Recurrent Idiopathic Intracranial Hypertension Dear Editor: Idiopathic intracranial hypertension (IIH), as defined using the modified Dandy criteria, is characterized by elevated intracra- nial pressure in the setting of a normal neuroimaging study and normal cerebrospinal fluid composition. 1 We review the Uni- versity of Iowa experience for recurrent cases of IIH within 10 years’ follow-up to generate hypotheses about possible precip- itating factors for recurrence (e.g., weight gain, pregnancy, hypertension, sleep apnea, medications). A retrospective chart review was conducted of all pa- tients with the diagnosis of intracranial hypertension from January 1982 to January 2006 at the University of Iowa Hospitals and Clinics, followed at the H. Stanley Thompson Neuro-ophthalmology Clinic. This study was performed with institutional review board approval. See Tables 1 and 2 (all tables available at http://aaojournal.org) for our study’s inclusion criteria and exclusion criteria, respectively. The charts were reviewed for demographic information, symptoms, associated potential risk factors, severity of dis- ease and visual outcome at both diagnosis and last follow-up (i.e., grade of edema, visual acuity, and visual field evaluation), compliance and tolerance of medical ther- apy, and treatment of recurrence (medical, surgical). Of the 810 charts reviewed with a presumed diagnosis of IIH, only 605 were of adult patients who satisfied the modified Dandy criteria. Of these, 476 had follow-up of 3 months and 410 had follow-up of 6 months. Of 410 patients, 34 (8.3%) had recurrence, as defined in our study (Table 1). Of these 34 recurrent IIH patients, 20% (7/34) recurred in the first year of follow-up, 65% (22/34) recurred from 1 to 5 years’ follow-up, and 15% (5/34) recurred from 5 to 10 years after follow-up. In our patients, the average patient age was 30 years (standard deviation 11), and 91% were female (31/34). The other clinical findings in these patients are described in Table 3. Of these, 80% (27/34) were retrospectively deemed to have been poorly compliant or noncompliant with treatment or intolerant to medical therapy. Patients were defined as intolerant to medi- cation for IIH if they were labeled intolerant in the chart and the medical record indicated a discontinuation of the medica- tion because of side effects. Likewise, patients were defined as noncompliant if there was specific notation in the chart that they were not taking their medication regularly or otherwise deemed poorly compliant or noncompliant in the chart. In addition, 44% (15/34) retrospectively reported weight gain before the recurrence, and 15% (5/34) had become pregnant before the recurrence. Interestingly, 12% (4/34) of our recurrent IIH patients reported a weight decrease before recurrence and 18% (6/34) reported some weight loss dur- ing the follow-up period. Four (12%) charts did not report weight gain or loss. Five (15%) patients had sleep apnea, of whom 2 were treated. Eight (23%) patients had hyperten- sion as a comorbidity. Two patients (6%) experienced a recurrence associated with exposure to a medication that might produce IIH (tetracycline [1] and minocycline [1]). Recurrence of IIH is relatively uncommon but not rare. The recurrence rate at our institution was comparable to the 10% recurrence rate in prior studies. 2– 4 In our series, most recur- rences were single events and typically occurred within 2 to 5 years of the original diagnosis of IIH. This finding is similar to those of other studies. The clinical symptoms and signs that were observed were similar to typical IIH as reported in the literature. 2,5 Both medical and surgical treatments for our re- current cohort were similar to the nonrecurrent IIH patients at our institution. In our study, 80% of the patients who recurred were retrospectively noted to be poorly compliant or noncom- pliant with treatment or intolerant to medical therapy. Weight gain was in 44% of our patients, and 15% (5/34) were pregnant at the time of recurrence. Although sleep apnea, hypertension, and exposure to tetracycline derivatives were noted as possible comorbidities for recurrence, a causal relationship can not be established for these factors based upon our retrospective re- view. We recognize the limitations of our work in Table 4. Despite the limitations of a retrospective review, 8.3% of our patients had a recurrence. Noncompliance or intolerance to medical therapy, weight gain, and pregnancy may be factors associated with an increased risk of recurrence. OTAR TAKTAKISHVILI, BS VINAY A. SHAH, MD REZA SHAHBAZ, BS ANDREW GO LEE, MD Iowa City, Iowa References 1. Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri. Population studies in Iowa and Louisiana. Arch Neu- rol 1988;45:875–7. 2. Kesler A, Hadayer A, Goldhammer Y, et al. Idiopathic intra- cranial hypertension: risk of recurrences. Neurology 2004;63: 1737–9. 3. Weisberg LA. Benign intracranial hypertension. Medicine (Baltimore) 1975;54:197–207. 4. Johnston I, Paterson A. Benign intracranial hypertension. I. Diagnosis and prognosis. Brain 1974;97:289 –300. 5. Radhakrishnan K, Thacker AK, Bohlaga NH, et al. Epidemi- ology of idiopathic intracranial hypertension: a prospective and case-control study. J Neurol Sci 1993;116:18 –28. 221
Welcome message from author
Hi everyone! Is this article helpful? Leave a comment!
Transcript
doi:10.1016/j.ophtha.2007.06.041Recurrent Idiopathic Intracranial Hypertension
Dear Editor: Idiopathic intracranial hypertension (IIH), as defined using the modified Dandy criteria, is characterized by elevated intracra- nial pressure in the setting of a normal neuroimaging study and normal cerebrospinal fluid composition.1 We review the Uni- versity of Iowa experience for recurrent cases of IIH within 10 years’ follow-up to generate hypotheses about possible precip- itating factors for recurrence (e.g., weight gain, pregnancy, hypertension, sleep apnea, medications).
A retrospective chart review was conducted of all pa- tients with the diagnosis of intracranial hypertension from January 1982 to January 2006 at the University of Iowa Hospitals and Clinics, followed at the H. Stanley Thompson Neuro-ophthalmology Clinic. This study was performed with institutional review board approval. See Tables 1 and 2 (all tables available at http://aaojournal.org) for our study’s inclusion criteria and exclusion criteria, respectively.
The charts were reviewed for demographic information, symptoms, associated potential risk factors, severity of dis- ease and visual outcome at both diagnosis and last follow-up (i.e., grade of edema, visual acuity, and visual field evaluation), compliance and tolerance of medical ther- apy, and treatment of recurrence (medical, surgical).
Of the 810 charts reviewed with a presumed diagnosis of IIH, only 605 were of adult patients who satisfied the modified Dandy criteria. Of these, 476 had follow-up of 3 months and 410 had follow-up of 6 months. Of 410 patients, 34 (8.3%) had recurrence, as defined in our study (Table 1). Of these 34 recurrent IIH patients, 20% (7/34) recurred in the first year of follow-up, 65% (22/34) recurred from 1 to 5 years’ follow-up, and 15% (5/34) recurred from 5 to 10 years after follow-up. In our patients, the average patient age was 30 years (standard deviation 11), and 91% were female (31/34). The other clinical findings in these patients are described in Table 3. Of these, 80% (27/34) were retrospectively deemed to have been poorly compliant or noncompliant with treatment or intolerant to medical therapy. Patients were defined as intolerant to medi- cation for IIH if they were labeled intolerant in the chart and the medical record indicated a discontinuation of the medica- tion because of side effects. Likewise, patients were defined as noncompliant if there was specific notation in the chart that they were not taking their medication regularly or otherwise deemed poorly compliant or noncompliant in the chart.
In addition, 44% (15/34) retrospectively reported weight gain before the recurrence, and 15% (5/34) had become
our recurrent IIH patients reported a weight decrease before recurrence and 18% (6/34) reported some weight loss dur- ing the follow-up period. Four (12%) charts did not report weight gain or loss. Five (15%) patients had sleep apnea, of whom 2 were treated. Eight (23%) patients had hyperten- sion as a comorbidity. Two patients (6%) experienced a recurrence associated with exposure to a medication that might produce IIH (tetracycline [1] and minocycline [1]).
Recurrence of IIH is relatively uncommon but not rare. The recurrence rate at our institution was comparable to the 10% recurrence rate in prior studies.2–4 In our series, most recur- rences were single events and typically occurred within 2 to 5 years of the original diagnosis of IIH. This finding is similar to those of other studies. The clinical symptoms and signs that were observed were similar to typical IIH as reported in the literature.2,5 Both medical and surgical treatments for our re- current cohort were similar to the nonrecurrent IIH patients at our institution. In our study, 80% of the patients who recurred were retrospectively noted to be poorly compliant or noncom- pliant with treatment or intolerant to medical therapy. Weight gain was in 44% of our patients, and 15% (5/34) were pregnant at the time of recurrence. Although sleep apnea, hypertension, and exposure to tetracycline derivatives were noted as possible comorbidities for recurrence, a causal relationship can not be established for these factors based upon our retrospective re- view.
We recognize the limitations of our work in Table 4. Despite the limitations of a retrospective review, 8.3% of our patients had a recurrence. Noncompliance or intolerance to medical therapy, weight gain, and pregnancy may be factors associated with an increased risk of recurrence.
OTAR TAKTAKISHVILI, BS VINAY A. SHAH, MD REZA SHAHBAZ, BS ANDREW GO LEE, MD Iowa City, Iowa
References
1. Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri. Population studies in Iowa and Louisiana. Arch Neu- rol 1988;45:875–7.
2. Kesler A, Hadayer A, Goldhammer Y, et al. Idiopathic intra- cranial hypertension: risk of recurrences. Neurology 2004;63: 1737–9.
3. Weisberg LA. Benign intracranial hypertension. Medicine (Baltimore) 1975;54:197–207.
4. Johnston I, Paterson A. Benign intracranial hypertension. I. Diagnosis and prognosis. Brain 1974;97:289–300.
5. Radhakrishnan K, Thacker AK, Bohlaga NH, et al. Epidemi- ology of idiopathic intracranial hypertension: a prospective
pregnant before the recurrence. Interestingly, 12% (4/34) of and case-control study. J Neurol Sci 1993;116:18–28.
Table 1. Inclusion
1. Clinical diagnosis of IIH and satisfaction of the modified Dandy criter 2. Documented raised ICP (opening pressure 20 cm of water) when m
possible. 3. Normal CSF analysis. 4. Normal cranial imaging with CT scan or MRI. 5. No other identifiable cause for the raised ICP. 6. Pediatric patients were excluded from the study (age 18 yrs). 7. Definitions of recurrence of IIH in our study. (1) Recurrence of optic dis
as documented in the chart after at least 3 mos (and 2 separate exami (grade 0 on the Frisen scale). The return of disc edema was document (by at least 1 Frisen grade) of previously stable optic disc edema for at recurrences were usually associated with return of symptoms known to transient visual obscurations) that had previously subsided as documen treatment during the recurrence.
8. Follow-up of at least 6 mos and at least 2 visits at the neuro-ophthalm
CSF cerebrospinal fluid; CT computed tomography; ICP intracr resonance imaging.
Table 2. Ex
1. Lack of a clinical diagnosis of IIH or inadequate satisfaction of the mo lack of documented opening pressure 20 cm of water; abnormal CS with CT scan or MRI with evidence of ventriculomegaly or a structur raised ICP.
2. Lack of recurrent optic disc edema. 3. Recurrent optic disc edema 3 mos after ophthalmoscopic resolution
delayed worsening rather than recurrence in our study.
CSF cerebrospinal fluid; CT computed tomography; ICP intracr
Criteria for the Study
ia for IIH. easured with the patient relaxed and in the lateral decubitus position, if
c edema in one or both eyes of a patient with or without return of symptoms nations) of ophthalmoscopic evidence of resolution of optic disc edema ed on at least 2 consecutive neuro-ophthalmic evaluations. (2) An increase least 3 mos and on at least 2 separate neuro-ophthalmic evaluations. These be associated with raised ICP (headache, pulse synchronous tinnitus, ted in the medical records. The patient may or may not have been on
ology clinic.
clusion Criteria
dified Dandy criteria for IIH. Symptoms and signs not consistent with ICP; F analysis (e.g., CSF protein, cell count, glucose); abnormal cranial imaging al cause for raised ICP; or presence of a secondary identifiable cause for the
of optic disc edema to baseline disc appearance, which was defined as
anial pressure; IIH idiopathic intracranial hypertension; MRI magnetic
resonance imaging.
Table 3. Clinical Features of Patients with Recurrent Idiopathic Intracranial Hypertension (IIH)
1. Average opening pressure at diagnosis was 36 cm of water (SD 9.3) 2. Average initial visual acuity, 20/32; final visual acuity, 20/25 3. Average Frisen grade of papilledema at the initial visit was 1.8 (SD 0.98), and the average of the maximum grade of papilledema was 2.3
(SD 1.15) 4. The presenting symptoms of patients with recurrent IIH were similar to those of typical IIH: headache (83%), pulse-synchronous tinnitus (52%),
transient visual obscurations (42%), and diplopia (24%)
SD standard deviation.
Table 4. Study Limitations
1. Despite the large number of patients with IIH at the University of Iowa, we were able to include only 34 charts with a documented recurrence of IIH. The University of Iowa being a tertiary care center and a center with a large referral area, many of our patients come for a second opinion or for primary diagnosis and then are locally observed and treated. This may account for the lower incidence of recurrence rate in our population.
2. Due to the retrospective nature of our study, we included only patients who had stability of the disease over 2 visits at least 3 mos apart. Thus, patients who were “cured” or stable at the last visit, who had an increase in disc edema with return of symptoms over the next visit, who had over 3 mos of stability, or who were observed locally were not included. This may account for the lower recurrence rate in the study.
3. All of the other limitations of a retrospective study, including ascertainment and selection bias, lack of follow-up, and lack of standardization of data collection.
4. Although intolerance or noncompliance with medical therapy was reported in 80% of our recurrences, this might be due to observer bias or recollection bias, and there was no standardized definition of either intolerance or noncompliance in our retrospective review.
5. Likewise, although weight gain and pregnancy are biologically plausible triggers for recurrent IIH, observer bias, recollection bias, and selection bias are limiting factors. Idiopathic intracranial hypertension is a disease of young obese females who are of childbearing age, and a certain percentage of the cohort would be expected by chance alone to be pregnant at the time of a recurrence of IIH. Two of our patients had tetracycline use before the recurrence, but we specifically excluded patients with IIH presumed secondary to medication, and thus, these 2 cases are not true rechallenge cases and cannot determine a cause-and-effect relationship. Although we believe that our retrospective work can generate hypotheses for possible risk factors for recurrent IIH, a case–control study might be better able to define a true cause-and-effect relationship.
CSF cerebrospinal fluid; CT computed tomography; ICP intracranial pressure; IIH idiopathic intracranial hypertension; MRI magnetic
resonance imaging.
Dear Editor: Idiopathic intracranial hypertension (IIH), as defined using the modified Dandy criteria, is characterized by elevated intracra- nial pressure in the setting of a normal neuroimaging study and normal cerebrospinal fluid composition.1 We review the Uni- versity of Iowa experience for recurrent cases of IIH within 10 years’ follow-up to generate hypotheses about possible precip- itating factors for recurrence (e.g., weight gain, pregnancy, hypertension, sleep apnea, medications).
A retrospective chart review was conducted of all pa- tients with the diagnosis of intracranial hypertension from January 1982 to January 2006 at the University of Iowa Hospitals and Clinics, followed at the H. Stanley Thompson Neuro-ophthalmology Clinic. This study was performed with institutional review board approval. See Tables 1 and 2 (all tables available at http://aaojournal.org) for our study’s inclusion criteria and exclusion criteria, respectively.
The charts were reviewed for demographic information, symptoms, associated potential risk factors, severity of dis- ease and visual outcome at both diagnosis and last follow-up (i.e., grade of edema, visual acuity, and visual field evaluation), compliance and tolerance of medical ther- apy, and treatment of recurrence (medical, surgical).
Of the 810 charts reviewed with a presumed diagnosis of IIH, only 605 were of adult patients who satisfied the modified Dandy criteria. Of these, 476 had follow-up of 3 months and 410 had follow-up of 6 months. Of 410 patients, 34 (8.3%) had recurrence, as defined in our study (Table 1). Of these 34 recurrent IIH patients, 20% (7/34) recurred in the first year of follow-up, 65% (22/34) recurred from 1 to 5 years’ follow-up, and 15% (5/34) recurred from 5 to 10 years after follow-up. In our patients, the average patient age was 30 years (standard deviation 11), and 91% were female (31/34). The other clinical findings in these patients are described in Table 3. Of these, 80% (27/34) were retrospectively deemed to have been poorly compliant or noncompliant with treatment or intolerant to medical therapy. Patients were defined as intolerant to medi- cation for IIH if they were labeled intolerant in the chart and the medical record indicated a discontinuation of the medica- tion because of side effects. Likewise, patients were defined as noncompliant if there was specific notation in the chart that they were not taking their medication regularly or otherwise deemed poorly compliant or noncompliant in the chart.
In addition, 44% (15/34) retrospectively reported weight gain before the recurrence, and 15% (5/34) had become
our recurrent IIH patients reported a weight decrease before recurrence and 18% (6/34) reported some weight loss dur- ing the follow-up period. Four (12%) charts did not report weight gain or loss. Five (15%) patients had sleep apnea, of whom 2 were treated. Eight (23%) patients had hyperten- sion as a comorbidity. Two patients (6%) experienced a recurrence associated with exposure to a medication that might produce IIH (tetracycline [1] and minocycline [1]).
Recurrence of IIH is relatively uncommon but not rare. The recurrence rate at our institution was comparable to the 10% recurrence rate in prior studies.2–4 In our series, most recur- rences were single events and typically occurred within 2 to 5 years of the original diagnosis of IIH. This finding is similar to those of other studies. The clinical symptoms and signs that were observed were similar to typical IIH as reported in the literature.2,5 Both medical and surgical treatments for our re- current cohort were similar to the nonrecurrent IIH patients at our institution. In our study, 80% of the patients who recurred were retrospectively noted to be poorly compliant or noncom- pliant with treatment or intolerant to medical therapy. Weight gain was in 44% of our patients, and 15% (5/34) were pregnant at the time of recurrence. Although sleep apnea, hypertension, and exposure to tetracycline derivatives were noted as possible comorbidities for recurrence, a causal relationship can not be established for these factors based upon our retrospective re- view.
We recognize the limitations of our work in Table 4. Despite the limitations of a retrospective review, 8.3% of our patients had a recurrence. Noncompliance or intolerance to medical therapy, weight gain, and pregnancy may be factors associated with an increased risk of recurrence.
OTAR TAKTAKISHVILI, BS VINAY A. SHAH, MD REZA SHAHBAZ, BS ANDREW GO LEE, MD Iowa City, Iowa
References
1. Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri. Population studies in Iowa and Louisiana. Arch Neu- rol 1988;45:875–7.
2. Kesler A, Hadayer A, Goldhammer Y, et al. Idiopathic intra- cranial hypertension: risk of recurrences. Neurology 2004;63: 1737–9.
3. Weisberg LA. Benign intracranial hypertension. Medicine (Baltimore) 1975;54:197–207.
4. Johnston I, Paterson A. Benign intracranial hypertension. I. Diagnosis and prognosis. Brain 1974;97:289–300.
5. Radhakrishnan K, Thacker AK, Bohlaga NH, et al. Epidemi- ology of idiopathic intracranial hypertension: a prospective
pregnant before the recurrence. Interestingly, 12% (4/34) of and case-control study. J Neurol Sci 1993;116:18–28.
Table 1. Inclusion
1. Clinical diagnosis of IIH and satisfaction of the modified Dandy criter 2. Documented raised ICP (opening pressure 20 cm of water) when m
possible. 3. Normal CSF analysis. 4. Normal cranial imaging with CT scan or MRI. 5. No other identifiable cause for the raised ICP. 6. Pediatric patients were excluded from the study (age 18 yrs). 7. Definitions of recurrence of IIH in our study. (1) Recurrence of optic dis
as documented in the chart after at least 3 mos (and 2 separate exami (grade 0 on the Frisen scale). The return of disc edema was document (by at least 1 Frisen grade) of previously stable optic disc edema for at recurrences were usually associated with return of symptoms known to transient visual obscurations) that had previously subsided as documen treatment during the recurrence.
8. Follow-up of at least 6 mos and at least 2 visits at the neuro-ophthalm
CSF cerebrospinal fluid; CT computed tomography; ICP intracr resonance imaging.
Table 2. Ex
1. Lack of a clinical diagnosis of IIH or inadequate satisfaction of the mo lack of documented opening pressure 20 cm of water; abnormal CS with CT scan or MRI with evidence of ventriculomegaly or a structur raised ICP.
2. Lack of recurrent optic disc edema. 3. Recurrent optic disc edema 3 mos after ophthalmoscopic resolution
delayed worsening rather than recurrence in our study.
CSF cerebrospinal fluid; CT computed tomography; ICP intracr
Criteria for the Study
ia for IIH. easured with the patient relaxed and in the lateral decubitus position, if
c edema in one or both eyes of a patient with or without return of symptoms nations) of ophthalmoscopic evidence of resolution of optic disc edema ed on at least 2 consecutive neuro-ophthalmic evaluations. (2) An increase least 3 mos and on at least 2 separate neuro-ophthalmic evaluations. These be associated with raised ICP (headache, pulse synchronous tinnitus, ted in the medical records. The patient may or may not have been on
ology clinic.
clusion Criteria
dified Dandy criteria for IIH. Symptoms and signs not consistent with ICP; F analysis (e.g., CSF protein, cell count, glucose); abnormal cranial imaging al cause for raised ICP; or presence of a secondary identifiable cause for the
of optic disc edema to baseline disc appearance, which was defined as
anial pressure; IIH idiopathic intracranial hypertension; MRI magnetic
resonance imaging.
Table 3. Clinical Features of Patients with Recurrent Idiopathic Intracranial Hypertension (IIH)
1. Average opening pressure at diagnosis was 36 cm of water (SD 9.3) 2. Average initial visual acuity, 20/32; final visual acuity, 20/25 3. Average Frisen grade of papilledema at the initial visit was 1.8 (SD 0.98), and the average of the maximum grade of papilledema was 2.3
(SD 1.15) 4. The presenting symptoms of patients with recurrent IIH were similar to those of typical IIH: headache (83%), pulse-synchronous tinnitus (52%),
transient visual obscurations (42%), and diplopia (24%)
SD standard deviation.
Table 4. Study Limitations
1. Despite the large number of patients with IIH at the University of Iowa, we were able to include only 34 charts with a documented recurrence of IIH. The University of Iowa being a tertiary care center and a center with a large referral area, many of our patients come for a second opinion or for primary diagnosis and then are locally observed and treated. This may account for the lower incidence of recurrence rate in our population.
2. Due to the retrospective nature of our study, we included only patients who had stability of the disease over 2 visits at least 3 mos apart. Thus, patients who were “cured” or stable at the last visit, who had an increase in disc edema with return of symptoms over the next visit, who had over 3 mos of stability, or who were observed locally were not included. This may account for the lower recurrence rate in the study.
3. All of the other limitations of a retrospective study, including ascertainment and selection bias, lack of follow-up, and lack of standardization of data collection.
4. Although intolerance or noncompliance with medical therapy was reported in 80% of our recurrences, this might be due to observer bias or recollection bias, and there was no standardized definition of either intolerance or noncompliance in our retrospective review.
5. Likewise, although weight gain and pregnancy are biologically plausible triggers for recurrent IIH, observer bias, recollection bias, and selection bias are limiting factors. Idiopathic intracranial hypertension is a disease of young obese females who are of childbearing age, and a certain percentage of the cohort would be expected by chance alone to be pregnant at the time of a recurrence of IIH. Two of our patients had tetracycline use before the recurrence, but we specifically excluded patients with IIH presumed secondary to medication, and thus, these 2 cases are not true rechallenge cases and cannot determine a cause-and-effect relationship. Although we believe that our retrospective work can generate hypotheses for possible risk factors for recurrent IIH, a case–control study might be better able to define a true cause-and-effect relationship.
CSF cerebrospinal fluid; CT computed tomography; ICP intracranial pressure; IIH idiopathic intracranial hypertension; MRI magnetic
resonance imaging.
Related Documents
![Interventions for recurrent idiopathic epistaxis ... · PDF file[Intervention Review] Interventions for recurrent idiopathic epistaxis (nosebleeds) in children Ali Qureishi1, Martin](https://static.cupdf.com/doc/110x72/5aa45ab87f8b9afa758bce4e/interventions-for-recurrent-idiopathic-epistaxis-intervention-review-interventions.jpg)