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Hippocampal sclerosis

MedGen UID:
1778719
Concept ID:
C1504404
Disease or Syndrome
Synonyms: Ammon Horn Scleroses; Ammon Horn Sclerosis; Hippocampal Scleroses; Hippocampal Sclerosis; Horn Sclerosis, Ammon; Sclerosis, Ammon Horn; Sclerosis, Hippocampal
 
HPO: HP:0033715

Definition

Hippocampal sclerosis is a neuropathologic finding characterized by neuronal loss and gliosis in the CA-1 and subiculum of the hippocampus. [from HPO]

Conditions with this feature

Epilepsy, familial adult myoclonic, 5
MedGen UID:
815704
Concept ID:
C3809374
Disease or Syndrome
Early-onset epilepsy-5 with or without developmental delay (EPEO5) is an autosomal recessive neurologic disorder characterized by the onset of various types of seizures late in the first decade or during adolescence. Focal seizures are common. Most affected individuals have developmental delay, variable impaired intellectual development, and/or behavioral and neuropsychiatric abnormalities (Stogmann et al., 2013; Abdulkareem et al., 2023). For a discussion of genetic heterogeneity of EPEO, see 617290.
Spastic ataxia 10, autosomal recessive
MedGen UID:
1851662
Concept ID:
C5882738
Disease or Syndrome
Autosomal recessive spastic ataxia-10 (SPAX10) is a slowly progressive movement disorder with a variable age at onset (range infancy to adulthood). Affected individuals present with gait abnormalities due to spasticity and hyperreflexia of the lower limbs and/or cerebellar gait and limb ataxia. More variable features may include dysarthria, saccadic eye movements, and mild cognitive impairment. Some patients show cerebellar atrophy on brain imaging. The disorder can be classified as a movement disorder on the ataxia-spasticity spectrum (ASS) (Cordts et al., 2022). For a discussion of genetic heterogeneity of spastic ataxia, see SPAX1 (108600).
Neurodevelopmental disorder with hypotonia, brain anomalies, distinctive facies, and absent language
MedGen UID:
1854654
Concept ID:
C5935628
Disease or Syndrome
ReNU syndrome (RENU), also known as neurodevelopmental disorder with hypotonia, brain anomalies, distinctive facies, and absent language (NEDHAFA), is characterized by hypotonia, global developmental delay, severely impaired intellectual development with poor or absent speech, delayed walking or inability to walk, feeding difficulties with poor overall growth, seizures (in most), dysmorphic facial features, and brain anomalies, including ventriculomegaly, thin corpus callosum, and progressive white matter loss (Greene et al., 2024; Schot et al., 2024; Chen et al., 2024).

Professional guidelines

PubMed

Riney K, Bogacz A, Somerville E, Hirsch E, Nabbout R, Scheffer IE, Zuberi SM, Alsaadi T, Jain S, French J, Specchio N, Trinka E, Wiebe S, Auvin S, Cabral-Lim L, Naidoo A, Perucca E, Moshé SL, Wirrell EC, Tinuper P
Epilepsia 2022 Jun;63(6):1443-1474. Epub 2022 May 3 doi: 10.1111/epi.17240. PMID: 35503725
Asadi-Pooya AA, Bartolini L
Int J Neurosci 2020 Nov;130(11):1151-1155. Epub 2020 Feb 27 doi: 10.1080/00207454.2020.1730370. PMID: 32053411
Montine TJ, Phelps CH, Beach TG, Bigio EH, Cairns NJ, Dickson DW, Duyckaerts C, Frosch MP, Masliah E, Mirra SS, Nelson PT, Schneider JA, Thal DR, Trojanowski JQ, Vinters HV, Hyman BT; National Institute on Aging; Alzheimer’s Association
Acta Neuropathol 2012 Jan;123(1):1-11. Epub 2011 Nov 20 doi: 10.1007/s00401-011-0910-3. PMID: 22101365Free PMC Article

Recent clinical studies

Etiology

Cheval M, Houot M, Chastan N, Szurhaj W, Marchal C, Catenoix H, Valton L, Gavaret M, Herlin B, Biraben A, Lagarde S, Mazzola L, Minotti L, Maillard L, Dupont S
J Neurol 2023 May;270(5):2715-2723. Epub 2023 Feb 10 doi: 10.1007/s00415-023-11603-7. PMID: 36763175
Singh G, Sander JW
Arq Neuropsiquiatr 2018 Nov;76(11):783-790. doi: 10.1590/0004-282X20180130. PMID: 30570023
Thom M
Neuropathol Appl Neurobiol 2014 Aug;40(5):520-43. doi: 10.1111/nan.12150. PMID: 24762203Free PMC Article
Urbach H
Clin Neuroradiol 2011 Sep;21(3):179-80. doi: 10.1007/s00062-011-0094-1. PMID: 21837499
Wyler AR
Neurosurgery 1997 Dec;41(6):1294-301; discussion 1301-2. doi: 10.1097/00006123-199712000-00012. PMID: 9402581

Diagnosis

Middlebrooks EH, Gupta V, Agarwal AK, Freund BE, Messina SA, Tatum WO, Sabsevitz DS, Feyissa AM, Mirsattari SM, Galan FN, Quinones-Hinojosa A, Grewal SS, Murray JV
AJNR Am J Neuroradiol 2024 Sep 9;45(9):1185-1193. doi: 10.3174/ajnr.A8214. PMID: 38383054Free PMC Article
Henning O, Heuser K, Larsen VS, Kyte EB, Kostov H, Marthinsen PB, Egge A, Alfstad KÅ, Nakken KO
Tidsskr Nor Laegeforen 2023 Jan 31;143(2) Epub 2023 Jan 30 doi: 10.4045/tidsskr.22.0369. PMID: 36718887
Bien CG
Handb Clin Neurol 2022;187:467-487. doi: 10.1016/B978-0-12-823493-8.00024-9. PMID: 35964988
Thom M
Neuropathol Appl Neurobiol 2014 Aug;40(5):520-43. doi: 10.1111/nan.12150. PMID: 24762203Free PMC Article
Guerrini R
Lancet 2006 Feb 11;367(9509):499-524. doi: 10.1016/S0140-6736(06)68182-8. PMID: 16473127

Therapy

Fang Y, Si X, Wang J, Wang Z, Chen Y, Liu Y, Yan Y, Tian J, Zhang B, Pu J
Neurology 2023 Jul 25;101(4):e399-e409. Epub 2023 May 24 doi: 10.1212/WNL.0000000000207423. PMID: 37225432Free PMC Article
Riney K, Bogacz A, Somerville E, Hirsch E, Nabbout R, Scheffer IE, Zuberi SM, Alsaadi T, Jain S, French J, Specchio N, Trinka E, Wiebe S, Auvin S, Cabral-Lim L, Naidoo A, Perucca E, Moshé SL, Wirrell EC, Tinuper P
Epilepsia 2022 Jun;63(6):1443-1474. Epub 2022 May 3 doi: 10.1111/epi.17240. PMID: 35503725
Morris G, Reschke CR, Henshall DC
EBioMedicine 2019 Jul;45:646-654. Epub 2019 Jul 9 doi: 10.1016/j.ebiom.2019.07.008. PMID: 31300345Free PMC Article
Guerrini R
Lancet 2006 Feb 11;367(9509):499-524. doi: 10.1016/S0140-6736(06)68182-8. PMID: 16473127
Yu H, Yen D, Yiu C, Chung W, Lirng J, Su M
Eur Neurol 1998 Jul;40(1):19-21. doi: 10.1159/000007950. PMID: 9693227

Prognosis

Middlebrooks EH, Gupta V, Agarwal AK, Freund BE, Messina SA, Tatum WO, Sabsevitz DS, Feyissa AM, Mirsattari SM, Galan FN, Quinones-Hinojosa A, Grewal SS, Murray JV
AJNR Am J Neuroradiol 2024 Sep 9;45(9):1185-1193. doi: 10.3174/ajnr.A8214. PMID: 38383054Free PMC Article
Bien CG
Handb Clin Neurol 2022;187:467-487. doi: 10.1016/B978-0-12-823493-8.00024-9. PMID: 35964988
Bernasconi A, Bernasconi N
Eur Neurol 2022;85(5):333-341. Epub 2022 Jun 15 doi: 10.1159/000525262. PMID: 35705017
Sidhu MK, Duncan JS, Sander JW
Curr Opin Neurol 2018 Aug;31(4):371-378. doi: 10.1097/WCO.0000000000000568. PMID: 29782369
Guerrini R
Lancet 2006 Feb 11;367(9509):499-524. doi: 10.1016/S0140-6736(06)68182-8. PMID: 16473127

Clinical prediction guides

Lewis DV, Voyvodic J, Shinnar S, Chan S, Bello JA, Moshé SL, Nordli DR Jr, Frank LM, Pellock JM, Hesdorffer DC, Xu Y, Shinnar RC, Seinfeld S, Epstein LG, Masur D, Gallentine W, Weiss E, Deng X, Sun S; FEBSTAT Study Team
Epilepsia 2024 Jun;65(6):1568-1580. Epub 2024 Apr 12 doi: 10.1111/epi.17979. PMID: 38606600Free PMC Article
Zhang M, Ganz AB, Rohde S, Lorenz L, Rozemuller AJM, van Vliet K, Graat M, Sikkes SAM, Reinders MJT, Scheltens P, Hulsman M, Hoozemans JJM, Holstege H
Alzheimers Dement 2023 Nov;19(11):5036-5047. Epub 2023 Apr 24 doi: 10.1002/alz.13087. PMID: 37092333
Zepeda N, Copitin N, Chávez JL, García F, Jaimes-Miranda F, Rincón-Heredia R, Paredes R, Solano S, Fernández AM, Molinari JL
J Helminthol 2019 Nov;93(6):690-696. Epub 2018 Aug 23 doi: 10.1017/S0022149X18000755. PMID: 30136641
Chatzikonstantinou A
Front Neurol Neurosci 2014;34:121-42. Epub 2014 Apr 16 doi: 10.1159/000356435. PMID: 24777136
Malmgren K, Thom M
Epilepsia 2012 Sep;53 Suppl 4:19-33. doi: 10.1111/j.1528-1167.2012.03610.x. PMID: 22946718

Recent systematic reviews

Li Y, Liu P, Lin Q, Zhou D, An D
Epilepsia 2023 Nov;64(11):2845-2860. Epub 2023 Sep 30 doi: 10.1111/epi.17757. PMID: 37611927
Chakravarty K, Ray S, Kharbanda PS, Lal V, Baishya J
Acta Neurol Scand 2021 Sep;144(3):236-250. Epub 2021 May 13 doi: 10.1111/ane.13455. PMID: 33987835
Korotkov A, Mills JD, Gorter JA, van Vliet EA, Aronica E
Sci Rep 2017 Sep 14;7(1):11592. doi: 10.1038/s41598-017-11510-8. PMID: 28912503Free PMC Article
Steve TA, Jirsch JD, Gross DW
Epilepsy Res 2014 Oct;108(8):1279-85. Epub 2014 Jul 23 doi: 10.1016/j.eplepsyres.2014.07.003. PMID: 25107686
Kauffman MA, Consalvo D, Moron DG, Lereis VP, Kochen S
Epilepsy Res 2010 Aug;90(3):234-9. Epub 2010 Jun 15 doi: 10.1016/j.eplepsyres.2010.05.007. PMID: 20554432

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