Clinical and pathophysiological implications of autoantibodies in autoimmune neuropathies.

Autoimmune neuropathies are a heterogeneous group of rare and disabling diseases in which the immune system targets peripheral nervous system antigens and that respond to immune therapies. This review focuses on Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, polyneuropathy associated with IgM monoclonal gammopathy, and autoimmune nodopathies. Autoantibodies targeting gangliosides, proteins in the node of Ranvier, and myelin-associated glycoprotein have been described in these disorders, defining subgroups of patients with similar clinical features and response to therapy. This topical review describes the role of these autoantibodies in the pathogenesis of autoimmune neuropathies and their clinical and therapeutic importance.

The expanding field of IgG4-mediated neurological autoimmune disorders.

At least 13 different disease entities affecting the central nervous system, peripheral nervous system and connective tissue of the skin or kidneys are associated with immunoglobulin G4 (IgG4) immune reactivity. IgG4 has always been considered a benign, non-inflammatory subclass of IgG, in contrast to the well-known complement-activating pro-inflammatory IgG1 subclass. A comprehensive review of these IgG4 autoimmune disorders reveals striking similarities in epitope binding and human leukocyte antigen (HLA) associations. Mechanical interference of extracellular ligand-receptor interactions by the associated IgG4 antibodies seems to be the common/converging disease mechanism in these disorders.

Long-lasting treatment effect of rituximab in MuSK myasthenia.

OBJECTIVE: Rituximab has emerged as an efficacious option for drug-resistant myasthenia gravis (MG). However, reports published only describe the short-term follow-up of patients treated and little is known about their long-term clinical and immunologic evolution. Our objective was to report the clinical and immunologic long-term follow-up of 17 patients (6 MuSK+MG and 11 AChR+MG) and compare the response between AChR+MG and MuSK+MG patients. METHODS: Myasthenia Gravis Foundation America postintervention status and changes in treatment and antibody titers were periodically determined. Lymphocyte subpopulations, total immunoglobulin, immunoglobulin G (IgG) anti-MuSK subclasses, and anti-tetanus toxoid IgG before and after treatment were also studied. RESULTS: After a mean post-treatment period of 31 months, 10 of the AChR+MG patients improved but 6 of them needed reinfusions. In contrast, all MuSK+MG patients achieved a remission (4/6) or minimal manifestations (2/6) status and no reinfusions were needed. Consequently, in the MuSK+MG group, prednisone doses were significantly reduced and concomitant immunosuppressants could be withdrawn. Clinical improvement was associated with a significant decrease in the antibody titers only in the 6 MuSK+MG patients. At last follow-up MuSK antibodies were negative in 3 of these patients and showed a decrease of over 80% in the other 3. CONCLUSION: In view of the long-lasting benefit observed in MuSK+MG patients, we recommend to use rituximab as an early therapeutic option in this group of patients with MG if they do not respond to prednisone. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that IV rituximab improves the clinical and immunologic status of patients with MuSK+MG.

Blood pressure is not associated with haematoma enlargement in acute intracerebral haemorrhage.

BACKGROUND AND PURPOSE: We performed an observational study that compared baseline and subsequent blood pressure (BP) measurements and its association with haematoma enlargement (HE) in patients with intracerebral haemorrhage (ICH). METHODS: We prospectively studied consecutive patients with supratentorial spontaneous ICH within the first 6 h after the onset of symptoms. HE was defined as an increase >or=33% in the volume of haematoma on the CT obtained 24-48 h after the onset of symptoms as compared with the CT at admission. We recorded systolic BP (SBP), diastolic BP (DBP) and mean BP (MBP) at admission and at 6, 12, 18 and 24 h after onset; the maximum SBP, DBP and MBP during the study period; the maximum SBP and DBP within intervals; the mean of all BP readings; administration of antihypertensive agents. RESULTS: We studied 60 patients whose mean age was 72.1 +/- 11.3 years. HE was observed in 27 (45%) patients. No statistically significant differences were observed in any of the analyses that compared BP parameters between the HE and non-HE groups (two-way anova). CONCLUSIONS: In an exploratory analysis, we did not find an association between BP and HE within the first 24 h after an acute ICH.

Infartos retiniano y cerebral secundarios a trombosis carotídea aguda en el síndrome de hiperestimulación ovárica / Retinal and brain infarctions secondary Retinal and brain infarctions secondary

Introducción. El síndrome de hiperestimulación ovárica(SHO) es un cuadro clínico yatrógeno infrecuente, pero potencialmentefatal, que puede aparecer tras el uso de inductoresde la ovulación en algunos tratamientos de fertilidad.Durante su curso pueden aparecer diversas complicaciones,algunas muy graves, y, entre ellas, diversos fenómenos tromboembólicos.Por tanto, y aunque con muy baja frecuencia,el SHO es una causa potencial de ictus.Caso clínico. Describimos el caso de una mujer de 34 años,en tratamiento inductor de la ovulación, que desarrolló duranteel curso de un SHO infartos retiniano y cerebral secundariosa trombosis carotídea aguda, tratados medianteanticoagulación, con buena evolución clínica, aunque conpersistencia de la oclusión en controles posteriores medianteangiorresonancia magnética.Conclusión. Se trata de la primera trombosis carotídeaen el contexto de este síndrome descrita en España y la octavapublicada en la literatura. Asimismo resumimos brevementelas diferentes publicaciones relacionadas con complicacionescerebrovasculares en el contexto del SHO (AU)

Introduction. Ovarian hyperstimulation syndrome(OHSS) is an uncommon but potentially life-threateningiatrogenic condition that may appear following ovulationinduction in the course of some fertility treatments.This may lead to further complications, some of whichmay be severe, such as thromboembolic events. Thoughrarely, it can therefore be a potential cause of stroke Clinical case. We report the case of a 34-year old womanunder ovulation induction treatment who developedretinal and brain infarctions secondary to internal carotidocclusion. Oral anticoagulation was administered and recoverywas good in spite of the persistence of carotid occlusionin follow-up magnetic ressonance imaging-angiographies.Conclusions. This is the first case of carotid occlusionfollowing an OHSS reported in Spain and the eighth onepublished in the literature. Current literature on cerebrovascularcomplications in OHSS is also briefly reviewed

[Retinal and brain infarctions secondary to acute carotid thrombosis in ovarian hyperstimulation syndrome]. / Infartos retiniano y cerebral secundarios a trombosis carotídea aguda en el síndrome de hiperestimulación ovárica.

INTRODUCTION: Ovarian hyperstimulation syndrome (OHSS) is an uncommon but potentially life-threatening iatrogenic condition that may appear following ovulation induction in the course of some fertility treatments. This may lead to further complications, some of which may be severe, such as thromboembolic events. Though rarely, it can therefore be a potential cause of stroke. CLINICAL CASE: We report the case of a 34-year old woman under ovulation induction treatment who developed retinal and brain infarctions secondary to internal carotid occlusion. Oral anticoagulation was administered and recovery was good in spite of the persistence of carotid occlusion in follow-up magnetic ressonance imaging- angiographies. CONCLUSIONS: This is the first case of carotid occlusion following an OHSS reported in Spain and the eighth one published in the literature. Current literature on cerebrovascular complications in OHSS is also briefly reviewed.