivity responses. The case in question illustrates that progressive infection may occur in the face of high antibody levels of all immunoglobulin types whereas the only demonstrable immunologic impairment was of delayed hypersensitivity.- - - - - - - - - - ranking = 4368.2604573731keywords = toxoplasmosis (Clic here for more details about this article) |
8/28. Ocular toxoplasmosis in an adult receiving long-term corticosteroid therapy.Sudden death of a 58-year-old woman who developed ocular toxoplasmosis while receiving long-term systemic corticosteroid therapy permitted correlation of early histopathologic lesions with their clinical counterpart recorded on fundus photographs. A wide-spread, paravascular, whitish, retinal opacification dominated the initial clinical picture. These lesions were represented histologically by focal zones of inner retinal necrosis located adjacent to arteries and veins. No associated inflammatory cell infiltrate was present, and numerous viable free toxoplasma organisms were consistently present at the interface between necrotic and healthy retina. Electron microscopy of formaldehyde-fixed tissue provided a useful technique for confirming the identity of the infecting organism in the absence of serologic or culture data.- - - - - - - - - - ranking = 4368.2604573731keywords = toxoplasmosis (Clic here for more details about this article) |
9/28. Whipple's disease with destructive arthritis, abdominal lymphadenopathy, and central nervous system involvement.We describe a patient with Whipple's disease who had an unusual erosive and destructive polyarthritis, massive abdominal lymphadenopathy, asymptomatic central nervous system involvement, and rare manifestations of orbital pseudotumor and orchitis with epididymitis. Taking oral therapy with trimethoprim-sulfamethoxazole he had recurrent flares of orbital pseudotumor, an episode of orchitis with epididymitis, and persistent polymerase chain reaction T. whipplei-positive cerebrospinal fluid. Resolution was achieved with a one month course of intravenous ceftriaxone and a 6 month course of azithromycin, and no relapse occurred during 24 months of followup.- - - - - - - - - - ranking = 5keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/28. 2-Deoxy-2-[18F]fluoro-D-glucose positron emission tomography uptake in systemic lupus erythematosus-associated adenopathy.Systemic lupus erythematosus (SLE) and lymphoma are disease entities that often have similar presenting signs and symptoms that can complicate or delay definitive diagnosis. 2-Deoxy-2-[(18)F]fluoro-D-glucose positron emission tomography (FDG-PET) has become a valuable tool in the diagnosis, staging, and evaluation of response to therapy in lymphoma patients. However, its utility in patients with SLE has been limited to the central nervous system. Significant FDG uptake has not been previously reported in lymphadenopathy associated with SLE. The case presented is an example of histologically proven benign adenopathy in a 16-year-old female with SLE that was hypermetabolic on FDG-PET imaging. It highlights the importance of recognizing that widespread inflammatory adenopathy in SLE can mimic the pattern of FDG uptake seen with lymphoma at PET imaging.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
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