Dandy-Walker syndrome: presentation of the congenital malformation in an older patient

P Reddy1, P S Vannemreddy, L Grier

  • 1Department of Internal Medicine, Louisiana State University Health Services Center, Shreveport, USA.

Insights

Dandy-Walker syndrome, a rare hindbrain malformation, can present in adulthood. This case highlights a 58-year-old woman diagnosed late, emphasizing extended asymptomatic periods for this congenital condition.

Area of Science:

  • Neuroscience
  • Developmental Biology
  • Genetics

Background:

  • Dandy-Walker syndrome is a congenital hindbrain malformation affecting the cerebellum and fourth ventricle.
  • It typically arises early in fetal development (before week 7) and is often diagnosed in infancy or childhood.

Observation:

  • A rare case of Dandy-Walker syndrome presenting in a 58-year-old woman is detailed.
  • This presentation is notable due to the patient's advanced age at diagnosis.

Findings:

  • The patient experienced a prolonged asymptomatic period following birth.
  • Late-onset symptomatic Dandy-Walker syndrome is possible, challenging typical diagnostic timelines.

Implications:

  • This case expands the known clinical spectrum of Dandy-Walker syndrome.
  • It suggests the need for considering Dandy-Walker syndrome in adult patients with unexplained neurological symptoms.
  • Further research into factors influencing the timing of Dandy-Walker syndrome symptom onset is warranted.

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