CSF-ACE activity in probable CNS neurosarcoidosis

Sarcoidosis Vasc Diffuse Lung Dis. 2002 Oct;19(3):191-7.


Objective: To redefine the utility of CSF-ACE as a selective indicator of probable CNS neurosarcoidosis.

Methods: The 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.

Results: The 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.

Conclusions: CSF-ACE activity is a useful biochemical marker of probable CNS neurosarcoidosis when brain and/or spinal cord MRI show diffuse enhancing lesions.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Aged
  • Biomarkers / cerebrospinal fluid
  • Central Nervous System / pathology
  • Central Nervous System Diseases / cerebrospinal fluid
  • Central Nervous System Diseases / diagnosis*
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Peptidyl-Dipeptidase A / cerebrospinal fluid*
  • Retrospective Studies
  • Sarcoidosis / cerebrospinal fluid
  • Sarcoidosis / diagnosis*
  • Sensitivity and Specificity


  • Biomarkers
  • Peptidyl-Dipeptidase A