Development of an Objective Structured Clinical Examination (OSCE) to evaluate the diagnosis announcement of chronic neurological disease by residents in neurology.

BACKGROUND: There is little consensus on how to make a diagnosis announcement of severe chronic disease in neurology. Other medical specialties, such as oncology, have developed assessment methods similar to the Objective Structured Clinical Examination (OSCE) to address this issue. Here we report the implementation of an OSCE focused on the diagnosis announcement of chronic disease in neurology by residents. OBJECTIVE: We aimed to evaluate the acceptability, feasibility and validity in routine practice of an OSCE combined with a theoretical course focused on diagnosis announcement in neurology. METHOD: Eighteen neurology residents were prospectively included between 2019 and 2022. First, they answered a questionnaire on their previous level of training in diagnosis announcement. Second, in a practical session with a simulated patient, they made a 15-min diagnosis announcement and then had 5mins of immediate feedback with an expert observer, present in the room. The OSCE consisted of 4 different stations, with standardized scenarios dedicated to the announcement of multiple sclerosis (MS), Parkinson's disease (PD), Alzheimer's disease (AD) and amyotrophic lateral sclerosis (ALS). Third, in a theory session, expert observers covered the essential theoretical points. All residents and expert observers completed an evaluation of the "practical session" and the "theory session". RESULTS: Residents estimated their previous level of diagnosis announcement training at 3.1/5. The most feared announcements were AD and ALS. The "practical session" was rated at a mean of 4.1/5 by the residents and 4.8/5 by the expert observers, and the "theory session" at a mean of 4.7/5 by the residents and 5/5 by the expert observers. After the OSCEs, 11 residents felt more confident about making an announcement. CONCLUSION: This study has shown a benefit of using an OSCE to learn how to make a diagnosis announcement of severe chronic disease in neurology. OSCEs could be used in many departments in routine practice and seem adapted to residents.

Migraine treatment: Position paper of the French Headache Society.

The French migraine management recommendations were published in 2021. However, in the last three years, new data have come to light and new drugs have been approved (eptinezumab, rimegepant and atogepant) by the European Medicines Agency that require us to take a position on their use and to update certain elements of the recommendations. The first important message concerns the position of the French Headache Society on the use of preventive treatments (monoclonal antibodies and gepants) targeting the calcitonin gene-related peptide (CGRP) pathway. In terms of efficacy and safety, and as suggested by other national headache societies, these treatments can be offered as first-line treatment, although the scope defined by the French national health authority for possible reimbursement is limited to patients with severe migraine, at least eight headache days per month and for whom two previous preventive treatments have failed. Another important change concerns the position of topiramate as a preventive treatment for migraine in women of childbearing age. This treatment has been proposed as a first-line treatment for chronic migraine. However, recent pharmacovigilance data have highlighted a potential adverse effect on neurodevelopment in children exposed in utero. As a result, this treatment is formally contraindicated during pregnancy and must be used with extreme caution in women of childbearing age (effective contraception, no therapeutic alternative available and annual follow-up as with valproate). It can therefore no longer be offered as first-line treatment for women of childbearing age.

Does post dural puncture headache exist in idiopathic intracranial hypertension? A pilot study.

BACKGROUND/OBJECTIVE: Occurrence of post-dural puncture headache (PDPH) after diagnostic lumbar puncture (LP) for idiopathic intracranial hypertension (IIH) may seem very unlikely in clinical practice. Nevertheless, it has been suggested by several studies, mainly in sub-group analyses. We aimed to evaluate the prevalence of PDPH in an IIH population and determine any eventual predictive factors of PDPH occurrence. METHODS: We conducted a retrospective multiple-center observational study. All newly diagnosed IIH patients who met the International Classification of Headache Disorders (ICHD-3) or the Dandy modified criteria were included from three different French hospitals. They all underwent LP following the same process with the same type of needle. We recorded PDPH occurring within five days after LP, as defined by ICHD-3 criteria. RESULTS: Seventy-four IIH patients were recruited, of whom 23 (31%) presented with PDPH. Neither classical risk factors for PDPH such as body mass index, age or gender, nor cerebrospinal fluid opening pressure, or specific IIH features were associated with occurrence of PDPH. CONCLUSION: PDPH can occur after LP in IIH patients. Clinicians should be aware of this possible event during the IIH diagnosis assessment and should not automatically reconsider IIH diagnosis. PDPH prevention using an atraumatic needle and dedicated PDPH treatment seem relevant in IIH patients.

Revised guidelines of the French headache society for the diagnosis and management of migraine in adults. Part 3: Non-pharmacological treatment.

The French Headache Society proposes updated French guidelines for the management of migraine. This article presents the third part of the guidelines, which is focused on the non-pharmacological treatment of migraine, including physical exercise, dietary supplements and plants, diets, neuromodulation therapies, acupuncture, behavioral interventions and mindfulness therapy, patent foramen ovale closure and surgical nerve decompression.

Revised guidelines of the French headache society for the diagnosis and management of migraine in adults. Part 2: Pharmacological treatment.

The French Headache Society proposes updated French guidelines for the management of migraine. This article presents the second part of the guidelines, which is focused on the pharmacological treatment of migraine, including both the acute treatment of attacks and the prophylaxis of episodic migraine as well as chronic migraine with and without medication overuse. The specific situations that can be encountered in women with migraine are also discussed, including pregnancy, menstrual migraine, contraception and hormonal replacement therapy.

Revised guidelines of the French Headache Society for the diagnosis and management of migraine in adults. Part 1: Diagnosis and assessment.

The French Headache Society proposes updated French guidelines for the management of migraine. The first part of these recommendations is focused on the diagnosis and assessment of migraine. First, migraine needs to be precisely diagnosed according to the currently validated criteria of the International Classification of Headache Disorders, 3d version (ICHD-3). Migraine-related disability has to be assessed and we suggest to use the 6 questions of the headache impact test (HIT-6). Then, it is important to check for risk factors and comorbidities increasing the risk to develop chronic migraine, especially frequency of headaches, acute medication overuse and presence of depression. We suggest to use a migraine calendar and the Hospital Anxiety and Depression scale (HAD). It is also necessary to evaluate the efficacy and tolerability of current migraine treatments and we suggest to systematically use the self-administered Migraine Treatment Optimization Questionnaire (M-TOQ) for acute migraine treatment. Finally, a treatment strategy and a follow-up plan have to be proposed. Guidelines for pharmacological and non-pharmacological treatments are presented in the second and third part of the recommendations.

Solving the conundrum. A migrainous infarction or an infarct-induced migrainous attack?

Solving the conundrum between a migrainous infarction (MI) and an infarct-induced migrainous attack (MA) is challenging. A 35-year-old woman with previous history of migraine with visual auras was addressed for acute aphasia followed by progressive right hemibody paresthesia and then by positive visual symptoms in her right visual field. These phenomena were followed by a migrainous headache. A perfusion CT performed during symptoms showed an extended hypoperfusion in the left temporo-occipital region corresponding to a migraine during an aura attack. An ASL sequence brain MRI undertaken 12 hours later (while the patient was only cephalalgic) showed an area of diffuse hyper-perfusion in the left hemisphere. DWI sequence showed a left middle cerebral artery territory infarction. We believe our case was most likely to have been an infarct-induced MA. To conclude, it is crucial to rule out cerebral infarction in cases where a patient experiences an atypical aura even in the context of established migraine.

Impact of an intrahospital mobile thrombolysis team on 3-month clinical outcomes in patients benefiting from intravenous thrombolysis for acute ischemic stroke.

INTRODUCTION/OBJECTIVE: The benefits of thrombolysis in patients presenting with acute ischemic stroke (IS) are highly time-dependent. The aim of our study was to evaluate the clinical benefit, after 3 months, of an intrahospital mobile thrombolysis team (MTT) for thrombolysis in IS. PATIENTS AND METHODS: A total of 95 consecutive patients treated with IV tPA for acute IS at the neurology department of Rouen University Hospital between 1 January and 31 December 2015 were retrospectively identified. Patients who had benefited from mechanical thrombectomy or hemicraniectomy were excluded. The study compared 33 patients who had benefited from our MTT (thrombolysis whatever the location and as soon as possible by a specific nurse) with 62 patients treated in the usual way (thrombolysis only at the stroke unit). Management timescales, inhospital and 3-month clinical outcomes, and imaging data were also compared between the two groups. RESULTS: Demographic data and factors known to influence the clinical course after thrombolysis were similar between the two groups (P>0.05). However, use of the MTT allowed significant decreases in the median onset-to-treatment (OTT) time of 26min and median door-to-needle (DTN) time of 27min (P<0.001). The proportion of patients with a DTN time<60min was higher in the MTT group than in the usual care (UC) group: 64% vs. 14%, respectively (P<0.001), according to American Heart Association/American Stroke Association guidelines. Although there was a smaller proportion of negative 3-month outcomes (modified Rankin Scale score: 6; 6% vs. 16%) and a larger proportion of highly favorable 3-month outcomes (mRS score: 0-1; 79% vs. 64%) in the MTT vs. UC groups, respectively, these differences were not statistically significant (P>0.05). DISCUSSION/CONCLUSION: Use of an MTT is a simple way to reduce thrombolysis delays, and the present results encourage us to improve the system to make it even more effective and available for all patients.

[Migraine and vascular risk]. / Migraine et risque vasculaire.

Migraine with aura is well known to be associated with an increased risk of ischemic stroke. This risk dramatically increases in smokers and uses of oral contraceptives. Brain MRI studies suggest that migraine is associated with silent brain infarcts. Migraine is also established as a risk factor for significantly more cerebral white matter lesions. Recent data suggest that migraine is also associated with coronary artery disease. Data concerning an increase risk of mortality in migraine are controversial. The pathophysiology of the increased risk of ischemic events is unclear. Migrainous infarcts seem to be very rare. Migraine is associated with an increased risk of cervical artery dissection. Classical vascular risk factors are more common in migraineurs. Triptans are not associated with an increased risk of ischemic events. Another explanation is a probable endothelial dysfunction and a lower level of endothelial progenitor cells in migraine. A prothrombotic state in migraine is also another possible explanation due to genetic polymorphisms. Appropriate management for primary vascular prevention in migraineurs is not different from that proposed for the general population. Oral contraceptives should be avoided in migraineurs with aura, especially if they smoke.

[Stroke and cornea verticillata revealing Fabry's disease in a female]. / Accident vasculaire cérébral ischémique et cornée verticillée révélant une maladie de Fabry chez une femme.

INTRODUCTION: Fabry's disease is a X-linked lysosomal storage disorder caused by an alpha-galactosidase A deficiency responsible for the accumulation of glycosphingolipids. Males are more severely and frequently affected than females. We report the case of a female who presented a stroke revealing Fabry's disease. CASE REPORT: An 53-year-old woman, with cardiovascular risk factors and two previous transient ischemic attacks, was admitted with a brutal right hemiparisia. Cerebral MRI showed multiple white matter lesions in the cerebral hemispheres with multiple lacunar infarcts and ectatic vessels, cardiac echography revealed a hypertrophic concentric cardiomyopathy, and slit-lamp examination demonstrated a cornea verticillata. The sequencing of the alpha-galactosidase gene (GLA) revealed the c.150_151del mutation responsible for a loss of function. DISCUSSION: As in the present case, ophthalmological examination is very useful to determine Fabry's disease as a cause of young onset stroke. Females may be affected by X-linked disease, as the Fabry's disease. Fabry's disease among females is mainly characterized by the involvement of the nervous and cardiovascular systems. The specific treatment is based on an enzyme replacement therapy by recombinant enzyme with cardiovascular benefit. Despite the presence of cardiovascular risk factors, this case demonstrates the importance of thorough standardized investigations including ophthalmological examination of young patients with stroke.

Clinical features of primary headache in children: a multicentre hospital-based study in France.

The aim of this study was to evaluate the concordance between clinical diagnosis and the International Classification of Headache Disorders, 2nd edn (ICHD-II) in children and adolescents with primary headaches. This 6-month prospective multicentre study of 486 patients (mean 9.8 +/- 3.1 years; 52.6% girls) assessed the headache features through a structured questionnaire. In 398 patients with a single type of headache, headaches were bilateral (78.1%), frontal (62.4%), pulsatile (56.1%), with associated symptoms in 84.4%. The most frequently assigned diagnoses were migraine without aura (50.8%), probable migraine (14.1%), migraine with aura (11.1%) and frequent episodic tension-type headache (7.5%). For most of the diagnostic categories, the consistency of the investigator's diagnosis with the ICHD-II criteria was good (kappa > 0.6 and < or = 0.8) or excellent (kappa > 0.8). We conclude that migraine was predominant with regard to headache diagnoses repartition and that the ICHD-II seems usable in practice for evaluation of primary headache in French children and adolescents.

Chronic cluster headache: a French clinical descriptive study.

BACKGROUND: Cluster headache (CH) is a relatively rare disease and episodic CH is more frequent than chronic CH. Few studies have described the characteristics of patients with chronic CH. METHODS: This was a descriptive study carried out by eight tertiary care specialist headache centres in France participating in the Observatory of Migraine and Headaches (OMH). From 2002 to 2005, OMH collected data from 2074 patients with CH, of whom 316 had chronic CH. From January to June 2005, 113 patients with chronic CH were interviewed using standardised questionnaires during a consultation. RESULTS: The male to female ratio was 4.65:1. Median age was 42 years. The majority of patients were smokers or former smokers (87%). 46% had primary chronic CH (chronic at onset) and 54% secondary chronic CH (evolving from episodic CH). Most patients had unilateral pain during attacks and 7% had sometimes bilateral pain during an attack. 48% reported a persisting painful state between attacks. Symptoms anteceding pain onset (mainly discomfort/diffuse pain, exhaustion, mood disorders) and auras were reported by 55% and 20% of patients, respectively. The functional impact of chronic CH was estimated as severe by 74% of patients, and 75.7% suffered from anxiety, as assessed by the Hospital Anxiety and Depression scale. There was no substantial difference in clinical presentation between primary and secondary CH. DISCUSSION: This study confirms the existence of auras and interictal signs and symptoms in patients with chronic CH, and male sex and smoking as CH risk factors. Primary and secondary chronic CH appear equally prevalent. Male sex does not appear to favour the shift from episodic to chronic CH.

Headache changes prior to aneurysmal rupture: A symptom of unruptured aneurysm?

BACKGROUND AND OBJECTIVES: The symptomatic status of unruptured aneurysms has to be looked for. The objective of this retrospective case-control study was to identify the headache semiologic characteristics of symptomatic aneurysms during the 3 months prior to patient admission. PATIENTS AND METHODS: The case cohort was composed of 40 consecutive patients admitted for the treatment of a ruptured intracranial aneurysm (IA) and able to answer a standardized questionnaire by the same neurologist. This cohort was matched with a control cohort of 40 patients operated on for a degenerative lumbar pathology. This questionnaire, using the criteria for headache characteristics according to the International Headache Society (IHS) enabled us to classify headaches during the previous 3 months prior to the rupture (study period) and during the year prior to the period studied (reference period) in both cohorts. Headache status was considered as unstable if there were modifications of semiologic headache characteristics, thunderclap headaches or de novo headaches, or on the contrary stable. RESULTS: During the status period, chronic headaches were reported by 31 patients (77.5%) in the studied cohort and 35 (87.5%) in the control cohort. During the study period, the cephalagia status was stable in 19 patients (47.5%) versus 35 patients (87.5%) in the control cohort (P<0.001). Modifications of chronic headaches were present in 11 patients (35.5%) in the studied cohort versus 4 patients (11.4%) in the control cohort (P=0.04). Thunderclap headaches were present in 7 patients (17.5%) in the studied cohort but none in the control cohort (P=0.006). DISCUSSION: Modifications of headaches semiologic characteristics during the 3 previous months were significantly more frequent in the studied cohort. This modification could be a sign of IA instability.

[Anti-Ri paraneoplastic syndrome associated with ophtalmoplegia, blepharospasm and palilalia]. / Ophtalmoplégie, blépharospasme et palilalie associés à des anticorps anti-Ri.

INTRODUCTION: Anti-Hu and anti-Yo are the most well-known anti-neuronal antibodies. The anti-Ri antibodies, which are less common, are generally found in subjects with opsoclonus-myoclonus, often associated with breast cancer. CASE REPORT: A 54-year-old woman presented anti-Ri antibodies associated with a paraneoplastic syndrome and unusual symptoms of ophthalmoplegia, blepharospasm, palilalia and ataxia. Adenocarcinoma of the breast was also found. After chemotherapy, radiotherapy, and several immunoglobulin infusions, the patient did not improve ten months after tumor surgery. CONCLUSION: Anti-Ri antibodies associated with paraneoplastic syndrome can be observed in patients who develop a rapidly progressive brainstem tumor. Breast or lung cancer and conduct to search a breast or pulmonary cancer.

[Pseudothromboangiitis obliterans and qualitative protein C deficiency: report of a case]. / Pseudothrombo-angéite oblitérante et déficit qualitatif en protéine C: a propos d'un cas.

Thromboangiitis obliterans is a segmental obliterating inflammatory arteritis, usually found in young (below 40) smoking males. Its diagnosis relies on patient history, clinical features, arterial angiography, and more rarely on pathological findings, though none of these is specific of the disease, and on the absence of other diseases such as early atheroma, thromboembolic processes, vascular malformation, trauma, collagen or blood disorders. Raynaud's phenomenon, digital arteritis, superficial and often migrating venous thromboses are further arguments for the disease. However, such associations can also occur during other diseases, especially congenital or acquired deficits in coagulation factors (antiphospholipid syndrome, S protein deficiency...). In our patient with suspected thromboangiitis obliterans, the occurrence of superior longitudinal and right lateral sinus thrombosis led to the discovery of a qualitative C protein defect. This observation stresses the need for careful elimination of a coagulation disorder before confirming the diagnosis of thromboangiitis obliterans.

[Clinical and evolutive aspects of cerebellar infarction]. / Aspects cliniques et évolutifs des infarctus du cervelet.

This study included 125 cases of cerebellar infarction followed during an average period of 4.3 years. The diagnosis was made by CT or MRI. Infarctions localized to the territory of the superior cerebellar artery (SCA) and the territory of the posterior inferior cerebellar artery (PICA) occurred with the same frequency. Transient ischemic attacks preceded infarction in 26% of cases. Symptoms and signs were usual with sudden association of headache, dizziness, unsteadiness and vomiting. Vestibular signs were more important in infarctions of the PICA territory; cerebellar signs and dysarthria were more frequent in infarction of the SCA territory. A decreased level of consciousness developed in only 21% of cases. Surgical operation was required in 9 cases. Investigations have showed the large responsibility of cardiac embolisms and atherosclerosis. Short term outcome was more often favourable: 116 patients were alive at the end of the first month; 80% of survivors were independent one year later. At 5 years, 73% of patients were alive. After the acute period, mortality was mainly due to cerebro-vascular and cardiac events.

[Continuous partial epilepsy disclosing diabetes mellitus]. / Epilepsie partielle continue révélatrice d'un diabète sucré.

Continuous partial epilepsy (CPE) is characterized by isolated, subintrant clonus focalized to a limited territory with critical focal electroencephalography in a concordant territory. CPE is observed in various cortical lesions but also in disorders of metabolism and notably decompensated diabetes mellitus. We report a case of CPE without focal lesion at MRI which revealed hyperglycaemia without ketosis. The 54-year old female patient was hospitalised for C.P.E.. Early CT and later MRI gave normal results. Biochemistry showed hyperglycaemia without kenoturia, acidosis or hyperosmolality. Insulin therapy rapidly brought glycaemia down to its normal level and the clonsism disappeared. Five months later, the patient had no other seizure and the EEG was normal. Epileptic seizures are frequent in hyperglycaemia without ketosis (25% of the cases) where they are mainly partial and motor (75 to 86% of the cases), rarely associated with a focal lesion (15% of the cases with CT scan). They are rare in patients with ketoacidosis. This apparent protective effect of ketoacidosis may be attributed to an increase of GABA bioavailability consecutive to acidosis. CPE is resistant to antiepileptic treatments. In CPE induced by hyperglycaemia without ketosis normalization of blood glucose level with insulin therapy is concomitant with a rapid cure of epilepsy. Thus glycaemia should be measured in all patients presenting with CPE, the aim being to diagnose hyperglycaemia without ketosis rapidly to avoid hyperosmolality and to prescribe an adequate treatment based exclusively on insulin and rehydration.