Hereditary elevation of angiotensin converting enzyme suggesting neurosarcoidosis

Abstract

We report a 49-year-old woman with arterial hypertension, transitory neurologic symptoms, and suspected neurosarcoidosis based on elevated plasma and CSF angiotensin converting enzyme (ACE) levels. Sarcoidosis is a multisystem disease of unknown etiology, histopathologically characterized by the formation of noncaseating granulomas. CNS involvement occurs in about 5% of patients, and neurosarcoidosis can present without systemic manifestation.1,2⇓ The most common clinical manifestations of neurosarcoidosis are cranial nerve palsies and CNS parenchymal disease.1,2⇓ MRI typically reveals diffuse meningeal involvement, mass lesions, or diffuse white matter lesions.1,2⇓ Plasma levels of ACE (enzyme commission 3.4.15.1) are increased in 70 to 80% of patients with systemic sarcoidosis. In patients with neurosarcoidosis, plasma ACE levels are increased in about 24%, and …