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Título

Late clinical manifestation of Dandy-Walker Syndrome : case report

RESUMO

Presentation
The Dandy-Walkér malformation was described by Virchow in 1863 and Fusari in 1891. However it was Dandy and Taggart and Walker who described the clinical and radiological elements necessary for the perfect characterization of the syndrome. This is an embryonic defect in the posterior fossa with absence from of the 4th ventricle, agenesis
of the lower part of the cerebellar vermis, very high situation of the lateral sinus and cerebellum tent, and dilation of the ventricular system. A case of late manifestation of the disease is reported. Female patient, 44 years old, complaining of insidious impairment of evolution of more than 15 years with progressive ataxia, dysarthria, scant speech, incoordination. Patient with incomplete higher education.

Discussion

Dandy-Walker Syndrome is a rare clinical and radiological disease that most commonly is the subject of study in the pediatric age group. However, this case is presented with a patient who arrived at the university until the last year of advocacy. Because the cerebellum is a vital part of movement, balance and body coordination, many people with Dandy-Walker syndrome have problems with their voluntary muscle movements and coordination. They may also experience difficulties with their motor skills, mood, and behavior, and may have limited intellectual development. About half of people with Dandy-Walker syndrome have a below-average IQ. Despite the classic symptoms, the 44-year-old G.M.O. patient was evaluated with a complaint of insidious ataxia, cognitive impairment, nystagmus, incoordination and scanted speech. He dropped out of college a few months before completing it. It observed losses in the performance of its domestic activities. When, already in his fifth decade of life, he sought medical assistance. Magnetic resonance imaging examination of the brain, at the time of the first visits in December 2019, revealed Dandy-Walker variant in the posterior fossa with limited development of the cerebellar vermis, right and left side of the cerebellum, enlargement of the fourth ventricle, development of a large, cyst-like formation at the base of the skull, where the brain stem and cerebelus are located.

Final Comments:

For most people, the signs and symptoms of Dandy-Walker syndrome are evident at birth or in the first year, but 10 to 20% of individuals may not develop symptoms until late childhood or early adulthood.

Palavras Chave

Dandy-Walker syndrome, late manifestation.