Sudden death can occur at any age but the patient is often young.
The presence of premature craniosynostosis may be known or it may be occult depending on when the sutures close.
The cause of death is a sudden increase in intracranial pressure with tonsillar herniation. This can occur with:
(1) head trauma
(2) drugs
(3) alcohol
(4) infection
The patient may present with headache, nausea, vomiting and signs of increased intracranial pressure.
Diagnostic features at autopsy:
(1) cerebral edema with tonsillar herniation
(2) in a child, the absence of suture lines
(3) increased scalloping (convolutional markings) of the internal table of the skull (impressiones digitatae)
(4) relatively small head
(5) exclusions of other causes
