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Background: The presence of inflammatory changes in the cerebrospinal fluid (CSF), including immunoglobulin intrathecal synthesis (IS), can support the diagnosis of autoimmune encephalitis (AE) and allow prompt treatment. The main aim of our study was to calculate the Kappa index as a marker of IS, in patients with AE.
Methods: Charts of patients undergoing a diagnostic work-up for suspected AE between 2009 and 2023 were reviewed and the Graus criteria applied. CSF and serum kappa free light chains were determined using the Freelite assay (The Binding Site Group) and the turbidimetric Optilite analyzer.
Results: We identified 34 patients with "definite" AE (9 anti-NMDAR AE and 25 limbic AE) and nine patients with "possible" AE. Five patients (15%) with definite AE had pleocytosis and twelve (34%) showed CSF-restricted oligoclonal bands (OCB) at isoelectric focusing. The Kappa index was >6 in 29.4% and > 3 in 50% of the definite AE patients. It was elevated (>3) in 36.4% of patients with definite AE who resulted negative to OCB testing and was the only altered parameter suggestive of an ongoing inflammatory process in the CNS in three definite AE patients with otherwise normal CSF findings (i.e. normal cell count and protein levels, no OCBs). In the possible AE group, one patient had a Kappa index >3 in the absence of OCB.
Conclusions: The Kappa index could be useful, as a more sensitive marker of IS and as a supportive marker of neuroinflammation, in the diagnostic work-up of suspected AE.
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http://dx.doi.org/10.1016/j.jns.2024.123146 | DOI Listing |
PLoS One
September 2025
Department of Health Services Research, and CAPHRI School for Public Health and Primary Care, Aging and Long Term Care Maastricht, Maastricht, the Netherlands.
Background: Older patients presenting with nonspecific complaints (NSC) in the Emergency Department (ED) pose diagnostic challenges. The lack of clear symptoms leads to high misdiagnosis rates, extended hospital stays, and functional impairment. However, limited research exists on diagnostic test utilization for this population.
View Article and Find Full Text PDFJACC Case Rep
September 2025
Department of Cardiovascular Disease, Wellstar Kennestone Regional Medical Center, Marietta, Georgia, USA.
Background: Paragangliomas are rare neuroendocrine tumors arising from chromaffin cells, often associated with excess catecholamine production. Cardiac paragangliomas are particularly uncommon, accounting for <1% of all cardiac tumors.
Case Summary: We report a case of a right ventricular mass that was initially misidentified as a pericardial cyst on imaging but was ultimately diagnosed as a benign paraganglioma deriving from hereditary paraganglioma and pheochromocytoma syndrome after further diagnostic evaluation and treatment.
Pulmonary hypertension is common in children with Trisomy 21, frequently with multifactorial aetiologies. Registry data provide better understanding of disease development, diagnostic workup and treatment patterns in children with Trisomy 21. TOPP (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension) is a centre-based, comprehensive registry.
View Article and Find Full Text PDFBMJ Case Rep
September 2025
Department of Clinical Neurology, Tbilisi State Medical University, Tbilisi, Georgia.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a brainstem syndrome marked by punctate 'pepper-and-salt' enhancement on MRI and corticosteroid responsiveness. Evidence suggests that CLIPPERS may represent a cancer-related immune reaction, occurring before tumour detection, in association with active malignancy or after treatment. We report the first case associated with papillary thyroid carcinoma (PTC).
View Article and Find Full Text PDFOccup Med (Lond)
September 2025
Department of Internal Diseases, Occupational Diseases Unit, Ministry of Health Ankara Etlik City Hospital, Ankara, Turkey.
Caplan syndrome, a rare condition characterized by the coexistence of pneumoconiosis and rheumatoid arthritis in individuals exposed to inorganic dust, remains a relevant diagnostic consideration in the 21st century. We present a case of a 41-year-old former coal miner who developed multiple cavitary pulmonary nodules years after leaving the mining sector, initially mimicking pulmonary malignancy. Extensive diagnostic work-up, including positron emission tomography-computed tomography, bronchoscopy and lung biopsy, eventually led to the diagnosis of Caplan syndrome, with seropositive rheumatoid arthritis emerging 8 years after exposure cessation.
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