Neurocognition in Congenital Central Hypoventilation Syndrome: influence of genotype and ventilation method.

Background: Congenital Central Hypoventilation Syndrome (CCHS) is characterized by central hypoventilation due to abnormal autonomic control of breathing and global dysautonomia. Patients harbour heterozygous PHOX-2B gene mutations which are polyalanine repeats of various lengths in most of the cases. A few previous studies have reported learning difficulties and neuropsychological disorders in patients with CCHS.

Snoring and Obstructive Sleep Apnea: Objective Efficacy and Impact of a Chairside Fabricated Mandibular Advancement Device.

Purpose: Obstructive sleep apnea (OSA) has been described as a common sleep respiratory disorder. Its prevalence in the adult population has been reported to be high, varying from 3% to 28%. Dental practitioners play a key role in the treatment of this disease, using tailor-made mandibular advancement devices (MADs).

EEG in children, in the laboratory or at the patient's bedside.

In pediatrics, EEG recordings are performed on patients from the neonatal period up to young adults. This means adapting techniques to many different conditions, concerning not only the patient's age, the need for asepsis and the patient's behavior, but also the environment (e.g.

Continuous EEG monitoring in children in the intensive care unit (ICU).

Pediatric EEG in the intensive care unit (ICU) requires specific technical requirements in order to yield relevant data depending upon clinical scenario: diagnosis of electroclinical or subclinical seizures, their quantification before and after therapeutic changes and sometimes evaluation of severity of cortical dysfunction. The urgent nature of these indications implies the rapid set-up of the EEG system by qualified staff and possibility of maintaining the electrodes in place during long periods of time. Various techniques are available today for EEG monitoring, the interpretation of which depends on the contribution of an experienced physician.

[French guidelines on electroencephalogram].

Electroencephalography allows the functional analysis of electrical brain cortical activity and is the gold standard for analyzing electrophysiological processes involved in epilepsy but also in several other dysfunctions of the central nervous system. Morphological imaging yields complementary data, yet it cannot replace the essential functional analysis tool that is EEG. Furthermore, EEG has the great advantage of being non-invasive, easy to perform and allows control tests when follow-up is necessary, even at the patient's bedside.

A comparison of recording modalities of P300 event-related potentials (ERP) for brain-computer interface (BCI) paradigm.

Aims Of The Study: A brain-computer interface aims at restoring communication and control in severely disabled people by identification and classification of EEG features such as event-related potentials (ERPs). The aim of this study is to compare different modalities of EEG recording for extraction of ERPs. The first comparison evaluates the performance of six disc electrodes with that of the EMOTIV headset, while the second evaluates three different electrode types (disc, needle, and large squared electrode).

Obstructive sleep apnoea syndrome: comparison between polysomnography and portable sleep monitoring based on jaw recordings.

Introduction: Obstructive sleep apnoea syndrome (OSAS) constitutes a new major public health problem because of its several pathophysiologic consequences such as cognitive disorders, excessive daytime sleepiness with risks of traffic accidents, cardiovascular implications, and decrease of quality of life. The necessity of a gold-standard polysomnography to ensure an accurate diagnosis implies an expensive, technical and time-consuming examination. Thus, it seems logical to develop new systems so as to diagnose SAS and to make it possible to detect apnoeas/hypopnoeas easily during sleep even at home.

Exploration of small fibers for testing diabetic neuropathies.

Introduction: Electrophysiological exploration of neuropathies is a standard method of investigating the dysfunction of myelinated larger fibers (Aalpha, Abeta). However, this method cannot test dysfunctions in other fibers. To evaluate the smaller (Adelta) and unmyelinated fiber (C-fibers) lesions a quantitative method has been perfected: the study of the sensory thresholds (quantitative sensory testing: QST).

[Indications of electroencephalogram in the newborn].

The electroencephalogram (EEG), an easy-to-use and non invasive cerebral investigation, is a useful tool for diagnosis and early prognosis in newborn babies. In newborn full term babies manifesting abnormal clinical signs, EEG can point focal lesions or specific aetiology. EEG background activity and sleep organization have a high prognostic value.

Evoked potentials in spinal muscular atrophy.

Visual evoked potentials, brainstem evoked responses, and somatosensory evoked potentials were evaluated in 22 children with spinal muscular atrophy, types I and II. Eleven of the children had the severe form of spinal muscular atrophy (type I) and 11 children had the intermediate form (type II). The results of visual evoked potentials, brainstem evoked responses, and somatosensory evoked potentials were compared with those obtained in a control group.

Prognostic value of evoked potentials and sleep recordings in the prolonged comatose state of children. Preliminary data.

Objectives: Sleep recordings and evoked potentials (EPs) were used in five comatose children to evaluate their predictive value for outcome following a severe comatose state.

Methods And Subjects: The protocol included EEG, Brainstem Evoked Responses (BERs), Somatosensory Evoked Potentials (SEPs) and polysomnography. From 10 to 15 days post-coma (D10 to D15), EEG and clinical examinations were carried out every second day, then one day in four from 15 to 30 days post-coma (D15 to D30), and one day in seven from D30 to six months (M6).

Sleep disorders caused by brainstem tumor: case report.

Few studies concerning sleep disorders in brainstem lesions or tumors have been published. We report the case of a girl who was operated on for a brainstem tumor at the age of 4 years. In postsurgery, she had hemiparesis of the left side, swallowing difficulties, and severe apneas requiring a tracheotomy with nocturnal ventilation.

[Electrophysiologic study of 10 cases of Miller Fisher syndrome].

The association of ophthalmoplegia, ataxia and areflexia was described by Miller Fisher in 1956. It is postulated as a variant of the Guillain Barré syndrome. We report 10 Miller Fisher syndrome patients admitted in an intensive care unit between June 1990 and February 1999 who were selected according to clinical criteria of Ropper and Wijdicks.

Hot water epilepsy: a benign and unrecognized form.

Hot water epilepsy is a reflex epilepsy. Seizures are provoked by hot water, and result from the association of both cutaneous and heat stimuli. Described mainly in India and Japan, the condition seems to be rare in Europe, where it occurs in young children.

[EEG anomalies, sleep modification and developmental dysphasia ].

The relation between severe developmental dysphasia and paroxystical anomalies was analyzed in 52 children, mean age 9 years (group I) suffering from developmental dysphasia, compared to a control group of 20 children, mean age 8 years (group II). The children of both groups were selected excluding cases with a prior history of epilepsy or neurological disease, and all had a cerebral MRI in the normal range. In group I, using the Rapin and Allen classification, we distinguished: 14 children with a syntactic-lexical syndrome and 34 children with a syntactic-phonological syndrome.

[Hot water epilepsy: a benign and underestimated form].

Background: Hot water epilepsy belongs to the group of reflex epilepsies. Seizures are provoked by hot water, due to the association of both cutaneous and heat stimuli. Described mainly in India and Japan, it seems to be rare in Europe where it occurs in young children.

Sleep EEG and developmental dysphasia.

To determine the relation between developmental dysphasia and EEG anomalies during sleep, we compared 52 subjects with dysphasia with a control group of 20 children by using the ambulatory EEG method. Whereas 50% of the children with dysphasia experienced paroxysmal activity (PA), only two of the control group did. It is likely that paroxysmal abnormalities and language impairment are related to architectural dysplasia and neuron-migration disturbances.

Evaluation of early motor and sensory evoked potentials in cervical spinal cord injury.

To determine the efficacy of motor evoked potentials (MEP) and sensory evoked potentials (SEP) in the assessment of severe cervical injury, 17 subjects with severe cervical injury were studied. During the 1st week post-injury and post-surgical treatment, all subjects were submitted to electromyogram (EMG) recordings, dermatomal somatosensory evoked potentials (D.SEP), posterior tibial nerve somatosensory evoked potentials (PTN.

[Visual, auditory and somatosensory potentials in the diagnosis of vitamin B12 deficiency].

We describe visual, brain stem auditory, and somatosensory evoked (VEP, BAEP, SEP) in a 49-year old male patient presenting with subacute degeneration of the spinal cord due to vitamin B12 deficiency. Neurological signs included tetraplegia with a C4-C5 spinal cord compression that was unchanged after surgical decompression. Before treatment, the duration of the bilateral VEP was slightly increased, though their amplitude and morphology were not modified.

[Value of somatosensory evoked potentials in spinal surgery monitoring in children and adolescents: 110 cases].

This study shows the results obtained in 110 patients, children and adolescents by monitoring somatosensory evoked potentials during spinal surgery: Cotrel-Dubousset instrumention, surgical anterior correction by plating, spondylolisthesis and hemivertebra surgery. The recordings were made in preoperative, peroperative and postoperative period; the anaesthetic and electrophysiological conditions allowed us to obtain reliable recordings. In the peroperative period, the recordings were made: after induction of anesthesia and exposure of the spine, after instrumentation, after maximum traction and at the end of the operation.