Diagnostic Criteria for Idiopathic Intracranial Hypertension (IIH)

The combination of raised intracranial pressure, without hydrocephalus or mass lesion, normal cerebrospinal fluid (CSF) composition and where no underlying aetiology is found are accepted criteria for the diagnosis of IIH.

Diagnostic Criteria for Idiopathic Intracranial Hypertension (IIH)

  1. Papilledema
  2. Normal neurological examination (except sixth cranial nerve palsy)
  3. Neuroimaging: normal brain parenchyma (no hydrocephalus, mass, structural lesion, or meningeal enhancement); venous thrombosis excluded in all
  4. Normal cerebrospinal fluid (CSF) constituents
  5. Elevated lumbar puncture pressure ≥25 cm CSF

Idiopathic Intracranial Hypertension Without Papilledema (IIHWOP)
Presence of criteria B–E for IIH plus:

  • Unilateral or bilateral sixth cranial nerve palsy.

Suggestion of possible IIHWOP if:
Presence of criteria B–E for IIH plus:

  • at least three of the following neuroimaging criteria are satisfied:
    • Empty sella
    • Flattening of the posterior aspect of the globe
    • Distention of the perioptic subarachnoid space with or without a tortuous optic nerve
    • Transverse venous sinus stenosis

Headache attributed to IIH (ICHD-3b)

  1. Any headache fulfilling criterion C
  2. IIH has been diagnosed with lumbar puncture pressure ≥25 cm CSF
  3. Evidence of causation demonstrated by at least 2 of:
    1. Headache developed in temporal relation to IIH
    2. Headache relieved by reducing ICP
    3. Headache exacerbated in temporal relationship to increased ICP
  4. Headache not accounted for by another ICHD-3 diagnosis

 

References:

  1. Mollan SP, Davies B, Silver NC, Shaw S, Mallucci CL, Wakerley BR, Krishnan A, Chavda SV, Ramalingam S, Edwards J, Hemmings K, Williamson M, Burdon MA, Hassan-Smith G, Digre K, Liu GT, Jensen RH, Sinclair AJ. Idiopathic intracranial hypertension: consensus guidelines on management. J Neurol Neurosurg Psychiatry. 2018 Oct;89(10):1088-1100. [Medline]
  2. Mollan SP, Hoffmann J, Sinclair AJ. Advances in the understanding of headache in idiopathic intracranial hypertension. Curr Opin Neurol. 2019 Feb;32(1):92-98. [Medline]

 

Created Sep 23, 2019.

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