• Albert J Tahmoush, Mary S Amir, William W Connor, James K Farry, Sevastian Didato, Alice Ulhoa-Cintra, Jennifer M Vasas, Robert J Schwartzman, Harold L Israel, and Herbert Patrick.
• Department of Neurology, MCP Hahnemann University, Philadelphia, PA 19102, USA. albert.tahmoush@drexel.edu
• Sarcoidosis Vasc Dif. 2002 Oct 1; 19 (3): 191-7.

ObjectiveTo redefine the utility of CSF-ACE as a selective indicator of probable CNS neurosarcoidosis.MethodsThe diagnosis of probable CNS neurosarcoidosis required: (a) biopsy evidence of systemic sarcoidosis, (b) cortical, brainstem, and/or spinal cord deficits, (c) enhancing lesions on brain and/or spinal cord MRI, and (d) exclusion of other etiologies which could account for the neurological deficits. Radioassay measurement of CSF-ACE activity was performed in 11 patients who met our criteria for probable CNS neurosarcoidosis and 207 control patients.ResultsThe M +/- SD for CSF-ACE activity was significantly higher (p < 0.05) for the 11 probable CNS neurosarcoidosis patients (9.5 +/- 6.9 nmol/mL/min) than for the control patients (2.9 +/- 2.7 nmol/mL/min). The optimal CSF-ACE activity discriminator value was 8 nmol/mL/min. At this value, the sensitivity and specificity of CSF-ACE activity was 55% and 94%, respectively.ConclusionsCSF-ACE activity is a useful biochemical marker of probable CNS neurosarcoidosis when brain and/or spinal cord MRI show diffuse enhancing lesions.

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