Facial pain as first manifestation of anti-Hu paraneoplastic syndrome.

The diagnosis of anti-Hu-associated encephalomyelitis/sensory neuropathy may be particularly difficult when cranial nerve involvement represents the first clinical manifestation of the disease. We report a case of a patient who presented with facial pain as the first manifestation of an anti-Hu paraneoplastic syndrome, which needs a rapid detection and treatment of the underlying tumour. We suggest that paraneoplastic neuropathy should be considered during the management of trigeminal neuropathic pain, especially when brain imagery is normal.

Brain responses to success and failure: Direct recordings from human cerebral cortex.

Evaluating the outcome of our own actions is a fundamental process by which we adapt our behavior in our interaction with the external world. fMRI and electrophysiological studies in monkeys have found feedback-specific responses in several brain regions, unveiling facets of a large-scale network predominantly distributed in the frontal lobes. However, a consensus has yet to be reached regarding the exact contribution of each region.

Pregabalin in partial seizures: a pragmatic 21-week, open-label study (PREPS).

Background And Purpose: Pregabalin has demonstrated efficacy in controlled trials as adjunctive treatment in patients with refractory seizures.

Methods: This open-label, 21-week study in adults with at least two partial seizures in the last 2 months, who were inadequately controlled with one to three antiepileptic drugs, evaluated pregabalin 150-600 mg/day (dosed twice daily). The study comprised a prospective or retrospective 8-week baseline phase, and 9-week dose optimization and 12-week maintenance periods.

Sudden unexpected death in epilepsy (SUDEP): update and reflections.

SUDEP remains a significant clinical problem, which, in the authors' opinion, is to some extent potentially preventable. Research needs to focus on interventions that may alter risk. Some wide-ranging suggestions for future developments that in time can translate to clinical practice have been presented.

Tumor necrosis factor-alpha participates in apoptosis in the limbic system after myocardial infarction.

This study was designed to determine the role of tumor necrosis factor-alpha (TNFalpha) in apoptosis observed in the myocardium and limbic system after myocardial ischemia. PEG sTNFRI, a recombinant, human, soluble p55 Type 1 TNF receptor (3 mg/kg) or vehicle (saline) was administered s.c.

Association between triallelic polymorphism of the serotonin transporter and [18F]MPPF binding potential at 5-HT1A receptors in healthy subjects.

Previous [(11)C]WAY100-635 PET studies have demonstrated that the short (S) and long (L) alleles of the serotonin transporter gene-linked polymorphic region (5-HTTLPR) were associated with distinct patterns of 5-HT(1A) receptor distribution in human. However, these studies reported discordant findings and did not take into account the recent description of two functional variants of the L allele (L(A)/L(G)). To further explore this issue, we investigated the triallelic functional polymorphism of the 5-HTTLPR in 38 healthy volunteers who underwent a [(18)F]MPPF PET study of 5-HT1A receptors.

Pregabalin for the management of partial epilepsy.

Pregabalin is one of the latest antiepileptic drugs introduced for the treatment of partial epilepsy. Its efficacy and safety as adjunctive therapy in refractory partial epilepsy have been established in four double-blind placebo-controlled trials (n = 1396) and 4 long-term open-label studies (n = 1480). In 3 fixed-dose trials, the proportion of patients with a >/=50% reduction in seizure frequency across the effective dose-range (150-600 mg/day) ranged between 14% and 51%, with a clear dose-response relationship.

Erythropoietin receptor expression is concordant with erythropoietin but not with common beta chain expression in the rat brain throughout the life span.

Brain effects of erythropoietin (Epo) are proposed to involve a heteromeric receptor comprising the classical Epo receptor (Epo-R) and the common beta chain (betac). However, data documenting the pattern of betac gene expression in the healthy brain, in comparison with that of the Epo-R gene, are still lacking. The present study is the first to investigate at the same time betac, Epo-R, and Epo gene expression within different rat brain areas throughout the life span, from neonatal to elderly stages, using quantitative RT-PCR for transcripts.

A novel three base-pair LGI1 deletion leading to loss of function in a family with autosomal dominant lateral temporal epilepsy and migraine-like episodes.

Mutations in LGI1 have been reported in several families with autosomal dominant lateral temporal epilepsy. In a family in which three patients also experienced migraine-like episodes we found a novel three base-pair deletion (c.377_379delACA), resulting in the deletion of an asparagine residue in the second leucine-rich repeat.

Once-daily lamotrigine extended release for epilepsy management.

In order to improve patient compliance and decrease toxicity of antiepileptic drugs (AEDs), the recent strategy for epilepsy management has been to expand the use of extended-release (XR) formulations. Lamotrigine (LTG), an AED with broad efficacy, is available as a twice-daily immediate-release formulation. A once-daily XR formulation of LTG (LTG-XR) is currently being developed.

[Excess mortality and sudden unexpected death in epilepsy].

Patients with epilepsy have a two to three fold increased risk of death as compared to the age-matched general population. This increased risk of death primarily affects young adults with drug resistant epilepsy. Sudden unexpected death in epilepsy (SUDEP) is one of the main cause of mortality in that population, and appears to be the direct consequence of a seizure.

SEEG-guided thermocoagulations: a palliative treatment of nonoperable partial epilepsies.

Objective: Intracranial stereotactic EEG recordings (SEEG) in presurgical epilepsy assessment are currently carried out in our department. The SEEG method generally used for exploration can also be used to perform radiofrequency thermocoagulations (RFTC) of the epileptic foci. To assess the indications of the RFTC procedure in the therapeutic arsenal of drug-resistant epilepsies, we report the results obtained in 41 patients to whom RFTC was proposed as a first therapeutic step before surgery or as a palliative treatment when surgery was not possible.

Foreign language ictal speech automatisms in nondominant temporal lobe epilepsy.

Background: Foreign language ictal speech automatism (FLISA) is a rare ictal sign that has been hitherto reported in five unilingual patients, all right handed men with right temporal lobe epilepsy (TLE), only one of whom has benefited from an intracerebral EEG investigation.

Methods: We report three unilingual French patients who consistently presented English spoken ictal speech automatisms and were investigated with intracerebral EEG recordings.

Results: All three patients were right-handed men with nondominant TLE originating in the right amygdala.

Sudden unexpected death in epilepsy: current knowledge and future directions.

Although largely neglected in earlier literature, sudden unexpected death in epilepsy (SUDEP) is the most important epilepsy-related mode of death, and is the leading cause of death in people with chronic uncontrolled epilepsy. Research during the past two to three decades has shown that incidence varies substantially depending on the epilepsy population studied, ranging from 0.09 per 1000 patient-years in newly diagnosed patients to 9 per 1000 patient-years in candidates for epilepsy surgery.

PET imaging of brain 5-HT1A receptors in the preoperative evaluation of temporal lobe epilepsy.

[(18)F]MPPF PET has previously been used to identify the epileptic lobe in temporal lobe epilepsy (TLE) patients at the group level. This study aims to validate the visual analysis of [(18)F]MPPF PET in the assessment of individual TLE patients for their suitability to undergo temporal lobe resection. Forty-two patients suffering from TLE and 18 control subjects matched for age and gender were prospectively enrolled for [(18)F]MPPF PET.

Comorbidity between temporal lobe epilepsy and depression: a [18F]MPPF PET study.

Brain and brainstem changes of serotoninergic 5-hydroxytryptophan (5-HT)(1A) receptor density have been reported in patients with major depressive disorder as well as in patients with temporal lobe epilepsy (TLE), using PET and the selective antagonist radiotracers [(11)C]WAY-100635 or [(18)F]FC-WAY. We used a distinct 5-HT(1A) antagonist, [(18)F]MPPF, whose binding potential depends on both receptor density and extracellular serotonin concentration, in 24 patients with drug-resistant TLE and MRI evidence of hippocampal sclerosis but without prior antidepressant exposure. Their Beck Depression Inventory (BDI-2) score ranged from 0 to 34, with nine patients having a score >11.

Olfactory hypersensitivity in migraineurs: a H(2)(15)O-PET study.

Olfactory hypersensitivity (OHS) may occur during migraine attacks and seems to be very specific to this form of headache. OHS is also observed during migraine-free periods and is associated with the presence of odour-triggered attacks. Yet the pathophysiology of OHS remains unknown.

Interictal brain 5-HT1A receptors binding in migraine without aura: a 18F-MPPF-PET study.

In this study we aimed to assess the brain distribution of 5-HT(1A) receptors in migraine patients without aura. Ten female migraine patients and 24 female healthy volunteers underwent magnetic resonance imaging and positron emission tomography using a radioligand antagonist of 5-HT(1A) receptors [4-(2'-methoxyphenyl)-1-[2'-(N-2-pirydynyl)-p-fluorobenzamido]-ethylpiperazine ((18)F-MPPF)]. A simplified reference tissue model was used to generate parametric images of 5-HT(1A) receptor binding potential (BP) values.

Greater response to placebo in children than in adults: a systematic review and meta-analysis in drug-resistant partial epilepsy.

Background: Despite guidelines establishing the need to perform comprehensive paediatric drug development programs, pivotal trials in children with epilepsy have been completed mostly in Phase IV as a postapproval replication of adult data. However, it has been shown that the treatment response in children can differ from that in adults. It has not been investigated whether differences in drug effect between adults and children might occur in the treatment of drug-resistant partial epilepsy, although such differences may have a substantial impact on the design and results of paediatric randomised controlled trials (RCTs).

Magnetic resonance imaging in clinical trials.

Purpose Of Review: The aim of this article is to review the latest clinical trials in neurological diseases where magnetic resonance imaging was used to assess treatment outcome.

Recent Findings: The unique sensitivity of magnetic resonance imaging for detecting disorders in the brain has made it an attractive noninvasive tool for assessing treatment efficacy in several diseases. Volumetric and functional magnetic resonance imaging have proved to represent robust biomarkers for the evaluation of anti-Alzheimer treatments, and have demonstrated a significant impact of cholinesterase inhibitors.

Paroxysmal exercise-induced dyskinesia and epilepsy is due to mutations in SLC2A1, encoding the glucose transporter GLUT1.

Paroxysmal exercise-induced dyskinesia (PED) can occur in isolation or in association with epilepsy, but the genetic causes and pathophysiological mechanisms are still poorly understood. We performed a clinical evaluation and genetic analysis in a five-generation family with co-occurrence of PED and epilepsy (n = 39), suggesting that this combination represents a clinical entity. Based on a whole genome linkage analysis we screened SLC2A1, encoding the glucose transporter of the blood-brain-barrier, GLUT1 and identified heterozygous missense and frameshift mutations segregating in this and three other nuclear families with a similar phenotype.

Analysis of clinical patterns and underlying epileptogenic zones of hypermotor seizures.

Background: Hypermotor seizures (HMS) are characterized by complex movements involving the proximal segment of the limbs and trunk. Although they are primarily reported in mesial frontal or orbitofrontal epilepsies, they have also been described in patients with temporal or insular epilepsies, questioning the localizing value of HMS in patients contemplating epilepsy surgery. Furthermore, HMS can include different forms of HM behaviors.