7.1.2 Headache attributed to intracranial hypertension secondary to metabolic, toxic or hormonal cause

Coded elsewhere:

Headache attributed to increased intracranial pressure due to head trauma, vascular disorder or intracranial infection is coded to whichever of these is the cause. Headache attributed to raised intracranial pressure occurring as a side-effect of medication is coded as 8.1.10 Headache attributed to long-term use of non-headache medication.

Description:

Headache caused by intracranial hypertension secondary to any of a variety of systemic disorders and accompanied by other symptoms and/or clinical and/or neuroimaging signs both of the intracranial hypertension and of the underlying causative disorder. It usually remits with resolution of the systemic disorder.

Diagnostic criteria:
  1. 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 metabolic, toxic or hormonal disorder1
  3. Evidence of causation demonstrated by either or both of the following:
    1. headache has developed in temporal relation to the increase in CSF pressure, or led to its discovery
    2. either or both of the following:
      • a) headache has significantly worsened in parallel with increasing CSF pressure
      • b) headache has significantly improved in parallel with reduction in CSF pressure
  4. Not better accounted for by another ICHD-3 diagnosis.
Note:

Potential metabolic, toxic or hormonal causes of intracranial hypertension include acute hepatic failure, renal failure, hypercarbia, acute hypertensive crisis, Reye’s hepatocerebral syndrome, cerebral venous sinus thrombosis, right heart failure, a range of substances (including thyroid hormone as replacement in children, all-trans retinoic acid, retinoids, tetracyclines and chlordecone), vitamin A toxicity and corticosteroid withdrawal.

Comment:

Removal of the inciting agent or treatment of the underlying causative disorder may not be sufficient to normalize the high intracranial pressure; additional treatment is often required to relieve headache and other symptoms and, more importantly, to prevent visual loss.