Pseudotumor Cerebri Support Group
Pseudotumor cerebri (PTC) is a neurological disorder that is characterized by increased intracranial pressure, in the absence of a tumor or other diseases affecting the brain or its lining. Diagnosis requires brain scans and lumbar puncture. Characteristic symptoms are headache, transient visual obscurations or transient visual loss in one or both eyes usually lasting...
http://www.neurology.org/content/82/11/1011.long
Editors' Note: Commenting on the updated diagnostic criteria for pseudotumor cerebri syndrome (PTCS), De Simone et al. explain why the lumbar puncture (LP) opening pressure (OP) is not sufficient to make the diagnosis of PTCS. Commenting on the same criteria, Dasheiff explains how table 1 in the article may be misleading and should reflect more the association of those conditions cited with PTCS rather than causality. Friedman et al., authors of the criteria, respond.Chafic Karam, MD, and Robert C. Griggs, MD
Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children
Roberto De Simone, Angelo Ranieri and Silvana Montella
+SHOW AFFILIATIONS
doi: 10.1212/WNL.0000000000000200
Neurology March 18, 2014 vol. 82 no. 11 1011-1012
While we appreciate the idiopathic intracranial hypertension (IIH) criteria update by Friedman et al.,1 OP alone does not negate the diagnosis of IIH/IIH without papilledema (IIHWOP). If an OP above 250 mm H2O is required, many cases will be missed.
Intracranial pressure (ICP) monitoring2,,5 demonstrated that ICP shows large diurnal fluctuations in IIH/IIHWOP patients, ranging from normal to definitely pathologic values. Many patients with papilledema fulfilling Friedman criteria AD might be classified as definite or probable depending on the timing of their unstable ICP estimation. Similarly, in chronic headache patients without papilledema presenting with abducens palsy (uncommon also in cases with papilledema) or with a combination of 3 out of 4 suggestive radiologic findings, the diagnosis of IIHWOP will vary from definite to not suggested largely depending on LP timing.
Considering ICP fluctuations and impracticability of routine ICP monitoring, OP, paradoxically, should never constitute the only reason to refute IIH/IIHWOP diagnosis. In a large chronic primary headache series,5 a continuous or intermittent ICP greater than 200 mm H2O was associated with sinus stenosis. We recommend keeping the 200 mm H2O cutoff value at least until the role of venous stenosis in IIH mechanisms is clarified.
Author Response
Deborah I. Friedman, Grant T. Liu and Kathleen B. Digre
We agree with De Simone et al. that LP OP alone is not suitable for making a diagnosis of IIH with or without papilledema and this was one of the main reasons that the new criteria are more stringent. The new criteria require papilledema or a sixth nerve palsy for a definite diagnosis of PTCS and more than one neuroimage result for patients without papilledema. The old cutoff value of 200 mm of CSF was arbitrary and based on a misinterpretation of a previous study that showed values of 250 mm CSF or greater were diagnostic, those less than 200 mm CSF were normal, and values between 200 and 249 mm CSF were nondiagnostic.6 Our cutoff values for OP are based on this previous study in adults with an unquestionable diagnosis of PTCS and are consistent with the previous criteria specifying an OP of 250 mm CSF in adults.7 The values for children are based on a reference range to determine normal OP. Those values were confirmed in a second study showing that most children with papilledema had OP above 280 mm CSF.8,9 Diplopia is more common in patients with papilledema than without papilledema but is sometimes the initial presentation of PTCS before papilledema develops. As De Simone et al. correctly pointed out, the presence of transverse venous sinus stenosis as an isolated neuroradiologic finding does not appear to distinguish true IIH/PTCS from chronic primary headache; thus 2 other accompanying imaging findings must be present to meet the imaging criteria.
Editors' Note: Commenting on the updated diagnostic criteria for pseudotumor cerebri syndrome (PTCS), De Simone et al. explain why the lumbar puncture (LP) opening pressure (OP) is not sufficient to make the diagnosis of PTCS. Commenting on the same criteria, Dasheiff explains how table 1 in the article may be misleading and should reflect more the association of those conditions cited with PTCS rather than causality. Friedman et al., authors of the criteria, respond.Chafic Karam, MD, and Robert C. Griggs, MD
Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children
Roberto De Simone, Angelo Ranieri and Silvana Montella
+SHOW AFFILIATIONS
doi: 10.1212/WNL.0000000000000200
Neurology March 18, 2014 vol. 82 no. 11 1011-1012
While we appreciate the idiopathic intracranial hypertension (IIH) criteria update by Friedman et al.,1 OP alone does not negate the diagnosis of IIH/IIH without papilledema (IIHWOP). If an OP above 250 mm H2O is required, many cases will be missed.
Intracranial pressure (ICP) monitoring2,,5 demonstrated that ICP shows large diurnal fluctuations in IIH/IIHWOP patients, ranging from normal to definitely pathologic values. Many patients with papilledema fulfilling Friedman criteria AD might be classified as definite or probable depending on the timing of their unstable ICP estimation. Similarly, in chronic headache patients without papilledema presenting with abducens palsy (uncommon also in cases with papilledema) or with a combination of 3 out of 4 suggestive radiologic findings, the diagnosis of IIHWOP will vary from definite to not suggested largely depending on LP timing.
Considering ICP fluctuations and impracticability of routine ICP monitoring, OP, paradoxically, should never constitute the only reason to refute IIH/IIHWOP diagnosis. In a large chronic primary headache series,5 a continuous or intermittent ICP greater than 200 mm H2O was associated with sinus stenosis. We recommend keeping the 200 mm H2O cutoff value at least until the role of venous stenosis in IIH mechanisms is clarified.
Author Response
Deborah I. Friedman, Grant T. Liu and Kathleen B. Digre
We agree with De Simone et al. that LP OP alone is not suitable for making a diagnosis of IIH with or without papilledema and this was one of the main reasons that the new criteria are more stringent. The new criteria require papilledema or a sixth nerve palsy for a definite diagnosis of PTCS and more than one neuroimage result for patients without papilledema. The old cutoff value of 200 mm of CSF was arbitrary and based on a misinterpretation of a previous study that showed values of 250 mm CSF or greater were diagnostic, those less than 200 mm CSF were normal, and values between 200 and 249 mm CSF were nondiagnostic.6 Our cutoff values for OP are based on this previous study in adults with an unquestionable diagnosis of PTCS and are consistent with the previous criteria specifying an OP of 250 mm CSF in adults.7 The values for children are based on a reference range to determine normal OP. Those values were confirmed in a second study showing that most children with papilledema had OP above 280 mm CSF.8,9 Diplopia is more common in patients with papilledema than without papilledema but is sometimes the initial presentation of PTCS before papilledema develops. As De Simone et al. correctly pointed out, the presence of transverse venous sinus stenosis as an isolated neuroradiologic finding does not appear to distinguish true IIH/PTCS from chronic primary headache; thus 2 other accompanying imaging findings must be present to meet the imaging criteria.
Was looking at ICD-10 7.1.1. HA attributed to IIH -- still says OP >20 for non-obese, >25 for obese; AND includes the language of "ANY of the following...." Wonder how Friedman reconciles this?
Hopefully that link allows you to access it.
I know I certainly would be excluded....at this point, we'll see what my OP comes back as on Friday.
"In these revised criteria, documentation of an elevated CSF opening pressure is required for the diagnosis of definite PTCS, but the diagnosis of probable PTCS may still be made in an otherwise typical patient if bilateral papilledema is present and the measured opening pressure is not elevated. If papilledema is not present, then the combination of an elevated CSF pressure and a sixth nerve palsy can be used to make the diagnosis of PTCS. Without papilledema or a sixth nerve palsy, a combination of an elevated CSF pressure and the presence of 3 of 4 radiologic criteria can only suggest the diagnosis. Notably, LP is always required in the workup of a patient considered to have PTCS."
On the bright side, it now seems likely that my newer NO has read this article. He seemed like he was behind the times when I was considering the modified Dandy criteria, but in fact he may have been viewing my case (and I arrived sans medical records, oops) from this viewpoint. So at least that would make him smarter than I initially thought, I couldn't figure out how an NO in the prominent position he is in could have such misconceptions but this makes more sense.
What Friedman doesn't do is go on to elaborate what should be done about the "in between" patients, i.e. the people who perhaps do not quite meet her redefined criteria for PTCS but still have evidence of a similar process. I can see the benefit of "cleaning up" the criteria - like she mentions not grouping together idiopathic IH and secondary IH - but what is her suggestion for all the patients who fall short of the criteria, but have a problem along the same lines? Is a new, additional diagnostic category to be added??? In the interim it seems to leave a lot of us SOL.
One sentence I will be highlighting and bringing to my next appt is "Papilledema may be less than expected or even absent if there is preexisting optic atrophy or previously resolved optic disc swelling."
Thanks for sharing!
RN: as you said, I think our NOs may have done better homework than we thought. All adding up. But again, how is this all reconciled with ICD-10 (which uses the mod Dandy)? Are the criteria now classified individually and treated as such? Headaches, tinnitus, etc. such in Case 2 of the report?
SOL indeed. Bah. All my docs were on board with IIH/PTC until I had an MRV followed by angiogram. Now it makes sense.