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A 51-year-old female patient complained of mild frontotemporal headache of insidious-onset for two years. One year ago, she had an episode of focal, tonic-clonic seizures (with right lower limb paresthesia) and was prescribed carbamazepine. Cerebrospinal fluid demonstrated increased protein levels and intrathecal immunoglobulin (IgG) synthesis, suggesting an inflammatory component. Magnetic resonance imaging was performed (Figure 1). Sarcoidosis is a multisystem disease of unknown etiology characterized by noncaseating granulomatous inflammation. There is a genetic predisposition, with T-lymphocyte receptor activation by some unknown antigen. The disease affects preferentially the respiratory system. In the lungs, granulomas are observed in the interstitial compartment, showing a perilymphatic distribution along the peribronchovascular sheaths, interlobular septa and pleural surface. It is estimated that in about 5% to 15% of cases sarcoidosis affects the central nervous system. Rarely the patient presents with exclusively neurological manifestations like in the present case. Most commonly, neurosarcoidosis is observed in cases of disseminated disease. The clinical manifestations of neurosarcoidosis are pleomorphic. Cranial nerve compromise, visual alterations, headache, weakness, paresis, paresthesia, psychiatric alterations and signs of meningeal irritation may be observed. Although rare, sympextraluminal extension of the tumor. The prognosis is poor, with mean survival time of approximately one year and a half after symptoms onset. Due to pulmonary artery occlusion and acute symptoms, surgical resection is generally the treatment of choice. In conclusion, the present case reinforces the important role of the imaging methods in the differentiation between pulmonary artery intimal sarcoma and chronic PTE. The relevant aspects for this differentiation, such as contrast enhancement, distention of the affect vessels and extraluminal extension, allow for a correct diagnosis, avoiding delay in the required surgical approach.

Central nervous system involvement in sarcoidosis