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Cortical blindness-intellectual disability-polydactyly syndrome

MedGen UID:
347487
Concept ID:
C1857568
Disease or Syndrome
Synonym: Cortical blindness, retardation, and postaxial polydactyly
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
 
Monarch Initiative: MONDO:0009024
OMIM®: 218010
Orphanet: ORPHA1389

Definition

A rare, genetic, multiple congenital anomalies/dysmorphic syndrome characterized by congenital, total, cortical blindness, intellectual disability, postaxial polydactyly of the hands and feet, pre- and postnatal growth delay, psychomotor developmental retardation, and mild facial dysmorphism (incl. prominent forehead, short nose, long philtrum, high-arched palate, and microretrognathia). Recurrent respiratory and intestinal infections, as well as moderate hypertonia and hyperreflexia, are also associated. There have been no further descriptions in the literature since 1985. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCortical blindness-intellectual disability-polydactyly syndrome
Follow this link to review classifications for Cortical blindness-intellectual disability-polydactyly syndrome in Orphanet.

Professional guidelines

PubMed

Qadir N, Sahetya S, Munshi L, Summers C, Abrams D, Beitler J, Bellani G, Brower RG, Burry L, Chen JT, Hodgson C, Hough CL, Lamontagne F, Law A, Papazian L, Pham T, Rubin E, Siuba M, Telias I, Patolia S, Chaudhuri D, Walkey A, Rochwerg B, Fan E
Am J Respir Crit Care Med 2024 Jan 1;209(1):24-36. doi: 10.1164/rccm.202311-2011ST. PMID: 38032683Free PMC Article
Ochani R, Asad A, Yasmin F, Shaikh S, Khalid H, Batra S, Sohail MR, Mahmood SF, Ochani R, Hussham Arshad M, Kumar A, Surani S
Infez Med 2021 Mar 1;29(1):20-36. PMID: 33664170
Thompson AJ, Banwell BL, Barkhof F, Carroll WM, Coetzee T, Comi G, Correale J, Fazekas F, Filippi M, Freedman MS, Fujihara K, Galetta SL, Hartung HP, Kappos L, Lublin FD, Marrie RA, Miller AE, Miller DH, Montalban X, Mowry EM, Sorensen PS, Tintoré M, Traboulsee AL, Trojano M, Uitdehaag BMJ, Vukusic S, Waubant E, Weinshenker BG, Reingold SC, Cohen JA
Lancet Neurol 2018 Feb;17(2):162-173. Epub 2017 Dec 21 doi: 10.1016/S1474-4422(17)30470-2. PMID: 29275977

Recent clinical studies

Etiology

Conrad J, Huppert A, Ruehl RM, Wuehr M, Schniepp R, Zu Eulenburg P
J Neurol 2023 Nov;270(11):5449-5460. Epub 2023 Jul 22 doi: 10.1007/s00415-023-11859-z. PMID: 37480400Free PMC Article
Pitsava G, Stratakis CA
Front Endocrinol (Lausanne) 2022;13:937793. Epub 2022 Aug 3 doi: 10.3389/fendo.2022.937793. PMID: 35992119Free PMC Article
Pattrapornpisut P, Avila-Casado C, Reich HN
Am J Kidney Dis 2021 Sep;78(3):429-441. Epub 2021 Jul 9 doi: 10.1053/j.ajkd.2021.01.024. PMID: 34247883
Rafal RD
Curr Opin Neurobiol 1994 Apr;4(2):231-6. doi: 10.1016/0959-4388(94)90078-7. PMID: 8038582
Doose H
Epilepsy Res Suppl 1992;6:163-8. PMID: 1418479

Diagnosis

Thompson AE, Thompson PD
Handb Clin Neurol 2023;196:443-455. doi: 10.1016/B978-0-323-98817-9.00008-9. PMID: 37620084
Pattrapornpisut P, Avila-Casado C, Reich HN
Am J Kidney Dis 2021 Sep;78(3):429-441. Epub 2021 Jul 9 doi: 10.1053/j.ajkd.2021.01.024. PMID: 34247883
Steinlein OK
Expert Rev Neurother 2009 Jun;9(6):825-33. doi: 10.1586/ern.09.49. PMID: 19496686
Beuschlein F, Reincke M
Ann N Y Acad Sci 2006 Nov;1088:319-34. doi: 10.1196/annals.1366.001. PMID: 17192577
Aubourg P, Scotto J, Rocchiccioli F, Feldmann-Pautrat D, Robain O
J Neurol Neurosurg Psychiatry 1986 Jan;49(1):77-86. doi: 10.1136/jnnp.49.1.77. PMID: 2420940Free PMC Article

Therapy

Kjeldsen T, Hvidt KJ, Bohn MB, Mygind-Klavsen B, Lind M, Semciw AI, Mechlenburg I
Physiotherapy 2024 Jun;123:69-80. Epub 2024 Jan 5 doi: 10.1016/j.physio.2024.01.001. PMID: 38295551
Pattrapornpisut P, Avila-Casado C, Reich HN
Am J Kidney Dis 2021 Sep;78(3):429-441. Epub 2021 Jul 9 doi: 10.1053/j.ajkd.2021.01.024. PMID: 34247883
Hon KL, Leung AKC, Torres AR
Recent Pat Inflamm Allergy Drug Discov 2018;12(2):128-135. doi: 10.2174/1872213X12666180508122450. PMID: 29745347
Andolino TP, Reid-Adam J
Pediatr Rev 2015 Mar;36(3):117-25; quiz 126, 129. doi: 10.1542/pir.36-3-117. PMID: 25733763
dos Santos RG, Hallak JE, Leite JP, Zuardi AW, Crippa JA
J Clin Pharm Ther 2015 Apr;40(2):135-43. Epub 2014 Dec 4 doi: 10.1111/jcpt.12235. PMID: 25475762

Prognosis

Pattrapornpisut P, Avila-Casado C, Reich HN
Am J Kidney Dis 2021 Sep;78(3):429-441. Epub 2021 Jul 9 doi: 10.1053/j.ajkd.2021.01.024. PMID: 34247883
Holden SK, Bettcher BM, Pelak VS
Curr Opin Neurol 2020 Feb;33(1):68-73. doi: 10.1097/WCO.0000000000000767. PMID: 31688098Free PMC Article
Hon KL, Leung AKC, Torres AR
Recent Pat Inflamm Allergy Drug Discov 2018;12(2):128-135. doi: 10.2174/1872213X12666180508122450. PMID: 29745347
Andolino TP, Reid-Adam J
Pediatr Rev 2015 Mar;36(3):117-25; quiz 126, 129. doi: 10.1542/pir.36-3-117. PMID: 25733763
Steinlein OK
Expert Rev Neurother 2009 Jun;9(6):825-33. doi: 10.1586/ern.09.49. PMID: 19496686

Clinical prediction guides

Thompson AE, Thompson PD
Handb Clin Neurol 2023;196:443-455. doi: 10.1016/B978-0-323-98817-9.00008-9. PMID: 37620084
Pattrapornpisut P, Avila-Casado C, Reich HN
Am J Kidney Dis 2021 Sep;78(3):429-441. Epub 2021 Jul 9 doi: 10.1053/j.ajkd.2021.01.024. PMID: 34247883
Andolino TP, Reid-Adam J
Pediatr Rev 2015 Mar;36(3):117-25; quiz 126, 129. doi: 10.1542/pir.36-3-117. PMID: 25733763
Lerario AM, Moraitis A, Hammer GD
Mol Cell Endocrinol 2014 Apr 5;386(1-2):67-84. Epub 2013 Nov 9 doi: 10.1016/j.mce.2013.10.028. PMID: 24220673Free PMC Article
Salloway S, Cummings J
J Neuropsychiatry Clin Neurosci 1994 Spring;6(2):93-9. doi: 10.1176/jnp.6.2.93. PMID: 8044049

Recent systematic reviews

Smart KM, Ferraro MC, Wand BM, O'Connell NE
Cochrane Database Syst Rev 2022 May 17;5(5):CD010853. doi: 10.1002/14651858.CD010853.pub3. PMID: 35579382Free PMC Article
Jacobsen A, Olabi B, Langley A, Beecker J, Mutter E, Shelley A, Worley B, Ramsay T, Saavedra A, Parker R, Stewart F, Pardo Pardo J
Cochrane Database Syst Rev 2022 Mar 11;3(3):CD013130. doi: 10.1002/14651858.CD013130.pub2. PMID: 35274741Free PMC Article
Smart KM, Wand BM, O'Connell NE
Cochrane Database Syst Rev 2016 Feb 24;2(2):CD010853. doi: 10.1002/14651858.CD010853.pub2. PMID: 26905470Free PMC Article
Dong W, Goost H, Lin XB, Burger C, Paul C, Wang ZL, Zhang TY, Jiang ZC, Welle K, Kabir K
Medicine (Baltimore) 2015 Mar;94(10):e510. doi: 10.1097/MD.0000000000000510. PMID: 25761173Free PMC Article
Canavan TN, Mathes EF, Frieden I, Shinkai K
J Am Acad Dermatol 2015 Feb;72(2):239-45. doi: 10.1016/j.jaad.2014.06.026. PMID: 25592340

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