Diagnoses associated with elevated angiotensin-converting enzyme in cerebrospinal fluid
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Athena Dao1, Andrew Liang1, Ryan Walsh1
1Department of Neurology, Medical College of Wisconsin, 9200 West Wisconsin Avenue, Milwaukee, WI 53226, United States.
Background:
Cerebrospinal fluid (CSF) angiotensin-converting enzyme (ACE) is commonly tested when neurosarcoidosis is suspected. However, diagnostic utility is debatable. CSF ACE elevation has been reported with other conditions, but has not been well-studied. We aimed to systematically investigate what diagnoses are associated with elevated CSF ACE.
Methods:
This was a single-center retrospective review of adult patients who underwent CSF ACE testing. IRB-approved institutional analytic tools identified patients and electronic medical records were manually reviewed. Patients with elevated CSF ACE (>2.5 U/L) underwent additional data extraction, and relevant diagnoses categorized.
Results:
Total of 1186 patients underwent CSF ACE testing with elevation in 66 (median level: 4.4 U/L, range 2.6-40.0 U/L). Forty (60.6%) female, 26 (39.4%) male, mean age 55.7 ± 14.2. Diagnostic categories included: meningitis (n = 13), central nervous system (CNS) tumors (n = 10), miscellaneous neurologic (n = 10), neurosarcoidosis (n = 9; 8 probable, 1 possible), structural pathology (n = 7), neuromuscular (n = 7), unclassified (n = 7; 5 incomplete workup, 2 lost to follow-up), CNS demyelinating diseases (n = 6), idiopathic meningeal enhancement (n = 4). Six noted to be taking neuroleptic medications. Twenty-four (36.4%) demonstrated meningeal enhancement on magnetic resonance imaging (10 leptomeningeal, 10 pachymeningeal, 4 both).
Conclusions:
Elevated CSF ACE was found in association with a variety of neurologic diagnoses, more often with meningitis (19.7%) or CNS tumors (15.2%) than neurosarcoidosis (13.6%), illustrating this biomarker is not unique to neurosarcoidosis. Furthermore, meningeal enhancement was a relatively common finding (36.4%), indicating CSF ACE may be more associated with meningeal pathology. Our findings can help clinicians with differential diagnostic considerations when CSF ACE is elevated.
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