The headache profile of idiopathic intracranial hypertension
Michael Wall
CEPHALALGIA Wall M. The headache profile of idiopathic intracranial hypertension. Cephalalgia 1990;10:331-5. Oslo. ISSN 0333-1024 The headache profile of idiopathic intracranial hypertension (IIH, pseudotumour cerebri) has not previously been prospectively studied. We administered a questionnaire to 63 cases at the time of diagnosis. Fifty-eight of the cases had headache and 93% of those with headache reported it to be their most severe ever. Patients characteristically noted a pulsatile headache of gradually increasing intensity that had awakened them. Daily headache occurred in 74% of those reporting headache. Pain in a nerve root distribution or retro-ocular pain with eye movement, uncommon with other headache disorders, help to differentiate this headache syndrome. • Headache, intracranial hypertension, migraine, pseudotumour cerebri Michael Wall, Tulane University School of Medicine, Department of Neurology & Psychiatry, 1430 Tulane Avenue, New Orleans, La. 70112 USA; Accepted 5 July 1990 There are many anecdotal reports of the headache of idiopathic intracranial hypertension (pseudotumour cerebri). Descriptions vary from mild to severe, pulsatile to throbbing and focal to generalized. The International Headache Society classification (Table 1) does not list a description for the headache of idiopathic intracranial hypertension or brain tumour (1). To determine a headache profile of idiopathic intracranial hypertension, we prospectively studied 63 patients that met the International Headache Society classification for the disorder. Methods
This series consisted of 63 consecutive patients referred to the Tulane University School of Medicine Neuro-ophthalmology Clinic between 1982 and 1989. All patients met the modified Dandy criteria for IIH (2), which also categorized them as having benign intracranial hypertension according to the International Headache Society criteria (Table 1) (1, 2). The patients answered a questionnaire at the time of their initial office visit. Fifty of these patients are the subject of another report primarily concerning their visual examination (3). We defined obesity as weight ³120% of the ideal weight for a medium-frame person according to the 1983 Metropolitan Life height-weight tables. Twenty-six of the 63 were referred directly from a population of a predominantly primary care facility (Charity Hospital of New Orleans). The remainder of the series was from a tertiary care referral practice (Tulane University Hospital and Clinic). The data from the questionnaire were entered into a database and tabulated. The statistical analysis was performed as follows. Differences between groups for nominal data were tested for statistical significance using a Fisher's exact test, as the expected frequency in one of the groups was five or less. Two-tailed independent t-tests were used for normally distributed quantitative data; the Mann Whitney test was used for corresponding non-parametric data. When multiple tests were performed on a data set, the results were corrected using the Bonferroni adjustment for multiple statistical tests. Differences between groups were interpreted as significant if the probability of their occurrence was less than 0.05. Results
Ninety-three percent of the patients were
Table 1. International Headache Society Classification for benign intracranial hypertension and the modified Dandy criteria for IIH. IHS diagnostic criteria A. Patient suffers from benign intracranial hypertension fulfilling the following criteria: 1. Increased intracranial pressure (>200 mm of water) measured by epidural or intraventricular pressure monitoring. 2. Normal neurologic examination except for papilloedema and possible sixth nerve palsy. 3. No mass lesion and no ventricular enlargement on neuroimaging. 4. Normal or low protein concentration and normal white cell count in CSF. 5. No clinical or neuroimaging suspicion of venous sinus thrombosis. B. Headache intensity and frequency related to variations of intracranial pressure with a time lag of less than 24 h. Modified Dandy criteria for IIH 1. Signs and symptoms of increased intracranial pressure. 2. Absence of localizing findings on neurologic examination. 3. Absence of deformity, displacement or obstruction of the ventricular system and otherwise normal neurodiagnostic studies, except for increased cerebrospinal fluid pressure. 4. Awake and alert patient. 5. No other cause of increased intracranial pressure present.
women and 93% were obese. The mean age at onset was 31 years (Fig. 1). Headache was reported by 92% of cases. Demographic data and associated symptoms in patients with and without headache are listed in Table 2. The only significant difference between the headache and non-headache groups, after correcting for performing multiple statistical tests, was that the non-headache group was older (p = 0.02). This group had less symptoms in general. The headache frequency of the patients is shown in Fig. 2. Forty-three of the 58 (73%) IIH patients that reported headache had daily headache. Ten patients reported that their headache was provoked by emotion. Two reported it was made worse by bending down and two with standing. Only one noted worsening with coughing or sneezing. Ten patients reported that various types of non-steroidal anti-inflammatory agents, including aspirin, relieved their headache. Sleep was effective in five and three reported that quietness alleviated their headache. Table 3 gives the headache characteristics and frequency of associated symptoms. This suggests a headache profile of IIH of a gradually increasing pulsatile headache different from previous head pain. It lasts longer than 1 h and the patients usually described it as the most severe head pain they had ever experienced. It may have a focal component. Nausea and neck stiffness were common. Discussion
Headache is the most common symptom of patients with IIH and is the usual presenting complaint. In patients with brain tumours and increased intracranial pressure, the headache due to the mass lesion is difficult to separate from the headache due to intracranial hypertension (4, 5). Previous reports of headache in IIH have not elucidated a distinct headache syndrome. Weisberg (6), in a retrospective chart review of 120 patients, reported the headache of IIH to
Table 2. Characteristics of IIH patients with and without headache. Headache Non-headache Characteristic group n = 58 group n = 5 p value Age 29.1 44.2 0.02 Weight 203.1 174.0 NS Weight gain in past year 18.3 1.6 NS Race NS white 62% 20% black 38% 80% Female 93% 60% NS Obese 93% 80% NS Pregnant 7% 0% NS Arterial hypertension 30% 20% NS Diabetes mellitus 5% 0% NS TVO 71% 40% NS Intracranial noises 60% 20% NS Diplopia 38% 0% NS Visual loss 31% 0% NS Most prominent symptom headache 72% TVO 10% 20% visual loss 7% 20% diplopia 7% none 4% 60% First symptom headache 78% TVO 10% 20% visual loss 5% 20% diplopia 5% poor night vision 2% none 60% TVO = transient visual obscurations. NS = not significant.
be generalized, episodic and throbbing. He noted that it was often worst in the morning and was aggravated by coughing, straining or change in position. It always preceded the visual symptoms, was commonly accompanied by nausea (40%) but rarely by vomiting (1%). Corbett (7, 8) stated that there was nothing specific about the headache of IIH. He observed the headache to be global or one-sided, mild or severe and it could worsen with a Valsalva manoeuvre. He notes that patients with migraine may have a worsening with IIH. The headache has been reported as seldom severe (9, 10) and may have a striking postural element (11, 12). Postural aggravating factors were seldom reported in our series when the patient was asked what made the headaches worse. The headache has been depicted as non-throbbing and increased when the head is given a "jolt" or sudden rotation (13). Bulens (10) described retro-ocular pain worsened by eye movement in five of 27
Table 3. The frequency of symptoms associated with headache in the 58 patients with headache. Symptom % with symptom Most severe headache ever 93 Last longer than 1 h 88 Different from previous headaches 86 Pulsatile 83 Intensity slowly increases 76 Awaken patient 62 Neck stiffness 59 Nausea 57 Retro-ocular pain 43 worse with eye movement 24 Generalized (some had focal pain in addition) 43 Vomiting 38 Radicular pain 17 Focal (some had generalized headache also) 70 hemicranial 16 fronto-orbital 31 occipital-nuchal 14 temporal 7 vertex 2 patients with IIH. The pain was severe but of short duration. In the present series, 43% of patients had retro-ocular pain that worsened on eye movements in 24%. Foley (9) recognized the association of neck stiffness in 7 of 31 patients. Round and Keane (14) reported painful neck stiffness in 31% of their patients. Forty-eight percent of our patients had neck stiffness. We did not ask if it was painful. Bortoluzzi (15) has reported "radicular pain" with intracranial hypertension; it radiates in the distribution of a spinal nerve root. Seventeen percent of our patients had this type of pain. Experimentally induced increased intracranial pressure in humans by cerebrospinal fluid infusion was found to produce pulsatile pain usually frontal or temporal that at times was associated with vomiting. In general, however, the infusion provoked variable headache responses (16). Johnston and Paterson (17) performed intracranial pressure monitoring in 20 patients using an intra-ventricular catheter. Thirteen cases had plateau waves. Eight cases had plateau-like waves. Neither waves were related to headache or any change in clinical symptoms. Our patients had a headache profile of severe daily, pulsatile pain that gradually increased in intensity. Nausea was common and vomiting less so. Most of those with headache reported that it was the worst head pain they had ever experienced. The headache had awakened the patient and was different from previous headaches. Postural aggravating factors were uncommon. Radicular pain or retrobulbar pain on eye movements, although not common, further differentiates this syndrome. We hope that these findings will serve as an initial groundwork to quantitate and classify the headache syndrome associated with intracranial hypertension. References
1.
Classification and diagnostic criteria for headache disorders, cranial neuralgias and facial pain. Cephalalgia 1988;8(suppl 7):1-96
2.
Smith JL. Whence pseudotumor cerebri? J Clin Neurol Ophthalmol 1985;5:55-6
3.
Wall M, George D. Idiopathic intracranial hypertension (pseudotumor cerebri): a prospective study of 50 patients. Brain (in press)
4.
Kunkle EC, Ray BS, Wolff HG. Studies on headache: The mechanisms and significance of the headache associated with brain tumor. Bull NY Acad Sci 1942;18:400-22
5.
Honig PJ, Charney EB. Children with brain tumor headaches. J Dis Child 1982;136:121-4
6.
Weisberg LA. Benign intracranial hypertension. Medicine 1975;54:197-207
7.
Corbett JJ. The 1982 Silversides lecture. Problems in the diagnosis and treatment of pseudotumor cerebri. Can J Neurol Sci 1983;10:221-9
8.
Corbett JJ, Savino PJ, Thompson HS, et al. Visual loss in pseudotumor cerebri. Follow-up of 57 patients from five to 41 years and a profile of 14 patients with permanent severe visual loss. Arch Neurol 1982;39:461-74
9.
Foley J. Benign forms of intracranial hypertension-"toxic" and "otitic" hydrocephalus. Brain 1955;78:1-41
10.
Bulens C, DeVries WA, van Crevel H. Benign intracranial hypertension. A retrospective and follow-up study. J Neurol Sci 1979;40:147-57
11.
Orcutt JC, Page NG, Sanders MD. Factors affecting visual loss in benign intracranial hypertension. Ophthalmology 1984;91:1303-12
12.
Dalessio DJ. Wolf's headache and other head pain. New York: Oxford University Press, 1980:310
13.
Raskin NH. Headache. New York: Churchill Livingstone, 1988:289
14.
Round R, Keane JR. The minor symptoms of increased intracranial pressure: 101 patients with benign intracranial hypertension. Neurology 1988;38:1461-4
15.
Bortoluzzi M, DiLauro L, Marini G. Benign intracranial hypertension with spinal and radicular pain. J Neurosurg 1982;57:833-6
16.
Fay T. A new test for the diagnosis of certain headaches. the cephalogram. Dis Nerv Sys 1940;1:312-15
17.
Johnston I, Paterson A. Benign intracranial hypertension. II. CSF pressure and circulation. Brain 1974;97:301-12
Michael Wall
CEPHALALGIA Wall M. The headache profile of idiopathic intracranial hypertension. Cephalalgia 1990;10:331-5. Oslo. ISSN 0333-1024 The headache profile of idiopathic intracranial hypertension (IIH, pseudotumour cerebri) has not previously been prospectively studied. We administered a questionnaire to 63 cases at the time of diagnosis. Fifty-eight of the cases had headache and 93% of those with headache reported it to be their most severe ever. Patients characteristically noted a pulsatile headache of gradually increasing intensity that had awakened them. Daily headache occurred in 74% of those reporting headache. Pain in a nerve root distribution or retro-ocular pain with eye movement, uncommon with other headache disorders, help to differentiate this headache syndrome. • Headache, intracranial hypertension, migraine, pseudotumour cerebri Michael Wall, Tulane University School of Medicine, Department of Neurology & Psychiatry, 1430 Tulane Avenue, New Orleans, La. 70112 USA; Accepted 5 July 1990 There are many anecdotal reports of the headache of idiopathic intracranial hypertension (pseudotumour cerebri). Descriptions vary from mild to severe, pulsatile to throbbing and focal to generalized. The International Headache Society classification (Table 1) does not list a description for the headache of idiopathic intracranial hypertension or brain tumour (1). To determine a headache profile of idiopathic intracranial hypertension, we prospectively studied 63 patients that met the International Headache Society classification for the disorder. Methods
This series consisted of 63 consecutive patients referred to the Tulane University School of Medicine Neuro-ophthalmology Clinic between 1982 and 1989. All patients met the modified Dandy criteria for IIH (2), which also categorized them as having benign intracranial hypertension according to the International Headache Society criteria (Table 1) (1, 2). The patients answered a questionnaire at the time of their initial office visit. Fifty of these patients are the subject of another report primarily concerning their visual examination (3). We defined obesity as weight ³120% of the ideal weight for a medium-frame person according to the 1983 Metropolitan Life height-weight tables. Twenty-six of the 63 were referred directly from a population of a predominantly primary care facility (Charity Hospital of New Orleans). The remainder of the series was from a tertiary care referral practice (Tulane University Hospital and Clinic). The data from the questionnaire were entered into a database and tabulated. The statistical analysis was performed as follows. Differences between groups for nominal data were tested for statistical significance using a Fisher's exact test, as the expected frequency in one of the groups was five or less. Two-tailed independent t-tests were used for normally distributed quantitative data; the Mann Whitney test was used for corresponding non-parametric data. When multiple tests were performed on a data set, the results were corrected using the Bonferroni adjustment for multiple statistical tests. Differences between groups were interpreted as significant if the probability of their occurrence was less than 0.05. Results
Ninety-three percent of the patients were
Table 1. International Headache Society Classification for benign intracranial hypertension and the modified Dandy criteria for IIH. IHS diagnostic criteria A. Patient suffers from benign intracranial hypertension fulfilling the following criteria: 1. Increased intracranial pressure (>200 mm of water) measured by epidural or intraventricular pressure monitoring. 2. Normal neurologic examination except for papilloedema and possible sixth nerve palsy. 3. No mass lesion and no ventricular enlargement on neuroimaging. 4. Normal or low protein concentration and normal white cell count in CSF. 5. No clinical or neuroimaging suspicion of venous sinus thrombosis. B. Headache intensity and frequency related to variations of intracranial pressure with a time lag of less than 24 h. Modified Dandy criteria for IIH 1. Signs and symptoms of increased intracranial pressure. 2. Absence of localizing findings on neurologic examination. 3. Absence of deformity, displacement or obstruction of the ventricular system and otherwise normal neurodiagnostic studies, except for increased cerebrospinal fluid pressure. 4. Awake and alert patient. 5. No other cause of increased intracranial pressure present.
women and 93% were obese. The mean age at onset was 31 years (Fig. 1). Headache was reported by 92% of cases. Demographic data and associated symptoms in patients with and without headache are listed in Table 2. The only significant difference between the headache and non-headache groups, after correcting for performing multiple statistical tests, was that the non-headache group was older (p = 0.02). This group had less symptoms in general. The headache frequency of the patients is shown in Fig. 2. Forty-three of the 58 (73%) IIH patients that reported headache had daily headache. Ten patients reported that their headache was provoked by emotion. Two reported it was made worse by bending down and two with standing. Only one noted worsening with coughing or sneezing. Ten patients reported that various types of non-steroidal anti-inflammatory agents, including aspirin, relieved their headache. Sleep was effective in five and three reported that quietness alleviated their headache. Table 3 gives the headache characteristics and frequency of associated symptoms. This suggests a headache profile of IIH of a gradually increasing pulsatile headache different from previous head pain. It lasts longer than 1 h and the patients usually described it as the most severe head pain they had ever experienced. It may have a focal component. Nausea and neck stiffness were common. Discussion
Headache is the most common symptom of patients with IIH and is the usual presenting complaint. In patients with brain tumours and increased intracranial pressure, the headache due to the mass lesion is difficult to separate from the headache due to intracranial hypertension (4, 5). Previous reports of headache in IIH have not elucidated a distinct headache syndrome. Weisberg (6), in a retrospective chart review of 120 patients, reported the headache of IIH to
Table 2. Characteristics of IIH patients with and without headache. Headache Non-headache Characteristic group n = 58 group n = 5 p value Age 29.1 44.2 0.02 Weight 203.1 174.0 NS Weight gain in past year 18.3 1.6 NS Race NS white 62% 20% black 38% 80% Female 93% 60% NS Obese 93% 80% NS Pregnant 7% 0% NS Arterial hypertension 30% 20% NS Diabetes mellitus 5% 0% NS TVO 71% 40% NS Intracranial noises 60% 20% NS Diplopia 38% 0% NS Visual loss 31% 0% NS Most prominent symptom headache 72% TVO 10% 20% visual loss 7% 20% diplopia 7% none 4% 60% First symptom headache 78% TVO 10% 20% visual loss 5% 20% diplopia 5% poor night vision 2% none 60% TVO = transient visual obscurations. NS = not significant.
be generalized, episodic and throbbing. He noted that it was often worst in the morning and was aggravated by coughing, straining or change in position. It always preceded the visual symptoms, was commonly accompanied by nausea (40%) but rarely by vomiting (1%). Corbett (7, 8) stated that there was nothing specific about the headache of IIH. He observed the headache to be global or one-sided, mild or severe and it could worsen with a Valsalva manoeuvre. He notes that patients with migraine may have a worsening with IIH. The headache has been reported as seldom severe (9, 10) and may have a striking postural element (11, 12). Postural aggravating factors were seldom reported in our series when the patient was asked what made the headaches worse. The headache has been depicted as non-throbbing and increased when the head is given a "jolt" or sudden rotation (13). Bulens (10) described retro-ocular pain worsened by eye movement in five of 27
Table 3. The frequency of symptoms associated with headache in the 58 patients with headache. Symptom % with symptom Most severe headache ever 93 Last longer than 1 h 88 Different from previous headaches 86 Pulsatile 83 Intensity slowly increases 76 Awaken patient 62 Neck stiffness 59 Nausea 57 Retro-ocular pain 43 worse with eye movement 24 Generalized (some had focal pain in addition) 43 Vomiting 38 Radicular pain 17 Focal (some had generalized headache also) 70 hemicranial 16 fronto-orbital 31 occipital-nuchal 14 temporal 7 vertex 2 patients with IIH. The pain was severe but of short duration. In the present series, 43% of patients had retro-ocular pain that worsened on eye movements in 24%. Foley (9) recognized the association of neck stiffness in 7 of 31 patients. Round and Keane (14) reported painful neck stiffness in 31% of their patients. Forty-eight percent of our patients had neck stiffness. We did not ask if it was painful. Bortoluzzi (15) has reported "radicular pain" with intracranial hypertension; it radiates in the distribution of a spinal nerve root. Seventeen percent of our patients had this type of pain. Experimentally induced increased intracranial pressure in humans by cerebrospinal fluid infusion was found to produce pulsatile pain usually frontal or temporal that at times was associated with vomiting. In general, however, the infusion provoked variable headache responses (16). Johnston and Paterson (17) performed intracranial pressure monitoring in 20 patients using an intra-ventricular catheter. Thirteen cases had plateau waves. Eight cases had plateau-like waves. Neither waves were related to headache or any change in clinical symptoms. Our patients had a headache profile of severe daily, pulsatile pain that gradually increased in intensity. Nausea was common and vomiting less so. Most of those with headache reported that it was the worst head pain they had ever experienced. The headache had awakened the patient and was different from previous headaches. Postural aggravating factors were uncommon. Radicular pain or retrobulbar pain on eye movements, although not common, further differentiates this syndrome. We hope that these findings will serve as an initial groundwork to quantitate and classify the headache syndrome associated with intracranial hypertension. References
1.
Classification and diagnostic criteria for headache disorders, cranial neuralgias and facial pain. Cephalalgia 1988;8(suppl 7):1-96
2.
Smith JL. Whence pseudotumor cerebri? J Clin Neurol Ophthalmol 1985;5:55-6
3.
Wall M, George D. Idiopathic intracranial hypertension (pseudotumor cerebri): a prospective study of 50 patients. Brain (in press)
4.
Kunkle EC, Ray BS, Wolff HG. Studies on headache: The mechanisms and significance of the headache associated with brain tumor. Bull NY Acad Sci 1942;18:400-22
5.
Honig PJ, Charney EB. Children with brain tumor headaches. J Dis Child 1982;136:121-4
6.
Weisberg LA. Benign intracranial hypertension. Medicine 1975;54:197-207
7.
Corbett JJ. The 1982 Silversides lecture. Problems in the diagnosis and treatment of pseudotumor cerebri. Can J Neurol Sci 1983;10:221-9
8.
Corbett JJ, Savino PJ, Thompson HS, et al. Visual loss in pseudotumor cerebri. Follow-up of 57 patients from five to 41 years and a profile of 14 patients with permanent severe visual loss. Arch Neurol 1982;39:461-74
9.
Foley J. Benign forms of intracranial hypertension-"toxic" and "otitic" hydrocephalus. Brain 1955;78:1-41
10.
Bulens C, DeVries WA, van Crevel H. Benign intracranial hypertension. A retrospective and follow-up study. J Neurol Sci 1979;40:147-57
11.
Orcutt JC, Page NG, Sanders MD. Factors affecting visual loss in benign intracranial hypertension. Ophthalmology 1984;91:1303-12
12.
Dalessio DJ. Wolf's headache and other head pain. New York: Oxford University Press, 1980:310
13.
Raskin NH. Headache. New York: Churchill Livingstone, 1988:289
14.
Round R, Keane JR. The minor symptoms of increased intracranial pressure: 101 patients with benign intracranial hypertension. Neurology 1988;38:1461-4
15.
Bortoluzzi M, DiLauro L, Marini G. Benign intracranial hypertension with spinal and radicular pain. J Neurosurg 1982;57:833-6
16.
Fay T. A new test for the diagnosis of certain headaches. the cephalogram. Dis Nerv Sys 1940;1:312-15
17.
Johnston I, Paterson A. Benign intracranial hypertension. II. CSF pressure and circulation. Brain 1974;97:301-12
