New headache, or a significant worsening of a pre-existing headache, caused by intracranial hypertension secondary to a chromosomal disorder and accompanied by other symptoms and/or clinical and/or neuroimaging signs both of the intracranial hypertension and of the underlying chromosomal disorder.
- New headache, or a significant worsening1 of a pre-existing headache, fulfilling criteria for 7.1 Headache attributed to increased cerebrospinal fluid (CSF) pressure and criterion C below
- Intracranial hypertension has been attributed to a chromosomal disorder2
- Evidence of causation demonstrated by at least two of the following:
- headache has developed in temporal relation to the intracranial hypertension, or led to its discovery
- headache is relieved by reducing the intracranial hypertension
- Not better accounted for by another ICHD-3 diagnosis.
- ”Significant worsening” implies a two-fold or greater increase in frequency and/or severity in accordance with the general rule on distinguishing secondary from primary headache.
- Chromosomal disorders associated with intracranial hypertension include Turner syndrome and Down syndrome.