Remote neurological evaluation reliably identifies patients eligible to endovascular therapy while non-eligible to intravenous thrombolysis.

INTRODUCTION/BACKGROUND: Early identification of suspected stroke patients who might be eligible for a reperfusion strategy is a daily challenge in the management of patient referrals. The aim of this study was to evaluate the performance of a remote medical assessment in identifying patients eligible for endovascular therapy (EVT) while not eligible for intravenous thrombolysis (IVT), compared with a decision based on bedside clinico-radiological data. METHODS: Patients admitted to the emergency department for acute neurological symptoms lasting for less than 24h were prospectively included. Assessment of the clinical severity and medical history was performed simultaneously by two vascular neurologists (VNs), one remotely using a mobile telemedicine solution (NOMADEEC), the other at the bedside. RACE score was calculated from the NIHSS score. At the end of the evaluation, both VNs quoted their treatment convictions (IVT/EVT). Final therapeutic decision following brain and vascular imaging was recorded and compared to remote and bedside predictions. The performances of three different conditions were evaluated: complete medical evaluation (NIHSS+medical history), NIHSS score alone, and RACE score alone. Remote and bedside performances were also compared. Diagnostic accuracy parameters (sensitivity, specificity, positive and negative predictive values) of each condition were estimated, along with their two-sided 95% binomial confidence intervals. RESULTS: Out of 215 enrolled patients, 186 had a complete evaluation, 91 (54.3%) were diagnosed with an ischemic stroke or transient ischemic attack and 46 (24.7%) had an intracranial occlusion. Considering the three conditions evaluated remotely, RACE score-based decision provided the best sensitivity 54.6% [95% CI 23.4; 83.2]/specificity 80.6% [73.9; 86.2] combination. However, the complete medical evaluation had the best specificity (88.6% [82.9; 92.9] compared to RACE scores alone (P=0.038). Remote and bedside performances did not differ (κ=0.68 [0.59; 0.77]). DISCUSSION/CONCLUSION: This real-life study performed in the setting of emergency demonstrates that remote medical evaluations including recording of extensive medical information and NIHSS examination to address patient's eligibility to revascularization treatment is swiftly feasible and is as effective as bedside prediction to EVT and/or IVT. Remote standardized medical evaluation might improve the decision of patients' primary orientation and avoid overcrowding of comprehensive stroke centres.

Tenecteplase in acute ischemic stroke: Review of the literature and expert consensus from the French Neurovascular Society.

BACKGROUND: Intravenous alteplase is the only thrombolytic treatment approved for patients with acute ischemic stroke (AIS). Although no randomized controlled trial (RCT) has shown the superiority of tenecteplase over alteplase in AIS, tenecteplase is increasingly used off-label in Stroke Units. The purpose of the present work was to provide an up-to-date set of expert consensus statements on the use of tenecteplase in AIS. METHODS: Members of the working group were selected by the French Neurovascular Society. RCTs comparing tenecteplase and alteplase in the treatment of AIS were reviewed. Recent meta-analysis and real-life experience data on tenecteplase published until 30th October 2021 were also analyzed. After a description of the available data, we tried to answer the subsequent questions about the use of tenecteplase in AIS: What dosage of tenecteplase should be preferred? How effective is tenecteplase for cerebral artery recanalization? What is the clinical effectiveness of tenecteplase? What is the therapeutic safety of tenecteplase? What are the benefits associated with tenecteplase ease of use? Then expert consensus statements for tenecteplase use were submitted. In October 2021 the working group was asked to review and revise the manuscript. In November 2021, the current version of the manuscript was approved. EXPERT CONSENSUS: A set of three expert consensus statements for the use of tenecteplase within 4.5hours of symptom onset in AIS patients were issued: (1) It is reasonable to use tenecteplase 0.25mg/kg when mechanical thrombectomy (MT) is planned. (2) Tenecteplase 0.25mg/kg can be used as an alternative to alteplase 0.9mg/kg in patients with medium- or small-vessel occlusion not retrievable with MT. (3) Tenecteplase 0.25mg/kg could be considered as an alternative to alteplase 0.9mg/kg in patients without vessel occlusion. CONCLUSIONS: These expert consensus statements could provide a framework to guide the clinical decision-making process for the use of tenecteplase according to admission characteristics of AIS patients. However, existing data are limited, requiring inclusions in ongoing RCTs or real-life registries.

High glycemic variability: An underestimated determinant of stroke functional outcome following large vessel occlusion.

BACKGROUND AND PURPOSE: Early glycemic variability (GV) in diabetic patients is a poor prognosis factor following cardiovascular events. However, its influence on the course of acute ischemic stroke (AIS) with large vessel occlusion remains unclear. We investigated the relationship between high GV during acute stroke and three-month functional outcome among patients treated with combined intravenous thrombolysis and endovascular therapy for large vessel occlusion. METHODS: A single-center retrospective analysis of AIS patients with proximal intracranial occlusion who underwent thrombolysis and mechanical thrombectomy between January 2015 and May 2017. Early GV was assessed using standard deviation (SD) of blood glucose levels for the first 24hours. The main outcome was functional status at three months as defined by the modified Rankin scale (mRS). Secondary outcomes were change in NIHSS score from baseline to 24hours and occurrence of severe hemorrhagic transformation. Multivariate logistic regression analyses including GV, admission glycemia and mean glycemia were performed. RESULTS: Among the 93 patients evaluated, 26 had early high GV (≥20.9mg/dl). High GV was associated with poor functional outcome (OR=8.00; 95%CI [1.34-47.89]; P=0.02) unlike admission glycemia and mean glycemia (OR=2.92; 95%CI [0.51-16.60]; P=0.23 and OR=0.36; 95%CI [0.05-2.6]; p=0.31, respectively). High GV was not associated with NIHSS at 24hours or hemorrhagic transformation. CONCLUSION: Acute high GV contributes to poorer functional outcome following AIS related to large vessel occlusion and should be considered as a new target in acute stroke management.

Carotid webs associated with ischemic stroke. Updated general review and research directions.

Carotid web (CaW) is an intimal variant of fibromuscular dysplasia strongly associated with ipsilateral cerebral infarction. Although considered rare, it is a recent and increasing concern for physicians involved in stroke diagnosis and management. The present general review relies on a systematic literature analysis and aims to update readers on the latest knowledge in the field of symptomatic CaW (syCaW). CaW associated with ipsilateral cerebral infarction or transient ischemic attack has been identified in 189 patients. Ischemic strokes (IS) mostly occur in middle age (mean 46 years) and predominately in females (66%). The high frequency of African descendant patients among case reports and series (58%) suggests an ethnic susceptibility for CaW development. CaW features are characterised by a shelf-like intraluminal defect on contrast sagittal imaging, a linear defect that splits the lumen on axial section, a post-contrast stagnation rostral to the lesion and a frequent contralateral mirrored CaW (26.6%). An artery-to-artery embolism mechanism is widely accepted via CaW blood stasis, thrombus formation and clot fragmentation scattered by blood flow. Therefore, cerebral infarctions are often large related to a high proportion of proximal occlusion (62.5%). CaW confers a high rate of IS recurrence despite standard anti-platelet treatment that reaches 33.3% of patients prospectively followed with a median time to event of one year. Although no randomised therapeutic studies are available, surgery (n=39) or stenting (n=50) have been often proposed and seem to avoid recurrences. CaW clearly emerges as a cause of cryptogenic embolic stroke and should be systematically investigated in routine. A large number of points remain to be elucidated and CaW patients should be steadily included in registries and randomised therapeutic studies.

Influence of inflammatory status in the acute phase of stroke on post-stroke depression.

BACKGROUND: Thirty percent of stroke patients will suffer from post-stroke depression (PSD). Recent data suggest that inflammation accounts for a substantial amount of depression. Our primary objective was to assess the association between standard inflammation biomarkers in the acute phase of stroke and PSD at three months. The secondary objective was to elaborate a predictive model of PSD from clinical, biological and radiological data. METHODS: We performed a retrospective analysis of a single-centre cohort of stroke patients with a three-month follow-up. Serum levels of C-reactive protein (CRP), fibrinogen, leukocyte count and neutrophil to lymphocyte ratio (NLR) were tested at admission and at peak. Mood was assessed at three months using the depression sub-scale of the Hospital Anxiety and Depression Scale (HADS). Association between inflammation biomarkers and HADS was evaluated with multi-linear regression adjusted on clinical and radiological parameters. Logistic predictive models of PSD at three months, with and without inflammation biomarkers, were compared. RESULTS: Three hundred and forty-eight patients were included, of whom 20.06% developed PSD. Baseline and peak values of all inflammatory markers were associated with the severity of PSD at three months. Area under the curve for the receiver operating characteristic curve of PSD prediction was 0.746 (CI 95% 0.592-0.803) with selected inflammation biomarkers and 0.744 (CI 95% 0.587-0.799) without. CONCLUSION: Most inflammation biomarkers are weakly associated with PSD, adding negligible value to predictive models. While they suggest the implication of inflammation in PSD pathogenesis, they are useless for the prediction of PSD, underscoring the need for more specific biomarkers.

Contribution of routine cardiac biological markers to the etiological workup of ischemic stroke.

BACKGROUND: Optimization of the detection of atrial fibrillation following stroke is mandatory. Unfortunately, access to long-term cardiac monitoring is limited in many centers. The aim of this study was to assess the potential usefulness of three routine biological markers, troponin, D-dimers and BNP, measured in acute stroke phase in the selection of patients at risk of cardio-embolic stroke. METHODS: Troponin, D-Dimers and BNP were measured within 48 h after admission for ischemic stroke in 634 patients. Stroke mechanism was defined at the 3 months follow-up visit using ASCOD classification using a standardized work-up. Association between clinical, radiological and biological markers and stroke mechanism was evaluated using logistic regression analyses. RESULTS: 159 patients (25.1% of total study population) had a cardiac mechanism. On multivariate analysis, admission initial stroke severity (OR 1.04, 95 CI% 1.004-1.07, p < 0.05) history of heart failure (OR 3.03, 95% CI 1.19-7.73, p < 0.05), ECG abnormalities and high BNP value (OR 4.34, 95% CI 2.59-7.29, p < 0.05) were associated with pure cardiac stroke mechanism. CONCLUSION: High BNP value measured within 48 h after stroke admission is an independent predictor of cardiac stroke mechanism. Its measurement might be used to improve the selection of patients for whom further cardiologic investigations such as continuous long term ECG monitoring would be the most useful. BNP should be added to the standard admission-work-up for stroke patients.

Magnetic resonance imaging characteristics of ischemic stroke in an Afro-Caribbean population: A 1-year prospective MRI study on 534 consecutive patients.

PURPOSES: Few population-based MRI studies on stroke, particularly in African-descent populations, are available. Based on a 1-year Afro-Caribbean population-based study MRI, ischemic stroke characteristics were extensively analyzed. METHODS: All strokes occurring in Martinique (390,371 inhabitants) were prospectively included. Ascertainment was based, whenever possible, on MRI. All patients were categorized as single- (subclassified as cortical, cortical-subcortical, subcortical, lacunar) or multiple-lesion pattern, and vascular (single, multiple or junctional) territory. Brain parenchyma was evaluated, based on visualization of macrobleeds, microbleeds, white-matter hyperintensities or stroke sequelae. Etiology was classified according to TOAST criteria. RESULTS: Among 596 ischemic stroke patients included, 534 (295 men, 239 women; mean age, 71 [range 23-110] years) underwent MRI (median delay 1 day). Four hundred and eighty-eight had single-type lesion (14.8% cortical, 42.4% cortical-subcortical, 14.5% subcortical, 16.6% lacunar), involving anterior cerebral (4%), middle cerebral (63.7%), posterior cerebral artery (10.4%) or basilar trunk (11.7%) territories, with 10.3% simultaneously involving multiple territories and 4.9% junctional infarction. Etiologies were LAA (11.2%), SVD (10.7%), CE (29.6%), rare (4.5%) or undetermined (44.1%). CONCLUSION: Our prospective, consecutive, ischemic stroke series gives a comprehensive description of ischemic stroke imaging patterns and etiologic distributions in an Afro-Caribbean population with high socio-economic status. Our patients' stroke characteristics are close to those of European-descent populations.

The impact of intravenous thrombolysis on outcome of patients with acute ischemic stroke after 90 years old.

BACKGROUND: Age increases the risk of mortality and poor prognosis following stroke. The benefit of intravenous thrombolysis in very old patients remains uncertain. The purpose of the study was to evaluate the efficacy and safety of thrombolysis in very old patients considering their perfusion-imaging profile. METHODS: We conducted a retrospective study including patients older than 90 y.o. admitted for an acute ischemic stroke. A computed tomography perfusion-imaging (CTP) was performed in patients who received thrombolysis. Primary outcome was the functional status at 3 months, assessed by the modified Rankin scale (mRS). Secondary outcomes were the rate of hemorrhagic transformations, duration of hospitalization and the rate of death in the first 7 days. Patients receiving thrombolysis were compared with an age-matched group of non-thrombolysed patients. RESULTS: 78 patients were included (31 % male, aged 92 ± 1.7 y.o). 37 patients received thrombolysis and among them, 30 had CTP with a mismatch. The three months mRS was not significantly different in the two groups (mRS 0-2: 5 % and 7 % in the thrombolysed and non-thrombolysed group, respectively). Hemorrhagic transformations were more frequent in the thrombolysed group (54 % versus 12 %, p = 0.002) and symptomatic intracranial hemorrhage tended to be associated with mRS at three months and death in the first 7 days. Duration of hospitalization was longer in the thrombolysed group (10 days ± 12 versus 7 days ± 9, p = 0.046). CONCLUSIONS: Patients who received thrombolysis did not have a better functional prognosis than non-thrombolysed patients.

Lucid dreams, an atypical sleep disturbance in anterior and mediodorsal thalamic strokes.

BACKGROUND: Cognitive, affective, and behavioural disturbances are commonly reported following thalamic strokes. Conversely, sleep disorders are rarely reported in this context. OBSERVATIONS: Herein, we report the cases of two young patients admitted for an ischemic stroke located in the territories of the left pre-mammillary and paramedian arteries. Together with aphasia, memory complaint, impaired attention and executive functions, they reported lucid dreams with catastrophic content or conflicting situations. CONCLUSION: Lucid dreams are an atypical presentation in thalamic strokes. These cases enlarge the clinical spectrum of sleep-wake disturbances potentially observed after an acute cerebrovascular event.

Different HLA class II (DRB1 and DQB1) alleles determine either susceptibility or resistance to NMO and multiple sclerosis among the French Afro-Caribbean population.

BACKGROUND: Despite similarities, neuromyelitis optica (NMO) can be distinguished from multiple sclerosis (MS) by clinical, radiological and serological findings. OBJECTIVE: This case-control study aimed to determine whether patients with NMO or with MS in an Afro-Caribbean population originating from French West Indies shared the same or different HLA class I and II pattern distribution. METHODS: The association with HLA class II (DRB1 and DQB1) alleles was tested in 42 NMO patients, 163 MS patients and 150 healthy controls. HLA-DRB1 and DQB1 typing was undertaken on genomic DNA extracted from peripheral blood leucocytes. RESULTS: By comparison with healthy controls, significantly increased frequency of HLA-DRB1 03 (26.2% vs. 13%, odds ratio 2.4, 95% confidence interval 1.31-4.28, p after correction, cp 0.045) was observed in patients with NMO. By contrast, in MS patients, HLA-DRB1 15 (24.8% vs. 13%, odds ratio 2.21, 95% CI 1.45-3.36, cp < 0.0015), but not DRB1 03 allele, was positively associated with the disease. Moreover, a modest protective effect of HLA-DRB1 11 in the MS group, independently of DRB1 15 association, was found (13.7% vs. 7% in controls, odds ratio 0.48, p 0.006), but did not survive Bonferroni correction. CONCLUSION: In conclusion, comparison of the HLA-DRB1 and DQB1 distribution in NMO and MS in this Afro-Caribbean population shows important differences in the HLA associations among NMO and MS.

Prevalence of carotid web in a French cohort of cryptogenic stroke.

BACKGROUND AND PURPOSE: Carotid webs (CaW) may be an under-recognized cause of anterior circulation cryptogenic ischemic stroke (ACIS). Prevalence is still unknown in European patients with ACIS. OBJECTIVE: To evaluate the prevalence of CaW in ACIS and describe patients with CaW phenotype in a cohort of patients from a French stroke center. METHODS: We conducted a retrospective monocentric cohort study from 01/01/2015 to 31/12/2019 (Montpellier University Hospital, France), in consecutive anterior ischemic stroke (AIS) patients ≤65 years old from a prospective stroke database. Using ASCOD phenotyping, ACIS patients were selected and cervical CTA were reviewed to find CaW. RESULTS: Among 1053 consecutive AIS patients, 266 ACIS patients with CTA were included. Among patients included (mean age 50, women 58%), CaW was in the ipsilateral carotid (iCaW) in 21 patients: 7.9% (95%CI [4.6-11.1]), (mean age 51, 11 women, 16 Caucasian). iCaW were uncovered during study review of CTA in 6/21 (29%) patients. Comparison between patients with iCaW and those without iCaW showed no differences except that of a higher rate of intracranial large vessel occlusion (LVO) (62.4 vs 37.6%; p = 0.03). Patients with iCaW under conservative medical therapy had an annualized stroke recurrence rate (SRR) of 11.4% (95%CI [8.4-15.1]. CONCLUSIONS: iCaW was identified as a source of stroke in about 8% of a French population ≤65 years with ACIS. iCaW was associated with a higher rate of LVO and a high SRR under conservative medical therapy.

[Reversibility of vertebrobasilar stenoses following treatment with corticosteroid therapy in patients with giant cell arteritis]. / Régression de sténoses intracrâniennes vertébrobasilaires symptomatiques sous corticothérapie au cours d'une maladie de Horton.

INTRODUCTION: Involvement of intracranial arteries in giant cell arteritis is a rare condition but often carries a fatal prognosis. Corticosteroids seem to be insufficient to avoid ischemic cerebral complications, and could even promote the occurrence of stroke. We report the case of a patient with giant cell arteritis who experienced recurrent cerebellar stroke caused by intracranial vertebrobasilar stenoses with a favorable outcome following treatment. CASE REPORT: A 77-year-old woman presented with a 3-month history of impaired general condition. She had new-onset headaches, jaw claudication and transient vertigo, especially when she woke-up. The brain MRI showed a recent cerebellar infarction. One week later, she was hospitalized for a clinical deterioration related to a recurrent cerebellar stroke caused by intracranial vertebro-basilar stenoses. Giant cell arteritis was confirmed on the temporal artery biopsy. A treatment with high-dose oral corticosteroids was begun associated with an intensive antiplatelet therapy. The clinical outcome was favorable with rapid improvement of gait imbalance together with a complete radiological regression of the intracranial stenoses. CONCLUSION: Ischemic stroke in giant cell intracranial arteritis is a severe condition without a well-defined treatment. Corticosteroid therapy improves intracranial stenoses caused by vasculitis but should be initially associated with an intensive antithrombotic therapy to avoid early recurrence of cerebral infarcts.

[Cannabis-induced cerebral and myocardial infarction in a young woman]. / Infarctus cérébral et myocardique après consommation de cannabis chez une femme jeune.

INTRODUCTION: Cannabis is the most consumed drug in the world particularly in young adults. Few reports have suggested a causal role of cannabis in the development of cerebral or cardiovascular events. We describe the first association of myocardial infarction and stroke after heavy cannabis consumption in a 45-year-old woman. OBSERVATION: Stroke occurred in relation with a right carotid and middle cerebral artery thrombosis after cannabis abuse. The patient was successfully treated with intravenous rt-PA. Two days after her admission, she presented a myocardial infarction due to a coronary thrombosis. Cerebral and coronary arteries were angiographically normal. Etiological tests were negative and a toxic cause in relation with cannabis consumption was concluded. CONCLUSION: Cannabis can be associated with vascular events by different mechanisms. Thrombosis may occur in cerebral and/or coronary arteries. We suggest that it might be useful to search for cannabis consumption systematically in young subjects victims of stroke and myocardial infarction.

[Bilateral intracerebral angioplasty in a patient with stroke caused by giant cell arteritis]. / Angioplastie carotidienne bilatérale chez une patiente avec infarctus cérébral sur maladie de Horton.

INTRODUCTION: Giant cell arteritis is the most frequent vasculitis and can cause stroke in about 4% of the patients. The mechanism is often hemodynamic in relation with an arterial stenosis or occlusion. Optimal treatment remains to be defined. Outcome is often poor. CASE REPORT: We report the case of a 78-year-old patient, with giant cell arteritis who developed neurological deterioration after the onset of the corticotherapy. Brain MRI revealed bilateral stroke caused by multiple preocclusive internal carotid stenosis. The patient was treated successfully with angioplasty and stenting. CONCLUSION: Endovascular treatment could constitute an interesting alternative for patients with giant cell arteritis associated with neurological deterioration after a first episode of stroke because of hemodynamic instability or at stroke recurrence. For these patients, the usual medical treatment appears to be insufficient.

Relapsing neuromyelitis optica: long term history and clinical predictors of death.

BACKGROUND: Relapsing neuromyelitis optica (RNMO) is an uncommon but devastating inflammatory disorder of the central nervous system. Long term history in a wide series of RNMO is required for better knowledge of the course of the disease and identification of patients at high risk of death. METHODS: Clinical features of patients with RNMO (88 women/eight men) obtained from the geographic Caribbean database (Cuba and French West Indies) were used to determine the progression of disability and to identify clinical predictors of death. RESULTS: Median age at onset of RNMO was 29.5 years (range 11-74). Median duration of disease was 9.5 years (1-40). Median relapse rate was 0.7 attack/patient/year (0.1-3). 66 patients experienced severe visual loss in at least one eye and 46 in both eyes. Median time from onset to unilateral and bilateral severe visual loss was 3 and 15 years, respectively. Median times to reach Kurtzke Disability Status Scale 3, 6 and 8 from onset of RNMO were 1, 8 and 22 years. There were 24 deaths (25%); within 5 years in 63% of cases. A higher attack frequency during the first year of disease (p = 0.009), blindness (p = 0.04) and sphincter signs at onset (p = 0.02) and lack of recovery of first attack (p = 0.003) were independently associated with a shorter time to death. CONCLUSION: RNMO is a very rapidly disabling disease affecting primarily young women. This study has identified clinical features that predict a poor outcome. These findings suggest that early and aggressive immunotherapy might be warranted in RNMO.

Evaluation of the 2005 McDonald MRI criteria for dissemination in space in Afro-Caribbean patients with clinically isolated syndromes.

BACKGROUND: In 2005, the McDonald MRI criteria for dissemination in space were revised to improve diagnosis of multiple sclerosis (MS) in non-Caucasians. METHODS: We included patients with a first clinically isolated syndrome (CIS) to assess their performance in the Afro-Caribbean population. Baseline brain and spine MRI examinations were available within 3 months after onset of CIS. The development of a second clinical event was used as the main outcome indicating clinically definite MS. RESULTS: A total of 66 patients (52F/14M) were included between January 1998 and January 2008 (mean age: 34.7; median follow-up: 34 months). CIS was classified as spinal cord (30.3%), optic neuritis (28.8%), brainstem (24.2%), multiregional (10.6%), hemispheric (4.5%), or undetermined (1.5%). Overall conversion rate was 42.4% (median: 11 months). The McDonald criteria revised for dissemination in space were fulfilled in 33.3% (sensitivity: 0.39 (+/-0.18); specificity: 0.66 (+/-0.15), positive predictive value: 0.46 (+/-0.20), negative predictive value: 0.60 (+/-0.15). CONCLUSION: The Afro-Caribbean population is characterized by a strong proportion of CIS in the spinal cord and a lower burden of disease on the baseline brain MRI. This may explain the low sensitivity of the 2005 McDonald criteria for dissemination in space. Further prospective studies emphasizing MRI spinal cord features are needed to improve diagnostic criteria in a population of African descent.

[Star fruit (Averrhoa carambola) toxic encephalopathy]. / Encéphalopathie toxique par ingestion de carambole (Averrhoa carambola).

Ingestion of star fruit (Averrhoa carambola) can induce severe intoxication in subjects with chronic renal failure. Oxalate plays a key role in the neurotoxicity of star fruit. We report the cases of two patients with unknown chronic renal insufficiency who developed severe encephalopathy after ingestion of star fruit. The two patients developed intractable hiccups, vomiting, impaired consciousness and status epilepticus. Diffusion-weighted MR imaging showed cortical and thalamic hyperintense lesions related to epileptic status. They improved after being submitted to continuous hemofiltration which constitutes the most effective treatment during the acute phase.

[Descriptive epidemiology of neuromyelitis optica in the Caribbean basin]. / Epidémiologie descriptive de la neuromyélite optique dans le bassin caraïbéen.

INTRODUCTION: Data on epidemiology of neuromyelitis optica (NMO) remained scarce in the last century, but the recent development of diagnostic criteria now enables inclusion of both monophasic and relapsing NMO in epidemiologic studies. Given the rarity of NMO, multicentric studies are needed to confirm a presumed higher frequency in women and in populations of black/Asian ancestry. The Caribbean basin is a suitable area for collecting a large NMO cohort and to assess the prevalence, incidence, and mortality of this disorder. PATIENTS AND METHODS: This population-based survey of the NMO spectrum in the French West Indies (FWI) and Cuba included 151 cases. RESULTS: Ninety-eight patients (female/male ratio: 9.8) had NMO. Age of onset in NMO patients was 30.9 years. Mean annual incidence of NMO in the French West Indies for the period July 2002 to June 2007 was 0.20/100,000 inhabitants (IC 95% 0.05-0.35). Incidence rates were steady in the FWI during the 1992 to 2007 period. Decreasing mortality in the FWI during the 1992 to 2007 period explained the increasing prevalence which was 4.20/100,000 inhabitants (IC 95% 3.7-5.7) in June 2007. The prevalence of NMO in Cuba on November302004 was 0.52/100,000 inhabitants. (IC 95% 0.39-0.67). Prevalence rates did not differ significantly by ethnic group in Cuba, however, black Cubans exhibited the highest prevalence. DISCUSSION: Epidemiologic studies on NMO in each population are needed to determine whether aggressive therapies can reduce the mortality of this devastating disorder. CONCLUSION: In the Caribbean basin, NMO involves almost exclusively young women; the epidemiologic data confirm its predilection for populations of African ancestry. In the FWI, recent and aggressive therapy has lowered mortality but with an increase in the prevalence of NMO.

MRI of the spinal cord in neuromyelitis optica and recurrent longitudinal extensive myelitis.

BACKGROUND AND PURPOSE: Neuromyelitis optica (NMO) is a severe inflammatory and necrotizing disease that clinically affects the optic nerves and spinal cord in a relapsing course. We assessed the baseline and follow-up MRI characteristics of cord attacks in NMO and recurrent longitudinal extensive myelitis (RLEM). METHODS: We retrospectively reviewed MRI data of 20 Afro-Caribbean patients diagnosed with either NMO or RLEM. MRI data from 51 cord or mixed attacks were evaluated, and 65 follow-up MRI studies were available for 30 baseline acute examinations. RESULTS: The cervical cord was involved in 63% of cases. Four attacks were limited to the brainstem. MRI of the spinal cord revealed longitudinal extensive signal abnormalities extending over three vertebral segments, associated with cord swelling in 67% of the 51 relapses. Gadolinium enhancement was observed, preferentially surrounding edema, in 69% of attacks. In the axial plane, signal abnormalities typically involved central areas of the cord. Cavitation was observed in 16% of attacks. Cord attacks recurred in the same or contiguous areas in 67% of cases. Follow-up MRI revealed a gradual decrease in cord swelling and T2 signal hyperintensity, with fragmentation of signal abnormalities in some cases. Cord atrophy was evident in 57% of the follow-up MRI. CONCLUSION: Given the poor prognosis of NMO and RLEM, radiologists need to be aware of the MRI pattern to prevent further attacks with the use of aggressive treatment.

[Steroid treatment in four cases of anti-GAD cerebellar ataxia]. / Traitement des ataxies cérébelleuses à anticorps anti-GAD par cures séquentielles de corticoïdes.

INTRODUCTION: Few neurological diseases are linked with anti-glutamic acid decarboxylase antibodies (GAD-ab); stiff man syndrome is an example. Cerebellar ataxia is a new feature of this expanding spectrum. No therapeutic trial is yet available in these diseases. We here report on four patients suffering from cerebellar ataxia linked with GAD-ab and review the data in the literature on this recently described syndrome. METHOD: We conducted an open trial with monthly pulsed steroids. Steroid pulses were given six months followed with placebo for another six months. Main clinical and biological parameters were monitored monthly (International Cooperative Cerebellar Ataxia Rating Scale [ICARS] and GAD-ab). RESULT: The clinical response was found limited and inconstant. Transient decline in GAD-ab level was noted in two patients. Moreover, GAD-ab level was found highly variable and did not correlate with clinical parameters. DISCUSSION: Cerebellar ataxia with GAD-ab is an increasingly described syndrome. Outcome can be severe, leading to definitive cerebellar atrophy. Diagnosis is supported by high level of serum GAD-ab with intrathecal secretion. Experimental data have suggested a direct excitotoxic effect of GAD-ab on Purkinje cells. Response to various treatments is often disappointing. Improvement has been obtained with veinoglobulins in individual patients. A weak clinical and biological response was associated with monthly steroid pulses.