Primary idiopathic CNS non-amyloidogenic light chain deposition disease complicated by treatment-resistant focal seizure disorder. (January 2019)

• Record Type: Journal Article
• Authors: Grainger, Brian T.; Issa, Samar; Anderson, Neil E.
• Is Part Of: Journal of clinical neuroscience. Volume 59(2019)
• Page Start: 313
• Page End: 315
• Publication Date: 2019-01
• Subjects: Brain -- Surgery -- Periodicals; Neurosciences -- Periodicals; Nervous system -- Surgery -- Periodicals; Brain -- surgery -- Periodicals; Neurosurgical Procedures -- Periodicals; Neurosciences -- Periodicals; Electronic journals; 616.8
• DOI: 10.1016/j.jocn.2018.10.123
• Languages: English
• ISSNs: 0967-5868

Abstract

Highlights: LCDD is disorder involving deposition of immunoglobulin light chains. CNS-restricted LCDD is among the rarest manifestations. This unique case is complicated focal onset epilepsy with for which therapy proved difficult. Abstract: Light chain deposition disease (LCDD) is a systemic disorder characterised by the pathologic deposition of immunoglobulin light chains, which is histologically distinguished from amyloidosis by failure to stain with Congo red. Central nervous system (CNS)-restricted LCDD is among the rarest manifestations. We describe a unique case complicated by focal onset epilepsy with impaired awareness for which control with anticonvulsant therapy proved difficult.