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Gene Review

DWS  -  dandy-walker syndrome

Homo sapiens

Synonyms: C3DELq22q24, DEL3q22q24
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Disease relevance of DWS


Psychiatry related information on DWS

  • The MDWS model is an extension of the Duifhuis, Willems, and Sluyter pitch meter (DWS) [J. Acoust. Soc. Am. 71, 1568-1580 (1982)] which is a practical implementation of Goldstein's optimum processor theory of pitch perception [J. Acoust. Soc. Am. 54, 1496-1516 (1973)] [6].
  • The Dandy-Walker syndrome is said to be associated with a high incidence of mental retardation and motor dysfunction leading some to suggest termination of the affected fetus in utero [7].

High impact information on DWS


Chemical compound and disease context of DWS


Biological context of DWS

  • The incidence of DWS was 1.0 per 100,000 live births per year [1].
  • The matrixes included 2 natural water samples [National Institute of Standards and Technology Standard Reference Material (NIST SRM) 1643e and NIST SRM 1640], 1 fortified standard solution (TMDA-64), 1 fortified water sample (TM-DWS), and 2 seawater samples (CASS-4 and NASS-5) [14].
  • Prenatal diagnosis of Dandy-Walker syndrome in early pregnancy presenting with increased nuchal translucency and generalized edema at 13 weeks of gestation [15].
  • Data used in this assessment were collected from literature sources and from the Drinking Water Surveillance Program (DWSP) of Ontario. For many common contaminants, there was insufficient environmental exposure (concentration) information in Ontario drinking water and groundwater [16].
  • We studied the blink reflex (especially the late components ipsi- and contralateral to stimulation site, R2 and R2') in 17 patients under 6 years of age; 12 with Chiari malformation, 1 with Dandy-Walker syndrome and 4 with brainstem tumor [17].

Anatomical context of DWS


Associations of DWS with chemical compounds

  • DWS microrheology of a linear polysaccharide [22].
  • Spin-echo images in the coronal, axial, and sagittal planes were obtained in two patients with Aicardi's syndrome and partial agenesis of the corpus callosum, in one patient with Dandy-Walker syndrome, and in two patients with septooptic dysplasia [23].
  • The rare finding of strong periventricular enhancement was observed by means of intraoperative CT cisternography using Iotrolan in a one-month-old boy with Dandy-Walker syndrome associated with occipital cephalocele [24].

Other interactions of DWS


Analytical, diagnostic and therapeutic context of DWS


  1. Dandy-Walker syndrome: incidence in a defined population of Tabuk, Saudi Arabia. Ohaegbulam, S.C., Afifi, H. Neuroepidemiology. (2001) [Pubmed]
  2. Biliary atresia and the Dandy-Walker anomaly in a neonate with 45,X Turner's syndrome. Molland, E.A., Purcell, M. J. Pathol. (1975) [Pubmed]
  3. Magnetic resonance demonstration of intracranial CSF flow in children. Curless, R.G., Quencer, R.M., Katz, D.A., Campanioni, M. Neurology (1992) [Pubmed]
  4. The Dandy-Walker and Arnold-Chiari malformations. Clinical, developmental, and teratological considerations. Gardner, E., O'Rahilly, R., Prolo, D. Arch. Neurol. (1975) [Pubmed]
  5. Ultrasound evaluation of cystic intracranial lesions in the neonate. Mack, L.A., Rumack, C.M., Johnson, M.L. Radiology. (1980) [Pubmed]
  6. Simulation of auditory analysis of pitch: an elaboration on the DWS pitch meter. Scheffers, M.T. The Journal of the Acoustical Society of America. (1983) [Pubmed]
  7. Dandy-Walker syndrome revisited. Maria, B.L., Zinreich, S.J., Carson, B.C., Rosenbaum, A.E., Freeman, J.M. Pediatric neuroscience. (1987) [Pubmed]
  8. Letter: Genesis of Dandy-Walker syndrome. Smith, M.T., Huntington, H.W. N. Engl. J. Med. (1976) [Pubmed]
  9. Classical embryological studies and modern genetic analysis of midbrain and cerebellum development. Zervas, M., Blaess, S., Joyner, A.L. Curr. Top. Dev. Biol. (2005) [Pubmed]
  10. Genomic location and variation of the gene for CRS, a complement binding protein in the M57 strains of Streptococcus pyogenes. Binks, M., McMillan, D., Sriprakash, K.S. Infect. Immun. (2003) [Pubmed]
  11. The neonatal cisterna magna: ultrasonic evaluation. Goodwin, L., Quisling, R.G. Radiology. (1983) [Pubmed]
  12. Aicardi's syndrome; radiologic manifestations. Phillips, H.E., Carter, A.P., Kennedy, J.L., Rosman, N.P., O'Connor, J.F. Radiology. (1978) [Pubmed]
  13. Congenital hydrocephalus mimicking Dandy-Walker syndrome induced by 6-aminonicotinamide injection in pregnant rat. Yamada, H., Oi, S., Tamaki, N., Matsumoto, S., Taomoto, K. Neurol. Med. Chir. (Tokyo) (1991) [Pubmed]
  14. Determination of inorganic arsenic(III) and arsenic(V) in water samples by ion chromatography/inductively coupled plasma-mass spectrometry. Yip, Y.C., Chu, H.S., Yuen, C.F., Sham, W.C. Journal of AOAC International (2007) [Pubmed]
  15. Prenatal diagnosis of Dandy-Walker syndrome in early pregnancy presenting with increased nuchal translucency and generalized edema at 13 weeks of gestation. Chen, S.H., Lin, M.Y., Chang, F.M. Prenat. Diagn. (2003) [Pubmed]
  16. Sources, pathways, and relative risks of contaminants in surface water and groundwater: a perspective prepared for the Walkerton inquiry. Ritter, L., Solomon, K., Sibley, P., Hall, K., Keen, P., Mattu, G., Linton, B. J. Toxicol. Environ. Health Part A (2002) [Pubmed]
  17. Blink reflex in young children with medullary kink in Chiari malformation. Tomita, Y., Tsukamoto, H., Takeshita, K., Takashima, S. Brain Dev. (1991) [Pubmed]
  18. The dysraphic state of the posterior fossa. Clinical review of the Dandy-Walker syndrome and the so-called arachnoid cysts. Carteri, A., Gerosa, M., Gaini, S.M., Villani, R. Journal of neurosurgical sciences. (1979) [Pubmed]
  19. Dandy-Walker syndrome in consecutive siblings: familial hindbrain malformation. Lehman, R.M. Neurosurgery (1981) [Pubmed]
  20. Occipital meningoceles in patients with the Dandy-Walker syndrome. Bindal, A.K., Storrs, B.B., McLone, D.G. Neurosurgery (1991) [Pubmed]
  21. Infratentorial arachnoid cysts. Galassi, E., Tognetti, F., Frank, F., Fagioli, L., Nasi, M.T., Gaist, G. J. Neurosurg. (1985) [Pubmed]
  22. DWS microrheology of a linear polysaccharide. Hemar, Y., Pinder, D.N. Biomacromolecules (2006) [Pubmed]
  23. MRI of corpus callosal syndromes. Curnes, J.T., Laster, D.W., Koubek, T.D., Moody, D.M., Ball, M.R., Witcofski, R.L. AJNR. American journal of neuroradiology. (1986) [Pubmed]
  24. Periventricular enhancement following intraoperative CT cisternography in a patient with Dandy-Walker syndrome. Case report. Koyama, T., Okudera, H., Tada, T., Wada, N., Kobayashi, S. Neurosurgical review. (1997) [Pubmed]
  25. Application of transmission diffusing wave spectroscopy to the study of gelation of milk by acidification and rennet. Alexander, M., Dalgleish, D.G. Colloids and surfaces. B, Biointerfaces. (2004) [Pubmed]
  26. Bilateral cystic nephroblastomas and multiple malformations with trisomy 8 mosaicism. Nakamura, Y., Nakashima, H., Fukuda, S., Hashimoto, T., Maruyama, M. Hum. Pathol. (1985) [Pubmed]
  27. Cerebellar vermian defects: antenatal sonographic appearance and clinical significance. Keogan, M.T., DeAtkine, A.B., Hertzberg, B.S. Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine. (1994) [Pubmed]
  28. Old age presentation of the Dandy-Walker syndrome associated with unilateral sudden sensorineural deafness and vertigo. Freeman, S.R., Jones, P.H. The Journal of laryngology and otology. (2002) [Pubmed]
  29. Incidence of short stature in children with hydrocephalus. Klauschie, J., Rose, S.R. Journal of pediatric endocrinology & metabolism : JPEM. (1996) [Pubmed]
  30. Dandy-walker syndrome diagnosed in utero by ultrasonography. Newman, G.C., Buschi, A.I., Sugg, N.K., Kelly, T.E., Miller, J.Q. Neurology (1982) [Pubmed]
  31. Dandy-Walker syndrome studied by computed tomography and pneumoencephalography. Masdeu, J.C., Dobben, G.D., Azar-Kia, B. Radiology. (1983) [Pubmed]
  32. Brainstem tethering in Dandy-Walker syndrome: a complication of cystoperitoneal shunting. Case report. Liu, J.C., Ciacci, J.D., George, T.M. J. Neurosurg. (1995) [Pubmed]
  33. The Dandy-Walker syndrome. Relevance to anaesthesia and intensive care. Ewart, M.C., Oh, T.E. Anaesthesia. (1990) [Pubmed]
  34. Enlarged fetal cisterna magna: appearance and significance. Comstock, C.H., Boal, D.B. Obstetrics and gynecology. (1985) [Pubmed]
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