Cases reported "Sarcoidosis"

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1/75. 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.
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2/75. 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.
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3/75. 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.
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4/75. 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.
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5/75. The optic pathway in neurosarcoidosis.

    A case is reported of a patient, age 40 yr, with progressive hypothalamic and visual failure. autopsy revealed widespread multisystemic sarcoidosis with involvement of the central nervous system. The intracranial portion of the optic pathway was diffusely infiltrated by sarcoid granulomas. Comparison of this case with previously reported autopsy and biopsy findings leads to the following conclusions: 1) The intraocular, intraorbital, and intracranial parts of the optic nerves may be involved independently; 2) lesions of the nerve head and of the retrobulbar part of the nerve may be unilateral; and 3) involvement of the intracranial portion is usually bilateral and forms part of more widespread sarcoidosis of the central nervous system.
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6/75. 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.
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7/75. Evolution of sarcoid granulomas of the retina.

    PURPOSE: To report a case of a young woman with a history of sarcoidosis who developed retinal granulomas. methods: Case report. RESULTS: A 33-year old woman with history of sarcoidosis with involvement of the central nervous system, confirmed by skin biopsy, bronchoscopy, and neuroimaging, presented with visual loss and was found to have choroidal and optic nerve granulomas in the left eye, and subsequently developed retinal granulomas in the left eye. CONCLUSION: Retinal granuloma is a rare manifestation of sarcoidosis.
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8/75. Mammary involvement in a patient with sarcoidosis.

    sarcoidosis is a systemic granulomatous disease of unknown etiology. sarcoidosis affects multiple organs; the lungs, lymphoid system, eyes and skin are usually involved. Commonly, any organ system may be affected. Sarcoid involvement in the nervous system, locomotor system, lacrimal and salivary glands, heart, kidney and liver has been recognized in patients with sarcoidosis. However, sarcoid involvement of the breast parenchyma has been extremely rare in patients with sarcoidosis. Herein, we report a patient with a sarcoid breast mass who had bilateral hilar lymphadenopathy and lymphocytosis in bronchoalveolar lavage analysis.
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9/75. amenorrhea-galactorrhea syndrome as an uncommon manifestation of isolated neurosarcoidosis.

    The involvement of the hypothalamic and/or the pituitary gland during granulomatous, infiltrative or autoimmune diseases is a rare condition of acquired hypothalamic dysfunction and non-tumoral-non-vascular hypopituitarism. sarcoidosis is a pathogen-free granulomatous disease which affects both central and peripheral nervous systems in 5-16% of patients. In most cases, nervous involvement by sarcoidosis occurs within a multi-systemic disease, although disease localization limited to the nervous system has also been reported. We observed a 26-year-old Italian woman presenting with low-grade fever, headache, visual disturbances, amenorrhea-galactorrhea syndrome and pituitary failure due to an infiltrative lesion involving the hypothalamus and the pituitary stalk. At first, the diagnosis of "probable" neurosarcoidosis was made according to the clinical picture, magnetic resonance imaging, and the study of cerebrospinal fluid lymphocyte sub-populations. In this case, hyperprolactinemic amenorrhea and galactorrhea combined with blunted responses of gonadotropins to luteinizing hormone releasing hormone and acquired growth hormone failure were to be considered as an infrequent manifestation of neurosarcoidosis limited to the medio-basal brain, without other disease localization. Forty-eight months after disease onset, the occurrence of mediastinal lymph node enlargement and increase of angiotensin converting enzyme in serum allowed us to confirm the diagnosis of sarcoidosis. Neurosarcoidosis is often hard to diagnose, especially when the neurological localization of the disease is not accompanied by other possible systemic signs, and when the lesion is too deep to obtain biopsy confirmation. The study of cerebrospinal fluid and blood lymphocyte sub-populations integrated by magnetic resonance imaging may be helpful for a correct diagnosis.
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10/75. Outlet obstruction of the fourth ventricle in sarcoidosis.

    central nervous system sarcoidosis and its varied neuroradiological manifestations are reviewed. Two cases of mechanical obstruction secondary to sarcoid granulomata at the outlet of the fourth ventricle are described.
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