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CS is an autosomal recessive multisystem disorder, which is mainly characterized by neurologic and sensory impairment, cachectic dwarfism, and photosensitivity. We describe the neuroimaging features (MR imaging, ¹H-MR spectroscopy, and CT) in the various clinical subtypes of CS from a cohort of genetically and biochemically proved cases. Hypomyelination,(More)
BACKGROUND Respiratory failure is the most serious short-term complication of Guillain-Barré syndrome and can require invasive mechanical ventilation in 20-30% of patients. We sought to identify clinical and electrophysiological predictors of respiratory failure in the disease. METHODS We prospectively assessed electrophysiological data and clinical(More)
BACKGROUND Little is known about the epidemiology and the prognostic factors of Guillain-Barré syndrome (GBS) following primary infection with cytomegalovirus (CMV-GBS). METHODS We prospectively followed up 506 patients with cases of GBS who were admitted to our center from 1996 through 2006. We diagnosed 63 (12.4%) CMV-GBS cases by immunoglobulin (Ig) M(More)
Slowness, fatigue, and learning difficulties are common in young patients with myotonic dystrophy type 1. These features may indicate poor sleep quality. The aim of this study was to search for sleep disorders in this population. This prospective study used questionnaires, genetic testing, night-time polysomnography and multiple sleep latency tests to(More)
BACKGROUND In Western countries, the cause of 60% of all Guillain-Barré syndrome (GBS) cases remains unidentified. The number of cases of unidentified cause peaks in winter, and these cases are commonly preceded by respiratory tract infection or influenza-like illness. We investigated the triggering role of influenza virus infection. METHODS Of 405(More)
The Bardet-Biedl syndrome (BBS) is a rare ciliopathy clinically defined by the association of retinitis pigmentosa, polydactyly, obesity, kidney disease and cognitive impairment. The cognitive functioning, behavioral phenotype, prevalence of psychiatric diseases and memory performances of a cohort of 34 patients with BBS were evaluated and a systemic brain(More)
OBJECTIVES Illness is often associated with anxiety, but few data exist about the prognostic significance of this phenomenon. To address this issue, we assessed whether patient anxiety is associated with subsequent need for intubation in Guillain-Barré syndrome (GBS). DESIGN Incident case-cohort study. SETTING Acute secondary care in a teaching hospital(More)
OBJECTIVES To study the incidence of clinical signs linked to botulinum toxin type A (BoNTA) spread from the injection site. METHODS Single-center, retrospective, cohort study. All patients who received BoNTA injections for spasticity treatment were assessed 1 month postinjection. Adverse effects indicative of BoNTA treatment were systematically sought.(More)
BACKGROUND Serial electrophysiology has been suggested as essential for accurate diagnosis in Guillain-Barré syndrome (GBS). However, whether more adapted electrophysiological criteria may allow a single study to be sufficient is unknown. METHODS We retrospectively reviewed records of 365 consecutive patients with GBS from Birmingham, U.K., and Garches,(More)
INTRODUCTION Sepsis is associated with increased mortality, delirium and long-term cognitive impairment in intensive care unit (ICU) patients. Electroencephalogram (EEG) abnormalities occurring at the acute stage of sepsis may correlate with severity of brain dysfunction. Predictive value of early standard EEG abnormalities for mortality in ICU septic(More)