Publications by authors named "Geneviève Demarquay"

27 Publications

  • Page 1 of 1

Headaches provoked by cortical stimulation: Their localizing value in focal epileptic seizures.

Epilepsy Behav 2021 Jun 15;122:108125. Epub 2021 Jun 15.

Lyon Neuroscience Research Center (CRNL), INSERM U1028, CNRS UMR5292, Lyon, France; Department of Neurology Neurological Hospital, Hospices Civils de Lyon and University of Lyon, France. Electronic address:

Objective: Electrical stimulations performed in awake patients identified dura mater, venous sinuses, and arteries as pain-sensitive intracranial structures. However, cephalic pain has been only occasionally reported in patients with epilepsy undergoing stereo-electroencephalography (SEEG) stimulations.

Methods: The aim of our study was to investigate whether headache can be triggered by SEEG stimulations and might be related to specific cortical areas. Data were gathered from 16 050 stimulations collected in 266 patients who underwent a SEEG as part of a presurgical assessment of their drug-resistant epilepsy.

Results: Two-hundred and eight stimulations (1.3%) evoked headaches. Pain was more frequently described as bilateral (42.31%) than ipsilateral (16.83%) or contralateral (14.42%) to the stimulated hemisphere. Headache was more frequently elicited during stimulation of the insulo-limbic regions such as the anterior and medial cingulate gyrus, the mesial part of temporal lobe, and the insula.

Conclusion: This study shows that cortical stimulation can evoke headache, mostly during stimulation of the temporo-frontal limbic regions. It suggests that brief epileptic headache can be an epileptic symptom caused by a cortical discharge involving somatic or visceral network and does not reflect only trigemino-vascular activation. Although not specific, the occurrence of a brief epileptic headache may point to a seizure origin in the temporo-frontal limbic regions.
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http://dx.doi.org/10.1016/j.yebeh.2021.108125DOI Listing
June 2021

KCNT1-related epilepsies and epileptic encephalopathies: phenotypic and mutational spectrum.

Brain 2021 Jun 11. Epub 2021 Jun 11.

Pediatric Neurology Department, Lyon University Hospital, 69500 Bron, France.

Variants in KCNT1, encoding a sodium-gated potassium channel (subfamily T member 1), have been associated with a spectrum of epilepsies and neurodevelopmental disorders. These range from familial autosomal dominant or sporadic sleep-related hypermotor epilepsy ((AD)SHE) to epilepsy of infancy with migrating focal seizures (EIMFS) and include developmental and epileptic encephalopathies (DEE). This study aims to provide a comprehensive overview of the phenotypic and genotypic spectrum of KCNT1 mutation-related epileptic disorders in 248 individuals, including 66 unpreviously published and 182 published cases, the largest cohort reported so far. Four phenotypic groups emerged from our analysis: i) EIMFS (152 individuals, 33 previously unpublished); ii) DEE other than EIMFS (non-EIMFS DEE) (37 individuals, 17 unpublished); iii) (AD)SHE (53 patients, 14 unpublished); iv) other phenotypes (6 individuals, 2 unpublished). In our cohort of 66 new cases, the most common phenotypic features were: a) in EIMFS, heterogeneity of seizure types, including epileptic spasms, epilepsy improvement over time, no epilepsy-related deaths; b) in non-EIMFS DEE, possible onset with West syndrome, occurrence of atypical absences, possible evolution to DEE with SHE features; one case of sudden unexplained death in epilepsy (SUDEP); c) in (AD)SHE, we observed a high prevalence of drug-resistance, although seizure frequency improved with age in some individuals, appearance of cognitive regression after seizure onset in all patients, no reported severe psychiatric disorders, although behavioural/psychiatric comorbidities were reported in about 50% of the patients, SUDEP in one individual; d) other phenotypes in individuals with mutation of KCNT1 included temporal lobe epilepsy, and epilepsy with tonic-clonic seizures and cognitive regression. Genotypic analysis of the whole cohort of 248 individuals showed only missense mutations and one inframe deletion in KCNT1. Although the KCNT1 mutations in affected individuals were seen to be distributed among the different domains of the KCNT1 protein, genotype-phenotype considerations showed many of the (AD)SHE-associated mutations to be clustered around the RCK2 domain in the C-terminus, distal to the NADP domain. Mutations associated with EIMFS/non-EIMFS DEE did not show a particular pattern of distribution in the KCNT1 protein. Recurrent KCNT1 mutations were seen to be associated with both severe and less severe phenotypes. Our study further defines and broadens the phenotypic and genotypic spectrums of KCNT1-related epileptic conditions and emphasizes the increasingly important role of this gene in the pathogenesis of early onset DEEs as well as in focal epilepsies, namely (AD)SHE.
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http://dx.doi.org/10.1093/brain/awab219DOI Listing
June 2021

Is Migraine Associated to Brain Anatomical Alterations? New Data and Coordinate-Based Meta-analysis.

Brain Topogr 2021 05 19;34(3):384-401. Epub 2021 Feb 19.

Lyon Neuroscience Research Center (CRNL), INSERM UMRS 1028, CNRS UMR 5292, Université Claude Bernard Lyon 1, Université de Lyon, Lyon, France.

A growing number of studies investigate brain anatomy in migraine using voxel- (VBM) and surface-based morphometry (SBM), as well as diffusion tensor imaging (DTI). The purpose of this article is to identify consistent patterns of anatomical alterations associated with migraine. First, 19 migraineurs without aura and 19 healthy participants were included in a brain imaging study. T1-weighted MRIs and DTI sequences were acquired and analyzed using VBM, SBM and tract-based spatial statistics. No significant alterations of gray matter (GM) volume, cortical thickness, cortical gyrification, sulcus depth and white-matter tract integrity could be observed. However, migraineurs displayed decreased white matter (WM) volume in the left superior longitudinal fasciculus. Second, a systematic review of the literature employing VBM, SBM and DTI was conducted to investigate brain anatomy in migraine. Meta-analysis was performed using Seed-based d Mapping via permutation of subject images (SDM-PSI) on GM volume, WM volume and cortical thickness data. Alterations of GM volume, WM volume, cortical thickness or white-matter tract integrity were reported in 72%, 50%, 56% and 33% of published studies respectively. Spatial distribution and direction of the disclosed effects were highly inconsistent across studies. The SDM-PSI analysis revealed neither significant decrease nor significant increase of GM volume, WM volume or cortical thickness in migraine. Overall there is to this day no strong evidence of specific brain anatomical alterations reliably associated to migraine. Possible explanations of this conflicting literature are discussed. Trial registration number: NCT02791997, registrated February 6th, 2015.
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http://dx.doi.org/10.1007/s10548-021-00824-6DOI Listing
May 2021

Cluster headache: state of the art of pharmacological treatments and therapeutic perspectives.

Fundam Clin Pharmacol 2021 Jun 24;35(3):595-619. Epub 2020 Dec 24.

Lyon Neuroscience Research Center (CRNL), Université de Lyon, CNRS, INSERM, Lyon, France.

Cluster headache (CH) is the most common form of trigeminal autonomic cephalalgia. Current treatments have several limitations, and new drugs are required. This article first briefly reviews present acute and preventive treatments in CH, their mechanism of action and limitations, then describes the state of the art in recent clinical drug trials since 2015, and ends with a critique of trials in the CH field. Research is limited by lack of knowledge of pathophysiology and lack of animal models. In the past 5 years, no brand-new treatment has emerged, but promising drugs, such as CGRP(R) antibodies, are under study. According to the literature and guidelines, clinicians and researchers should be aware of many limitations in study protocols: concomitant medication, patient sample size, patients' protocol compliance, and study designs that tend to restrict patient recruitment.
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http://dx.doi.org/10.1111/fcp.12636DOI Listing
June 2021

Auditory attention alterations in migraine: A behavioral and MEG/EEG study.

Clin Neurophysiol 2020 08 18;131(8):1933-1946. Epub 2020 Jun 18.

Lyon Neuroscience Research Center (CRNL), INSERM UMRS 1028, CNRS UMR 5292, Université Claude Bernard Lyon 1, Université de Lyon, Lyon, France.

Objectives: To evaluate alterations of top-down and/or bottom-up attention in migraine and their cortical underpinnings.

Methods: 19 migraineurs between attacks and 19 matched control participants performed a task evaluating jointly top-down and bottom-up attention, using visually-cued target sounds and unexpected task-irrelevant distracting sounds. Behavioral responses and magneto- and electro-encephalography signals were recorded. Event-related potentials and fields were processed and source reconstruction was applied to event-related fields.

Results: At the behavioral level, neither top-down nor bottom-up attentional processes appeared to be altered in migraine. However, migraineurs presented heightened evoked responses following distracting sounds (orienting component of the N1 and Re-Orienting Negativity, RON) and following target sounds (orienting component of the N1), concomitant to an increased recruitment of the right temporo-parietal junction. They also displayed an increased effect of the cue informational value on target processing resulting in the elicitation of a negative difference (Nd).

Conclusions: Migraineurs appear to display increased bottom-up orienting response to all incoming sounds, and an enhanced recruitment of top-down attention.

Significance: The interictal state in migraine is characterized by an exacerbation of the orienting response to attended and unattended sounds. These attentional alterations might participate to the peculiar vulnerability of the migraine brain to all incoming stimuli.
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http://dx.doi.org/10.1016/j.clinph.2020.05.024DOI Listing
August 2020

Treatment Responsiveness in KCNT1-Related Epilepsy.

Neurotherapeutics 2019 07;16(3):848-857

Division of Child Neurology, Department of Neurology, University of Rochester School of Medicine, Rochester, NY, USA.

Pathogenic variants in KCNT1 represent an important cause of treatment-resistant epilepsy, for which an effective therapy has been elusive. Reports about the effectiveness of quinidine, a candidate precision therapy, have been mixed. We sought to evaluate the treatment responsiveness of patients with KCNT1-related epilepsy. We performed an observational study of 43 patients using a collaborative KCNT1 patient registry. We assessed treatment efficacy based upon clinical seizure reduction, side effects of quinidine therapy, and variant-specific responsiveness to treatment. Quinidine treatment resulted in a > 50% seizure reduction in 20% of patients, with rare patients achieving transient seizure freedom. Multiple other therapies demonstrated some success in reducing seizure frequency, including the ketogenic diet and vigabatrin, the latter particularly in patients with epileptic spasms. Patients with the best quinidine response had variants that clustered distal to the NADP domain within the RCK2 domain of the protein. Half of patients did not receive a quinidine trial. In those who did, nearly half did not achieve therapeutic blood levels. More favorable response to quinidine in patients with KCNT1 variants distal to the NADP domain within the RCK2 domain may suggest a variant-specific response.
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http://dx.doi.org/10.1007/s13311-019-00739-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6694367PMC
July 2019

Migraine with brainstem aura: Why not a cortical origin?

Cephalalgia 2018 09 26;38(10):1687-1695. Epub 2017 Oct 26.

1 Department of Neurology, Hospices Civils de Lyon, Lyon, France.

Background Migraine with brainstem aura is defined as a migraine with aura including at least two of the following symptoms: dysarthria, vertigo, tinnitus, hypacusis, diplopia, ataxia and/or decreased level of consciousness. Aim The aim of this study is to review data coming from clinical observations and functional mapping that support the role of the cerebral cortex in the initiation of brainstem aura symptoms. Results Vertigo can result from a vestibular cortex dysfunction, while tinnitus and hypacusis can originate within the auditory cortex. Diplopia can reflect a parieto-occipital involvement. Dysarthria can be caused by dysfunctions located in precentral gyri. Ataxia can reflect abnormal processing of vestibular, sensory, or visual inputs by the parietal lobe. Alteration of consciousness can be caused by abnormal neural activation within specific consciousness networks that include prefrontal and posterior parietal cortices. Conclusion Any symptom of so-called brainstem aura can originate within the cortex. Based on these data, we suggest that brainstem aura could have a cortical origin. This hypothesis would explain the co-occurrence of typical and brainstem aura during attacks and would fit with the theory of cortical spreading depression. We propose that migraine with brainstem aura should be classified as a typical migraine aura.
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http://dx.doi.org/10.1177/0333102417738251DOI Listing
September 2018

Marked EEG worsening following Levetiracetam overdose: How a pharmacological issue can confound coma prognosis.

Clin Neurol Neurosurg 2017 Jan 9;152:1-4. Epub 2016 Nov 9.

Hospices Civils de Lyon, Hopital de la Croix Rousse, Service de Reanimation Médicale, Lyon, France; Université de Lyon, Université Claude Bernard Lyon 1, Villeurbanne, France. Electronic address:

Levetiracetam is an anti-epileptic drug commonly used in intensive care when seizure is suspected as a possible cause of coma. We propose to question the cofounding effect of Levetiracetam during the prognostication process in a case of anoxic coma. We report the story of a young woman presenting a comatose state following a hypoxic cardiac arrest. After a first EEG presenting an intermediate EEG pattern, a seizure suspicion led to prescribe Levetiracetam. The EEG showed then the appearance of burst suppression, which was compatible with a very severe pattern of post-anoxic coma. This aggravation was in fact related to an overdose of Levetiracetam (the only medication introduced recently) and was reversible after Levetiracetam cessation. The increased plasmatic dosages of Levetiracetam confirming this overdose could have been favoured by a moderate reduction of renal clearance, previously underestimated because of a low body-weight. This EEG dynamic was unexpected under Levetiracetam and could sign a functional instability after anoxia. Burst suppression is classically observed with high doses of anaesthetics, but is not expected after a minor anti-epileptic drug. This report proposes that Levetiracetam tolerance might not be straightforward after brain lesions and engages us to avoid confounding factors during the awakening prognostication, which is mainly based on the severity of the EEG. Hence, prognosis should not be decided on an isolated parameter, especially if the dynamic is atypical after a new prescription, even for well-known drugs. For any suspicion, the drug's dosage and replacement should be managed before any premature care's withdrawal.
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http://dx.doi.org/10.1016/j.clineuro.2016.11.004DOI Listing
January 2017

The Paradoxical Significance of Headache in Hypertension.

Am J Hypertens 2016 09 19;29(9):1109-16. Epub 2016 Apr 19.

Cardiology Department, European Society of Hypertension Excellence Center, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, Lyon, France; Université de Lyon, CREATIS, CNRS UMR5220, Inserm U1044, INSA-Lyon, Université Claude Bernard Lyon 1, Hospices Civils de Lyon, Lyon, France;

Background: The cardiovascular prognostic value of various types of headache, particularly migraine, in the general population remains controversial. The aim of the present study was to assess their prognostic value for all-cause, cardiovascular and stroke mortalities in hypertensive patients.

Methods: A total of 1,914 hypertensive individuals were first categorized according to the absence or presence of headache and thereafter according to the 3 subtypes of headache: migraine, daily headache, and other headache.

Results: Multiple regression analysis demonstrated that all headache types were predicted by gender (women), diastolic blood pressure, absence of diabetes, secondary hypertension, and a trend for severe retinopathy. After 30 years of follow-up, 1,076 deaths were observed, 580 of whom were from cardiovascular cause and 97 from acute stroke. In a multivariable Cox model adjusted for major confounders, patients having headache had a decreased risk for all-cause mortality (hazard ratio (HR) 0.82; 95% confidence interval (CI) 0.73-0.93) and cardiovascular mortality (HR 0.80; 95% CI 0.68-0.95), but not for stroke mortality (HR 1.00; 95% CI 0.70-1.43). When considering only patients with headache, "daily headache" had a nonsignificant better prognostic value for all-cause and cardiovascular mortality than "other headache" (HR 0.83; 95% CI 0.68-1.01; HR 0.89; 95% CI 0.69-1.16, respectively) and "migraine" (HR 0.85; 95% CI 0.65-1.11; HR 0.78; 95% CI 0.55-1.10, respectively).

Conclusion: Presence of nonspecific headache in hypertensive patients has a paradoxical significance in that it is associated with a high-risk profile but does not result in a worse prognosis over the long term.
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http://dx.doi.org/10.1093/ajh/hpw041DOI Listing
September 2016

[Cluster headache and brain imagery].

Presse Med 2015 Nov 12;44(11):1185-7. Epub 2015 Nov 12.

Hospices civils de Lyon, hôpital de la Croix-Rousse, consultation céphalées-migraine, service de neurologie, 103, grande rue de la Croix-Rousse, 69317 Lyon cedex 04, France; Lyon Neuroscience Research Center (CRNL), Brain Dynamics and Cognition Team (Dycog), Inserm U1028, CNRS UMR5292, 69000 Lyon, France.

Cluster headache is defined on clinical international criteria developed by International Headache Society (IHS, 2013). The realization of a brain MRI with arterial angio-MRI is required according to the French recommendations (Donnet et al., 2014) based on recent the literature. Numerous causes or diseases can mimic typical or atypical AVF (Edvardsson, 2014). Identification of these causes allows an appropriate treatment in addition with symptomatic treatment.
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http://dx.doi.org/10.1016/j.lpm.2015.05.007DOI Listing
November 2015

Central Nervous System Underpinnings of Sensory Hypersensitivity in Migraine: Insights from Neuroimaging and Electrophysiological Studies.

Headache 2016 Oct 9;56(9):1418-1438. Epub 2015 Sep 9.

Neurological Hospital Pierre Wertheimer: Functional Neurology and Epilepsy Department, Hospices Civils de Lyon and Claude Bernard Lyon1 University, Lyon, France, and Lyon Neuroscience Research Center (CRNL), Neuropain team, INSERM U1028, CNRS UMR5292, Lyon, France.

Whereas considerable data have been generated about the pathophysiology of pain processing during migraine attacks, relatively little is known about the neural basis of sensory hypersensitivity. In migraine, the term "hypersensitivity" encompasses different and probably distinct pathophysiological aspects of sensory sensitivity. During attacks, many patients have enhanced sensitivity to visual, auditory and/or olfactory stimuli, which can enhance headache while interictally, migraineurs often report abnormal sensitivity to environmental stimuli that can cause nonpainful discomfort. In addition, sensorial stimuli can influence and trigger the onset of migraine attacks. The pathophysiological mechanisms and the origin of such sensitivity (individual predisposition to develop migraine disease or consequence of repeated migraine attacks) are ill understood. Functional neuroimaging and electrophysiological studies allow for noninvasive measures of neuronal responses to external stimuli and have contributed to our understanding of mechanisms underlying sensory hypersensitivity in migraine. The purpose of this review is to present pivotal neuroimaging and neurophysiological studies that explored the basal state of brain responsiveness to sensory stimuli in migraineurs, the alterations in habituation and attention to sensory inputs, the fluctuations of responsiveness to sensory stimuli before and during migraine attacks, and the relations between sensory hypersensitivity and clinical sensory complaints.
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http://dx.doi.org/10.1111/head.12651DOI Listing
October 2016

Attention orienting dysfunction with preserved automatic auditory change detection in migraine.

Clin Neurophysiol 2014 Mar 8;125(3):500-11. Epub 2013 Nov 8.

INSERM, U1028, Lyon Neuroscience Research Center, Brain Dynamics and Cognition Team, Lyon F-69000, France; CNRS, UMR5292, Lyon Neuroscience Research Center, Brain Dynamics and Cognition Team, Lyon F-69000, France; Université Lyon 1, Lyon F-69000, France.

Objective: To investigate automatic event-related potentials (ERPs) to an auditory change in migraine patients.

Methods: Auditory ERPs were recorded in 22 female patients suffering from menstrually-related migraine and in 20 age-matched control subjects, in three sessions: in the middle of the menstrual cycle, before and during menses. In each session, 200 trains of tone-bursts each including two duration deviants were presented in a passive listening condition.

Results: In all sessions, duration deviance elicited a mismatch negativity (MMN) showing no difference between the two groups. However, migraine patients showed an increased N1 orienting component to all incoming stimuli and a prolonged N2b to deviance. They also presented a different modulation of P3a amplitude along the menstrual cycle, which tended to normalise during migraine attacks. None of the studied ERP components showed a default of habituation.

Conclusions: This passive paradigm highlighted increased automatic attention orienting to auditory changes but normal auditory sensory processing in migraineurs.

Significance: Our observations suggest normal auditory processing up to attention triggering but enhanced activation of attention-related frontal networks in migraineurs.
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http://dx.doi.org/10.1016/j.clinph.2013.05.032DOI Listing
March 2014

[Acute and chronic headache].

Rev Prat 2013 Apr;63(4):551-8

Service de neurologie, hôpital Croix-Rousse, 69004 Lyon, France.

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April 2013

Primary cough headache, primary exertional headache, and primary headache associated with sexual activity: a clinical and radiological study.

Neuroradiology 2013 Feb 2;55(3):297-305. Epub 2012 Nov 2.

Service de Neurologie et Pathologie du Mouvement, Pole Neurosciences Cliniques, Hôpital Timone, 264 Boulevard Saint-Pierre, 13385 Marseille Cedex 05, France.

Introduction: The purposes of this study are to describe clinical features of primary cough headache, primary exertional headache, and primary headache associated with sexual activity and to evaluate potential association with abnormalities in the cerebral or cervical venous circulation.

Methods: This multicentre, observational, non-interventional consecutive cohort study included patients fulfilling ICHD-II criteria for primary cough headache (N = 10), primary exertional headache (N = 11), or primary headache associated with sexual activity (N = 20), as well as 16 headache-free controls. Each patient was evaluated clinically and underwent craniocervical MRV of the cranial circulation. All scans were interpreted centrally by blinded raters, using the Farb criteria proposed for idiopathic intracranial hypertension. Stenosis was defined as a Farb score <3 in left or right transverse sinuses or jugular veins.

Results: In all primary headache groups, headaches were most frequently diffuse, severe, or very severe. Headache duration was significantly shorter in patients with cough headache (median 6.5 versus 20 and 60 min). An exploitable magnetic resonance venogram was obtained for 36 patients. Stenosis was detected in none of the control group, but in 5/7 patients with primary cough headache group, 2/10 patients with primary exertion headache, and 12/19 patients with primary headache associated with sexual activity. The frequency of stenosis was significantly different from the control group in the primary cough headache and primary headache associated with sexual activity groups.

Conclusions: Headaches provoked by cough and sexual activity are possibly associated with venous abnormalities in a significant subgroup of affected patients. As the literature shows conflicting results, this venous stenosis can be considered as a promoting factor.
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http://dx.doi.org/10.1007/s00234-012-1110-0DOI Listing
February 2013

Exacerbated attention orienting to auditory stimulation in migraine patients.

Clin Neurophysiol 2011 Sep 10;122(9):1755-63. Epub 2011 Mar 10.

Hospices Civils de Lyon, Croix-Rousse Hospital, Neurology Department, Lyon F-69000, France.

Objective: To investigate long-term (LTH) and short-term (STH) habituation of auditory event-related potentials (ERPs) during a migraine cycle, using a classic habituation paradigm.

Methods: In 22 patients suffering from menstrually-related migraine and in 20 age-matched control subjects, auditory ERPs were recorded in 3 sessions: in the middle of the menstrual cycle, before menses, and during menses. In 12 patients, a migraine attack occurred during one of the peri-menses sessions. In each session, 200 trains of stimuli were presented, with an average of 10 stimuli per train.

Results: In response to the first stimuli of the trains, migraineurs exhibited in all sessions a larger orienting component of N1 than matched controls and a larger P3a in the interictal session, which normalized during attacks. They also showed a residual orienting component in response to the subsequent stimuli inside the trains. In contrast, the sensory component of N1 showed no difference between the two groups, with similar STH and LTH.

Conclusions: Migraineurs show an exacerbated attention orienting to auditory stimulation, without any habituation deficit.

Significance: Previous migraine studies reported interictal habituation deficits of ERPs, but demonstrated in the auditory modality only in paradigms testing intensity dependence. Previous and current results can be interpreted as an increased attention orienting, possibly relying on an abnormal involvement of frontal cortex in auditory processing.
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http://dx.doi.org/10.1016/j.clinph.2011.02.013DOI Listing
September 2011

Positional cluster-like headache. A case report of a neurovascular compression between the third cervical root and the vertebral artery.

Cephalalgia 2010 Dec 8;30(12):1509-13. Epub 2010 Jun 8.

Université Lyon 1, France.

Symptomatic cluster-like headaches have been described with lesions of the trigeminal and parasympathetic systems. Here, we report the case of a 44-year-old woman with continuous auricular pain and a positional cluster-like headache associated with red ear syndrome. Clinical data and morphological investigations raised the hypothesis of a neurovascular compression between the C3 root and vertebral artery. Neurosurgical exploration found a fibrosis surrounding both the C3 root and the vertebral artery. The excellent outcome after microvascular cervical decompression suggests a causal relationship between the cluster-like headache and the vertebral constraint on the C3 root.
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http://dx.doi.org/10.1177/0333102410373158DOI Listing
December 2010

Facial pain as first manifestation of anti-Hu paraneoplastic syndrome.

J Headache Pain 2010 Aug 13;11(4):355-7. Epub 2010 Apr 13.

Service de Neurologie, Hôpital de la Croix-Rousse, 103, Grande Rue de la Croix-Rousse, 69004 Lyon, France.

The diagnosis of anti-Hu-associated encephalomyelitis/sensory neuropathy may be particularly difficult when cranial nerve involvement represents the first clinical manifestation of the disease. We report a case of a patient who presented with facial pain as the first manifestation of an anti-Hu paraneoplastic syndrome, which needs a rapid detection and treatment of the underlying tumour. We suggest that paraneoplastic neuropathy should be considered during the management of trigeminal neuropathic pain, especially when brain imagery is normal.
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http://dx.doi.org/10.1007/s10194-010-0212-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3476344PMC
August 2010

Impact of a public sensitization campaign on migraine management in France.

J Headache Pain 2009 Aug 30;10(4):241-7. Epub 2009 May 30.

Department of Neurology, La Timone Hospital, 264 Boulevard Saint-Pierre, 13385 Marseille Cedex, France.

Migraine is a common and frequently disabling condition. Nevertheless, many migraine sufferers do not consult for migraine, are not medically followed up and self-treat the attacks. "Tour de France of migraine" consisted of free-access conferences held in six large towns in France following a wide public information campaign. This sensitization campaign aimed at providing participants with educational information on migraine disease and on current therapies. Headache sufferers were then invited to respond to two consecutive questionnaires delivered at the end of the conferences and 3 months later to assess the influence of the information delivered on their migraine management. Tour de France of migraine recruited mainly severe migraine sufferers, most of whom had already consulted and were medically followed up. However, migraine management was often suboptimal in these subjects since most of them found their acute treatment of attacks ineffective and only few of them received a prophylactic treatment. Three months after the conferences, more than half of respondents had consulted for headaches. There was a significant improvement in migraine-related disability, as reflected by a significant decrease in mean Headache Impact Test 6-item score, which might have been related to the higher proportion of subjects receiving a prophylactic treatment of migraine. The Tour de France of migraine campaign revealed the difficulty in sensitizing migraine sufferers towards the necessity of being medically followed up. Mainly patients with severe migraine attended the conferences and derived clinical benefit from the educational program. Other strategies should be developed to reach a wider population of migraine sufferers.
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http://dx.doi.org/10.1007/s10194-009-0128-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3451749PMC
August 2009

Nocturnal hypermotor seizures, suggesting frontal lobe epilepsy, can originate in the insula.

Epilepsia 2006 Apr;47(4):755-65

Department of Functional Neurology and Epileptology, Hospices Civils de Lyon and Université Claude Bernard Lyon 1, Lyon, France.

Purpose: To report three patients with drug-resistant nocturnal hypermotor seizures (NHSs), no detectable brain lesion, and clinically defined nocturnal frontal lobe epilepsy (NFLE) or autosomal dominant NLFE (ADNFLE), whose intracerebral EEG ictal onset primarily involved the insula, rather than the mesial or orbital frontal cortex.

Methods: Fourteen to 15 intracerebral electrodes were implanted in each patient, primarily sampling the frontal lobes with 80 to 91 recording leads covering the most likely side of seizure onset, and two to six leads placed within the ipsilateral insula. Electrical stimulation was used to test the epileptic threshold of frontal and insular brain regions at the various recording sites.

Results: In all three patients, a low-voltage fast activity was recorded within the anterosuperior aspect of the insula at ictal onset, either in isolation, or extending to the nearby frontal operculum in the ADNFLE patient. The role of the insula was further supported in all three patients either by the presence of high-amplitude spikes that clearly predominated over that region (n = 2) or by triggering the patient's typical aura or seizure when applying an electrical stimulation at that site, selectively (n = 2).

Conclusions: The anterosuperior portion of the insula might play a pivotal role in generating nocturnal hypermotor seizures in some patients with nonlesional drug-resistant epilepsy suggesting NFLE or ADNFLE. Whether these patients are amenable to successful surgery remain an open issue.
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http://dx.doi.org/10.1111/j.1528-1167.2006.00510.xDOI Listing
April 2006

Involvement of medial pulvinar thalamic nucleus in human temporal lobe seizures.

Epilepsia 2006 Jan;47(1):98-107

Department of Functional Neurology and Epileptology, Federative Institute of Neurosciences (INSERM IFR 19), Bron, France.

Purpose: Several animal studies suggest that the thalamus might be involved in the maintenance and propagation of epileptic seizures. However, electrophysiologic evidence for this implication in human partial epileptic seizures is still lacking. Considering the rich and reciprocal connectivity of the medial pulvinar (PuM) with the temporal lobe, we evaluated a potential participation of this thalamic nucleus in temporal lobe epilepsy (TLE).

Methods: The electrophysiologic activity of PuM was recorded during stereoelectroencephalographic exploration of spontaneous temporal lobe seizures in 14 patients referred for presurgical assessment of refractory TLE.

Results: We recorded PuM ictal activity in 80% of the 74 seizures that we analyzed. This activity was characterized by rhythmic slow-waves or rhythmic spikes (RSW-RS) or both or by low-voltage fast activity (LVFA) in 64% and 36% of seizures, respectively. RSW-RS occurred mostly in seizures arising from mesiotemporal structures, whereas LVFA was more frequently observed in seizures of neocortical origin. In the 15 seizures without PuM ictal activity, spreading of the seizure outside the onset zone never occurred, whereas it did in 78% of seizures with PuM ictal involvement. Discharge propagation was systematic when PuM involvement corresponded to LVFA, whatever the seizure onset zone was, whereas it represented only 66% of the seizures when PuM exhibited RSW-RS.

Conclusions: This study shows that ictal changes in PuM activity are frequently observed during temporal lobe seizures and suggests that this thalamic nucleus might participate in their propagation.
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http://dx.doi.org/10.1111/j.1528-1167.2006.00375.xDOI Listing
January 2006

Ipsilateral head deviation in frontal lobe seizures.

Epilepsia 2005 Nov;46(11):1750-3

Department of Functional Neurology and Epileptology, Neurological Hospital, Lyon, France.

Purpose: The lateralizing value of ictal head deviation (HD) in frontal lobe epilepsy (FLE) is a matter of debate. Although FLE is typically associated with tonic or clonic HD contralateral to seizure onset, ipsilateral HD has been noted in numerous reports. Whether both types of HD can be distinguished according to their clinical patterns has not yet been specifically investigated.

Methods: We studied the clinical pattern and time of occurrence of HD of 129 seizures in 13 consecutive patients, who underwent successful surgery for FLE, including 12 investigated with an intracerebral stereotactic EEG procedure.

Results: Ictal HD was ipsilateral to the epileptogenic zone (EZ) in four (30%) patients and 27 (20.9%) seizures and contralateral in five (38%) patients and 15 (11.6%) seizures. Ipsilateral HD was rarely tonic and never associated with clonic manifestation. Contralateral HD was always tonic, unnatural, and associated with hemifacial clonic movements in 86% of seizures. Ipsilateral HD occurred earlier than contralateral HD (p < 0.03), with a mean delay of 1 +/- 2 s after the first detectable ictal sign, as compared with a delay of 17 +/- 11 s for contralateral HD. Moreover, ipsilateral HD always occurred before contralateral HD when both signs coexisted in the same seizure. Our patients with ipsilateral HD demonstrated either an anterior or dorsolateral frontal EZ.

Conclusions: Ipsilateral HD is a common ictal sign during FLE and can be distinguished from contralateral HD by its time of occurrence at or immediately after seizure onset and its lacking association with clonic movements.
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http://dx.doi.org/10.1111/j.1528-1167.2005.00293.xDOI Listing
November 2005

Ictal dysprosody and the role of the non-dominant frontal operculum.

Epileptic Disord 2005 Sep;7(3):193-7

Service de Neurologie Fonctionnelle et d'Epileptologie, Lyon, France.

Prosody is an important feature of language and refers to variations in the acoustic properties of timing, intensity and fundamental frequency that are used to convey affective or linguistic information. The prosodic component of ictal speech has not yet been specifically studied in epileptic patients. We report the case of a patient with right mesial temporal lobe epilepsy who developed ictal, recurrent speech utterances associated with an altered prosody during both spontaneous seizures and a very localized stimulation-induced discharge of the non-dominant precentral operculum, a finding consistent with results of functional magnetic resonance imaging studies in healthy subjects. This should prompt future studies, with the aim of better evaluating the localizing value of ictal dysprosody in patients with drug resistant, partial epilepsy.
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September 2005

Ipsilateral head deviation related to orbito-frontal and fronto-polar seizures.

Epileptic Disord 2005 Jun;7(2):97-102

Department of Functional Neurology and Epileptology, Neurological Hospital Pierre Wetheimer, Lyon, France.

The localizing and lateralizing values of eye and head ictal deviations during frontal lobe seizures are still matters of debate. In particular, no specific data regarding the origin of ipsilateral head turning in frontal lobe seizures are available. We report a patient with frontal lobe seizures associated with reproducible, early, ipsilateral head deviation, where imaging and video-stereo-electroencephalography data, as well as surgical outcome, demonstrated the fronto-polar and orbito-frontal origin of the epileptic discharge. We conclude that early ipsilateral head deviation, in the context of frontal lobe epilepsy, raises the possibility of fronto-polar or orbito-frontal seizure onset.[Published with video sequences].
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June 2005

Ethical issues of informed consent in acute stroke. Analysis of the modalities of consent in 56 patients enrolled in urgent therapeutic trials.

Cerebrovasc Dis 2005 11;19(2):65-8. Epub 2005 Jan 11.

Service d'Urgences Neurovasculaires, Hôpital Neurologique de Lyon, Lyon, France.

Background: Many patients may be mentally incompetent or physically unable to give informed consent at the acute stage of stroke. Accordingly, we aimed to investigate the modalities of informed consent in urgent therapeutic stroke trials, the awareness of patients and relatives regarding stroke clinical trials and the impact of decision making on patients and relatives.

Methods: We present a study of 56 acute ischemic stroke patients who were randomized in 4 trials (2 trials testing neuroprotective agents, 1 testing thrombolysis and 1 testing antithrombotic agents). A standardized questionnaire was used to assess the modalities of informed consent in this setting.

Results: The mean age was 67.1 (SD 12.6) years. The mean baseline Scandinavian Stroke Scale (SSS) score was 23.8 (SD 10.5). Only 13 patients (23% of cases) gave consent while relatives gave consent for 43 patients (77%). The main reason for not getting consent from the patient was aphasia in 29 patients (67.4%). Multiple logistic regression analysis showed that the two independent factors influencing the ability to give consent are age and baseline neurological deficit as assessed by the SSS score. Concerning the psychological impact of consent, none of the 10 patients who answered our questionnaire declared feeling uncomfortable when giving consent, while 7 out of the 13 relatives who could be reached declared they felt uncomfortable, mainly because of the psychological stress induced by urgent decision making.

Conclusions: Our study emphasizes the specific ethical difficulties of informed consent in the setting of acute stroke research. Only a minority of patients are able to give consent at the acute stage. Increasing age and neurological deficit are independent predictors of inability to give consent. Thus, the responsibility for consent usually relies on relatives with potential inaccuracy of decision concerning the patient's wish or even conflict of interest. Further evaluation of the psychological impact of decision on relatives is needed in this setting of acute stroke.
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http://dx.doi.org/10.1159/000083250DOI Listing
April 2005

Cerebellar ataxia with oculomotor apraxia type 1: clinical and genetic studies.

Brain 2003 Dec 23;126(Pt 12):2761-72. Epub 2003 Sep 23.

Fédération de Neurologie, Hôpital de la Salpêtrière AP-HP, Paris, France.

Ataxia with ocular motor apraxia type 1 (AOA1) is an autosomal recessive cerebellar ataxia (ARCA) associated with oculomotor apraxia, hypoalbuminaemia and hypercholesterolaemia. The gene APTX, which encodes aprataxin, has been identified recently. We studied a large series of 158 families with non-Friedreich progressive ARCA. We identified 14 patients (nine families) with five different missense or truncating mutations in the aprataxin gene (W279X, A198V, D267G, W279R, IVS5+1), four of which were new. We determined the relative frequency of AOA1 which is 5%. Mutation carriers underwent detailed neurological, neuropsychological, electrophysiological, oculographic and biological examinations, as well as brain imaging. The mean age at onset was 6.8 +/- 4.8 years (range 2-18 years). Cerebellar ataxia with cerebellar atrophy on MRI and severe axonal sensorimotor neuropathy were present in all patients. In contrast, oculomotor apraxia (86%), hypoalbuminaemia (83%) and hypercholesterolaemia (75%) were variable. Choreic movements were frequent at onset (79%), but disappeared in the course of the disease in most cases. However, a remarkably severe and persistent choreic phenotype was associated with one of the mutations (A198V). Cognitive impairment was always present. Ocular saccade initiation was normal, but their duration was increased by the succession of multiple hypometric saccades that could clinically be confused with 'slow saccades'. We emphasize the phenotypic variability over the course of the disease. Cerebellar ataxia and/or chorea predominate at onset, but later on they are often partially masked by severe neuropathy, which is the most typical symptom in young adults. The presence of chorea, sensorimotor neuropathy, oculomotor anomalies, biological abnormalities, cerebellar atrophy on MRI and absence of the Babinski sign can help to distinguish AOA1 from Friedreich's ataxia on a clinical basis. The frequency of chorea at onset suggests that this diagnosis should also be considered in children with chorea who do not carry the IT15 mutation responsible for Huntington's disease.
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http://dx.doi.org/10.1093/brain/awg283DOI Listing
December 2003
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