Diagnostic Criteria for Pseudotumor Cerebri Syndrome (PTCS)

The pseudotumor cerebri syndrome (PTCS) may be primary (idiopathic intracranial hypertension) or arise from an identifiable secondary cause. Characterization of typical neuroimaging abnormalities, clarification of normal opening pressure in children, and features distinguishing the syndrome of intracranial hypertension without papilledema from intracranial hypertension with papilledema have furthered our understanding of this disorder.

Diagnostic Criteria for Pseudotumor Cerebri Syndrome

1. Required for diagnosis of pseudotumor cerebri syndrome

  1. Papilledema
  2. Normal neurologic examination except for cranial nerve abnormalities
  3. Neuroimaging: Normal brain parenchyma without evidence of hydrocephalus, mass, or structural lesion and no abnormal meningeal enhancement on MRI, with and without gadolinium, for typical patients (female and obese), and MRI, with and without gadolinium, and magnetic resonance venography for others; if MRI is unavailable or contraindicated, contrast-enhanced CT may be used
  4. Normal CSF composition
  5. Elevated lumbar puncture opening pressure (≥250 mm CSF in adults and ≥280 mm CSF in children [250 mm CSF if the child is not sedated and not obese]) in a properly performed lumbar puncture

2. Diagnosis of pseudotumor cerebri syndrome without papilledema
In the absence of papilledema, a diagnosis of pseudotumor cerebri syndrome can be made if B–E from above are satisfied, and in addition the patient has a unilateral or bilateral abducens nerve palsy
In the absence of papilledema or sixth nerve palsy, a diagnosis of pseudotumor cerebri syndrome can be suggested but not made if B–E from above are satisfied, and in addition at least 3 of the following neuroimaging criteria are satisfied:

  1. Empty sella
  2. Flattening of the posterior aspect of the globe
  3. Distention of the perioptic subarachnoid space with or without a tortuous optic nerve
  4. Transverse venous sinus stenosis

A diagnosis of pseudotumor cerebri syndrome is definite if the patient fulfills criteria A–E. The diagnosis is considered probable if criteria A–D are met but the measured CSF pressure is lower than specified for a definite diagnosis

 

References:

  1. Burkett JG, Ailani J. An Up to Date Review of Pseudotumor Cerebri Syndrome. Curr Neurol Neurosci Rep. 2018 May 2;18(6):33. [Medline]
  2. Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 2013 Sep 24;81(13):1159-65. [Medline]
  3. Friedman DI. Pseudotumor cerebri. Neurol Clin. 2004 Feb;22(1):99-131. [Medline]

 

Created Apr 30, 2019.