@Article{Matjašič2021,
journal="Folia Neuropathologica",
issn="1641-4640",
volume="59",
number="2",
year="2021",
title="Brain aggregoma with clonal B-cell perivascular proliferation
detected by next-generation sequencing. A case report
and review of the literature",
abstract="Light-chain deposition disease (LCDD), a rare type of monoclonal immunoglobulin deposition disease, can be presented as systemic or localized, very rarely affecting central nervous system (CNS). Only 10 cases of CNS-LCDD have been described so far. We present an eleventh case of cerebral tumour-like LCDD, called aggregoma, and compare it with previously reported cases. A 49-year-old patient was admitted to the hospital due to a first generalized epileptic seizure. Magnetic resonance imaging (MRI) showed focal lesion in the right occipital lobe. Abundant parenchymal aggregates of pale eosinophilic material were observed, Congo red negative, Thioflavin T moderately positive, and l-light chain positive, but k negative in immunofluorescence with mild perivascular lymphoplasmacytic infiltrates in the intervening brain tissue. Clonality testing by next-generation sequencing showed the monoclonal nature of B-lymphocytes. Electron microscopy showed a finely granular ultrastructure of the aggregates without deposition in the vessel walls. A whole-body workup did not show any extra-cerebral immune dyscrasias.",
author="Matjašič, Alenka
and Wechtersbach, Karmen
and Kavalar, Rajko
and Voršič, Matjaž
and Mlakar, Jernej
and Ravnik, Janez
and Popović, Mara",
pages="205--211",
doi="10.5114/fn.2021.106405",
url="http://dx.doi.org/10.5114/fn.2021.106405"
}