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.

Response to interferon-Beta treatment in afro-caribbeans with multiple sclerosis.

Background. Multiple sclerosis (MS) patients of African ancestry have a more aggressive disease course than white patients and could be resistant to interferon-beta (INFB). Methods. We studied the impact of INFB in treatment-naive Afro-Caribbean (AC) with clinically definite MS using our European Database for Multiple Sclerosis (EDMUS) (2003-2010). Main outcome measures were annual relapse rate after 2 years of treatment, proportion of exacerbation-free subjects 48 weeks after initiating INFB, and time to first relapse. Results. 76 AC-MS (59F/17M) were identified. Annual relapse rate of 1.29 decreased to 0.83 (-35.6%) after 2 years of treatment. The proportion of relapse-free patients at 48 weeks was 46.2%. Median time to first relapse was 52 weeks. Conclusion. INFB is not strong enough to control AC-MS patients in many cases which is problematic in a population of worse MS prognosis.

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.

[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.

[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.

[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.

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.

[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.

[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.

[Failure of rituximab in relapsing neuromyelitis optica: case report with two-year prospective follow-up]. / Echec thérapeutique du rituximab dans un cas de neuromyélite optique rémittente: suivi prospectif longitudinal sur deux ans.

Neuromyelitis optica (Devic's disease, NMO) is an inflammatory disease of the central nervous system preferentially involving the spinal cord and optic nerves in either a monophasic or relapsing-remitting course. B-cell induced pathogenesis was recently described for NMO. Rituximab is a chimeric monoclonal antibody directed against the CD20 antigen, which causes depletion of B-cells. Rituximab might be effective in treatment of NMO. We report the two-year clinicoradiological and biological follow-up data from a patient included in July 2005 in a prospective trial of rituximab for severe NMO refractory to immunosuppressant therapy. Blood B-cell depletion after rituximab induction was maintained for 10 months. Seven attacks occurred during a two-year follow-up. EDSS increased from 7.0 to 8.0. Left visual acuity decreased from 20/20 degrees to 20/60 degrees. Spinal cord atrophy worsened.

Thrombotic stroke following snake bites by the "Fer-de-Lance"Bothrops lanceolatus in Martinique despite antivenom treatment: a report of three recent cases.

BACKGROUND: The severity of envenoming from Bothrops lanceolatus is determined by the development of cerebral, myocardial or pulmonary infarctions, and occasionnaly by serious local envenoming. Introduction of specific antivenom has resulted in a dramatic improvement in the prognosis of this envenoming. Against this background, we report 3 recent cases of patients bitten by B. lanceolatus who developed cerebral infarctions despite early administration of antivenom. METHODS: In 1991 a protocol was designed to apply the same evaluation and treatment to all envenomed patients. The clinical results have been continuously monitored. RESULTS: Between April 1993 and July 2003, 128 envenomed patients (age 6-83 (mean 45) years) were treated. No coagulopathy, thrombotic complication or death occurred in patients who were given early antivenom therapy--up to 6h following the bite--and 126 patients recovered. Between August 2003 and October 2004, 10 additional patients (18-66 (mean 46) years) were given antivenom at the time of admission at hospital. Of these, 3 developed cerebral infarctions within 24h. Effectiveness of antivenom was tested on mouse, and found to be lower than specified by the manufacturer. DISCUSSION: Our data shows that recently the antivenom may have lost some of its efficacy. Possible mechanisms include variability in venom composition or loss of activity of the antibodies produced more than 15 years ago. The question is whether we should attempt to produce improved antivenom. This could include activity against the venom of Bothrops caribbaeus from the neighbouring island of St Lucia, which shares a monophyletic group with B. lanceolatus and whose venom produces a similar thrombotic syndrome. CONCLUSION: Prevention of systemic vessels thrombosis remains the main therapeutic challenge of B. lanceolatus envenoming in Martinique.