1/98. The pitfall of silent neurosarcoidosis.sarcoidosis in childhood is seldom reported. Most cases are observed in older children and preadolescents as bilateral pulmonary disease and eye lesions. Arthritic features are more likely to be observed in infants and children younger than 4 years of age who do not develop pulmonary disease. Neurosarcoidosis is exceptional in this age group and seldom suspected when the neurologic symptoms are present. The authors report a pediatric patient with systemic sarcoidosis who developed a severe but silent neurologic involvement. Numerous masslike lesions were discovered on systematic cranial magnetic resonance imaging. The authors recommend a complete screening of extrapulmonary manifestations in children with sarcoidosis. The proper management of patients with incidentally discovered neurosarcoidosis has yet to be established.- - - - - - - - - - ranking = 1keywords = sarcoid, sarcoidosis (Clic here for more details about this article) |
2/98. Radiation therapy for neurosarcoidosis: report of three cases from a single institution.sarcoidosis is a chronic, multisystemic disorder of unknown etiology. The incidence of central nervous system involvement is as high as 5%. Although steroids have been the cardinal treatment for sarcoidosis, many patients become symptomatically unresponsive to them. Other patients may suffer from glucose intolerance, cataracts, and obesity, which are adverse effects of high-dose steroids. Various reports in the literature suggest that some chemotherapeutic agents and/or radiation may be useful in these situations. We present three patients with neurosarcoidosis who were treated with radiation at a single institution. We also review previous reports on radiation-treated neurosarcoid patients. While the results vary, some patients clearly derive symptomatic benefits from low-dose radiation. Since the side effects of low-dose cranial irradiation are minimal, it may be prudent to use radiation therapy for patients who are refractory to steroids or who suffer adversely from high-dose steroids.- - - - - - - - - - ranking = 0.81717545248944keywords = sarcoid, sarcoidosis (Clic here for more details about this article) |
3/98. Cerebral herniation after lumbar puncture in sarcoid meningitis.A patient with chronic meningitis due to neurosarcoidosis became comatose within minutes of a lumbar puncture and died 24 h later. The diagnosis of neurosarcoidosis was made post mortem. Development of cerebral herniation may have been exacerbated by lumbar puncture. It was proposed that arachnoid villi dysfunction may have contributed to very high intracranial pressures in this patient, since post mortem examination revealed communication between the ventricles and outlet foramina of the fourth ventricle, and that herniation was in part due to an acute pressure differential caused by lumbar puncture.- - - - - - - - - - ranking = 0.51870180995775keywords = sarcoid, sarcoidosis (Clic here for more details about this article) |
4/98. Five cases with central diabetes insipidus and hypogonadism as first presentation of neurosarcoidosis.OBJECTIVES: We retrospectively reviewed 5 patients with neurosarcoidosis, who all presented with central diabetes insipidus and hypogonadism. DESIGN: This was a single-centre, retrospective analysis of 5 cases with a minimum follow-up of 2 years. methods: Case analysis included clinical, biochemical, and endocrinological evaluation and frequent CT/MRI scans of involved organs as primary evaluation and in response to immunosuppressive therapy. RESULT: Neurosarcoidosis was diagnosed in all patients. Two patients had no proven extracerebral manifestation and had a stable disease over 3 and 5 years. One patient showed deterioration with corticosteroids alone but partial remission after additional cyclophosphamide. Pituitary dysfunction remained unchanged in all patients, despite total clinical and radiological remission in two patients. However, one of these patients died of acute granulomatous meningoencephalitis after two years of follow-up. CONCLUSION: Although the presenting symptoms of neurosarcoidosis may vary, the occurrence of central diabetes insipidus associated with typical radiological features is suggestive of neurosarcoidosis. However, there is an increasing number of case reports on lymphocytic hypophysitis. Without the bioptic diagnosis, the differentiation between potentially lethal isolated neurosarcoidosis and lymphocytic hypophysitis is difficult. These cases demonstrate the difficulties in diagnosing neurosarcoidosis and reflect experiences with follow-up parameters.- - - - - - - - - - ranking = 1.25keywords = sarcoid, sarcoidosis (Clic here for more details about this article) |
5/98. A vascular sarcoid mass mimicking a convexity meningioma.We report a 48-year-old woman with a left posterior temporal extra-axial mass that had the imaging characteristics of a meningioma on preoperative CT, MRI and angiography. However, a biopsy diagnosis of sarcoidosis was made. This case illustrates that dural-based sarcoid masses can be very vascular and radiographically indistinguishable from meningiomas. Characteristic imaging features of extra- and intra-axial sarcoid lesions are discussed.- - - - - - - - - - ranking = 0.52805271493663keywords = sarcoid, sarcoidosis (Clic here for more details about this article) |
6/98. Micturitional disturbance in a patient with neurosarcoidosis.We report a case of neurosarcoidosis in which urodynamic studies showed neurogenic bladder dysfunction. A 30-year-old man began to have slowly progressive gait ataxia of vestibular origin, deafness, and hallucination, which developed into versive seizure and stupor. brain computed tomography and magnetic resonance imaging showed the anteromedial frontal lobe lesion with mild ventricular enlargement. The cerebrospinal fluid examination revealed pleocytosis with raised total protein and angiotensin-converting enzyme levels. Endoscopic lung biopsy showed epithelioid granuloma. Oral prednislone (60 mg/day) ameliorated his symptoms. After tapering steroids, however, he developed urinary urgency, frequency, urge urinary incontinence, and a relapse of gait ataxia. The urodynamic study showed detrusor hyperreflexia. prednisolone treatment again improved his urinary and neurological symptoms. The anteromedial frontal lobe lesion seems to be responsible for the micturitional disturbance in our patient with neurosarcoidosis.- - - - - - - - - - ranking = 0.75keywords = sarcoid, sarcoidosis (Clic here for more details about this article) |
7/98. Necrotizing sarcoid granulomatosis mimicking an intracranial neoplasm: clinicopathologic features and review of the literature.We present a unique case of biopsy-proven necrotizing sarcoidosis involving the central nervous system (CNS) in a 52-year-old woman. The patient presented with a 3-month history of left-sided headache and sharp, shooting pains on the left side of her face. She also has a previous history of sarcoidosis, histopathologically confirmed on parotid gland biopsy 24 years before. Imaging studies of the present lesion revealed a 1.8 x 1.4-cm mass in the left temporal lobe with signal intensity suggestive of meningioma or low-grade glial neoplasm. Surgical resection was initiated, and intraoperative consultation with frozen sections revealed granulomata. The lesion was biopsied, and surgical intervention was terminated. Permanent sections failed to reveal bacteria, mycobacteria, fungi, or foreign bodies. A diagnosis of necrotizing neurosarcoidosis was rendered. The patient was administered steroid therapy and clinically responded favorably. At the most recent follow-up almost 2 years later, there was no evidence of recurrence or progression. Necrotizing sarcoidosis has been reported most commonly in the lungs and rarely in other organ systems. We report the first histologically proven case involving the CNS as well as a rare example of sarcoidosis and necrotizing sarcoid granulomatosis in the same patient. sarcoidosis and its necrotizing variant should be considered in the differential diagnosis of a granulomatous mass lesion involving the CNS, particularly in the context of a history of systemic disease.- - - - - - - - - - ranking = 0.96087726244719keywords = sarcoid, sarcoidosis (Clic here for more details about this article) |
8/98. susac syndrome: microangiopathy of the retina, cochlea and brain.BACKGROUND: susac syndrome is characterized by the triad of branch retinal arterial occlusions, encephalopathy and cochlear microangiopathy. The underlying process is believed to be a small vessel vasculitis causing microinfarcts in the retina, brain and cochlea. methods: Analysis of two male and two female cases of susac syndrome recognized in australia. RESULTS: In this series the epidemiology, mode of presentation, ophthalmologic features, neurologic and cochleo-vestibular features, radiologic characteristics, cerebrospinal fluid findings, therapeutic interventions, clinical course and outcome of susac syndrome is examined. Key ophthalmologic differential diagnoses include systemic lupus erythematosis (SLE), Behcet's syndrome and other vasculitides such as sarcoidosis, tuberculosis, syphilis and lymphoma. Neuro-otologic features are most frequently misdiagnosed as multiple sclerosis. CONCLUSION: susac syndrome, first described in 1979, is becoming an increasingly recognized condition. Early recognition of the syndrome is important because treatment with systemic immunosuppression may minimize permanent cognitive, audiologic and visual sequelae.- - - - - - - - - - ranking = 0.125keywords = sarcoid, sarcoidosis (Clic here for more details about this article) |
9/98. Unusual clincal presentation of cerebral-isolated sarcoidosis. Case report and review of the literature.The authors report the case of a 31-year-old man, who presented an hemorrhagic onset of a cerebral-isolated sarcoidosis, to date never described in the literature. Unusual clinical manifestation, neuroradiological study and direct histological confirmation are presented. Moreover, gallium-67 Single Photon Emission Computed tomography (Ga-67 SPECT) contribution is indicated to complete the evaluation of patients with cerebral inflammatory lesions, such as neurosarcoidosis, and in cases of equivocal neuroradiological imaging, to evaluate uptake activity of the inflammatory tissue. The patient did well and reported no further progression of his disease during 7 months of follow-up.- - - - - - - - - - ranking = 0.75keywords = sarcoid, sarcoidosis (Clic here for more details about this article) |
10/98. Central diabetes insipidus as the first manifestation of neurosarcoidosis in a 10-year-old girl.Central diabetes insipidus with thickening of the pituitary stalk has been demonstrated in adults with Langerhans cell histiocytosis (LCH), tuberculosis or sarcoidosis, and in children with LCH. We present a 10.5-year-old girl with central diabetes insipidus. magnetic resonance imaging (MRI) revealed thickening of the central part of the pituitary stalk and multiple hyperintense lesions in the frontal white matter on T(2)-weighted images. Laboratory findings were normal except for an elevated serum angiotensin-converting enzyme (ACE) level. The MRI findings together with the elevated serum ACE level highly suggest the diagnosis of sarcoidosis. We conclude that central diabetes insipidus can be the first clinical manifestation of sarcoidosis in children.- - - - - - - - - - ranking = 0.875keywords = sarcoid, sarcoidosis (Clic here for more details about this article) |
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