"Dandy-Walker Syndrome" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
A congenital abnormality of the central nervous system marked by failure of the midline structures of the cerebellum to develop, dilation of the fourth ventricle, and upward displacement of the transverse sinuses, tentorium, and torcula. Clinical features include occipital bossing, progressive head enlargement, bulging of anterior fontanelle, papilledema, ataxia, gait disturbances, nystagmus, and intellectual compromise. (From Menkes, Textbook of Child Neurology, 5th ed, pp294-5)
Descriptor ID |
D003616
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MeSH Number(s) |
C10.228.140.252.300 C10.228.140.602.500 C10.500.205 C16.131.666.205
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Concept/Terms |
Dandy-Walker Syndrome- Dandy-Walker Syndrome
- Dandy Walker Syndrome
- Dandy-Walker Complex
- Dandy Walker Complex
- Dandy-Walker Complices
- Luschka-Magendie Foramina Atresia
- Luschka Magendie Foramina Atresia
- Dandy-Walker Deformity
- Dandy Walker Deformity
- Dandy-Walker Deformities
- Hydrocephalus, Internal, Dandy-Walker Type
- Hydrocephalus, Noncommunicating, Dandy-Walker Type
- Dandy-Walker Malformation
- Dandy Walker Malformation
- Malformation, Dandy-Walker
- Dandy-Walker Cyst
- Cyst, Dandy-Walker
- Cysts, Dandy-Walker
- Dandy-Walker Cysts
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Below are MeSH descriptors whose meaning is more general than "Dandy-Walker Syndrome".
Below are MeSH descriptors whose meaning is more specific than "Dandy-Walker Syndrome".
This graph shows the total number of publications written about "Dandy-Walker Syndrome" by people in this website by year, and whether "Dandy-Walker Syndrome" was a major or minor topic of these publications.
To see the data from this visualization as text,
click here.
Year | Major Topic | Minor Topic | Total |
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1995 | 1 | 1 | 2 |
1996 | 0 | 1 | 1 |
2001 | 1 | 0 | 1 |
2003 | 0 | 1 | 1 |
2004 | 1 | 0 | 1 |
2005 | 1 | 0 | 1 |
2006 | 0 | 1 | 1 |
2008 | 2 | 0 | 2 |
2009 | 1 | 0 | 1 |
2013 | 2 | 0 | 2 |
2016 | 0 | 1 | 1 |
2017 | 1 | 0 | 1 |
2019 | 0 | 1 | 1 |
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click here.
Below are the most recent publications written about "Dandy-Walker Syndrome" by people in Profiles.
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Spatiotemporal expansion of primary progenitor zones in the developing human cerebellum. Science. 2019 10 25; 366(6464):454-460.
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Phenotypic outcomes in Mouse and Human Foxc1 dependent Dandy-Walker cerebellar malformation suggest shared mechanisms. Elife. 2017 01 16; 6.
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A novel rasopathy caused by recurrent de novo missense mutations in PPP1CB closely resembles Noonan syndrome with loose anagen hair. Am J Med Genet A. 2016 09; 170(9):2237-47.
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AP1S2 is mutated in X-linked Dandy-Walker malformation with intellectual disability, basal ganglia disease and seizures (Pettigrew syndrome). Eur J Hum Genet. 2014 Mar; 22(3):363-8.
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Mutations in extracellular matrix genes NID1 and LAMC1 cause autosomal dominant Dandy-Walker malformation and occipital cephaloceles. Hum Mutat. 2013 Aug; 34(8):1075-9.
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FOXC1 is required for normal cerebellar development and is a major contributor to chromosome 6p25.3 Dandy-Walker malformation. Nat Genet. 2009 Sep; 41(9):1037-42.
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Cobblestone-like brain dysgenesis and altered glycosylation in congenital cutis laxa, Debre type. Neurology. 2008 Nov 11; 71(20):1602-8.
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Linkage to chromosome 2q36.1 in autosomal dominant Dandy-Walker malformation with occipital cephalocele and evidence for genetic heterogeneity. Hum Genet. 2008 Apr; 123(3):237-45.
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PHACES association. J Am Acad Dermatol. 2006 Dec; 55(6):1072-4.
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Endoscopic, single-catheter treatment of Dandy-Walker syndrome hydrocephalus: technical case report and review of treatment options. Pediatr Neurosurg. 2005 Sep-Oct; 41(5):264-8.