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Isolated intracerebral light chain deposition disease: novel imaging and pathologic findings

      Abstract

      Light chain deposition disease (LCDD) is a rare clinicopathologic entity first described in 1976 and is characterized by a monoclonal gammopathy resulting in nonamyloid immunoglobulin light chain tissue deposition. Only four cases of intracerebral LCDD have been previously reported, all in the setting of a known plasma cell dyscrasia or in the presence of local mature plasma cells. We present the first case of intracranial LCDD in the absence of a known plasma cell dyscrasia or local mature plasma cells.

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      References

        • Randall RE
        • Williamson WC
        • Mullinax F
        • Tung MY
        • Still WJ
        Manifestations of systemic light chain deposition.
        Am J Med. 1976; 60: 293-299
        • Jimenez-Zepeda VH
        Light chain deposition disease: novel biological insights and treatment advances.
        Int J Lab Hematol. 2012; 34: 347-355
        • Rho L
        • Qiu L
        • Strauchen JA
        • Gordon RE
        • Teirstein AS
        Pulmonary manifestations of light chain deposition disease.
        Respirology. 2009; 14: 767-770
        • Kijner CH
        • Yousem SA
        Systemic light chain deposition disease presenting as multiple pulmonary nodules. A case report and review of the literature.
        Am J Surg Pathol. 1988; 12: 405-413
        • Skardelly M
        • Pantazis G
        • Bisdas S
        • Feigl GC
        • Schuhmann MU
        • Tatagiba MS
        • et al.
        Primary cerebral low-grade B-cell lymphoma, monoclonal immunoglobulin deposition disease, cerebral light chain deposition disease and “aggregoma”: an update on classification and diagnosis.
        BMC Neurol. 2013; 13: 107
        • Gertz MA
        Immunoglobulin light chain amyloidosis: 2013 update on diagnosis, prognosis, and treatment.
        Am J Hematol. 2013; 88: 416-425
        • Gandhi D
        • Wee R
        • Goyal M
        CT and MR imaging of intracerebral amyloidoma: case report and review of the literature.
        AJNR Am J Neuroradiol. 2003; 24: 519-522
        • Fischer B
        • Palkovic S
        • Rickert C
        • Weckesser M
        • Wassmann H
        Cerebral AL lambda-amyloidoma: clinical and pathomorphological characteristics. Review of the literature and of a patient.
        Amyloid. 2007; 14: 11-19
        • Westermark P
        Aspects on human amyloid forms and their fibril polypeptides.
        FEBS J. 2005; 272: 5942-5949
        • Westermark P
        • Benson MD
        • Buxbaum JN
        • Cohen AS
        • Frangione B
        • Ikeda S
        • et al.
        Amyloid: toward terminology clarification. Report from the Nomenclature Committee of the International Society of Amyloidosis.
        Amyloid. 2005; 12: 1-4
        • Landau D
        • Avgeropoulos N
        • Ma J
        Cerebral amyloidoma mimicking intracranial tumor: a case report.
        J Med Case Rep. 2010; 4: 308
        • Lee J
        • Krol G
        • Rosenblum M
        Primary amyloidoma of the brain: CT and MR presentation.
        AJNR Am J Neuroradiol. 1995; 16: 712-714
        • Caerts B
        • Mol V
        • Sainte T
        • Wilms G
        • Van den Bergh V
        • Stessens L
        CT and MRI of amyloidoma of the CNS.
        Eur Radiol. 1997; 7: 474-476
        • Fischer L
        • Korfel A
        • Stoltenburg-Didinger G
        • Ransco C
        • Thiel E
        A 19-year-old male with generalized seizures, unconsciousness and a deviation of gaze.
        Brain Pathol. 2006; 16 ([7]): 185-186
        • Popovic M
        • Tavcar R
        • Glavac D
        • Volavsek M
        • Pirtosek Z
        • Vizjak A
        Light chain deposition disease restricted to the brain: the first case report.
        Hum Pathol. 2007; 38: 179-184
        • Vital A
        • Ellie E
        • Loiseau H
        A 61-year-old man with instability of gait and right hand clumsiness.
        Brain Pathol. 2010; 20: 273-274