The teaching of headache medicine in France: A questionnaire-based study.

BACKGROUND: The treatment of patients with headache represents an important part of a neurologist's activity. It requires sufficient training for neurology residents. In France, residents in neurology can complete this training by attending specialized consultations or by participating in a postgraduate training program called "Diplôme Inter-Universitaire Migraine et Céphalées" (DIUMC). OBJECTIVE: The objective of this cross-sectional study was to investigate the French residents' knowledge in headache medicine and the impact of different types of training in headache medicine that are available in France. METHODS: An anonymous survey was carried out among 548 French residents in neurology. RESULTS: The questionnaires of 121 residents (22.1%) were analyzed. Among them, 54.5% (66/121) had no complementary training apart from the internship (Group 1), 21.5% (26/121) had attended only specialized consultations (Group 2), and 24% (29/121) had participated in the DIUMC (Group 3). There was no difference between all groups regarding the knowledge of the prevalence of primary or chronic headaches. There was almost no difference between the groups in the management of episodic migraine. In contrast, the management of tension-type headache and chronic headache was better known by residents of Group 3 than residents of Group 1. In these two diseases, residents of Group 3 offered prophylactic treatment more often. Almost 29% of the residents (35/121) had read the French guidelines for the diagnosis and management of migraine. In Group 3, residents had read them significantly more often (1.6% in Group 1, 38.5% in Group 2 and 62.1% in Group 3, p < 0.001). CONCLUSION: This study shows the lack of knowledge among French neurology residents regarding headache medicine. It highlights the interest of specific training programs that could improve the practical and theoretical knowledge of future neurologists.

A retrospective observation on 105 patients with chronic cluster headache receiving indomethacin.

BACKGROUND: Indomethacin (IMC) as a prophylactic treatment is considered to be ineffective in cluster headache (CH). However, small series suggested the interest of IMC in CH. Some authors support that an IMC test is useful in all trigeminal autonomic cephalalgias. We described clinical features of IMC responders in a retrospective cohort of chronic cluster headache (CCH). METHODS: This single-center and retrospective study was conducted in a tertiary care specialist headache center in France. Patients were selected between January 2007 and December 2008. We included all patients fulfilling CCH criteria (ICHD-3-beta). Data were collected from medical records. We recorded all the prescriptions of IMC as a prophylactic treatment. Responders were defined by 50% reduction in attack frequency; complete response was defined by disappearance of the attacks. The non-responders must have received at least 100 mg daily during 7 days. RESULTS: The study consisted of 324 CCH, 121 female (37%) and 203 males (63%) with an average age at onset of 33.93 (± 14.71) years. Of the patients, 105 were treated with IMC. Thirty patients (29%) were responders. Thirty-four patients (32%) were non-responders. Responding status was undefined for 41 patients (39%). Twelve patients (11%) had a complete response. Responders were composed by 18 women (60%) and 12 men (40%) and had on average 44.89 (± 12.88) years. The minimal effective dose was 86.11 mg daily (± 48.72). DISCUSSION: This study shows the interest of IMC in CCH patients. We recommend an IMC test as a third-line treatment in CCH.

Contribution of Otoacoustic Emissions for Diagnosis of Atypical or Recurrent Intracranial Hypotension. A Cases Series.

BACKGROUND: The diagnosis of spontaneous or post-traumatic intracranial hypotension mainly rely on clinical features and neuroimaging. In atypical presentations, other non-invasive tests are missing to support the diagnosis of intracranial hypotension. The assessment of otoacoustic emissions (OAE) shown interest to monitor intracranial pressure mainly in cases of intracranial hypertension. This non-invasive method was also assessed in response to pressure change after lumbar puncture. A few reports showed abnormal results of distortion product otoacoustic emissions (DPOAE) in cases of spontaneous or post-traumatic intracranial hypotension. We describe additional results in a series of intracranial hypotension cases. We discuss the interest of DPOAE assessment in atypical presentations of intracranial hypotension. We review the other non-invasive tests reported in literature. METHODS: We studied 4 atypical or recurrent cases of spontaneous or post-traumatic intracranial hypotension by recording OAE in sitting then supine position. RESULTS: Unilateral or bilateral abnormal changes of DPOAE were recorded in all cases, in response to postural test. These changes were present even in the absence of vestibular symptoms. CONCLUSION: The study of OAE may be a non-invasive tool for the diagnosis of spontaneous intracranial hypotension.

Sneezing in Primary Headaches With Cranial Autonomic Symptoms: Pathophysiological Considerations. A Series of Case Reports.

OBJECTIVE: To report a case series of sneezing systematically occurring in two cases with migraine and cranial autonomic symptoms and two with cluster headache. BACKGROUND: Sneezing occurring systematically during cluster headache or migraine is a rare condition. Sneezing has been described as a specific trigger for migraine. METHODS: Case reports. CASE SERIES: We describe four patients with primary headaches, presenting systematic sneezing at a definite time of premonitory, postdromal, or headache phase. All of these headaches were associated with cranial autonomic symptoms. CONCLUSION: Sneezing is a symptom associated with primary headaches. The definite place of sneezing in these patients suggests a particular implication of hypothalamus in the pathophysiology of these cases.

Cyclic Vomiting Syndrome in Children.

PURPOSE OF REVIEW: Cyclic vomiting syndrome (CVS) is a misrecognized and probably underdiagnosed episodic syndrome associated with migraine, occurring preferentially in childhood. Attacks are stereotyped for each individual, with predictable periodicity. This review summarizes recent clinical and paraclinical observations in this syndrome, and current approaches in explorations and therapeutics. RECENT FINDINGS: Clinical phenotype during prodromal, vomiting, and recovery phases contains visceral and neuropsychological symptoms, but also cranial and systemic symptoms. Some clinical arguments as circadian or circannual periodicity suggest a chronobiological disease. Red flags in clinical presentation are proposed to distinguish other etiologies of recurrent gastrointestinal disturbances and guide paraclinical explorations. Functional magnetic resonance imaging in both CVS and migraine displayed diminished insular connectivity with the sensorimotor network, suggesting a common pathophysiology. Pathophysiology of CVS is not well defined, and there is probably a multifactorial origin. Distinction with other differential diagnoses is a challenge for clinicians. Further research, in particular with functional imaging, are required to define pathophysiology of CVS. Control trials are missing in pediatric population. Injectable or intranasal sumatriptan are often effective. For prophylaxis, amitriptyline, cyproheptadine, or propranolol are the most common treatments, depending on age and comorbidities. Non-pharmacologic measures as lifestyle modification also seem to be effective as preventive treatment.

Cyclic Vomiting Syndrome in Adults and Children: A Hypothesis.

OBJECTIVE: To see how patients' clinical descriptions compare to the third classification of International Classification of Headache Disorders (ICHD-III beta). BACKGROUND: CVS is an episodic syndrome associated with migraine. It is a rare disorder of childhood and adulthood. New criteria are proposed in the third classification of International Classification of Headache Disorders. METHODS: This study describes children and adults' CVS characteristics at a French tertiary-care center, according to the ICHD-II and ICHD-III beta criteria. Clinical features, and results of paraclinical tests were characterized. RESULTS: Fifteen patients were included: 8 adults, 2 children, and 5 adolescents. Familial history of migraine or CVS was respectively noticed in 12 and 4 patients. The duration of premonitory, emetic, and postdromal phases was evaluated from hours to days. Some symptoms described in theses phases are also described in migrainous population, with a comparable frequency. Cranial or systemic autonomic symptoms were respectively reported in 47% and 93%. Nine patients described a circadian periodicity, and six patients described a circannual periodicity. The predictable periodicity was from 1 week to 12 months. A brain perfusion SPECT showed an increased hypothalamic perfusion during emetic phase in one patient. CONCLUSIONS: CVS is a rare episodic syndrome associated with migraine. Some clinical features and paraclincal tests suggest a chronobiological disease.

Cyclic vomiting syndrome, a common language? A 20-year bibliometric study.

BACKGROUND: Cyclic vomiting syndrome (CVS) is recognized as an episodic syndrome associated with migraine in the last version of the International Classification of Headache Disorders (ICHD-3). It manifests as stereotypical episodes of intense nausea and vomiting, occurring preferentially in childhood. Over the last 2 decades, the knowledge of this disorder has increased. The diagnostic criteria have been modified, through the evolution of several successive classifications. Actually, two classifications are prominent in the literature: the ICHD-3 and the Rome IV classification. The predictable periodicity of episodes is only recognized in the ICHD-3. OBJECTIVE: We aimed to analyze the evolution of CVS literature in the last 2 decades, with a focus on CVS criteria used in these papers. METHODS: We conducted a bibliometric study. We searched in the Web of Science database all papers in English literature with the term CVS in the abstract or title, in the category "article" or "review", published from 2001 to 2020. We searched within the paper which classification was used or mentioned. RESULTS: In total, 213 papers were analyzed. 116 papers exclusively concerned childhood and adolescence CVS, or were written by pediatric practitioners. For most of the papers, the corresponding author was specialized in the field of gastroenterology. The Rome III classification was the main classification used or mentioned. The ICHD-3 and its beta version were mostly used or mentioned by the authors affiliated to the neurologic field. CONCLUSION: This study shows the growth in the number of publications on CVS. It highlights the lack of reference to the ICHD, in particular by practitioners in the field of gastroduodenal disorders. This should encourage the achievement of a common classification with the different scientific societies.

Hemiplegic Migraine Associated With PRRT2 Variations: A Clinical and Genetic Study.

BACKGROUND AND OBJECTIVE: PRRT2 variants have been reported in a few cases of patients with hemiplegic migraine. To clarify the role of PRRT2 in familial hemiplegic migraine, we studied this gene in a large cohort of affected probands. METHODS: PRRT2 was analyzed in 860 probands with hemiplegic migraine, and PRRT2 variations were identified in 30 probands. Genotyping of relatives identified a total of 49 persons with variations whose clinical manifestations were detailed. RESULTS: PRRT2 variations were found in 12 of 163 probands who previously tested negative for CACNA1A, ATP1A2, and SCN1A variations and in 18 of 697 consecutive probands screened simultaneously on the 4 genes. In this second group, pathogenic variants were found in 105 individuals, mostly in ATP1A2 (42%), followed by CACNA1A (26%), PRRT2 (17%), and SCN1A (15%). The PRRT2 variations included 7 distinct variants, 5 of which have already been described in persons with paroxysmal kinesigenic dyskinesia and 2 new variants. Eight probands had a deletion of the whole PRRT2 gene. Among the 49 patients with variations in PRRT2, 26 had pure hemiplegic migraine and 16 had hemiplegic migraine associated with another manifestation: epilepsy (8), learning disabilities (5), hypersomnia (4), or abnormal movement (3). Three patients had epilepsy without migraine: 2 had paroxysmal kinesigenic dyskinesia without migraine, and 1 was asymptomatic. DISCUSSION: PRRT2 should be regarded as the fourth autosomal dominant gene for hemiplegic migraine and screened in any affected patient, together with the 3 other main genes. Further studies are needed to understand how the same loss-of-function PRRT2 variations can lead to a wide range of neurologic phenotypes, including paroxysmal movement disorder, epilepsy, learning disabilities, sleep disorder, and hemiplegic migraine.

The neurologist and the hydrops.

The presence of endolymphatic hydrops has been studied in many neurological disorders. The pathophysiological mechanisms may involve CSF pressure variations, transmitted to the innear ear. This hydrops could play a role in vestibular or cochlear symptoms. For the ENT specialist, the etiological diagnosis of endolymphatic hydrops is a challenge, and neurological etiologies must be known. The treatment of these neurological causes could be effective on cochleo-vestibular symptoms. The knowledge of endolymphatic hydrops could also be a target for noninvasive tests, able to estimate CSF pressure variations. For the neurologist, this could represent a useful tool for the diagnosis and follow-up, in some of these neurological disorders, related to a CSF pressure imbalance. The purpose of this paper is to summarize literature data on endolymphatic hydrops in neurological disorders. We define some neurological conditions, for which there is a particular interest in noninvasive investigations of endolymphatic hydrops.

Stroke after spontaneous intracranial hypotension: Not a single mechanism. Case report and review of literature.

Spontaneous intracranial hypotension (SIH) is a rare syndrome, typically manifests as orthostatic headache. Sometimes considered asbenignillness, neurological complications are well described, in particular subdural hematoma and cerebral venous sinus thrombosis. Brain infarction as complication of SIH is rarely reported. The main mechanism supported in the literature is the stretching of arteries due to the sagging of the brain. We report a case of SIH followed with brain infarction, with a distinct presentation from previous literature, suggesting a different mechanism. A 35 year-old had severe orthostatic headache, responsible for prolonged bed rest. One month later, he had acute left hemiparesis secondary to stroke and right posterior cerebral artery occlusion. Stroke MRI showed arguments for intracranial hypotension (thickened meninges). He was successfully treated with intravenous rtPA thrombolysis. Headache were resolved after an epidural blood patch. A patent foramen ovale was detected. Clinical features of this description were compared with previous literature. This case suggest a different mechanism for cerebral infarction after intracranial hypotension. In case of prolonged lying down due to intracranial hypotension, the presence of patent foramen ovale could be a risk factor for embolic stroke.