Cancer and multiple sclerosis: 2023 recommendations from the French Multiple Sclerosis Society.

BACKGROUND: Epidemiological data reveal that 45% of persons with multiple sclerosis (PwMS) in France are more than 50 years. This population more than 50 is more susceptible to cancer, and this risk may be increased by frequent use of immunosuppressive drugs. Consequently, concerns have arisen about the potential increased risk of cancer in PwMS and how patients should be screened and managed in terms of cancer risk. OBJECTIVE: To develop evidence-based recommendations to manage the coexistence of cancer and multiple sclerosis (MS). METHODS: The French Group for Recommendations in MS collected articles from PubMed and university databases covering the period January 1975 through June 2022. The RAND/UCLA method was employed to achieve formal consensus. MS experts comprehensively reviewed the full-text articles and developed the initial recommendations. A group of multidisciplinary health care specialists then validated the final proposal. RESULTS: Five key questions were addressed, encompassing various topics such as cancer screening before or after initiating a disease-modifying therapy (DMT), appropriate management of MS in the context of cancer, recommended follow-up for cancer in patients receiving a DMT, and the potential reintroduction of a DMT after initial cancer treatment. A strong consensus was reached for all 31 recommendations. CONCLUSION: These recommendations propose a strategic approach to managing cancer risk in PwMS.

Stroke in a young adult: looking beyond the diffusion-weighted imaging sequence.

A 37-year-old migrainous woman was referred several hours after a sudden dysarthria lasting less than 1 min. Brain MRI undertaken a few hours later showed no acute cerebral infarction on the diffusion-weighted imaging (DWI) sequence. However, an intraluminal thrombus within a distal branch of the middle cerebral artery (MCA) was seen on the T2*-weighted gradient echo images as well as hyperintense vessels related to slow flow beyond the occlusion's site. Control MRI 24 h later confirmed the presence of a small infarct affecting the distal portion of the MCA territory but the thrombus was no longer visible. A comprehensive etiologic workup revealed the presence of a patent foramen ovale, as the underlying cause of the patient's stroke. Though the sensitivity of DWI sequence for acute ischemia is high, false negative exist particularly in cases of transient ischemic attack, brainstem or lacunar strokes. A careful brain MRI examination or the use of perfusion weighted imaging sequences are useful to avoid misdiagnosis particularly in young patients.

Pregnancy and multiple sclerosis: 2022 recommendations from the French multiple sclerosis society.

OBJECTIVE: The objective of this study was to develop evidence-based recommendations on pregnancy management for persons with multiple sclerosis (MS). BACKGROUND: MS typically affects young women in their childbearing years. Increasing evidence is available to inform questions raised by MS patients and health professionals about pregnancy issues. METHODS: The French Group for Recommendations in Multiple Sclerosis (France4MS) reviewed PubMed and university databases (January 1975 through June 2021). The RAND/UCLA appropriateness method was developed to synthesise the scientific literature and expert opinions on healthcare topics; it was used to reach a formal agreement. Fifty-six MS experts worked on the full-text review and initial wording of recommendations. A group of 62 multidisciplinary healthcare specialists validated the final proposal of summarised evidence. RESULTS: A strong agreement was reached for all 104 proposed recommendations. They cover diverse topics, such as pregnancy planning, follow-up during pregnancy and postpartum, delivery routes, locoregional analgesia or anaesthesia, prevention of postpartum relapses, breastfeeding, vaccinations, reproductive assistance, management of relapses and disease-modifying treatments. CONCLUSION: The 2022 recommendations of the French MS society should be helpful to harmonise counselling and treatment practice for pregnancy in persons with MS, allowing for better and individualised choices.

Pregnancy and neuromyelitis optica spectrum disorders: 2022 recommendations from the French Multiple Sclerosis Society.

BACKGROUND: In 2020, the French Multiple Sclerosis (MS) Society (SFSEP) decided to develop a national evidence-based consensus on pregnancy in MS. As neuromyelitis optica spectrum disorders (NMOSD) shares a series of commonalities with MS, but also some significant differences, specific recommendations had to be developed. OBJECTIVES: To establish recommendations on pregnancy in women with NMOSD. METHODS: The French Group for Recommendations in Multiple Sclerosis (France4MS) reviewed PubMed and universities databases (January 1975 through June 2021). The RAND/UCLA appropriateness method, which was developed to synthesise the scientific literature and expert opinions on health care topics, was used to reach a formal agreement. Fifty-six MS experts worked on the full-text review and initial wording of recommendations. A sub-group of nine NMOSD experts was dedicated to analysing available data on NMOSD. A group of 62 multidisciplinary healthcare specialists validated the final proposal of summarised evidence. RESULTS: A strong agreement was reached for all 66 proposed recommendations. They cover diverse topics, such as pregnancy planning, follow-up during pregnancy and postpartum, delivery routes, loco-regional analgesia or anaesthesia, prevention of postpartum relapses, breastfeeding, vaccinations, reproductive assistance, management of relapses, and disease-modifying treatments. CONCLUSION: Physicians and patients should be aware of the new and specific evidence-based recommendations of the French MS Society for pregnancy in women with NMOSD. They should help harmonise counselling and treatment practise, allowing for better individualised choices.

Bilateral facial palsy in an older person.

Lyme disease is an infectious disease caused by the Borrelia burgdorferi spirochetes and other related species that are transmitted through an infected tick bite. We report the case of an older patient presenting with bilateral facial palsy due to Lyme disease. Multiple non-specific clinical signs preceded facial palsy with falls, fatigue and pain of both legs especially during the night. Our case illustrates the difficulty to diagnose this infectious disease, especially in older patients who have rare outdoor activities and a low risk of tick exposure.

Clinically suspected concomitant spinal cord and vertebrobasilar infarctions caused by fibrocartilaginous embolism.

Fibrocartilaginous embolism (FCE) is a rare and probably under diagnosed cause of spinal cord infarction presumably due to acute embolization of nucleus pulposus fragments into the spinal circulation. Concomitant cerebral involvement is much rarer and often asymptomatic. Although the definitive diagnosis is histologic, certain criteria have been proposed to support the diagnosis in living patients, such as absence of vascular risk factors, acute onset or antecedent of valsalva maneuver before the episode and the exclusion of potential differential diagnoses. A 56 years-old patient, without any medical history was referred for sudden back pain while carrying heavy load at work. Clinical examination showed a Brown-Sequard syndrome. Brain and spine MRI disclosed spinal cord infarction at the C4-C5 level associated with brain infarctions involving exclusively the vertebrobasilar circulation. The exhaustive etiological assessment was normal. In our case, the acute symptoms onset, the clinical and imaging data and lack of evidence for other plausible diagnoses in the setting of a valsalva-like maneuver are highly suggestive of FCE diagnosis.

Isolated peripheral-type facial palsy due to contralateral precentral gyrus cavernoma hemorrhage.

Facial palsy is a condition frequently encountered in neurological daily practice. Typically, telling apart peripheral facial palsy (PFP) from central facial palsy is easy, and depends on the presence of the upper face involvement. However weakness of eye closure can be seen also in cases of central facial palsies, making the diagnosis more difficult. When the facial involvement is isolated, that constitutes a crucial diagnosis challenge because it can be misdiagnosed as Bell's palsy, particularly in young patients. However, subtle clinical findings such as a predominant involvement of the lower face and the presence of a dissociation of emotional and volitional facial movements points towards a central etiology as in our patient. We report here a case of isolated peripheral-type facial palsy due to acute bleeding of supratentorial cavernoma. To our knowledge, such case has not yet been reported.

A case of right internal carotid agenesis with associated transcavernous anastomosis revealed by transient ischemic attack.

Agenesis of the internal carotid artery (ICA) is a rare congenital vascular disorder of the cerebral circulation. CT scan of the skull base disclosing complete absence of the bony carotid canal helps to differentiate an agenesis from aplasia or hypoplasia. Although most of the patients remain asymptomatic (thanks to the sufficient collateral circulation provided by the circle of Willis) cerebral infarcts, transient ischemic attacks or intracranial aneurysms have been rarely described in association with agenesis of the ICA. Most often, the vascular territory of the involved ICA is supplied by the contralateral carotid artery and from the vertebrobasilar circulation through the anterior and posterior communicating arteries, respectively. However, collateral supply can also be provided thanks to a transcavernous anastomosis, an aberrant vascular communication between the cavernous portions of the ICAs coursing through the sella turcica. We report here the case of a 55-year-old man with right carotid agenesis and associated transcavernous anastomosis revealed by transient ischemic attack. Embryogenesis, imaging findings, possibilities of collateral circulation and potential complications have also been discussed.

Is there a timing for sensitivity to acute cerebral ischemia in migraine patients?

BACKGROUND: Migraine may be a factor of increased cerebral sensitivity to ischemia. Previous studies were conducted within 6 to 72 after stroke onset. We aimed to determine if an accelerated infarct growth exists in migraine patients within the first 4.5 h. METHOD: A retrospective case-control study was conducted where all patients admitted for acute stroke started <4.5 h before and who underwent perfusion CT were assessed. The hypoperfusion and necrosis volumes on initial CT perfusion were analyzed, as well as the final infarct volume on MRI performed within 72 h after admission. A no-mismatch pattern was defined as a ratio necrosis/hypoperfusion volume > 83%. RESULTS: 24 patients with personal history of migraine were identified, 8 of them with aura. The control cohort included 51 patients. No difference was found between groups in terms of demographics, initial severity or outcome or presumed cause of stroke. Mean time to CT scan was 125 min in migraine patients and 127 min in the control group. A no-mismatch pattern was equally found in migraine patients and controls, even after adjustment for age, sex and presence of proximal occlusion (p = .22). The final infarct volume was also similar in both groups. CONCLUSIONS: Migraine patients did not display more no-mismatch pattern than controls within the 4.5 h of stroke onset. This deviates from previous studies and may be due to our earlier time from stroke onset to CT scan. A history of migraine may lead to malignant progression of ischemia but occurring only after several hours.

Trigeminal Neuralgia and Charcot-Marie-Tooth Disease: An Intriguing Association. Lessons From a Large Family Case Report and Review of Literature.

We report a case of familial trigeminal neuralgia (TN) and Charcot-Marie-Tooth disease (CMT) caused by an identified MPZ mutation with a review of previous cases described in the literature. BACKGROUND: The association of TN in CMT patients has previously been reported in a few cases. The pathophysiological link can be detailed with recent use of genetic analysis in CMT. METHODS: We report a large family including 7 members affected by CMT, 4 of whom also presented with TN. We then performed a literature review of literature by search of Pubmed from 1950 to September 2018, using the search terms "trigeminal neuralgia" and "Charcot-Marie-Tooth" and the references of relevant articles. RESULTS: Overall, we found 29 previously published TN cases in 12 CMT families. Among them, only 7 families (69%) included several affected members, suggesting that not all mutations involved in CMT predispose to TN. TN in this context seems to present with specific characteristics, including earlier age of onset, bilateral presentation, and poor tolerance to preventive treatments with gait disturbance exacerbated by the underlying neuropathy. CONCLUSION: This report of familial TN in CMT with identified MPZ mutation highlighted specific characteristics of this association. Considered as a rare association in the literature, it may be underestimated and the clinician should be aware of its specific pattern, including earlier age of onset, bilateral presentation, and poor tolerance to preventive treatments.

Atypical MRI presentation of posterior reversible encephalopathy syndrome with predominant brainstem involvement.

We report here the case of a 80-year-old woman for the assessment of an acute confusional state since 2 days asssociated with diffuse and gradual headache. Brain MRI disclosed isolated hyperintense signal on fluid-attenuated inversion recovery sequence involving the medulla and the right inferior cerebellar peduncle with moderate swelling, consistent with vasogenic oedema, without abnormalities on diffusion-weighted imaging sequence. PRES diagnosis was suspected and antihypertensive therapy was introduced to achieve a blood pressure goal < 140/90 mmHg, allowing a quickly favourable clinical course. Three months later, brain MRI demonstrated a complete clearance of the abnormalities, confirming the PRES diagnosis. Atypical MRI findings are possible with involvement of basal ganglia, brainstem or cerebellum, but in these cases, oedema is most of the time accompanied by the classical parieto-occipital region involvement. Vasogenic oedema strictly unilateral or involving exclusively the brainstem or the cerebellum are very rare and should prompt suspicion for an alternative diagnosis. That constitutes a crucial diagnosis challenge in neurology.

In-hospital delays to stroke thrombolysis: Out of hours versus regular hours and reduction in treatment times through the creation of a 24/7 mobile thrombolysis team.

BACKGROUND: The main aim of this study was to evaluate the impact of the implementation of a mobile thrombolysis team (MTT) on time to thrombolysis treatment depending on patient admission time: regular hours (RH) or out of hours (OH). METHODS: 504 consecutive patients treated with IV tPA or with combined IV tPA and mechanical thrombectomy for acute ischemic stroke were retrospectively included between 1st January 2013 and 31st December 2017. Three sub-periods were identified: 2013-2014, 2015-2016, and 2017 during which patients were treated with the usual care (UC), by the MTT or with UC according to their time of admission, or by the MTT, in the three time periods respectively. We compared in-hospital delays according to patient admission time. RESULTS: In 2013-2014, 133 patients were included. Both median door-to-needle (DTN) and imaging to needle (ITN) times were shorter for patients admitted during RH than OH, respectively 75 min versus 85 min and 52 min versus 57 min (P < 0.05), and the proportion of patients with DTN ≤ 60 min was 23% versus 9% (P < 0.05), respectively. In 2015-2016, 223 patients were included. DTN and ITN times were shorter for patients admitted during RH and treated by the MTT than during OH with UC, respectively 54 min versus 78 min and 24 min versus 47 min (P < 0.001), and the proportion of patients with DTN ≤ 60 min was 64% versus 21% (P < 0.001), respectively. In 2017, there was no difference concerning in-hospital delays regardless of patient admission time (P > 0.05). DISCUSSION: DTN time was significantly longer for patients admitted OH. We suggest that the implementation of an around-the-clock MTT would allow a reduction of in-hospital delays and similar times to thrombolysis treatment regardless of admission time.

Unusual Aspect of Posterior Reversible Encephalopathy Syndrome in an Elderly Adult.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) classically occurs in patients presenting with a sudden increase of arterial pressure or eclampsia, but the range of etiologies is very wide. Although the mechanisms underlying PRES remain unclear, research suggests that when the extent of hypertension exceeds the threshold of cerebral blood flow autoregulation, this induces blood-brain barrier disruption that leads to brain edema. Unusual presentations on magnetic resonance imaging (MRI) are possible, including the involvement of frontal or temporal lobes, cerebellar hemispheres, basal ganglia, brainstem, or deep white matter. However, in these atypical locations, edema is mostly accompanied by the classical parieto-occipital region involvement. CASE REPORT: We report the case of an elderly adult presenting with an unusual presentation of PRES with exclusive involvement of the frontal lobes, associated with severe hypertension. In our case, computed tomography and MRI abnormalities were exclusively localized in the frontal regions without involvement of the classical parieto-occipital regions. A favorable clinical course after hypertension management and complete clearance on an MRI scan at 3 months' follow-up confirmed the diagnosis. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: PRES is a condition potentially encountered by many doctors, especially in the emergency department. However, atypical clinical or imaging presentations are possible and could delay the diagnosis. These must be known to begin adequate treatment as quickly as possible.

Predictive Factors of Headache Resolution After Chiari Type 1 Malformation Surgery.

BACKGROUND: Headache is the main and often isolated symptom of patients with Chiari type 1 malformation (CM1). Classically described as occipital and exacerbated by cough, headaches may be poorly characterized, making it difficult to establish CM1 as the underlying cause. Current guidelines for surgical posterior fossa decompression are undefined. The challenge is to distinguish headaches related to CM1 from headaches coincidentally coexisting with CM1. We aimed to determine predictive factors of headache resolution after surgery and applied to our cohort the Chiari Severity Index, a recently developed predictive prognostic score. METHODS: This retrospective study enrolled 49 patients with CM1 and preoperative headache. Standardized telephone interviews regarding headaches before and after surgery were conducted by the same neurologist; magnetic resonance imaging morphometric analyses were performed by an independent neuroradiologist. Headache resolution was defined as ≥50% reduction in frequency of headache days. RESULTS: Preoperative factors of headache resolution after multivariate analysis were attack duration <5 minutes (P = 0.001), triggering by Valsalva maneuvers (P = 0.003), severe intensity of attack (P = 0.05), occipital location (P = 0.05), and greater number of headache days per month (P = 0.04). These characteristics are part of International Headache Society diagnostic criteria for headache attributed to CM1. No radiologic predictive factor was demonstrated. Postoperative improvement was inversely correlated with Chiari Severity Index. CONCLUSIONS: This study confirms the relevance of International Headache Society criteria to identify headaches related to CM1. We propose their systematic use in a preoperative questionnaire.