PS9: DANDY–WALKER SYNDROME (DWS)

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AUTHORS
Ausra Bagdonaite 1, Valentinas Mačiulis 1, 3, Gintaras Naujokas 2, Jūratė Lengvenienė 1, Aldona Šiurkutė 1, 3, Bernadeta Viesulaite 4, Sandra Simkunaite 1

1Republican Vilnius Psychiatric Hospital, Vilnius, Lithuania
2Private Limited Company Žemaitijos Mental Health Center, Telšiai Lithuania
3Vilnius University Medical Faculty Psychiatric Clinic, Vilnius, Lithuania
4Klaipeda Seamen’s Hospital, Klaipeda, Lithuania

ABSTRACT DESCRIPTION

Objective: The authors review a clinical case of Dandy-Walker syndrome variant subtype. DWS is a rare group of congenital human brain malformations which affects the cerebellum (cerebellar vermis aplasia or hypoplasia), fourth ventricle enlargement, cyst formation near the internal base of the skull. There are three subtypes of this syndrome – DWS malformation, DWS mega cisterna magna, and DWS variant. While the first two types are considered a severe presentation of the syndrome, the variant type can be silent for years as in this clinical case.

Method: A clinical case description.

Clinic: A seventy nine year old man was hospitalized to the Republican Vilnius Psychiatric hospital in 2013 due to a severe delirium. Hallucinatory behavior, psychomotor agitation was present. Family members reported that the patient for all of his life worked as a tractor driver. In 2006, the patient had several episodes of seizures and after one of them, the patient was examined in the neurology center. An EEG showed waves close to epileptiformic. The head CT showed vascular leukoencephalopathy, cerebellum porencephalic formation (rated as of injuring); brain and cerebellum atrophy. Until late 2012, the patient was able to care for himself – there were no signs of dementia. Dementia appeared and progressed quickly until delirium developed and the patient was hospitalized in the psychiatric hospital.

Results: Delirium was treated and the patient was discharged from the hospital after 9 days of intensive care and treatment. A head CT scan showed Dandy-Walker variant, vascular leukoencephalopathy. MMSE – 14. After treatment, the patient showed signs of dysarthria.

Conclusions: DWS is a rare group congenital brain malformations which manifests with various symptoms according to subtype but usually diagnosed early. This case illustrates DWS variant type which has been silent for years, has not been diagnosed even after head CT was made at neurology center because of symptomatic seizures which occurred and were diagnosed only accidentally when dementia evolved.

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