Primary central nervous system lymphoma mimicking autoimmune encephalitis: the role of autoantibody testing
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LETTER TO THE EDITOR
Primary central nervous system lymphoma mimicking autoimmune encephalitis: the role of autoantibody testing Eleonora Rigoni 1
&
Lisa Farina 2 & Paola Bini 3 & Patrizia Morbini 4 & Diego Franciotta 5 & Matteo Gastaldi 1,5
Received: 24 March 2020 / Accepted: 18 September 2020 # Fondazione Società Italiana di Neurologia 2020
Professor A. Federico Editor-in-Chief of Neurological Sciences Dear Professor: Autoimmune encephalitis (AE) comprises a group of inflammatory disorders of the CNS pre-eminently characterized by neuropsychiatric symptoms, and often associated with antibodies to neuronal cell-surface antigens. The detection of such antibodies is fundamental for a reliable diagnosis of these diseases, whose outcome improves if they are early identified and adequately treated [1]. Early diagnosis is so important that clinical criteria for AE have been introduced to start timely therapies even when the tests for the detection of the AE-associated antibodies are not available, turnaround times are excessively long, or results are negative [1]. However, on the one hand, many conditions can mimic AE, and on the other hand, nonstandardized laboratory techniques for the antibody detection can yield both false negative and false positive results, with risk of improper patient classification and management [1, 2]. Antigen-specific cell-based assays (CBAs) are fundamental for the detection of antibodies to neuronal cell-surface antigens [1]. In addition, screening tissue-based assays (TBAs)
* Eleonora Rigoni [email protected] 1
Department of Neurology, IRCCS Mondino Foundation, University of Pavia, via Mondino 2, 27100 Pavia, Italy
2
Department of Neuroradiology, IRCCS Mondino Foundation, Pavia, Italy
3
Department of Neuro-Oncology, IRCCS Mondino Foundation, Pavia, Italy
4
Unit of Pathology, Department of Molecular Medicine, IRCCS Policlinico San Matteo Foundation, University of Pavia, 27100 Pavia, Italy
5
Laboratory of Neuroimmunology, IRCCS Mondino Foundation, Pavia, Italy
exploiting immunohistochemistry on lightly fixed murine brain is recommended to improve the diagnostic accuracy of testing for AE [2], concomitantly allowing the identification of uncharacterized autoantibody reactivities [1], whose definite clinical meaning is still unclear. We describe a patient with primary CNS lymphoma (PCNSL), whose clinical and paraclinical manifestations at onset fulfilled the criteria for “probable but antibody negative AE” [1]. The assays for both the known intracellular and cell-surface neuronal antibodies were negative, but hippocampal neuropilar staining on TBA further favored an AE diagnosis, which was eventually excluded by the results of a brain biopsy.
Case report A 47-year-old man was admitted to our Institute for subacute onset of drowsiness and confusion. Previous medical history was remarkable for laparoscopic cholecystectomy and juvenile hypertension. The patient presented severe short-term memory loss, confabulation, and striking hypersomnia. Mini Mental State
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