U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Congenital bilateral perisylvian syndrome(CBPS; BPPX)

MedGen UID:
337000
Concept ID:
C1845668
Disease or Syndrome
Synonyms: Bilateral perisylvian polymicrogyria; Perisylvian syndrome
SNOMED CT: Congenital bilateral perisylvian syndrome (438583008)
 
Related genes: ADGRG1, PI4KA
 
HPO: HP:0032407
Monarch Initiative: MONDO:0020340
OMIM®: 300388
Orphanet: ORPHA98889

Definition

Polymicrogyria (PMG) is a malformation of cortical development in which the brain surface is irregular and the normal gyral pattern replaced by multiple small, partly fused gyri separated by shallow sulci. Microscopic examination shows a simplified 4-layered or unlayered cortex. Several patterns of PMG, including bilateral frontal, bilateral perisylvian, and bilateral mesial occipital PMG, have been described on the basis of their topographic distribution. All but the perisylvian form appear to be rare. Bilateral perisylvian PMG (BPP) often results in a typical clinical syndrome that is manifested by mild mental retardation, epilepsy, and pseudobulbar palsy, which causes difficulties with expressive speech and feeding (Kuzniecky et al., 1993). PMG may be a feature of other conditions as well (see, e.g., 300643). [from OMIM]

Clinical features

From HPO
Dysarthria
MedGen UID:
8510
Concept ID:
C0013362
Mental or Behavioral Dysfunction
Dysarthric speech is a general description referring to a neurological speech disorder characterized by poor articulation. Depending on the involved neurological structures, dysarthria may be further classified as spastic, flaccid, ataxic, hyperkinetic and hypokinetic, or mixed.
Pseudobulbar paralysis
MedGen UID:
10989
Concept ID:
C0033790
Disease or Syndrome
Bilateral impairment of the function of the cranial nerves 9-12, which control musculature involved in eating, swallowing, and speech. Pseudobulbar paralysis is characterized clinically by dysarthria, dysphonia, and dysphagia with bifacial paralysis, and may be accompanied by Pseudobulbar behavioral symptoms such as enforced crying and laughing.
Polymicrogyria
MedGen UID:
78605
Concept ID:
C0266464
Congenital Abnormality
Polymicrogyria is a congenital malformation of the cerebral cortex characterized by abnormal cortical layering (lamination) and an excessive number of small gyri (folds).
Cognitive impairment
MedGen UID:
90932
Concept ID:
C0338656
Mental or Behavioral Dysfunction
Abnormal cognition is characterized by deficits in thinking, reasoning, or remembering.
Delayed speech and language development
MedGen UID:
105318
Concept ID:
C0454644
Finding
A degree of language development that is significantly below the norm for a child of a specified age.
Dyslexia
MedGen UID:
96906
Concept ID:
C0476254
Mental or Behavioral Dysfunction
A learning disorder characterized primarily by difficulties in learning to read and spell. Dyslectic children also exhibit a tendency to read words from right to left and to confuse letters such as b and d whose orientation is important for their identification. Children with dyslexia appear to be impaired in phonemic skills (the ability to associate visual symbols with the sounds they represent).
Bilateral tonic-clonic seizure
MedGen UID:
141670
Concept ID:
C0494475
Sign or Symptom
A bilateral tonic-clonic seizure is a seizure defined by a tonic (bilateral increased tone, lasting seconds to minutes) and then a clonic (bilateral sustained rhythmic jerking) phase.
Atypical absence seizure
MedGen UID:
108888
Concept ID:
C0595948
Disease or Syndrome
An atypical absence seizure is a type of generalized non-motor (absence) seizure characterized by interruption of ongoing activities and reduced responsiveness. In comparison to a typical absence seizure, changes in tone may be more pronounced, onset and/or cessation may be less abrupt, and the duration of the ictus and post-ictal recovery may be longer. Although not always available, an EEG often demonstrates slow (<3 Hz), irregular, generalized spike-wave activity.

Conditions with this feature

Rolandic epilepsy, intellectual disability, and speech dyspraxia, X-linked
MedGen UID:
337150
Concept ID:
C1845070
Disease or Syndrome
Megalencephaly-polydactyly syndrome
MedGen UID:
1855924
Concept ID:
C5935591
Disease or Syndrome
Megalencephaly-polydactyly syndrome (MPAPA) is an autosomal dominant disorder characterized by megalencephaly, ventriculomegaly, postaxial polydactyly, and, notably, neuroblastoma during infancy (summary by Nishio et al., 2023).

Professional guidelines

PubMed

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

Yasuda CL, Guimarães CA, Guerreiro MM, Boscariol M, Oliveira EP, Teixeira KC, Costa AL, Beltramini GC, Cendes F
J Neurol 2014 Jul;261(7):1374-80. Epub 2014 Apr 30 doi: 10.1007/s00415-014-7356-1. PMID: 24781839
Saporta AS, Kumar A, Govindan RM, Sundaram SK, Chugani HT
Pediatr Neurol 2011 Apr;44(4):270-4. doi: 10.1016/j.pediatrneurol.2010.11.006. PMID: 21397168
Brandão-Almeida IL, Hage SR, Oliveira EP, Guimarães CA, Teixeira KC, Abramides DV, Montenegro MA, Santos NF, Cendes F, Lopes-Cendes I, Guerreiro MM
Neuropediatrics 2008 Jun;39(3):139-45. Epub 2008 Nov 7 doi: 10.1055/s-0028-1085462. PMID: 18991192
Gropman AL, Barkovich AJ, Vezina LG, Conry JA, Dubovsky EC, Packer RJ
Neuropediatrics 1997 Aug;28(4):198-203. doi: 10.1055/s-2007-973700. PMID: 9309709
Kuzniecky R, Andermann F
AJNR Am J Neuroradiol 1994 Jan;15(1):139-44. PMID: 8141045Free PMC Article

Diagnosis

Bonardi CM, Bayat A, Madsen CG, Hammer TB, Reale C, Gardella E, Marjanovic D, Beniczky S, Møller RS, Rubboli G
Epileptic Disord 2022 Jun 1;24(3):577-582. doi: 10.1684/epd.2022.1423. PMID: 35770758
Chatur C, Balani A
Neurol India 2018 Nov-Dec;66(6):1847-1849. doi: 10.4103/0028-3886.246254. PMID: 30504605
Saporta AS, Kumar A, Govindan RM, Sundaram SK, Chugani HT
Pediatr Neurol 2011 Apr;44(4):270-4. doi: 10.1016/j.pediatrneurol.2010.11.006. PMID: 21397168
Brandão-Almeida IL, Hage SR, Oliveira EP, Guimarães CA, Teixeira KC, Abramides DV, Montenegro MA, Santos NF, Cendes F, Lopes-Cendes I, Guerreiro MM
Neuropediatrics 2008 Jun;39(3):139-45. Epub 2008 Nov 7 doi: 10.1055/s-0028-1085462. PMID: 18991192
Kim HI, Palmini A, Choi HY, Kim YH, Lee JC
J Korean Med Sci 1994 Aug;9(4):335-40. doi: 10.3346/jkms.1994.9.4.335. PMID: 7848582Free PMC Article

Therapy

Junming Z, Yuanyuan Z, Fang F, Weiming F, Ryan H, Jianmin Z, Li F, Xiao J, Shuda C
Turk Neurosurg 2014;24(1):70-4. doi: 10.5137/1019-5149.JTN.6362-12.1. PMID: 24535795
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
Margari L, Presicci A, Ventura P, Buttiglione M, Andreula C, Perniola T
Brain Dev 2005 Jan;27(1):53-7. doi: 10.1016/j.braindev.2004.03.006. PMID: 15626542
Kim HI, Palmini A, Choi HY, Kim YH, Lee JC
J Korean Med Sci 1994 Aug;9(4):335-40. doi: 10.3346/jkms.1994.9.4.335. PMID: 7848582Free PMC Article
Kuzniecky R, Andermann F, Guerrini R
J Child Neurol 1994 Oct;9(4):420-3. doi: 10.1177/088307389400900418. PMID: 7822736

Prognosis

Bonardi CM, Bayat A, Madsen CG, Hammer TB, Reale C, Gardella E, Marjanovic D, Beniczky S, Møller RS, Rubboli G
Epileptic Disord 2022 Jun 1;24(3):577-582. doi: 10.1684/epd.2022.1423. PMID: 35770758
Margari L, Presicci A, Ventura P, Buttiglione M, Andreula C, Perniola T
Brain Dev 2005 Jan;27(1):53-7. doi: 10.1016/j.braindev.2004.03.006. PMID: 15626542
Kuzniecky R, Andermann F, Guerrini R
Neurology 1994 Mar;44(3 Pt 1):379-85. doi: 10.1212/wnl.44.3_part_1.379. PMID: 8145902
Kim HI, Palmini A, Choi HY, Kim YH, Lee JC
J Korean Med Sci 1994 Aug;9(4):335-40. doi: 10.3346/jkms.1994.9.4.335. PMID: 7848582Free PMC Article
Kuzniecky R, Andermann F, Guerrini R
J Child Neurol 1994 Oct;9(4):420-3. doi: 10.1177/088307389400900418. PMID: 7822736

Clinical prediction guides

Margari L, Presicci A, Ventura P, Buttiglione M, Andreula C, Perniola T
Brain Dev 2005 Jan;27(1):53-7. doi: 10.1016/j.braindev.2004.03.006. PMID: 15626542
Yekeler E, Ozmen M, Genchellac H, Dursun M, Acunas G
Pediatr Radiol 2004 Nov;34(11):908-11. Epub 2004 May 28 doi: 10.1007/s00247-004-1221-9. PMID: 15168097
Kuzniecky R, Andermann F, Guerrini R
Neurology 1994 Mar;44(3 Pt 1):379-85. doi: 10.1212/wnl.44.3_part_1.379. PMID: 8145902
Kuzniecky R, Andermann F
AJNR Am J Neuroradiol 1994 Jan;15(1):139-44. PMID: 8141045Free PMC Article
Kim HI, Palmini A, Choi HY, Kim YH, Lee JC
J Korean Med Sci 1994 Aug;9(4):335-40. doi: 10.3346/jkms.1994.9.4.335. PMID: 7848582Free PMC Article

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.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...