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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.
Abstract:
Dandy-Walker syndrome, a congenital malformation of the hindbrain involving the cerebellum and the fourth ventricle, is a rare cranial abnormality that commonly occurs before the sixth or seventh week of development. It is usually diagnosed at birth or in early childhood; however, an occasional patient may first become symptomatic in adult life. A case of Dandy-Walker syndrome in a 58-year-old woman is reported because of the older age at presentation and relatively long asymptomatic period after birth.
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