Mathieu Zuber

Botulinum antibodies in dystonic patients treated with type A botulinum toxin : frequency and significance

We measured serum antibodies to botulinum toxin (ABT) in 96 patients with focal dystonia who had been treated with type A botulinum toxin. The frequency of detectable ABT was 3% (three patients). Patients with ABT had received more than 50 ng of botulinum toxin, and the shortest time between two injections was significantly less than in patients without ABT. The clinical evolution of the three patients was heterogeneous: one had decreased effectiveness with repeated injections, another had persistent improvement, and the third never responded to toxin injections.

Factor V Leiden Mutation in Cerebral Venous Thrombosis

BACKGROUND AND PURPOSE Resistance to activated protein C is a common inherited risk factor for venous thrombosis, which is due to a mutation in coagulation factor V (factor V Leiden mutation). It is present in approximately 20% of unselected consecutive patients with deep vein thrombosis. The rate of resistance to activated protein C in patients with cerebral venous thrombosis (CVT) is unknown. METHODS We investigated the association of factor V mutation with CVT using a case-control study. Nineteen unselected patients with CVT and 57 healthy control subjects were tested for the point mutation. RESULTS The mutation was found in a heterozygous form in 4 of the 19 patients with CVT (21%) and in only 1 of the 57 control subjects (2%) (P = .02, Fishers exact test). The prevalence of the coagulation defect found in our patients with CVT was consistent with that observed in previous studies in patients with deep vein thrombosis. In 3 of the 4 patients positive for the mutation, CVT developed in the presence of an acquired prothrombotic state, including oral contraceptive use in 2 patients and puerperium in the third. CONCLUSIONS Factor V Leiden mutation is a risk factor for CVT and may be the most common inherited coagulation defect associated with this condition.

Patent Foramen Ovale Closure or Anticoagulation vs. Antiplatelets after Stroke

BACKGROUND Trials of patent foramen ovale (PFO) closure to prevent recurrent stroke have been inconclusive. We investigated whether patients with cryptogenic stroke and echocardiographic features representing risk of stroke would benefit from PFO closure or anticoagulation, as compared with antiplatelet therapy. METHODS In a multicenter, randomized, open‐label trial, we assigned, in a 1:1:1 ratio, patients 16 to 60 years of age who had had a recent stroke attributed to PFO, with an associated atrial septal aneurysm or large interatrial shunt, to transcatheter PFO closure plus long‐term antiplatelet therapy (PFO closure group), antiplatelet therapy alone (antiplatelet‐only group), or oral anticoagulation (anticoagulation group) (randomization group 1). Patients with contraindications to anticoagulants or to PFO closure were randomly assigned to the alternative noncontraindicated treatment or to antiplatelet therapy (randomization groups 2 and 3). The primary outcome was occurrence of stroke. The comparison of PFO closure plus antiplatelet therapy with antiplatelet therapy alone was performed with combined data from randomization groups 1 and 2, and the comparison of oral anticoagulation with antiplatelet therapy alone was performed with combined data from randomization groups 1 and 3. RESULTS A total of 663 patients underwent randomization and were followed for a mean (±SD) of 5.3±2.0 years. In the analysis of randomization groups 1 and 2, no stroke occurred among the 238 patients in the PFO closure group, whereas stroke occurred in 14 of the 235 patients in the antiplatelet‐only group (hazard ratio, 0.03; 95% confidence interval, 0 to 0.26; P<0.001). Procedural complications from PFO closure occurred in 14 patients (5.9%). The rate of atrial fibrillation was higher in the PFO closure group than in the antiplatelet‐only group (4.6% vs. 0.9%, P=0.02). The number of serious adverse events did not differ significantly between the treatment groups (P=0.56). In the analysis of randomization groups 1 and 3, stroke occurred in 3 of 187 patients assigned to oral anticoagulants and in 7 of 174 patients assigned to antiplatelet therapy alone. CONCLUSIONS Among patients who had had a recent cryptogenic stroke attributed to PFO with an associated atrial septal aneurysm or large interatrial shunt, the rate of stroke recurrence was lower among those assigned to PFO closure combined with antiplatelet therapy than among those assigned to antiplatelet therapy alone. PFO closure was associated with an increased risk of atrial fibrillation. (Funded by the French Ministry of Health; CLOSE ClinicalTrials.gov number, NCT00562289.)

Primary angiitis of the central nervous system: description of the first fifty-two adults enrolled in the French cohort of patients with primary vasculitis of the central nervous system.

To describe characteristics and outcomes of a multicenter cohort of patients diagnosed as having primary angiitis of the central nervous system (PACNS).

Cerebral vasculopathy with aneurysm formation in HIV-infected young adults.

The article by Kossorotoff et al. highlights the importance of vigilance for HIV-associated cerebral aneurysms in adults as a cause of stroke.1 They discuss two patients with HIV, ages 23 and 32, who had strokes and were found to have ectasias and aneurysms of the large cerebral arteries (which they term CVA [cerebral vasculopathy with aneurysm]). Although the definitive cause of CVA in HIV-infected persons remains unclear, there is evidence that immune reconstitution in patients with AIDS leads to a vasculopathy complicating a chronic endothelial infection.2 Immune reconstitution inflammatory syndrome (IRIS) is …

Reversible cerebral angiopathy: efficacy of nimodipine.

Reversible cerebral angiopathy (RCA) is responsible for disabling headache and potential stroke complications. Most patients respond poorly to analgesics. We describe four patients with typical RCA whose headache rapidly disappeared after IV nimodipine treatment was initiated.

Carotid artery dissection due to elongated styloid process

Internal carotid artery (ICA) dissection during a long-lasting telephone conversation is probably related to lateral flexion of the head.1 We observed a case in which the likely mechanism of arterial dissection in this position could be elicited; specifically, mechanical injury of the ICA due to an elongated styloid process. This mechanism of injury could have been previously underestimated in ICA dissection. Shortly following a long-lasting phone conversation (more than 1 hour), a previously healthy 43-year-old psychiatrist suddenly complained of left transient monocular blindness followed by left pulsatile tinnitus and motor aphasia. Throughout the conversation, he held the …

Séquences IRM « SWAN, SWI et VenoBOLD » exploitant le phénomène de susceptibilité magnétique : principes techniques et applications cliniques

Susceptibility-weighted MR sequences, T2 star weighted angiography (SWAN, General Electric), Susceptibility weighted imaging (SWI, Siemens) and venous blood oxygen level dependant (VenoBOLD, Philips) are 3D spoiled gradient-echo sequence that provide a high sensitivity for the detection of blood degradation products, calcifications, and iron deposits. For all these sequences, an appropriate echo time allows for the visualization of susceptibility differences between adjacent tissues. However, each of these sequences presents a specific technical background. The purpose of this review was to describe 1/the technical aspects of SWAN, VenoBOLD and SWI sequences, 2/the differences observed in term of contrast within the images, 3/the key imaging findings in neuroimaging using susceptibility-weighted MR sequences.

RevueSéquences IRM « SWAN, SWI et VenoBOLD » exploitant le phénomène de susceptibilité magnétique : principes techniques et applications cliniquesSusceptibility weighted magnetic resonance sequences “SWAN, SWI and VenoBOLD”: Technical aspects and clinical applications

Susceptibility-weighted MR sequences, T2 star weighted angiography (SWAN, General Electric), Susceptibility weighted imaging (SWI, Siemens) and venous blood oxygen level dependant (VenoBOLD, Philips) are 3D spoiled gradient-echo sequence that provide a high sensitivity for the detection of blood degradation products, calcifications, and iron deposits. For all these sequences, an appropriate echo time allows for the visualization of susceptibility differences between adjacent tissues. However, each of these sequences presents a specific technical background. The purpose of this review was to describe 1/the technical aspects of SWAN, VenoBOLD and SWI sequences, 2/the differences observed in term of contrast within the images, 3/the key imaging findings in neuroimaging using susceptibility-weighted MR sequences.

Clinical and genetic heterogeneity of Charcot-Marie-Tooth disease.

The autosomal dominant forms of hereditary motor and sensory neuropathies include the hypertrophic form (CMT1) and the neuronal form of Charcot-Marie-Tooth disease (CMT2). While at least two distinct loci have been shown to be linked to the CMT1 phenotype (CMT1A and CMT1B, on chromosomes 17 and 1, respectively), whether the CMT2 phenotype results from mutations allelic to either of the CMT1 genes remains unknown. Studying one CMT1 and two CMT2 pedigrees, we were able to exclude the CMT2 disease locus from the region of chromosome 17 (Z = -2.80 at theta = 0.05 for D17S58) where the CMT1A gene maps (Z = +3.67 at theta = 0.00). Similarly, negative lod score values were obtained in CMT2 for the region of chromosome 1 where the CMT1B gene has been located (Z = -3.09 at theta = 0.05 for D1S61). The present study therefore provides evidence for genetic heterogeneity between the hypertrophic and the neuronal forms of Charcot-Marie-Tooth disease and demonstrates that the CMT2 gene is not allelic to either of the CMT1 genes mapped to date.