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Cerebellar Cognitive Affective Syndrome in Dandy-Walker Variant Disorder
Heiko Graf, M.D.; Beate Franke, M.D.; Birgit Abler, M.D.
The Journal of Neuropsychiatry and Clinical Neurosciences 2013;25:E45-E46. doi:10.1176/appi.neuropsych.12070179
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Dr. Graf, Dr. Franke, and Dr. Abler report no financial or other relationship relevant to the subject of this article.

Dept. of Psychiatry
Ulm University Hospital
Ulm University
Ulm, Germany

Corresponding author: Heiko Graf, M.D. Department of Psychiatry Ulm University Leimgrubenweg 12-14 89075 Ulm Germany Phone: + 49 731 500 61553 Fax: + 49 731 500 61402 E-mail: heiko.graf@uni-ulm.de

Copyright © 2013 American Psychiatric Association


To the Editor: The Dandy-Walker malformation (DWM) is a congenital malformation characterized by the following triad: hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle, and enlarged posterior fossa. The DWM, the Dandy-Walker variant (DWV) without enlargement of the posterior fossa, and the megacisterna magna are classified as a continuum of developmental anomalies termed as Dandy-Walker complex (DWC).1 Clinical manifestations are dominated by neurological symptoms, developmental delays, and vegetative symptoms. Besides mental retardation, psychiatric symptoms are rarely mentioned in psychiatric literature. A few case reports describe bipolar/affective, psychotic, and attention-deficit disorders in DWM-patients.2 However, whether psychiatric symptoms occur incidentally or are indeed integral to the syndrome, remains unclear. As recent literature supports a prominent role of the cerebellum in the organization of higher-order functions,3 one might speculate about psychiatric symptoms as an integral feature. Here, we present a case of a DWV patient who was formerly diagnosed with comorbid intellectual disability, impulse-control problems, and attention-deficit disorder.

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FIGURE 1. MR Scan of Patient With Dandy-Walker Variant

[A] shows enlargement of the fourth ventricle (white arrow) and mild dilatation of other cerebral spinal fluid spaces, but no enlargement of the posterior fossa. [B] shows hypoplasia of the cerebellar vermis (white arrow).



Sasaki-Adams  D;  Elbabaa  SK;  Jewells  V  et al:  The Dandy-Walker variant: a case series of 24 pediatric patients and evaluation of associated anomalies, incidence of hydrocephalus, and developmental outcomes.  J Neurosurg Pediatr 2008; 2:194–199
[CrossRef] | [PubMed]
Tadakamadla  J;  Kumar  S;  Mamatha  GP:  Dandy-Walker malformation: an incidental finding.  Indian J Hum Genet 2010; 16:33–35
[CrossRef] | [PubMed]
Tavano  A;  Grasso  R;  Gagliardi  C  et al:  Disorders of cognitive and affective development in cerebellar malformations.  Brain 2007; 130:2646–2660
[CrossRef] | [PubMed]
Schmahmann  JD:  The role of the cerebellum in cognition and emotion: personal reflections since 1982 on the dysmetria of thought hypothesis, and its historical evolution from theory to therapy.  Neuropsychol Rev 2010; 20:236–260
[CrossRef] | [PubMed]
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