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Leptomeningeal enhancement

MedGen UID:
1671079
Concept ID:
C4732837
Anatomical Abnormality
HPO: HP:0032070

Definition

Contrast material enhancement of the pia mater or enhancement that extends into the subarachnoid spaces of the sulci and cisterns is leptomeningeal enhancement. Leptomeningeal enhancement is usually associated with meningitis, which may be bacterial, viral, or fungal. The primary mechanism of this enhancement is breakdown of the blood-brain barrier without angiogenesis. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLeptomeningeal enhancement

Conditions with this feature

Familial amyloid neuropathy
MedGen UID:
414031
Concept ID:
C2751492
Disease or Syndrome
Hereditary transthyretin (ATTR) amyloidosis is characterized by a slowly progressive peripheral sensorimotor and/or autonomic neuropathy as well as non-neuropathic changes of cardiomyopathy, nephropathy, vitreous opacities, and CNS amyloidosis. The disease usually begins in the third to fifth decade in persons from endemic foci in Portugal and Japan; onset is later in persons from other areas. Typically, sensory neuropathy starts in the lower extremities with paresthesias and hypesthesias of the feet, followed within a few years by motor neuropathy. In some persons, particularly those with early-onset disease, autonomic neuropathy is the first manifestation of the condition; findings can include: orthostatic hypotension, constipation alternating with diarrhea, attacks of nausea and vomiting, delayed gastric emptying, sexual impotence, anhidrosis, and urinary retention or incontinence. Cardiac amyloidosis is mainly characterized by progressive cardiomyopathy. Individuals with leptomeningeal amyloidosis may have the following CNS findings: dementia, psychosis, visual impairment, headache, seizures, motor paresis, ataxia, myelopathy, hydrocephalus, or intracranial hemorrhage.

Professional guidelines

PubMed

Martín-Jiménez P, Sánchez-Tornero M, Llamas-Velasco S, Guerrero-Molina MP, González-Sánchez M, Herrero-San Martín A, Blanco-Palmero V, Calleja-Castaño P, Francisco-Gonzalo J, Hilario A, Ramos A, Salvador E, Toldos Ó, Hernández-Lain A, Pérez-Martínez DA, Villarejo-Galende A
Neurologia (Engl Ed) 2023 Oct;38(8):550-559. Epub 2023 Jul 10 doi: 10.1016/j.nrleng.2023.07.002. PMID: 37437655
Meena DS, Kumar D, Sharma M, Malik M, Ravindra A, Santhanam N, Bohra GK, Garg MK
Orphanet J Rare Dis 2023 Jun 2;18(1):133. doi: 10.1186/s13023-023-02744-z. PMID: 37269006Free PMC Article
Del Brutto OH, Nash TE, White AC Jr, Rajshekhar V, Wilkins PP, Singh G, Vasquez CM, Salgado P, Gilman RH, Garcia HH
J Neurol Sci 2017 Jan 15;372:202-210. Epub 2016 Nov 21 doi: 10.1016/j.jns.2016.11.045. PMID: 28017213

Recent clinical studies

Etiology

Elsbernd P, Cacciaguerra L, Krecke KN, Chen JJ, Gritsch D, Lopez-Chiriboga AS, Sechi E, Redenbaugh V, Morris PP, Carter JL, Wingerchuk DM, Tillema JM, Valencia-Sanchez C, Thakolwiboon S, Pittock SJ, Flanagan EP
J Neurol Neurosurg Psychiatry 2023 Dec 14;95(1):14-18. doi: 10.1136/jnnp-2023-331137. PMID: 37221051Free PMC Article
Filippi M, Preziosa P, Arnold DL, Barkhof F, Harrison DM, Maggi P, Mainero C, Montalban X, Sechi E, Weinshenker BG, Rocca MA
J Neurol 2023 Mar;270(3):1286-1299. Epub 2022 Nov 24 doi: 10.1007/s00415-022-11488-y. PMID: 36427168Free PMC Article
Valencia-Sanchez C, Guo Y, Krecke KN, Chen JJ, Redenbaugh V, Montalvo M, Elsbernd PM, Tillema JM, Lopez-Chiriboga S, Budhram A, Sechi E, Kunchok A, Dubey D, Pittock SJ, Lucchinetti CF, Flanagan EP
Ann Neurol 2023 Feb;93(2):297-302. Epub 2022 Dec 2 doi: 10.1002/ana.26549. PMID: 36372941Free PMC Article
Gravier-Dumonceau A, Ameli R, Rogemond V, Ruiz A, Joubert B, Muñiz-Castrillo S, Vogrig A, Picard G, Ambati A, Benaiteau M, Rulquin F, Ciron J, Deiva K, de Broucker T, Kremer L, Kerschen P, Sellal F, Bouldoires B, Genet R, Biberon J, Bigot A, Duval F, Issa N, Rusu EC, Goudot M, Dutray A, Devoize JL, Hopes L, Kaminsky AL, Philbert M, Chanson E, Leblanc A, Morvan E, Andriuta D, Diraison P, Mirebeau G, Derollez C, Bourg V, Bodard Q, Fort C, Grigorashvili-Coin I, Rieul G, Molinier-Tiganas D, Bonnan M, Tchoumi T, Honnorat J, Marignier R
Neurology 2022 Feb 8;98(6):e653-e668. Epub 2021 Nov 19 doi: 10.1212/WNL.0000000000013087. PMID: 34799461Free PMC Article
Deuble M, Aquilina C, Norton R
Am J Trop Med Hyg 2013 Sep;89(3):535-9. Epub 2013 Jul 8 doi: 10.4269/ajtmh.12-0559. PMID: 23836574Free PMC Article

Diagnosis

Filippi M, Preziosa P, Arnold DL, Barkhof F, Harrison DM, Maggi P, Mainero C, Montalban X, Sechi E, Weinshenker BG, Rocca MA
J Neurol 2023 Mar;270(3):1286-1299. Epub 2022 Nov 24 doi: 10.1007/s00415-022-11488-y. PMID: 36427168Free PMC Article
Nehme A, Boulanger M, Aouba A, Pagnoux C, Zuber M, Touzé E, de Boysson H
Rev Neurol (Paris) 2022 Dec;178(10):1041-1054. Epub 2022 Sep 22 doi: 10.1016/j.neurol.2022.05.003. PMID: 36156251
David C, Sacré K, Henri-Feugeas MC, Klein I, Doan S, Cohen FA, Jouvent E, Papo T
Autoimmun Rev 2022 Jun;21(6):103097. Epub 2022 Apr 10 doi: 10.1016/j.autrev.2022.103097. PMID: 35413469
Bot JCJ, Mazzai L, Hagenbeek RE, Ingala S, van Oosten B, Sanchez-Aliaga E, Barkhof F
Neuroradiology 2020 Mar;62(3):283-300. Epub 2020 Jan 10 doi: 10.1007/s00234-019-02335-5. PMID: 31925469Free PMC Article
Deuble M, Aquilina C, Norton R
Am J Trop Med Hyg 2013 Sep;89(3):535-9. Epub 2013 Jul 8 doi: 10.4269/ajtmh.12-0559. PMID: 23836574Free PMC Article

Therapy

Ineichen BV, Tsagkas C, Absinta M, Reich DS
Neuroimage Clin 2022;33:102939. Epub 2022 Jan 10 doi: 10.1016/j.nicl.2022.102939. PMID: 35026625Free PMC Article
Al Malik YM
Neurosciences (Riyadh) 2020 Oct;25(5):406-411. doi: 10.17712/nsj.2020.5.20200090. PMID: 33459292Free PMC Article
Castillo JJ, Treon SP
Br J Haematol 2019 Nov;187(3):277-285. Epub 2019 Aug 20 doi: 10.1111/bjh.16167. PMID: 31430829
Bagnato F, Stern BJ
Expert Rev Neurother 2015 May;15(5):533-48. doi: 10.1586/14737175.2015.1037288. PMID: 25936846
Deuble M, Aquilina C, Norton R
Am J Trop Med Hyg 2013 Sep;89(3):535-9. Epub 2013 Jul 8 doi: 10.4269/ajtmh.12-0559. PMID: 23836574Free PMC Article

Prognosis

Filippi M, Preziosa P, Arnold DL, Barkhof F, Harrison DM, Maggi P, Mainero C, Montalban X, Sechi E, Weinshenker BG, Rocca MA
J Neurol 2023 Mar;270(3):1286-1299. Epub 2022 Nov 24 doi: 10.1007/s00415-022-11488-y. PMID: 36427168Free PMC Article
Gao X, Pan R, Chen M, Zhao J, Zhong W, Wang H, Si X, Zhang X, Zhang L, Xu Y, Wang M
Thorac Cancer 2022 Apr;13(7):1059-1066. Epub 2022 Mar 3 doi: 10.1111/1759-7714.14362. PMID: 35238486Free PMC Article
Gravier-Dumonceau A, Ameli R, Rogemond V, Ruiz A, Joubert B, Muñiz-Castrillo S, Vogrig A, Picard G, Ambati A, Benaiteau M, Rulquin F, Ciron J, Deiva K, de Broucker T, Kremer L, Kerschen P, Sellal F, Bouldoires B, Genet R, Biberon J, Bigot A, Duval F, Issa N, Rusu EC, Goudot M, Dutray A, Devoize JL, Hopes L, Kaminsky AL, Philbert M, Chanson E, Leblanc A, Morvan E, Andriuta D, Diraison P, Mirebeau G, Derollez C, Bourg V, Bodard Q, Fort C, Grigorashvili-Coin I, Rieul G, Molinier-Tiganas D, Bonnan M, Tchoumi T, Honnorat J, Marignier R
Neurology 2022 Feb 8;98(6):e653-e668. Epub 2021 Nov 19 doi: 10.1212/WNL.0000000000013087. PMID: 34799461Free PMC Article
Alrobaian MA, Henderson AD
J Neuroophthalmol 2021 Dec 1;41(4):e584-e590. doi: 10.1097/WNO.0000000000001071. PMID: 32868572
Castillo JJ, Treon SP
Br J Haematol 2019 Nov;187(3):277-285. Epub 2019 Aug 20 doi: 10.1111/bjh.16167. PMID: 31430829

Clinical prediction guides

Elsbernd P, Cacciaguerra L, Krecke KN, Chen JJ, Gritsch D, Lopez-Chiriboga AS, Sechi E, Redenbaugh V, Morris PP, Carter JL, Wingerchuk DM, Tillema JM, Valencia-Sanchez C, Thakolwiboon S, Pittock SJ, Flanagan EP
J Neurol Neurosurg Psychiatry 2023 Dec 14;95(1):14-18. doi: 10.1136/jnnp-2023-331137. PMID: 37221051Free PMC Article
Filippi M, Preziosa P, Arnold DL, Barkhof F, Harrison DM, Maggi P, Mainero C, Montalban X, Sechi E, Weinshenker BG, Rocca MA
J Neurol 2023 Mar;270(3):1286-1299. Epub 2022 Nov 24 doi: 10.1007/s00415-022-11488-y. PMID: 36427168Free PMC Article
Valencia-Sanchez C, Guo Y, Krecke KN, Chen JJ, Redenbaugh V, Montalvo M, Elsbernd PM, Tillema JM, Lopez-Chiriboga S, Budhram A, Sechi E, Kunchok A, Dubey D, Pittock SJ, Lucchinetti CF, Flanagan EP
Ann Neurol 2023 Feb;93(2):297-302. Epub 2022 Dec 2 doi: 10.1002/ana.26549. PMID: 36372941Free PMC Article
Jiang W, Sun X, Huang H, Sun H, Zhang S, He M, Yu X, Huang D, Wu L
J Neuroimmunol 2022 Aug 15;369:577898. Epub 2022 May 20 doi: 10.1016/j.jneuroim.2022.577898. PMID: 35717737
Gravier-Dumonceau A, Ameli R, Rogemond V, Ruiz A, Joubert B, Muñiz-Castrillo S, Vogrig A, Picard G, Ambati A, Benaiteau M, Rulquin F, Ciron J, Deiva K, de Broucker T, Kremer L, Kerschen P, Sellal F, Bouldoires B, Genet R, Biberon J, Bigot A, Duval F, Issa N, Rusu EC, Goudot M, Dutray A, Devoize JL, Hopes L, Kaminsky AL, Philbert M, Chanson E, Leblanc A, Morvan E, Andriuta D, Diraison P, Mirebeau G, Derollez C, Bourg V, Bodard Q, Fort C, Grigorashvili-Coin I, Rieul G, Molinier-Tiganas D, Bonnan M, Tchoumi T, Honnorat J, Marignier R
Neurology 2022 Feb 8;98(6):e653-e668. Epub 2021 Nov 19 doi: 10.1212/WNL.0000000000013087. PMID: 34799461Free PMC Article

Recent systematic reviews

Wiśniewski K, Brandel MG, Gonda DD, Crawford JR, Levy ML
Childs Nerv Syst 2022 Sep;38(9):1663-1673. Epub 2022 Jul 22 doi: 10.1007/s00381-022-05600-w. PMID: 35867118
Ineichen BV, Tsagkas C, Absinta M, Reich DS
Neuroimage Clin 2022;33:102939. Epub 2022 Jan 10 doi: 10.1016/j.nicl.2022.102939. PMID: 35026625Free PMC Article
Gombolay GY, Gadde JA
J Neuroimmunol 2021 Sep 15;358:577653. Epub 2021 Jul 1 doi: 10.1016/j.jneuroim.2021.577653. PMID: 34229204
Lewis A, Jain R, Frontera J, Placantonakis DG, Galetta S, Balcer L, Melmed KR
J Neuroimaging 2021 Sep;31(5):826-848. Epub 2021 Jun 8 doi: 10.1111/jon.12880. PMID: 34105198Free PMC Article
Arai N, Kagami H, Mine Y, Ishii T, Inaba M
World Neurosurg 2018 Sep;117:386-393. Epub 2018 Jun 26 doi: 10.1016/j.wneu.2018.06.138. PMID: 29959081

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