U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Diffuse subcortical band heterotopia

MedGen UID:
1689721
•Concept ID:
C5139327
•
Disease or Syndrome
Synonym: Subcortical band heterotopia diffuse
 
HPO: HP:0032413

Definition

Longitudinal bands of gray matter located deep to the cerebral cortex and separated from it by a thin layer of normal appearing white matter visible along the whole brain. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDiffuse subcortical band heterotopia

Professional guidelines

PubMed

Targeted re-sequencing in malformations of cortical development: genotype-phenotype correlations.
Accogli A, Severino M, Riva A, Madia F, Balagura G, Iacomino M, Carlini B, Baldassari S, Giacomini T, Croci C, Pisciotta L, Messana T, Boni A, Russo A, Bilo L, Tonziello R, Coppola A, Filla A, Mecarelli O, Casalone R, Pisani F, Falsaperla R, Marino S, Parisi P, Ferretti A, Elia M, Luchetti A, Milani D, Vanadia F, Silvestri L, Rebessi E, Parente E, Vatti G, Mancardi MM, Nobili L, Capra V, Salpietro V, Striano P, Zara F
Seizure 2020 Aug;80:145-152. Epub 2020 Jun 3 doi: 10.1016/j.seizure.2020.05.023. PMID: 32570172
Treatment of epilepsy in severely disabled children with bilateral brain malformations.
Saito Y, Sugai K, Nakagawa E, Sakuma H, Komaki H, Sasaki M, Maegaki Y, Ohno K, Sato N, Kaneko Y, Otsuki T
J Neurol Sci 2009 Feb 15;277(1-2):37-49. Epub 2008 Nov 26 doi: 10.1016/j.jns.2008.10.009. PMID: 19036389

Recent clinical studies

Etiology

Clinical and neuroimaging findings in patients with lissencephaly/subcortical band heterotopia spectrum: a magnetic resonance conventional and diffusion tensor study.
Chiba E, Kimura Y, Shimizu-Motohashi Y, Miyagawa N, Ota M, Shigemoto Y, Ohnishi M, Nakaya M, Nakagawa E, Sasaki M, Sato N
Neuroradiology 2022 Apr;64(4):825-836. Epub 2021 Oct 25 doi: 10.1007/s00234-021-02836-2. PMID: 34693484
Targeted re-sequencing in malformations of cortical development: genotype-phenotype correlations.
Accogli A, Severino M, Riva A, Madia F, Balagura G, Iacomino M, Carlini B, Baldassari S, Giacomini T, Croci C, Pisciotta L, Messana T, Boni A, Russo A, Bilo L, Tonziello R, Coppola A, Filla A, Mecarelli O, Casalone R, Pisani F, Falsaperla R, Marino S, Parisi P, Ferretti A, Elia M, Luchetti A, Milani D, Vanadia F, Silvestri L, Rebessi E, Parente E, Vatti G, Mancardi MM, Nobili L, Capra V, Salpietro V, Striano P, Zara F
Seizure 2020 Aug;80:145-152. Epub 2020 Jun 3 doi: 10.1016/j.seizure.2020.05.023. PMID: 32570172
Neuronal migration disorders.
Guerrini R, Parrini E
Neurobiol Dis 2010 May;38(2):154-66. Epub 2009 Feb 23 doi: 10.1016/j.nbd.2009.02.008. PMID: 19245832
Treatment of epilepsy in severely disabled children with bilateral brain malformations.
Saito Y, Sugai K, Nakagawa E, Sakuma H, Komaki H, Sasaki M, Maegaki Y, Ohno K, Sato N, Kaneko Y, Otsuki T
J Neurol Sci 2009 Feb 15;277(1-2):37-49. Epub 2008 Nov 26 doi: 10.1016/j.jns.2008.10.009. PMID: 19036389
Focal cortical resection in malformations of cortical development.
Russo GL, Tassi L, Cossu M, Cardinale F, Mai R, Castana L, Colombo N, Bramerio M
Epileptic Disord 2003 Sep;5 Suppl 2:S115-23. PMID: 14617430

Diagnosis

Unusual presentation of acute encephalopathy with biphasic seizures and late reduced diffusion in Miller-Dieker syndrome.
Kobayashi S, Kamishima M, Yokoi K, Suzuki S
BMJ Case Rep 2022 Mar 16;15(3) doi: 10.1136/bcr-2021-248190. PMID: 35296495Free PMC Article
Electrographic pattern recognition: A simple tool to predict clinical outcome in children with lissencephaly.
Jauhari P, Farmania R, Chakrabarty B, Kumar A, Gulati S
Seizure 2020 Dec;83:175-180. Epub 2020 Oct 31 doi: 10.1016/j.seizure.2020.10.020. PMID: 33161247
Targeted re-sequencing in malformations of cortical development: genotype-phenotype correlations.
Accogli A, Severino M, Riva A, Madia F, Balagura G, Iacomino M, Carlini B, Baldassari S, Giacomini T, Croci C, Pisciotta L, Messana T, Boni A, Russo A, Bilo L, Tonziello R, Coppola A, Filla A, Mecarelli O, Casalone R, Pisani F, Falsaperla R, Marino S, Parisi P, Ferretti A, Elia M, Luchetti A, Milani D, Vanadia F, Silvestri L, Rebessi E, Parente E, Vatti G, Mancardi MM, Nobili L, Capra V, Salpietro V, Striano P, Zara F
Seizure 2020 Aug;80:145-152. Epub 2020 Jun 3 doi: 10.1016/j.seizure.2020.05.023. PMID: 32570172
Diffuse malformations of cortical development.
Bahi-Buisson N, Guerrini R
Handb Clin Neurol 2013;111:653-65. doi: 10.1016/B978-0-444-52891-9.00068-3. PMID: 23622213
Pigmentary mosaicism, subcortical band heterotopia, and brain cystic lesions.
Ruggieri M, Roggini M, Spalice A, Addis M, Iannetti P
Pediatr Neurol 2009 May;40(5):383-6. doi: 10.1016/j.pediatrneurol.2008.11.006. PMID: 19380077

Therapy

Parkinsonism induced by VNS in a child with double-cortex syndrome.
Cukiert A, Mariani PP, Burattini JA, Cukiert CM, Forster C, Baise C, Argentoni-Baldochi M, Mello V
Epilepsia 2009 Dec;50(12):2667-9. Epub 2009 Aug 8 doi: 10.1111/j.1528-1167.2009.02255.x. PMID: 19674051
Treatment of epilepsy in severely disabled children with bilateral brain malformations.
Saito Y, Sugai K, Nakagawa E, Sakuma H, Komaki H, Sasaki M, Maegaki Y, Ohno K, Sato N, Kaneko Y, Otsuki T
J Neurol Sci 2009 Feb 15;277(1-2):37-49. Epub 2008 Nov 26 doi: 10.1016/j.jns.2008.10.009. PMID: 19036389
Focal cortical resection in malformations of cortical development.
Russo GL, Tassi L, Cossu M, Cardinale F, Mai R, Castana L, Colombo N, Bramerio M
Epileptic Disord 2003 Sep;5 Suppl 2:S115-23. PMID: 14617430

Prognosis

Early suppression of excitability in subcortical band heterotopia modifies epileptogenesis in rats.
Hardy D, Buhler E, Suchkov D, Vinck A, Fortoul A, Watrin F, Represa A, Minlebaev M, Manent JB
Neurobiol Dis 2023 Feb;177:106002. Epub 2023 Jan 14 doi: 10.1016/j.nbd.2023.106002. PMID: 36649744
Electrographic pattern recognition: A simple tool to predict clinical outcome in children with lissencephaly.
Jauhari P, Farmania R, Chakrabarty B, Kumar A, Gulati S
Seizure 2020 Dec;83:175-180. Epub 2020 Oct 31 doi: 10.1016/j.seizure.2020.10.020. PMID: 33161247
Surgical resection for intractable epilepsy in "double cortex" syndrome yields inadequate results.
Bernasconi A, Martinez V, Rosa-Neto P, D'Agostino D, Bernasconi N, Berkovic S, MacKay M, Harvey AS, Palmini A, da Costa JC, Paglioli E, Kim HI, Connolly M, Olivier A, Dubeau F, Andermann E, Guerrini R, Whisler W, de Toledo-Morrell L, Morrell F, Andermann F
Epilepsia 2001 Sep;42(9):1124-9. doi: 10.1046/j.1528-1157.2001.39900.x. PMID: 11580758
Subcortical band heterotopia in rare affected males can be caused by missense mutations in DCX (XLIS) or LIS1.
Pilz DT, Kuc J, Matsumoto N, Bodurtha J, Bernadi B, Tassinari CA, Dobyns WB, Ledbetter DH
Hum Mol Genet 1999 Sep;8(9):1757-60. doi: 10.1093/hmg/8.9.1757. PMID: 10441340

Clinical prediction guides

Clinical and neuroimaging findings in patients with lissencephaly/subcortical band heterotopia spectrum: a magnetic resonance conventional and diffusion tensor study.
Chiba E, Kimura Y, Shimizu-Motohashi Y, Miyagawa N, Ota M, Shigemoto Y, Ohnishi M, Nakaya M, Nakagawa E, Sasaki M, Sato N
Neuroradiology 2022 Apr;64(4):825-836. Epub 2021 Oct 25 doi: 10.1007/s00234-021-02836-2. PMID: 34693484
Electrographic pattern recognition: A simple tool to predict clinical outcome in children with lissencephaly.
Jauhari P, Farmania R, Chakrabarty B, Kumar A, Gulati S
Seizure 2020 Dec;83:175-180. Epub 2020 Oct 31 doi: 10.1016/j.seizure.2020.10.020. PMID: 33161247
Pigmentary mosaicism, subcortical band heterotopia, and brain cystic lesions.
Ruggieri M, Roggini M, Spalice A, Addis M, Iannetti P
Pediatr Neurol 2009 May;40(5):383-6. doi: 10.1016/j.pediatrneurol.2008.11.006. PMID: 19380077
Focal cortical resection in malformations of cortical development.
Russo GL, Tassi L, Cossu M, Cardinale F, Mai R, Castana L, Colombo N, Bramerio M
Epileptic Disord 2003 Sep;5 Suppl 2:S115-23. PMID: 14617430
Subcortical band heterotopia in rare affected males can be caused by missense mutations in DCX (XLIS) or LIS1.
Pilz DT, Kuc J, Matsumoto N, Bodurtha J, Bernadi B, Tassinari CA, Dobyns WB, Ledbetter DH
Hum Mol Genet 1999 Sep;8(9):1757-60. doi: 10.1093/hmg/8.9.1757. PMID: 10441340

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...