Coexistence of migraine and idiopathic intracranial hypertension without papilledema

Headache, papilledema, and elevated CSF pressure above 200 mm of water in a patient with normal neurologic examination and neuro-imaging meet the International Headache Society (IHS) diagnostic criteria for idiopathic intracranial hypertension (IIH). ^l Transient visual obscurations (TVOs), diplopia, visual loss, and noises in the head can occur in many of these patients. ^2–6 Papilledema is the most consistent neurologic sign of IIH but, rarely, IIH can occur without papilledema. ^7–10

Headache is the most common symptom of IIH, occurring in 90% of patients, ^2–5 and on a daily basis in 75%. ¹¹ Although there has been considerable attention given to the visual symptomatology of IIH, only one study has detailed the headache profile. Pulsatile, predominantly focal (retro-ocular), sometimes generalized headache, waking the patient up in the morning, with nausea and vomiting, occurred in the majority. ¹¹ This headache profile is not strikingly different from that of migraine. ¹² Corbett ³ and Sorenson and Corbett ¹³ noted that preexisting migraine may worsen with IIH, and prophylactic migraine drugs, especially beta blockers and naproxen sodium, are useful in the treatment of headache from intracranial hypertension. In a related condition, namely slit ventricle syndrome, antimigraine agents were highly effective in controlling headache. ¹⁴ Ramadan ¹⁵ reported transformation of episodic migraine into chronic daily headache (CDH) in a patient who developed IIH without papilledema. Marcelis and Silberstein ¹⁰ described 10 patients with IIH without papilledema who presented with CDH. Many of their patients had a headache profile similar to that of migraine and were treated with antimigraine agents. Silberstein and Corbett ¹⁶ emphasized the importance of performing lumbar puncture in patients with intractable CDH to rule out IIH without papilledema.

Patients

The Houston Headache Clinic is a referral center. We use uniform standardized forms to obtain headache, general medical, neurologic, and family histories. Information regarding comorbid and trigger factors is also recorded in the intake form. The interviews and physical and neurologic examinations were conducted by neurologists, both initially and on follow-up visits.

Among the patients with the transformed migraine type of CDH ^l7,18 treated at the Houston Headache Clinic between 1986 and 1993, 85 who were considered refractory form the cases for the present study. All these patients gave a history of migraine in the past but developed daily headache subsequently. All patients had daily headache at the time of study. The response to abortive antimigraine agents such as ergotamine, sumatriptan, and dihydroergotamine (DHE) during acute phases were satisfactory in most of these patients but, over time, their daily headaches persisted. They developed repeated prolonged periods of very frequent severe migraine-like and nondescript headaches resulting in over-medication. This resulted in frequent clinic and emergency room visits and sometimes hospitalization. This group of 85 chronic refractory patients was subjected to lumbar puncture as part of a further work-up to rule out chronic meningitis or increased intracranial pressure. All patients had normal CT or MRI.

After obtaining informed consent, lumbar puncture was performed with patients positioned in the lateral decubitus position on a level surface. A standard 20-gauge spinal needle was used with the manometer positioned at a 90-degree angle to the spinal cord. The opening pressure was measured with the patient's knee and hips in the extended position and neck straightened.

Patients with elevated CSF pressure were treated with acetazolamide and furosemide in combination with abortive antimigraine agents (ergotamine, DHE, or sumatriptan), and prophylactic agents such as beta blockers, amitriptyline, or methysergide.

Results

Of the 85 patients with refractory chronic headache with migrainous features who underwent spinal tap, 12 had elevated CSF pressure. None had papilledema, TVOs, or diplopia. The visual fields in all these patients were normal. The clinical and headache profiles of these 12 patients are given in table 1.

The IHS classification assigned to these patients, the duration of treatment at our clinic prior to the spinal tap, and duration of follow-up period after the spinal tap are given in table 2. All the patients had more than one IHS diagnosis. Using Metropolitan Life Insurance criteria for the diagnosis of obesity, 5 of 10 females in the series were considered obese. Both males in the series were obese by the same criteria.

The ages ranged from 13 to 54 years. All patients gave a long history of headache ranging from 3 to 30 years. All had a history of migraine-five with attacks with aura (four with visual aura, one with sensory aura, and one with both sensory and visual aura). The others had migraine without aura. Nine patients gave a positive family history of migraine. History of menstrual aggravation or hormonally influenced headache aggravation was obtained in 9 of the 10 females. Ten patients had associated comorbidity including depression, anxiety, panic attacks, and hypertension. Four patients reported noises in the head (pulse synchronous tinnitus). Empty sella was found in one (patient 8).

Findings

Table 4 shows the CSF findings in this series. All patients had elevated CSF. Repeat spinal tap in all 12 patients revealed continuing intracranial hypertension. There was no pleocytosis in the spinal fluid, nor was there any other abnormality except in one (patient 6) whose spinal fluid protein was 59 in the first lumbar puncture and 43 in the second. A brief summary of patient 6 is given below.

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Table 4 IIH without papilledema

Patient 6. This 48-year-old obese white male complained of recurrent headache for many years. Headache had become severe and daily for 4 months prior to referral to our center. There was no history of fever or any systemic symptoms. He was not a diabetic. Neurologic examination, MRI of the brain, and CT of the sinus were normal. CSF pressure was 280 mm of water during the first lumbar puncture and 285 mm of water during the second. CSF protein was 59 during the first lumbar puncture and 43 during the second. There were no cells. CSF cytology, India ink preparation, and bacterial, fungal, and tuberculosis cultures were normal. Because of the elevated protein, a cerebral arteriogram was performed, which revealed no evidence of cerebral vasculitis. During the 13-month follow-up, the patient showed no evidence of any other underlying disease to account for the elevated CSF protein.

The figure shows the pressure values in the present series compared with the normal values in obese and nonobese patients published by Corbett and Mehta. ¹⁹

Figure. Compares the CSF pressure values of patients in the current series with CSF pressure in normal nonobese and obese persons (data from Corbett and Mehta, ¹⁹ 1983).

The lumbar punctures resulted in transient improvement of headache in 4 of the 12 patients. Three patients had post-lumbar puncture headache, of which two required epidural blood patch.

Discussion

One of the criticisms that can be raised in the diagnosis of IIH without papilledema is the limitations of direct ophthalmoscopy in detecting early papilledema. Indirect ophthalmoscopy or stereo fundus photography is the most reliable method of identifying early papilledema. In this series, absence of papilledema was confirmed based only on careful and repeated direct ophthalmoscopy.

The diagnosis of IIH without papilledema, TVOs, or any visual changes ^7–10 is impossible without a spinal tap. The headache profiles of patients suffering from increased intracranial hypertension with and without papilledema have no specificity or diagnostic characteristics. Our series indicates that the headache profile meeting the IHS criteria for migraine (with and without aura) and chronic tension-type headache can occur in patients with IIH. The similarity between the headache profile in our patients and that of migraine patients from an epidemiologic survey by Rasmussen and Olesen ¹² is striking. Thus, in a given case, the headache profile of IIH may be indistinguishable from that of mixed migraine and chronic tension-type headache.

We found that abortive antimigraine medications such as sumatriptan, DHE, and ergotamine are useful in the management of acute exacerbations of headache in patients with coexisting IIH and migraine. This response is comparable with that of the CDH patients without IIH. All the patients were on prophylactic antimigraine agents prior to the diagnosis of IIH with partial results; however, addition of acetazolamide and furosemide resulted in further improvement of the overall headache control. In other words, in these patients, the response to migraine prophylactic agents alone is not as good as in CDH patients with normal pressure.

Previously reported beneficial response to antimigraine medications in cases with IIH ¹³ and slit ventricle syndrome, ¹⁴ combined with our experience with the present series, links migraine and IIH. Van Alphen ²⁰ argued, based on his experience in 40 patients, that migraine results from some unknown disturbance in the CSF circulation causing increased pressure in the basal cisterns and posterior fossa. He postulated that this resulted in transient acquired cerebellar herniation and spasm of the vertebral arteries accounting for headache and the neurologic manifestation of migraine, but he did not present adequate convincing data to substantiate his postulations. In many of our patients, the spinal fluid pressure remained above the normal range during the treatment, and the CSF pressure had no correlation with the degree of improvement they exhibited with antimigraine and diuretic therapy.

The practical conclusion from the study of this series is that any patient with CDH with migrainous features who is refractory to conventional therapy with prophylactic antimigraine agents should have a spinal tap to exclude coexisting IIH. This leads to the question of whether there is a shared pathophysiology between IIH and migraine. The clinical observations in our series suggest that further study is required to clarify if IIH is an entity in the migraine spectrum.