ISSN 2398-2969      

Dandy-Walker like syndrome

icanis
Contributor(s):

Mark Lowrie

Laurent Garosi


Introduction

  • Congenital malformations of the cerebellum can include aplasia (absence of cerebellar tissue) and partial agenesis or hypoplasia (partial or uniform lack of cerebellar tissue). 
  • Cerebellar hypoplasia has been associated with infection or toxin exposure during a critical stage of cerebellum development in utero
  • Attempts at amplifying parvovirus from dogs with midline malformation and vermian defects have been unsuccessful. 
  • Dandy-Walker syndrome in people classically defines complete or partial agenesis of the cerebellar vermis with an upward displacement and rotation of the remnants of the vermis, cyst-like dilation of the fourth ventricle and enlargement of the posterior fossa, with upward displacement of the tentorium cerebelli osseum, transverse sinuses and torcula (the confluence of the sinuses). 
  • Caudal vermian hypoplasia is described in some dogs with associated ventricular dilatation and is defined as Dandy-Walker like syndrome.  
  • A causal genetic mutation (VLDLR) for this condition has been identified in the Eurasier breed with recessive inheritance. 

Pathogenesis

Etiology

  • Dandy-Walker like syndrome is a congenital problem Brain: cerebellum - congenital disorders assumed to be associated with abnormal embryogenesis (primarily parenchymal midline developmental field defect of unknown origin). 
  • Represents partial or complete absence of the vermis of the cerebellum. 
  • Associated with these vermal defects is cyst-like dilation of the fourth ventricle. In addition many of these patients have dilated third and lateral ventricles (hydrocephalus), stenosis of the aqueduct, and absence of the corpus callosum. 

Predisposing factors

Specific

Pathophysiology

  • Failure of normal cerebellar development. 
  • There is a malformation resulting in a cyst-like abnormality in the cerebellum. The lateral and third ventricles are commonly dilated concurrently. 
  • In Eurasier dogs, a mutation in the VLDLR gene (very low density lipoprotein receptor) was found to be causative. This gene codes for receptors which influence neuroblast migration in the cerebral cortex and cerebellum. The absence of these receptors results in an underdeveloped central nervous system. The mutation has autosomal recessive inheritance. 

Timecourse

  • Slowly progressive over months in some 
  • Can stabilize and improve in other dogs. 

Diagnosis

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Treatment

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Prevention

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Outcomes

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Further Reading

Publications

Refereed Papers

  • Recent references from PubMed and VetMedResource.
  • Prikryl M, Caine A, Palus V (2020) Transient postural vestibulo-cerebellar syndrome in three dogs with presumed cerebellar hypoplasia. Front Vet Sci 7, 453 www.ncbi.nlm.nih.gov/pmc/articles/PMC7419425/.
  • Kwiatkowska M, Rose J H, Pomianowski A (2019) Dandy-Walker malformation in Polish hunting dogs: long-term prognosis and quality of like. Veterinarni Medicina 64, 37-43.
  • Tamura S, Nakamoto Y, Uemura T et al (2016) Head tilting elicited by head turning in three dogs with hypoplastic cerebellar nodulus and ventral uvula. Front Vet Sci 3, 104 www.ncbi.nlm.nih.gov/pmc/articles/PMC5120117/
  • Gerber M, Fischer A, Jagannathan V, Drögemüller M, Drögemüller C et al (2015) A deletion in the VLDLR gene in Eurasier dogs with cerebellar hypoplasia resembling a Dandy-Walker-like malformation (DWLM). PLoS One 10(2), e0108917 PubMed.
  • Bernardino F, Rentmeister K, Schmidt M J, Brühschwein A, Matiasek K et al (2015) Cerebellar hypoplasia resembling Dandy-Walker-like malformation in purebed Eurasier dogs with familial nonprogressive ataxia: a retrospective and prospective clinical cohort study. PLoS One 10(2), e0117670 PubMed.
  • Kobatake Y, Miyabayashi T, Yada N, Kachi S, Ohta G et al (2013) Magnetic resonance imaging diagnosis of Dandy-Walker-like syndrome in a Wire-haired Miniature Dachshund. J Vet Med Sci 75, 1379-1381 PubMed.
  • Lim J H, Kim D Y, Yoon J H, Kim W H, Kweon O K (2008) Cerebellar vermian hypoplasia in a Cocker Spaniel. J Vet Sci 9, 215-217 PubMed.
  • Schmidt M J, Jawinski S, Wigger A, Kramer M (2008) Imaging diagnosis—Dandy-Walker malformation. Vet Radiol Ultrasound 49, 264-266 PubMed.
  • Noureddine C, Harder R, Olby N J, Spaulding K, Brown T (2004) Ultrasonographic appearance of Dandy-Walker-like syndrome in a Boston Terrier. Vet Radiol Ultrasound 45, 336-339 PubMed.
  • Choi H, Sangkyu K, Seongmok J, Sungwhan C, Kichang L et al (2007) Imaging diagnosis- cerebellar vermis hypoplasia in a miniature Schnauzer. Vet Radiol Ultrasound 48, 129-131 PubMed.
  • Schmid V, Lang J, Wolf M (1992) Dandy-Walker-like syndrome in four dogs: cisternography as a diagnostic aid. JAAHA 28(4), 355-360.
  • Regnier A M, Delahitte M J D, Delisle M B, Dubois G G (1993) Dandy-Walker Syndrome in a Kitten. JAAHA 29, 514-518.
  • Kornegay J N (1986) Cerebellar vermian hypoplasia in dogs. Vet Pathol 23, 374-379 PubMed.
  • Pass D A, Howell J M, Thompson R R (1981) Cerebellar malformation in two dogs and a sheep. Vet Pathol 18, 405-407 PubMed.

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