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.

Impact of previous statin use on first intracerebral hemorrhage in cerebral amyloid angiopathy.

OBJECTIVES: Statins have been associated with an increased risk of spontaneous intracerebral hemorrhage (ICH), but without dedicated study in cerebral amyloid angiopathy (CAA). We aimed to evaluate the association between previous statin treatment and radiological hemorrhagic lesions in a CAA population during a first lobar ICH event. MATERIALS AND METHODS: We retrospectively included all patients meeting the modified Boston criteria for probable CAA and admitted for a first lobar ICH between 2010 and 2021 at Rouen University Hospital. Patients were classified as having previous statin treatment or not. We compared the ICH volume, the number of associated cerebral microbleeds (CMBs), and cortical superficial siderosis (CSS) according to previous statin treatment or not. We also compared functional outcomes and ICH recurrence during the follow-up period between the two groups. RESULTS: We included 99 patients, 27 of whom had statin treatment prior to their ICH. The ICH volume and the number of CMBs did not differ between groups. Disseminated CSS was initially more frequent in the statin group (88% versus 57%; P=0.019), but this was no longer significant after adjustment for antiplatelet treatment (P=0.13). The long-term outcome was similar between the two groups with no increased risk of ICH recurrence in the statin-treated group (29.63% versus 23.61%, P=0.54). CONCLUSIONS: Previous statin treatment was not associated with more severe hemorrhagic lesions in CAA in terms of ICH volume or number of microbleeds, but a trend for increased disseminated CSS was highlighted, which will require further larger studies.

Different clinical outcomes between cerebral amyloid angiopathy-related inflammation and non-inflammatory form.

OBJECTIVE: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare manifestation related to CAA, thought to be more severe. We aimed to compare the clinical and radiological outcomes of CAA-ri and non-inflammatory CAA. MATERIALS AND METHODS: We retrospectively included all patients with CAA-ri from 13 French centers. We constituted a sex- and age-matched control cohort with non-inflammatory CAA and similar disease duration. Survival, autonomy and cognitive evolution were compared after logistic regression. Cerebral microbleeds (CMB), intracerebral hemorrhage, cortical superficial siderosis and hippocampal atrophy were analyzed as well as CSF biomarker profile and APOE genotype when available. Outcomes were compared using Kaplan-Meier curves and log-rank tests. RESULTS: Data from 48 CAA-ri patients including 28 already reported and 20 new patients were analyzed. Over a mean of 3.1 years, 11 patients died (22.9%) and 18 (37.5%) relapsed. CAA-ri patients were more frequently institutionalized than non-inflammatory CAA patients (30% vs 8.3%, p < 0.001); mortality rates remained similar. MMSE and modified Rankin scale scores showed greater severity in CAA-ri at last follow-up. MRI showed a higher number of CMB at baseline and last follow-up in CAA-ri (p < 0.001 and p = 0.004, respectively). CSF showed lower baseline levels of Aß42 in CAA-ri than non-inflammatory CAA (373.3 pg/ml vs 490.8 pg/ml, p = 0.05). CAA-ri patients more likely carried at least one APOE ε4 allele (76% vs 37.5%, adjusted p = 0.05) particularly as homozygous status (56% vs 6.2%, p < 0.001). INTERPRETATION: CAA-ri appears to be more severe than non-inflammatory CAA with a significant loss of autonomy and global higher amyloid burden, shown by more CMB and a distinct CSF profile. This burden may be partially promoted by ε4 allele.

Validation of a non-motor fluctuations questionnaire in Parkinson's disease.

INTRODUCTION: Non-motor fluctuations (NMF) in Parkinson's disease (PD) remain poorly recognized but have a high impact on patients' quality of life. The lack of assessment tools limits our understanding of NMF, compromising appropriate management. Our objective was to validate a hetero-questionnaire for NMF in PD patients at different stages of the disease: without treatment, without motor fluctuations, with motor fluctuations. METHODS: We included patients in 15 centers in France. Our questionnaire, NMF-Park, resulted from previous studies, allowing us to identify the more pertinent NMF for evaluation. Patients reported the presence (yes or no) of 22 selected NMF, and their link with dopaminergic medications. The assessment was repeated at one and two years to study the progression of NMF. We performed a metrological validation of our questionnaire. RESULTS: We included 255 patients (42 without treatment, 88 without motor fluctuations and 125 with motor fluctuations). After metrological validation, three dimensions of NMF were found: dysautonomic; cognitive; psychiatric. The sensory/pain dimension described in the literature was not statistically confirmed by our study. DISCUSSION: Our questionnaire was validated according to clinimetric standards, for different stages of PD. It was clinically coherent with three homogeneous dimensions. It highlighted a link between fatigue, visual accommodation disorder, and cognitive fluctuations; and the integration of sensory/pain fluctuations as part of dysautonomic fluctuations. It focused exclusively on NMF, which is interesting considering the described differences between non-motor and motor fluctuations. CONCLUSION: Our study validated a hetero-questionnaire of diagnosis for NMF for different stages of PD.

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.

Practice of stereoelectroencephalography (sEEG) in drug-resistant epilepsy: Retrospective series with surgery and thermocoagulation outcomes.

OBJECTIVE: The aim of this study was to determine the success rate of sEEG in locating the epileptogenic zone (EZ) in patients with pharmaco-resistant epilepsy. Secondary objectives were to analyze sEEG-related morbidity and outcomes for post-sEEG thermocoagulation and cortical resection. METHODS: Data were collected on 49 sEEGs from 46 consecutive patients between 2010 and 2018. Following sEEG, either resective or palliative surgery with vagus nerve stimulation was performed. In 8 patients, EZ thermocoagulation was performed before EEG leads were withdrawn. Outcomes were collected based on the Engel and ILAE outcome scales. RESULTS: sEEG was contributive in 45 of 49 recordings, with a success rate of 92% in locating the EZ. Minor complications, such as transient neurologic deficit and electrode implantation failures, occurred in 6%. One major complication occurred, with death due to atypical late hematoma. Thermocoagulation was performed in 8 patients and stopped or significantly reduced seizure frequency in 7 (88%). Outcome of surgical resection (n=33) was good, with 20 (61%) seizure-free patients and 32 (97%) with definite improvement. CONCLUSIONS: Our findings suggest that sEEG is an effective technique for EZ location in patients with drug-resistant epilepsy. sEEG was contributive in up to 92% of patients, allowing thermocoagulation and/or surgical resection that resulted in seizure-freedom in two-thirds and seizure-reduction in one-third of cases. This study highlights the need for strict selection of implantation candidates, with strong initial hypothesis as to EZ location.

Acute hemichorea revealing atrial flutter.

Transient ischemic attacks (TIAs) typically present with easily recognizable neurological focal deficits. Symptoms such as paroxysmal involuntary movements are not usually considered to be a manifestation of TIA. We report a case with video documentation of TIA due to permanent atrial flutter presenting as acute left hemichorea. To our knowledge, such a case has not yet been reported. The present case constitutes a crucial diagnostic challenge in neurological practice in order to prevent a high risk of subsequent ischemic stroke.

The pathophysiological mechanisms of motivational deficits in Parkinson's disease.

Parkinson's disease (PD) is a progressive degenerative disorder that leads to disabling motor symptoms and a wide variety of neuropsychiatric symptoms. Apathy is the most common psychiatric disorder in the early stages of untreated PD and can be defined as a hypodopaminergic syndrome, which also includes anxiety and depression. Apathy is also considered the core feature of the parkinsonian triad (apathy, anxiety and depression) of behavioural non-motor signs, including a motivational deficit. Moreover, apathy is recognised as a distinct chronic neuropsychiatric behavioural disorder based on specific diagnostic criteria. Given the prevalence of apathy in approximately 40% of the general Parkinson's disease population, this appears to be a contributing factor to dementia in PD; also, apathy symptoms are factors that potentially contribute to morbidity, leading to a major impairment of health-related quality of life, thus stressing the importance of understanding the pathophysiology of this disease. Several studies have clearly established a prominent role for DA-mediated signals in PD apathy. However, synergistic interaction between dopaminergic impairment resulting from the neurodegenerative process and deep brain stimulation of the subthalamic nucleus may cause or exacerbate apathy. Furthermore, serotoninergic mechanism signalling is also likely to be of importance in this pathophysiology.

Management and outcome of cerebral venous thrombosis after head trauma: A case series.

Cerebral venous thrombosis (CVT) is an underdiagnosed complication of head trauma. To date, initiation of anticoagulation is still a matter of debate because of the risk of worsening traumatic hemorrhage. This report describes a case series of five patients admitted for head injury complicated by CVT. The main associated radiological signs were skull fractures crossing the venous sinus and adjacent traumatic hematoma. In four patients, anticoagulation was introduced within 48-72h of CVT diagnosis, with no subsequent hemorrhagic complications. The present report and data from the literature raise the question of systematic additional venoscans when confronted by associated radiological features of post-traumatic CVT. The safety of anticoagulation in selected patients is also discussed.

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.

Adult-onset stereotypical motor behaviors.

Stereotypies have been defined as non-goal-directed movement patterns repeated continuously for a period of time in the same form and on multiple occasions, and which are typically distractible. Stereotypical motor behaviors are a common clinical feature of a variety of neurological conditions that affect cortical and subcortical functions, including autism, tardive dyskinesia, excessive dopaminergic treatment of Parkinson's disease and frontotemporal dementia. The main differential diagnosis of stereotypies includes tic disorders, motor mannerisms, compulsion and habit. The pathophysiology of stereotypies may involve the corticostriatal pathways, especially the orbitofrontal and anterior cingulated cortices. Because antipsychotics have long been used to manage stereotypical behaviours in mental retardation, stereotypies that present in isolation tend not to warrant pharmacological intervention, as the benefit-to-risk ratio is not great enough.

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.

French consensus procedure for assessing cognitive function in Parkinson's disease.

INTRODUCTION: One of the objectives of the French expert centers for Parkinson's disease (NS-Park) network was to determine a consensus procedure for assessing cognitive function in patients with Parkinson's. This article presents this procedure and briefly describes the selected tests. METHODS: A group of 13 experts used the Delphi method for consensus building to define the overall structure and components of the assessment procedure. For inclusion in the battery, tests had to be validated in the French language, require little motor participation, have normative data and be recognized by the international community. Experimental tasks and tests requiring specific devices were excluded. RESULTS: Two possibilities were identified, depending on whether an abbreviated or comprehensive assessment of cognitive function was necessary. For an abbreviated assessment, the experts recommended the Montreal Cognitive Assessment (MoCA) as a screening test for cognitive impairment or dementia. For a comprehensive neuropsychological assessment, the experts recommended assessing global efficiency plus the five main cognitive domains (attention and working memory, executive function, episodic memory, visuospatial function and language) that may be impaired in Parkinson's disease, using two tests for each domain. DISCUSSION AND CONCLUSION: A common procedure for assessing cognitive function is now available across the French network dedicated to Parkinson's disease, and is recommended for both research and clinical practice. It will also help to promote standardization of the neuropsychological assessment of Parkinson's disease.