1/251. Solitary sarcoid granuloma of the cerebellopontine angle: a case report.BACKGROUND: sarcoidosis involves the nervous system about 5% of the time and usually manifests as a granulomatous inflammation of the basal meninges and hypothalamus. Cases which are strictly isolated to the central nervous system occur infrequently; rarely, they may present as an intracranial mass. methods: We present the case of a solitary sarcoid granuloma at the cerebellopontine angle in a 42-year-old female who presented with headache, facial numbness, and hearing loss. RESULTS: A suboccipital craniectomy was performed and the lesion was noted to be grossly adherent to the lower cranial nerves and skull base. The lesion was misdiagnosed as a meningioma with preoperative magnetic resonance imaging and intraoperative histology, and perhaps additional morbidity resulted. CONCLUSION: We present this case in order to demonstrate the importance of differentiating these dural-based lesions and propose that cases of neurosarcoidosis presenting as a solitary granuloma be treated with surgical debulking and immunosuppression.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/251. Bilateral optic disk edema caused by sarcoidosis mimicking pseudotumor cerebri.PURPOSE: To present a case of retrobulbar optic nerve and chiasm sarcoidosis that mimicked pseudotumor cerebri. methods: A 34-year-old, thin, black woman presented with transient visual obscurations, normal visual acuity, bilateral optic disk edema, and enlarged blind spots. Clinical, medical, and radiologic evaluations were consistent with pseudotumor cerebri. The patient improved while taking acetazolamide, but 6 months later her symptoms worsened. neuroimaging disclosed enhancement of the optic nerve and chiasm. RESULTS: Despite administration of intravenous corticosteroids, the patient's vision worsened. Bilateral optic nerve sheath fenestrations were performed, and pathology disclosed sarcoidosis. CONCLUSION: sarcoidosis of the optic nerves and chiasm may mimic pseudotumor cerebri.- - - - - - - - - - ranking = 4keywords = nerve (Clic here for more details about this article) |
3/251. sarcoidosis with selective involvement of a second liver allograft: report of a case and review of the literature.A case of sarcoidosis recurrent in a patient's second liver allograft is described. There was no granulomatous disease seen in the patient's first liver allograft. After the second orthotopic liver transplantation (OLT), the patient was successfully treated for acute rejection, aspergillus infection, and cytomegalovirus viremia. Approximately 2 months after the second OLT, the patient was treated with long-term interferon-alpha for recurrent hepatitis c. Five years after the operation, he experienced liver failure secondary to recurrent hepatitis and underwent a third OLT. This is only the second reported case of sarcoidosis recurrent in the liver parenchyma of a transplanted organ and the first in which interferon-alpha might have played a role.- - - - - - - - - - ranking = 0.55311151855682keywords = organ (Clic here for more details about this article) |
4/251. The clinical management of sarcoidosis. A 50-year experience at the Johns Hopkins Hospital.sarcoidosis is an enigmatic disease with extremely variable manifestations in pattern, severity and course. Since Longcope and Freiman's descriptive monograph in 1952 (50) summarizing the clinical findings of the first half of this century, new dimensions of assessing the disease and treatment have been added. The impact of corticosteroids is central. The present review extends the studies to the second half of this century. Earlier diagnosis is facilitated and treatment often reverses many of the disease manifestations and improves the quality and extent of life for the patient. The management issues and guidelines outlined in this paper for both intrathoracic and extrathoracic disease are based on several longitudinal studies of the sarcoidosis patients summarized here, and 50 years of clinical experience by the senior author (CJJ) at Johns Hopkins Hospital, a tertiary referral center with an active Sarcoid Clinic. case reports are presented in the appendix. It is clear that corticosteroids are the most effective therapeutic agent for sarcoidosis, usually with impressive and prompt response. This represents the dramatic difference in this disease after 1950. No more specific or effective immunosuppressive or antiinflammatory agents have been identified. Undesirable side effects are minimal if excessive doses are avoided. The effectiveness of "steroid-sparing agents" such as methotrexate is uncertain. Although irreversible tissue damage from the disease may limit the effectiveness of treatment, benefits of corticosteroids greatly exceed the negative side effects. Since spontaneous remissions without treatment do occur, a period of observation of 2 years are more is warranted if the patient is relatively asymptomatic. Gradual radiographic progression for 2 or more years, even without major symptoms or reduction in pulmonary function, indicates the need for a trial of corticosteroid treatment, especially in white patients where symptoms may lag behind the radiographic changes. Relapses as treatment is withdrawn are frequent, especially in African-American patients, who tend to have more severe and more prolonged disease than white patients. A minimum of 1 year of treatment is recommended unless no improvement is noted after 3 months. Continued low-dose prednisone at daily doses of 10-15 mg is helpful in preventing relapses and further progression of disease. Periodic attempts at tapering are justified. Repeated relapses may indicate the need for life-long treatment. When irreversible changes are present, especially in the presence of chronic fibrotic disease, changing goals of treatment to provide optimal supportive care may represent better management than having unrealistic expectations from increased corticosteroid dosage or the addition of other potentially toxic immunosuppressive agents. Many agents related to sarcoidosis require further research. The most important question facing sarcoid researchers today is etiology. It is difficult to design specific therapy when the fundamental causes and disease mechanisms are not established. Rather than being a single disease with a single cause, it is possible that a number of genetic factors and environmental or infectious agents may result in an immune response that is manifested as sarcoidosis. Understanding basic causal mechanisms may help explain the varied disease manifestations and aid in designing curative treatments. Such etiologic questions should be explored from both a basic science and an epidemiologic approach. Therapeutic trials of new drugs such as pentoxyfylline and possibly thalidomide are needed to address their potential as well as limitations of steroid therapy. Finally, for patients who have progressed to organ failure, the problems of sarcoid recurrence in transplanted tissue, increased allograft rejection, and long-term prognosis of solid organ transplants have yet to be resolved. (ABSTRACT TRUNCATED)- - - - - - - - - - ranking = 1.1062230371136keywords = organ (Clic here for more details about this article) |
5/251. central nervous system sarcoidosis--diagnosis and management.A series of 68 patients with neurosarcoidosis is reported, with particular emphasis on clinical aspects, diagnosis and treatment. A classification system based on clinical diagnostic probability is proposed, consisting of probable and definite disease, the latter being dependent on finding sarcoid granulomas on nervous system histology, which was obtained in 12 patients (18%). The role of investigations, including magnetic resonance imaging (MRI), chest radiography, Kveim skin test, gallium 67 isotope scanning and cerebrospinal fluid (CSF) studies, is considered. Sixty-two percent of patients presented with nervous system disease, most commonly affecting the optic nerve and chiasm. Other common presentations included cranial nerve palsies, spinal cord and brainstem manifestations. Investigations yielding most diagnostic information included the kveim test (41/48, 85% positive), raised CSF protein and/or cells (50/62, 81%) and gallium 67 scan (14/31, 45%). Eleven out of 29 patients (38%) patients showed meningeal enhancement on MRI scanning and 43% of scans demonstrated multiple white-matter lesions. Mean follow-up for the group was 4.6 years. Forty-seven patients were seen for > 18 months, and over half of these patients progressed despite corticosteroid and other immunosuppressive therapies. The benefit of a large patient database prospectively studied, with extended follow-up is discussed in order to learn more about prognosis and advance therapy in neurosarcoidosis.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
6/251. polyradiculopathy in sarcoidosis.We present three new and 14 retrospective cases of polyradiculopathy in sarcoidosis. Of these, 71% had weakness and 59% areflexia of the lower extremities, and 35% had sphincter dysfunction. Cases often were associated with central nervous system sarcoidosis. All cases involved thoracolumbar or lumbosacral roots, except a single case of cervical polyradiculopathy. Of 14 treated patients, nine improved with corticosteroids, laminectomy, or both. polyradiculopathy complicating sarcoidosis: (1) is uncommon; (2) primarily involves thoracic and lumbar roots; (3) may arise from contiguous, hematogenous, or gravitational nerve root sleeve seeding; (4) may be asymptomatic; and (5) may improve with corticosteroids. Differential diagnosis of weakness in patients with sarcoidosis should include nerve root involvement from the primary process by direct sarcoid involvement.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
7/251. Sarcoid-like pulmonary disorder in human immunodeficiency virus-infected patients receiving antiretroviral therapy.We report two cases of hiv-infected patients who presented with diffuse interstitial micronodular lesions on chest X-ray after institution of protease inhibitor-containing highly active antiretroviral therapy (HAART). Granulomatous pulmonary disorder mimicking sarcoidosis was diagnosed on histopathological studies revealing noncaseating granuloma and bronchoalveolar lavage analysis showing an intense CD4( ) lymphocyte alveolitis. Causative agents such as infectious organisms and environmental compounds were excluded. The relationship between sarcoid-like reaction and immune reconstitution under HAART is discussed.- - - - - - - - - - ranking = 0.55311151855682keywords = organ (Clic here for more details about this article) |
8/251. Membranous nephropathy associated with childhood sarcoidosis.sarcoidosis is a chronic multisystemic granulomatous disease of unknown etiology. It is relatively rare in children. Renal involvement in sarcoidosis is described less commonly than other organ involvement such as pulmonary, eye, musculoskeletal, and skin. We report a 13-year-old girl with sarcoidosis and nephrotic syndrome. Renal biopsy showed findings of membranous nephropathy. She received intravenous pulse methylprednisolone and oral cyclophosphamide with resolution of the symptoms of fever and edema, and improvement of the proteinuria. Her condition is stable with no progression of her renal disease. To the best of our knowledge, this is the first report of membranous nephropathy associated with childhood sarcoidosis.- - - - - - - - - - ranking = 0.55311151855682keywords = organ (Clic here for more details about this article) |
9/251. Whipple's disease presenting as sarcoidosis and valvular heart disease.Whipple's disease is a rare systemic disorder that can present in a variety of ways and is often difficult to diagnose. It can involve almost any organ system and can mimic other diseases both in its symptoms and pathologic appearance. We present a case initially diagnosed as sarcoidosis that was found to be Whipple's disease after pathologic examination of the patient's mitral and aortic valves.- - - - - - - - - - ranking = 0.55311151855682keywords = organ (Clic here for more details about this article) |
10/251. Nodular sarcoid myositis of skeletal muscle diagnosed by fine needle aspiration biopsy. A case report.BACKGROUND: Symptomatic striated muscle involvement in sarcoidosis is rare. Muscle biopsy is usually required for the diagnosis. Fine needle aspiration biopsy (FNAB) has been successfully used in diagnosing soft tissue lesions. To the best of our knowledge, FNAB of sarcoid myositis has not been reported. CASE: A 31-year-old, black female with a history of sarcoidosis presented with an enlarging, painful, left calf mass. Infected thrombi were suspected. FNAB showed numerous loosely arranged epithelioid histiocytes, multinucleated giant cells and skeletal muscle cells. The overall cytologic picture was that of granulomatous myositis. The cytologic features coupled with the patient's history and magnetic resonance imaging findings suggested sarcoid myositis. Subsequent muscle biopsy showing noncaseating granulomata and negative stains for organisms confirmed the diagnosis of nodular sarcoid myositis. CONCLUSION: Nodular sarcoid myositis can be suggested by FNAB cytology in a patient with a past history of sarcoidosis.- - - - - - - - - - ranking = 0.55311151855682keywords = organ (Clic here for more details about this article) |
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