U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Unilateral polymicrogyria

MedGen UID:
870513
Concept ID:
C4024960
Disease or Syndrome
Synonym: unilateral polymicrogyria
SNOMED CT: Unilateral polymicrogyria (715905006)
 
HPO: HP:0006927
Monarch Initiative: MONDO:0017092
Orphanet: ORPHA268943

Definition

Excessive number of small gyri (convolutions) on the surface of one side of the brain. [from HPO]

Conditions with this feature

Complex cortical dysplasia with other brain malformations 7
MedGen UID:
765150
Concept ID:
C3552236
Disease or Syndrome
Complex cortical dysplasia with other brain malformations-7 is an autosomal dominant, clinically heterogeneous disorder showing a wide spectrum of abnormalities of cortical brain development. The most severely affected patients are fetuses with microlissencephaly, absence of the cortical plate, agenesis of the corpus callosum, and severely hypoplastic brainstem and cerebellum. Other patients have lissencephaly, polymicrogyria, cortical dysplasia, or neuronal heterotopia. Those with less severe malformations can survive, but usually have some degree of neurologic impairment, such as mental retardation, seizures, and movement abnormalities (summary by Chang et al., 2006; Fallet-Bianco et al., 2014). For a discussion of genetic heterogeneity of CDCBM, see CDCBM1 (614039).

Recent clinical studies

Etiology

Scarabello M, Righini A, Severino M, Pinelli L, Parazzini C, Scola E, Palumbo G, Di Maurizio M, D'Errico I, Rossi A, Triulzi F, Griffiths PD
AJNR Am J Neuroradiol 2021 Jun;42(6):1151-1156. Epub 2021 Mar 11 doi: 10.3174/ajnr.A7062. PMID: 33707279Free PMC Article
Foesleitner O, Nenning KH, Traub-Weidinger T, Feucht M, Bonelli S, Czech T, Dorfer C, Prayer D, Kasprian G
AJNR Am J Neuroradiol 2018 Aug;39(8):1530-1535. Epub 2018 Jun 28 doi: 10.3174/ajnr.A5715. PMID: 29954815Free PMC Article
Caraballo RH, Reyes G, Falsaperla R, Ramos B, Ruiz AC, Fernandez CA, Peretti G, Beltran L
Seizure 2016 Feb;35:88-92. Epub 2016 Jan 15 doi: 10.1016/j.seizure.2016.01.012. PMID: 26803282
Caraballo RH, Cersósimo RO, Fortini PS, Ornella L, Buompadre MC, Vilte C, Princich JP, Fejerman N
Epileptic Disord 2013 Dec;15(4):417-27. doi: 10.1684/epd.2013.0612. PMID: 24184770
Caraballo RH, Cersósimo RO, Fejerman N
Brain Dev 2004 Apr;26(3):151-7. doi: 10.1016/S0387-7604(03)00113-X. PMID: 15030902

Diagnosis

Scarabello M, Righini A, Severino M, Pinelli L, Parazzini C, Scola E, Palumbo G, Di Maurizio M, D'Errico I, Rossi A, Triulzi F, Griffiths PD
AJNR Am J Neuroradiol 2021 Jun;42(6):1151-1156. Epub 2021 Mar 11 doi: 10.3174/ajnr.A7062. PMID: 33707279Free PMC Article
Hiraiwa A, Matsui K, Nakayama Y, Komatsubara T, Magara S, Kobayashi Y, Hojo M, Kato M, Yamamoto T, Tohyama J
Brain Dev 2021 Mar;43(3):448-453. Epub 2020 Nov 21 doi: 10.1016/j.braindev.2020.11.003. PMID: 33229101
Caraballo RH, Cersósimo RO, Fortini PS, Ornella L, Buompadre MC, Vilte C, Princich JP, Fejerman N
Epileptic Disord 2013 Dec;15(4):417-27. doi: 10.1684/epd.2013.0612. PMID: 24184770
Ishitobi M, Nakasato N, Yoshimoto T, Iinuma K
Brain Dev 2005 Jan;27(1):22-9. doi: 10.1016/j.braindev.2004.02.013. PMID: 15626537
Pascual-Castroviejo I, Pascual-Pascual SI, Viaño J, Martinez V, Palencia R
Brain Dev 2001 Jul;23(4):216-22. doi: 10.1016/s0387-7604(01)00211-x. PMID: 11376999

Therapy

Caraballo RH, Reyes G, Falsaperla R, Ramos B, Ruiz AC, Fernandez CA, Peretti G, Beltran L
Seizure 2016 Feb;35:88-92. Epub 2016 Jan 15 doi: 10.1016/j.seizure.2016.01.012. PMID: 26803282
Fortini S, Corredera L, Pastrana AL, Reyes G, Fasulo L, Caraballo RH
Seizure 2013 Sep;22(7):565-71. Epub 2013 May 2 doi: 10.1016/j.seizure.2013.04.006. PMID: 23642405
Fejerman N, Caraballo R, Cersósimo R, Ferraro SM, Galicchio S, Amartino H
Epilepsia 2012 Jul;53(7):1156-61. Epub 2012 Apr 17 doi: 10.1111/j.1528-1167.2012.03458.x. PMID: 22509732
Bast T, Ramantani G, Boppel T, Metzke T, Ozkan O, Stippich C, Seitz A, Rupp A, Rating D, Scherg M
Neuroimage 2005 May 1;25(4):1232-41. doi: 10.1016/j.neuroimage.2004.12.059. PMID: 15850741

Prognosis

Foesleitner O, Nenning KH, Traub-Weidinger T, Feucht M, Bonelli S, Czech T, Dorfer C, Prayer D, Kasprian G
AJNR Am J Neuroradiol 2018 Aug;39(8):1530-1535. Epub 2018 Jun 28 doi: 10.3174/ajnr.A5715. PMID: 29954815Free PMC Article
Caraballo RH, Cersósimo RO, Fortini PS, Ornella L, Buompadre MC, Vilte C, Princich JP, Fejerman N
Epileptic Disord 2013 Dec;15(4):417-27. doi: 10.1684/epd.2013.0612. PMID: 24184770
Mavili E, Coskun A, Per H, Donmez H, Kumandas S, Yikilmaz A
Childs Nerv Syst 2012 Jun;28(6):905-9. doi: 10.1007/s00381-012-1703-2. PMID: 22286201
Caraballo RH, Cersósimo RO, Fejerman N
Brain Dev 2004 Apr;26(3):151-7. doi: 10.1016/S0387-7604(03)00113-X. PMID: 15030902
Caraballo R, Cersósimo R, Fejerman N
Epilepsia 1999 Jul;40(7):865-71. doi: 10.1111/j.1528-1157.1999.tb00792.x. PMID: 10403209

Clinical prediction guides

Foesleitner O, Nenning KH, Traub-Weidinger T, Feucht M, Bonelli S, Czech T, Dorfer C, Prayer D, Kasprian G
AJNR Am J Neuroradiol 2018 Aug;39(8):1530-1535. Epub 2018 Jun 28 doi: 10.3174/ajnr.A5715. PMID: 29954815Free PMC Article
Mavili E, Coskun A, Per H, Donmez H, Kumandas S, Yikilmaz A
Childs Nerv Syst 2012 Jun;28(6):905-9. doi: 10.1007/s00381-012-1703-2. PMID: 22286201
Ishitobi M, Nakasato N, Yoshimoto T, Iinuma K
Brain Dev 2005 Jan;27(1):22-9. doi: 10.1016/j.braindev.2004.02.013. PMID: 15626537
Pascual-Castroviejo I, Pascual-Pascual SI, Viaño J, Martinez V, Palencia R
Brain Dev 2001 Jul;23(4):216-22. doi: 10.1016/s0387-7604(01)00211-x. PMID: 11376999
Caraballo R, Cersósimo R, Fejerman N
Epilepsia 1999 Jul;40(7):865-71. doi: 10.1111/j.1528-1157.1999.tb00792.x. PMID: 10403209

Supplemental Content

Table of contents

    Clinical resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...