Posttransplant Acute Limbic Encephalitis

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Retrieved

2021-01-23

Source

Orphanet

Trials

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Genes

ACAT1, IKBKG, RPL35, KLF1, SLC19A2, TCIRG1, SH2B3, KIF23, LRAT, PRPF4, AIP, CACNA1H, JRK, TNFSF11, CASK, SLC25A11, ACO2, VHL, UCP2, HNF1A, ABCC8, SPTB, SPTA1, SLC4A1, SLC2A1, SDHD, SDHC, SDHB, SDHA, RPS29, KIF1B, VPS13A, SF3B1, TNPO3, HGSNAT, TTC8, SLC46A1, IFT43, TSR2, RAX2, C15orf41, TMEM126A, PUS1, CDH23, AGBL5, WDR19, ALS2, KLHL7, TMEM127, POMGNT1, STEAP3, FANCI, MSTO1, SDHAF2, TET2, ADA2, TRNT1, SNX10, MMADHC, MMACHC, PRPF6, RPS28, RPS27, RPS26, HNF4A, HEXA, HBG2, HBG1, HBB, HBA2, HBA1, GATA2, GATA1, GABRG2, GABRB3, GABRA1, G6PD, FH, FDXR, FBP1, F8, EPB42, EPB41, DLST, DHFR, CLCN7, CALR, CACNA1A, ATP7A, AIRE, ANK1, ALAS2, HMGCL, JAK2, RPS24, KCNJ11, RPS19, RPS17, RPS15A, RPS10, RPS7, RPL35A, RPL27, RPL26, RPL18, RPL15, RPL11, RPL5, RET, RAG2, RAG1, PTS, PIGA, PGK1, OPA1, NRL, MYD88, MPL, MEN1, MDH2, MAX, KIF5A, KCNQ2, TMPRSS6

Drugs

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Posttransplant acute limbic encephalitis is a rare, acquired, non-paraneoplastic limbic encephalitis disorder, that develops in the setting of treatment-related immunosuppression, typically after allogeneic hemapoietic stem cell transplantation, characterized by onset of confusion, headache, anterograde amnesia, seizures and/or loss of consciousness 2-6 weeks following transplantation. Bilateral, non-enhancing T2 hyperintensities in limbic structures are observed on magnetic resonance imaging. Mild cerebrospinal fluid pleocytosis and syndrome of inappropriate antidiuretic hormone secretion may also be associated.