7.1.3 Headache attributed to intracranial hypertension secondary to chromosomal disorder

Description:

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.

Diagnostic criteria:
  1. 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
  2. Intracranial hypertension has been attributed to a chromosomal disorder2
  3. Evidence of causation demonstrated by at least two of the following:
    1. headache has developed in temporal relation to the intracranial hypertension, or led to its discovery
    2. headache is relieved by reducing the intracranial hypertension
    3. papilloedema
  4. Not better accounted for by another ICHD-3 diagnosis.
Notes:
  1. ”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.
  2. Chromosomal disorders associated with intracranial hypertension include Turner syndrome and Down syndrome.