Abstract
To assess the prevalence and possible pathogenetic involvement of raised intracranial pressure in patients presenting with unresponsive chronic migraine (CM), we evaluated the intracranial opening pressure (OP) and clinical outcome of a single cerebrospinal fluid withdrawal by lumbar puncture in 44 consecutive patients diagnosed with unresponsive chronic/transformed migraine and evidence of sinus stenosis at magnetic resonance venography. The large majority of patients complained of daily or near-daily headache. Thirty-eight (86.4 %) had an OP >200 mmH2O. Lumbar puncture-induced normalization of intracranial pressure resulted in prompt remission of chronic pain in 34/44 patients (77.3 %); and an episodic pattern of headache was maintained for 2, 3 and 4 months in 24 (54.6 %), 20 (45.4 %) and 17 (38.6 %) patients, respectively. The medians of overall headache days/month and of disabling headache days/month significantly decreased (p < 0.0001) at each follow-up versus baseline. Despite the absence of papilledema, 31/44 (70.5 %) patients fulfilled the ICHD-II criteria for “Headache attributed to Intracranial Hypertension”. Our findings indicate that most patients diagnosed with unresponsive CM in specialized headache clinics may present an increased intracranial pressure involved in the progression and refractoriness of pain. Moreover, a single lumbar puncture with cerebrospinal fluid withdrawal results in sustained remission of chronic pain in many cases. Prospective controlled studies are needed before this procedure can be translated into clinical practice. Nonetheless, we suggest that intracranial hypertension without papilledema should be considered in all patients with almost daily migraine pain, with evidence of sinus stenosis, and unresponsive to medical treatment referred to specialized headache clinics.Electronic supplementary materialThe online version of this article (doi:10.1007/s00415-014-7355-2) contains supplementary material, which is available to authorized users.
Highlights
Idiopathic intracranial hypertension without papilledema (IIHWOP) and chronic migraine (CM) are often clinically indistinguishable [1,2,3]
To assess the prevalence and possible pathogenetic involvement of raised intracranial pressure in patients presenting with unresponsive chronic migraine (CM), we evaluated the intracranial opening pressure (OP) and clinical outcome of a single cerebrospinal fluid withdrawal by lumbar puncture in 44 consecutive patients diagnosed with unresponsive chronic/transformed migraine and evidence of sinus stenosis at magnetic resonance venography
We recently proposed a model of sinus stenosis-associated IIH pathogenesis whereby a self-limiting venous collapse feedback-loop leads to a coupled increase of venous and cerebrospinal fluid (CSF) pressures [17]
Summary
Idiopathic intracranial hypertension without papilledema (IIHWOP) and chronic migraine (CM) are often clinically indistinguishable [1,2,3]. We recently proposed a model of sinus stenosis-associated IIH pathogenesis whereby a self-limiting venous collapse feedback-loop leads to a coupled increase of venous and cerebrospinal fluid (CSF) pressures [17]. This model could explain the sustained remissions of IIH syndromes reported after sinus venous stenting [18] and not infrequently observed after serial or even after a single lumbar puncture (LP) with CSF withdrawal [19, 20]. The rate of responders and the duration of the clinical benefit after a single CSF withdrawal by LP in IIH/IIHWOP patients are unknown
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