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How good at neurology are you? — Answers

1. The CT shows a defect in the left squamous temporal bone where the knife has penetrated, and a small sliver of bone lifted from the petrous temporal bone. There is opacity in the mastoid air cells due to blood. Not shown is a small temporal lobe haemorrhage. When you re-examine the patient you realise there is a knife entry point in front of her left ear.
   
   
2. Only (1) is false. Tangier disease is named after an island in Chesapeake Bay, Virginia, USA—home to the first two probands. Nearly all affected children have been identified on the basis of large lobulated yellow-orange tonsils. In adults, when the tonsils have been previously removed, proctoscopy consistently demonstrates a pale rectal mucosa studded with 1–2mm discrete orange-brown spots.
   Adults usually present with a neuropathy. It may have a syringomyelia-like distribution and a slowly progressive course, a multifocal relapsing and remitting pattern, or a crural . . . [Full text of this article]