Isolated neurosarcoidosis - MR findings and pathologic correlation: A case report

Department of Radiology, Keski-Pohjanmaa Central Hospital, Kokkola, Finland.
Acta Radiologica (Impact Factor: 1.6). 12/2001; 42(6):563-7. DOI: 10.1080/028418501127347386
Source: PubMed


Neurosarcoidosis is a diagnostic challenge, especially if systemic symptoms are absent. We present a 49-year-old woman with isolated neurosarcoidosis. The main symptom was loss of vision in the left eye. Brain MR imaging showed 6 high-signal white matter lesions frontotemporally on proton density and T2-weighted turbo spin-echo images. Coronal fat-saturated turbo FLAIR images of the orbits showed a swollen left optic nerve with increased signal intensity, which finding has not been previously published in sarcoid optic neuropathy. A control MR examination showed meningeal enhancement of the left optic nerve and leptomeningeal enhancing lesions around the brain stem. Spinal MR revealed leptomeningeal enhancement throughout the spinal cord and asymptomatic enhancing cauda equina lesions, mimicking subarachnoid tumour seeding, and an enhancing nerve root mass at Th12/L1. Biopsy of the latter lesion revealed non-caseating granulomas consistent with sarcoidosis.

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    ABSTRACT: Sarcoidosis is now recognised as a member of a large family of granulomatous disorders and has been reported from all parts of the world. Current evidence points to genetic predisposition and exposure to yet unknown transmissible agent(s) and/or environmental factors as etiological agents. Depending on the organ systems involved, patients with sarcoidosis present themselves to various specialities; pulmonary involvement is by far the commonest and occurs in about 90 per cent of the patients. The diagnosis is based on a compatible clinical and/or radiological picture, histopathological evidence of non-caseating granulomas in tissue biopsy specimens and exclusion of other diseases capable of producing similar clinical or histopathological appearances. Sarcoidosis is an underdiagnosed disease in India. However, owing to the increasing awareness, it is being diagnosed more frequently than a few decades ago. Most Indian patients with sarcoidosis are males and present in the fourth or fifth decade of life. The disease runs a benign course with spontaneous remission of the activity though some degree of residual pulmonary function abnormality persists. Only a minority of the patients develop complicated disease. Corticosteroids remain the mainstay of treatment. For asymptomatic patients with pulmonary sarcoidosis, no therapy is required. Corticosteroid treatment should be considered in symptomatic patients with evidence of radiological or pulmonary function deterioration. Treatment under close clinical monitoring should be tailored to suit the needs of the individual patient. Sometimes steroid-sparing alternative treatments may be beneficial. In this review, the current understanding of the global scenario and Indian perspective of sarcoidosis are critically reviewed.
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    ABSTRACT: Sarcoidosis is a systemic disorder of unknown cause with a wide variety of clinical and radiologic manifestations. The diagnosis is usually made on the basis of these manifestations supported by histologic findings. Systemic manifestations (eg, Löfgren syndrome, Heerfordt syndrome) are commonly seen at clinical examination. Bilateral hilar lymphadenopathy is the most common radiologic finding-frequently with associated pulmonary infiltrates-and typically has a characteristic perivascular distribution at high-resolution chest computed tomography. Radiologic findings in the short tubular bones of the hands and feet and magnetic resonance imaging findings of nodular involvement of muscle are often sufficient to raise suspicion for sarcoidosis. In the liver, spleen, kidneys, and scrotum, coalescing granulomas form nodules whose imaging features may occasionally be nonspecific, although familiarity with the relevant clinical settings will be helpful in recognizing the presence of sarcoidosis. Radiologic recognition of cardiac and central nervous system involvement is also important because patients may be only mildly symptomatic. The clinical course and prognosis of sarcoidosis are highly variable, often correlating with the mode of onset. Familiarity with the clinical and radiologic features of sarcoidosis in various anatomic locations plays a crucial role in diagnosis and management.
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