Version 1
: Received: 22 July 2023 / Approved: 25 July 2023 / Online: 26 July 2023 (10:37:24 CEST)
How to cite:
Chamoun, V.; Thompson, E.; Giovannoni, G. New Evidence-Based Diagnostic Criteria for Neurosarcoidosis. Preprints2023, 2023071760. https://doi.org/10.20944/preprints202307.1760.v1
Chamoun, V.; Thompson, E.; Giovannoni, G. New Evidence-Based Diagnostic Criteria for Neurosarcoidosis. Preprints 2023, 2023071760. https://doi.org/10.20944/preprints202307.1760.v1
Chamoun, V.; Thompson, E.; Giovannoni, G. New Evidence-Based Diagnostic Criteria for Neurosarcoidosis. Preprints2023, 2023071760. https://doi.org/10.20944/preprints202307.1760.v1
APA Style
Chamoun, V., Thompson, E., & Giovannoni, G. (2023). New Evidence-Based Diagnostic Criteria for Neurosarcoidosis. Preprints. https://doi.org/10.20944/preprints202307.1760.v1
Chicago/Turabian Style
Chamoun, V., Edward Thompson and Gavin Giovannoni. 2023 "New Evidence-Based Diagnostic Criteria for Neurosarcoidosis" Preprints. https://doi.org/10.20944/preprints202307.1760.v1
Abstract
Background: Neurosarcoidosis is a great mimic, and its distinction from multiple sclerosis and other disorders can be particularly troublesome. We investigated whether cerebrospinal fluid (CSF) examination could help distinguish these two conditions and propose new diagnostic criteria. Methods: Stringent hierarchical diagnostic criteria were formulated. These were then applied prospectively to 531 neurological patients in whom neurosarcoidosis entered the differential diagnosis. The CSF total protein (TP), white cell count (WCC) and angiotensin-converting enzyme (ACE) levels were measured. The CSF and serum IgG patterns after isoelectric focusing were compared. Not all CSF parameters were measured in every patient. Results: 85/531 (16%) cases examined were diagnosed with neurosarcoidosis. Only 22 of these 85 (26%) met stringent diagnostic criteria. Intrathecal synthesis of oligoclonal IgG was evident in only 1/19 (5%) who fulfilled these criteria compared to 19/59 (32%) cases who did not (p = 0.03). CSF TP levels > 1g/L were more common in patients meeting the stringent criteria compared to the remainder of the patients, i.e. 11/22 (50%) vs 14/58 (24%) (p<0.05). Eleven out of 20 cases (55%) fulfilling stringent criteria had elevated CSF WCCs, and 6/12 cases (50%) had raised CSF ACE, neither significantly different from the remaining cases. Mean CSF TP, WCC, and ACE levels did not differ significantly between any groups of patients. Interpretation: Large elevations in CSF total protein, white cell count, and ACE occur in neurosarcoidosis but are rare in multiple sclerosis. However, the diagnostic use of these tests is limited since minimal changes may occur in both conditions. In contrast, intrathecal synthesis of oligoclonal IgG is a powerful discriminator because it is very uncommon in neurosarcoidosis whilst occurring in 95-98% of cases of multiple sclerosis. This observation implies that the immunopathogenesis of the two disorders is quite different. We suggest caution in diagnosing neurosarcoidosis when intrathecal oligoclonal IgG synthesis is detected. We propose new diagnostic criteria for neurosarcoidosis for future research.
Medicine and Pharmacology, Neuroscience and Neurology
Copyright:
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
