Comparison of juvenile and adult myasthenia gravis in a French cohort with focus on thymic histology.

Myasthenia gravis (MG) is an autoimmune disease characterized by muscle fatigability due to acetylcholine receptor (AChR) autoantibodies. To better characterize juvenile MG (JMG), we analyzed 85 pre- and 132 post-pubescent JMG (with a cutoff age of 13) compared to 721 adult MG patients under 40 years old using a French database. Clinical data, anti-AChR antibody titers, thymectomy, and thymic histology were analyzed.

Not all patients with convulsive status epilepticus intubated in pre-hospital settings meet the criteria for refractory status epilepticus.

Introduction: Mechanically ventilated patients admitted to the intensive care unit (ICU) for generalized convulsive status epilepticus (GCSE) are a heterogeneous population. Our objective was to evaluate the number of patients who fulfilled the diagnostic criteria for refractory GCSE and describe their initial management and prognosis.

Methods: This multicenter retrospective study was conducted in four French ICUs in Pitié-Salpêtrière University Hospital in Paris and in the Hospital of Jossigny.

Regulatory B cells in myasthenia gravis are differentially affected by therapies.

We analyzed the number and functionality of regulatory B (Breg) cells in well-defined myasthenia gravis patients. We first showed a decreased number of circulating CD19 CD24 CD38 Breg cells and an altered functionality of Breg cells in untreated myasthenia gravis patients. Next, we demonstrated that the proportion of circulating Breg cells was restored in myasthenia gravis patients after thymectomy, probably as Breg cells could be sequestered in the myasthenia gravis thymus.

Surgery and risk of Guillain-Barré syndrome: A French nationwide epidemiologic study.

Objective: To assess the association between Guillain-Barré syndrome (GBS) and recent surgery based on French nationwide data.

Methods: Data were extracted from the French health administrative databases (SNIIRAM/PMSI). All patients hospitalized for GBS between 2009 and 2014 were identified by ICD-10 code G61.

Survival and consciousness recovery are better in the minimally conscious state than in the vegetative state.

Background: The prognosis value of early clinical diagnosis of consciousness impairment is documented by an extremely limited number of studies, whereas it may convey important information to guide medical decisions.

Objective: We aimed at determining if patients diagnosed at an early stage (<90 days after brain injury) as being in the minimally conscious state (MCS) have a better prognosis than patients in the vegetative state/Unresponsive Wakefulness syndrome (VS/UWS), independent of care limitations or withdrawal decisions.

Methods: Patients hospitalized in ICUs of the Pitié-Salpêtrière Hospital (Paris, France) from November 2008 to January 2011 were included and evaluated behaviourally with standardized assessment and with the Coma Recovery Scale-Revised as being either in the VS/UWS or in the MCS.

Regulatory T cell frequency, but not plasma IL-33 levels, represents potential immunological biomarker to predict clinical response to intravenous immunoglobulin therapy.

Background: Intravenous immunoglobulin (IVIG) is a polyspecific pooled immunoglobulin G preparation and one of the commonly used therapeutics for autoimmune diseases including those of neurological origin. A recent report in murine model proposed that IVIG expands regulatory T (T) cells via induction of interleukin 33 (IL-33). However, translational insight on these observations is lacking.

Resistant myasthenia gravis and rituximab: A monocentric retrospective study of 28 patients.

This retrospective study evaluated the efficiency and tolerance of rituximab in the management of resistant myasthenia gravis (MG). All patients who received rituximab for the treatment of MG between 2004 and 2015 at Pitié-Salpétrière University Hospital (Paris, France) were included. The efficacy of rituximab was evaluated every 6 months by the myasthenic muscle score (MMS), the Myasthenia Gravis Foundation of America - Clinical Classification (MGFA-CC), the MGFA Therapy Status and the Postintervention Status (PIS).

Guillain-Barré syndrome in France: a nationwide epidemiological analysis based on hospital discharge data (2008-2013).

Guillain-Barré syndrome (GBS) is potentially life threatening and typically occurs after an infection. No detailed information is available concerning the epidemiological characteristics of GBS in France. We estimated age- and sex-specific incidence rates (IRs) based on a French nationwide hospital discharge database.

Translation, cross-cultural adaptation, and validation of the french version of the 15-item Myasthenia Gravis Quality Of life scale.

Introduction: Evaluation of quality of life (QOL) has become essential in healthcare. Currently no MG-specific QOL measure exists in French. The aim of this study was to translate, culturally adapt, and evaluate the psychometric properties of the French version of the 15-Item Myasthenia Gravis Quality of Life Scale (MG-QOL15) scale for French myasthenia patients.

Multidrug-resistant bacteria transmitted through high-density EEG in ICU.

Purpose: Prevention of multidrug resistant (MDR) bacterial contamination remains a major challenge in ICUs. Many hospital outbreaks involving MDR transmitted through environmental contamination have been reported. Bedside high-density EEG allow for dynamic cognitive evaluation in brain-injured patients and is used more and more frequently in clinical practice to evaluate brain function and predict outcome in severely neurologically impaired patients.

Prehospital treatment with levetiracetam plus clonazepam or placebo plus clonazepam in status epilepticus (SAMUKeppra): a randomised, double-blind, phase 3 trial.

Background: Generalised convulsive status epilepticus (GCSE) should be treated quickly. Benzodiazepines are the only drug treatment available so far that is effective before admission to hospital. We assessed whether addition of the antiepileptic drug levetiracetam to the benzodiazepine clonazepam would improve prehospital treatment of GCSE.

Post-traumatic stress symptoms in Guillain-Barré syndrome patients after prolonged mechanical ventilation in ICU: a preliminary report.

Thirty percent of Guillain-Barré syndrome (GBS) patients require mechanical ventilation (MV) in intensive care unit (ICU). Post-traumatic stress disorder (PTSD) is found in ICU survivors, and the traumatic aspects of intubation and MV have been previously reported as risk factors for PTSD after ICU. Our objective was to determine long-term PTSD or post-traumatic stress symptoms (PTSS) in GBS patients after prolonged MV in ICU.

Intravenous immunoglobulin exerts reciprocal regulation of Th1/Th17 cells and regulatory T cells in Guillain-Barré syndrome patients.

Guillain-Barré syndrome (GBS) is an acute, autoimmune inflammatory disorder of peripheral nervous system characterized by a severe functional motor weakness. Treatment with intravenous immunoglobulin (IVIg) is one of the approved and preferred therapeutic strategies for GBS. However, the mechanisms underlying the therapeutic benefit with IVIg in GBS are not completely understood.

Granulomatous angiitis of the CNS revealing a Hodgkin lymphoma.

Apart from the iatrogenic effects of treatment, neurologic complications of Hodgkin lymphoma (HL) can be divided into direct (meningeal or intracranial/spinal localization) and indirect (paraneoplastic/immune complications).(1) Here, we present a patient with granulomatous angiitis of the CNS (GANS) associated with HL that dramatically improved after the treatment of the angiitis by cyclophosphamide, methylprednisolone, and specific chemotherapy.

Event related potentials elicited by violations of auditory regularities in patients with impaired consciousness.

Improving our ability to detect conscious processing in non communicating patients remains a major goal of clinical cognitive neurosciences. In this perspective, several functional brain imaging tools are currently under development. Bedside cognitive event-related potentials (ERPs) derived from the EEG signal are a good candidate to explore consciousness in these patients because: (1) they have an optimal time resolution within the millisecond range able to monitor the stream of consciousness, (2) they are fully non-invasive and relatively cheap, (3) they can be recorded continuously on dedicated individual systems to monitor consciousness and to communicate with patients, (4) and they can be used to enrich patients' autonomy through brain-computer interfaces.

A prehospital randomized trial in convulsive status epilepticus.

Therapeutic strategies for patients with generalized convulsive status epilepticus (GCSE) need to be improved. We present the design of an add-on, randomized, double-blind, placebo-controlled, phase III clinical trial, to compare the efficacy for GCSE of intravenous levetiracetam in association with clonazepam versus clonazepam alone. In the therapeutic arm, 1 mg clonazepam is injected together with 2500 mg levetiracetam over 5 min.

Prominent plasmacytosis following intravenous immunoglobulin correlates with clinical improvement in Guillain-Barré syndrome.

Background: High doses of pooled polyclonal IgG are commonly used to treat numerous autoimmune diseases. Their mode of action nevertheless remains only partially explained. At the same time, until now, no early biological marker has been able to predict their efficacy.

Acute disseminated encephalomyelitisin the intensive care unit:clinical features and outcome of 20 adults.

Objective: Because acute disseminated encephalomyelitis (ADEM) is a rare disease in adults admitted to the intensive care unit (ICU), we describe its characteristics and patient outcomes.

Design And Setting: A retrospective (2000-2006), observational, multicenter study was conducted in seven medical ICUs. Clinical, biological and neuroimaging features of patients diagnosed with ADEM were evaluated.