BMJ Case Reports 2012; doi:10.1136/bcr-2012-007152
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Ataxia, hypoplastic vermis and keyhole connection sign: is it the variant form of Dandy-Walker complex?

  1. Osama S M Amin
  1. Department of Neurology, Sulaimaniya General Teaching Hospital, Sulaimaniya City, Iraq
  1. Correspondence to Dr Osama S M Amin, dr.osama.amin{at}


A 12-year-old girl has a progressive instability of stance and gait since the age of 2 years. She demonstrates mild mental subnormality and signs of cerebellar incoordination. A diagnosis of an ataxic form of cerebral palsy was made at the age of 3 years. When she was 9 years old, she started to develop occasional vomiting and headache which were ascribed to ‘hydrocephalus’. Accordingly, shunting surgery was performed (figure 1). Both headaches and vomiting remarkably improved; however, the cerebellar dysfunction remained. Her up-to-date brain MRI is shown (figure 2).

Figure 1

Axial non-contrast CT brain scan of the patient, which was done 3 years ago. Note the site of the shunt (yellow arrow), which was placed …

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