Headache in idiopathic intracranial hypertension. A CGRP-dependent head pain?
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HEADACHES IN CSF DYNAMICS DISORDERS
Headache in idiopathic intracranial hypertension. A CGRP-dependent head pain? Roberto De Simone 1 & Mattia Sansone 1 & Vincenzo Bonavita 2
# Fondazione Società Italiana di Neurologia 2020
Abstract Headache is the most frequent and often the most severe symptom of idiopathic intracranial hypertension (IIH) clinical presentation, although pain characteristics are very variable among sufferers and the pain may even lack in some cases. Whatever the headache features, refractoriness to treatments, pain worsening in the recumbent position, and frequent awakenings with severe headache late in the night are the specific complains of such patients. However, a migraine or probable migraine headache, mostly with a chronic headache pattern, can be diagnosed in about 2/3 of the cases. In IIH cases without papilledema (IIHWOP), this leads to a high rate of misdiagnosis with primary chronic migraine (CM). Mechanisms responsible for the shared migrainous presentation of CM and IIH/IIHWOP may rely on a pathologic CGRP release from the rich trigemino-vascular innervated dural sinuses, congested in the course of raised intracranial pressure. The possible role of IIHWOP as a powerful and modifiable risk factor for migraine progression is discussed. Further studies investigating the possible efficacy of anti CGRP/receptor antibodies in IIH/IIHWOP headache treatment are needed. Keywords Idiopathic intracranial hypertension . Starling resistor . CGRP . Chronic migraine . Cerebral perfusion autoregulation . Risk factor
Background Idiopathic intracranial hypertension (IIH) is an infrequent condition characterized by a raised intracranial pressure without a detectable cause. Current incidence is 2,4/100,000 on general population, but in obese women of working age, the incidence rise up to 22/100,000 [1]. IIH may run without papilledema (IIHWOP). Considered an infrequent variant of a rare disease [2], IIHWOP may instead be a much more common condition due to a high prevalence of asymptomatic, overlooked, or misdiagnosed cases [3]. Female sex, obesity, and sleep disturbances are well-established IIH risk factors [4]. Headache represents the most common symptom and is the main responsible for the reduced quality of life in IIH patients [5]. Other symptoms include blurred vision, diplopia, transient visual * Roberto De Simone [email protected] 1
Department of Neuroscience, Reproductive Sciences and Odontostomatology, Headache Centre, University Federico II of Naples, Via Pansini, 5, 80131 Naples, Italy
2
Istituto di Diagnosi e Cura Hermitage Capodimonte, Naples, Italy
obscuration, ear fullness [6], tinnitus, and vestibular derangement. The condition is not so “benign” as permanent visual deficit occurs in up to 25% of the cases.
Advancement in IIH mechanisms A raised dural sinus pressure (DSp) of whichever etiology is considered the universal mechanism leading to the increase of intracranial pressure (ICp), because it reduces the cerebrospinal fluid (CSF)/dural sinus transmural pressure gradient requ
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