Diagnostic Value of Bereitschaftspotential in People With Functional Seizures.

Purpose: Bereitschaftspotential (BP) or readiness potential in people with functional movement disorders can aid diagnostic workup. We evaluated the diagnostic value of BP as an interictal EEG marker in people with functional seizures (FS).

Methods: We recorded and analyzed BP interictal before intended movements in 17 adults with FS and 17 controls with alternative diagnoses.

Intraoperative mapping of epileptogenic foci and tumor infiltration in neuro-oncology patients with epilepsy.

Background: Epileptogenesis and glioma growth have a bidirectional relationship. We hypothesized people with gliomas can benefit from the removal of epileptic tissue and that tumor-related epileptic activity may signify tumor infiltration in peritumoral regions. We investigated whether intraoperative electrocorticography (ioECoG) could improve seizure outcomes in oncological glioma surgery, and vice versa, what epileptic activity (EA) tells about tumor infiltration.

Boosting brain-computer interfaces with functional electrical stimulation: potential applications in people with locked-in syndrome.

Individuals with a locked-in state live with severe whole-body paralysis that limits their ability to communicate with family and loved ones. Recent advances in brain-computer interface (BCI) technology have presented a potential alternative for these people to communicate by detecting neural activity associated with attempted hand or speech movements and translating the decoded intended movements to a control signal for a computer. A technique that could potentially enrich the communication capacity of BCIs is functional electrical stimulation (FES) of paralyzed limbs and face to restore body and facial movements of paralyzed individuals, allowing to add body language and facial expression to communication BCI utterances.

Association between lesion location and sensorimotor rhythms in stroke - a systematic review with narrative synthesis.

Background: Stroke causes alterations in the sensorimotor rhythms (SMRs) of the brain. However, little is known about the influence of lesion location on the SMRs. Understanding this relationship is relevant for the use of SMRs in assistive and rehabilitative therapies, such as Brain-Computer Interfaces (BCIs).

Resective Epilepsy Surgery, QUality of life and Economic evaluation (RESQUE): the change in quality of life after resective epilepsy surgery-protocol for a multicentre, prospective cohort study.

Introduction: Resective epilepsy surgery is often seen as a last resort when treating drug-resistant epilepsy. Positive results on quality of life (QoL) and economic benefits after surgery argue for a less restrictive attitude towards epilepsy surgery for drug-resistant epilepsy. QoL and economic benefits are country-dependent.

Towards predicting ECoG-BCI performance: assessing the potential of scalp-EEG.

. Implanted brain-computer interfaces (BCIs) employ neural signals to control a computer and may offer an alternative communication channel for people with locked-in syndrome (LIS). Promising results have been obtained using signals from the sensorimotor (SM) area.

Distinctive tics suppression network in Gilles de la Tourette syndrome distinguished from suppression of natural urges using multimodal imaging.

Background And Objectives: Gilles de la Tourette syndrome (GTS) is a neuropsychiatric disorder characterized by tics. A hallmark of GTS is the ability to voluntarily suppress tics. Our aim was to distinguish the neural circuits involved in the voluntary suppression of ocular tics in GTS patients from blink suppression in healthy subjects.

Specific EEG markers in POLG1 Alpers' syndrome.

Objective: To examine whether rhythmic high-amplitude delta with superimposed (poly)spikes (RHADS) in EEG allow a reliable early diagnosis of Alpers-Huttenlocher syndrome (AHS) and contribute to recognition of this disease.

Methods: EEGs of nine patients with DNA-proven AHS and fifty age-matched patients with status epilepticus were retrospectively examined by experts for the presence of RHADS and for accompanying clinical signs and high-frequency ripples. Reproducibility of RHADS identification was tested in a blinded panel.

Familial Cortical Myoclonic Tremor and Epilepsy, an Enigmatic Disorder: From Phenotypes to Pathophysiology and Genetics. A Systematic Review.

Background: Autosomal dominant familial cortical myoclonic tremor and epilepsy (FCMTE) is characterized by distal tremulous myoclonus, generalized seizures, and signs of cortical reflex myoclonus. FCMTE has been described in over 100 pedigrees worldwide, under several different names and acronyms. Pathological changes have been located in the cerebellum.

Safety and efficiency of medication withdrawal at home prior to long-term EEG video-monitoring.

Purpose: Long-term video-EEG monitoring (LTM) is frequently used for diagnostic purposes and in the workup of epilepsy surgery to determine the seizure onset zone. Different strategies are applied to provoke seizures during LTM, of which withdrawal of anti-epileptic drugs (AED) is most effective. Remarkably, there is no standardized manner of AED withdrawal.

Randomised controlled trial of escitalopram for cervical dystonia with dystonic jerks/tremor.

Objective: Trials for additional or alternative treatments for cervical dystonia (CD) are scarce since the introduction of botulinum neurotoxin (BoNT). We performed the first trial to investigate whether dystonic jerks/tremor in patients with CD respond to the selective serotonin reuptake inhibitor (SSRI) escitalopram.

Methods: In a randomised, double-blind, crossover trial, patients with CD received escitalopram and placebo for 6 weeks.

Clinical decision-making in functional and hyperkinetic movement disorders.

Objective: Functional or psychogenic movement disorders (FMD) present a diagnostic challenge. To diagnose FMD, clinicians must have experience with signs typical of FMD and distinguishing features from other hyperkinetic disorders. The aim of this study was to clarify the decision-making process of expert clinicians while diagnosing FMD, myoclonus, and tics.

Which patients with epilepsy are at risk for psychogenic nonepileptic seizures (PNES)? A multicenter case-control study.

Objective: We sought to examine the clinical and electrographic differences between patients with combined epileptic (ES) and psychogenic nonepileptic seizures (PNES) and age- and gender-matched patients with ES-only and PNES-only.

Methods: Data from 138 patients (105 women [77%]), including 46 with PNES/ES (39±12years), 46 with PNES-only (39±11years), and 46 with ES-only (39±11years), were compared using logistic regression analysis after adjusting for clustering effect.

Results: In the cohort with PNES/ES, ES antedated PNES in 28 patients (70%) and occurred simultaneously in 11 (27.

RELN rare variants in myoclonus-dystonia.

Background: Myoclonus-dystonia (M-D) is a hyperkinetic movement disorder with predominant myoclonic symptoms combined with dystonia of the upper part of the body. A proportion of M-D cases are caused by mutations in the epsilon-sarcoglycan gene. In remaining M-D patients, no genetic factor has been established, indicating genetic heterogeneity.

Propriospinal myoclonus: clinical reappraisal and review of literature.

Objective: Propriospinal myoclonus (PSM) is a rare disorder with repetitive, usually flexor arrhythmic brief jerks of the trunk, hips, and knees in a fixed pattern. It has a presumed generation in the spinal cord and diagnosis depends on characteristic features at polymyography. Recently, a historical paradigm shift took place as PSM has been reported to be a functional (or psychogenic) movement disorder (FMD) in most patients.

Jerky periods: myoclonus occurring solely during menses.

Background: In this case report, we describe an unusual case of a patient with myoclonus only occurring during menses.

Case Report: A 41-year-old female, known to have neurological sequelae after a car accident 1 year earlier, presented with myoclonic movements of the right arm and hand only during menses. Brain magnetic resonance imaging is compatible with head trauma.

Functional MRI study of response inhibition in myoclonus dystonia.

Background: Myoclonus-dystonia (MD) is a movement disorder characterized by myoclonic jerks, dystonic postures and psychiatric co-morbidity. A mutation in the DYT11 gene underlies half of MD cases. We hypothesize that MD results from a dysfunctional basal ganglia network causing insufficient inhibitory motor control.

Neuropsychological profile of psychogenic jerky movement disorders: importance of evaluating non-credible cognitive performance and psychopathology.

Background: Psychogenic movement disorders are disorders of movements that cannot be explained by a known neurological disorder and are assumed to be associated with psychiatric symptoms such as depression and anxiety.

Objective: To examine the neuropsychological profile of patients with psychogenic movement disorders.

Methods: We examined cognitive functioning using neuropsychological tests in 26 patients with clinically established psychogenic jerky movement disorders (PMD).

The eye of the beholder: inter-rater agreement among experts on psychogenic jerky movement disorders.

Objective: The current criteria for conversion disorder in the Diagnostic and Statistical Manual of Mental Disorders rely on the assumption that neurological disorders can be distinguished from conversion disorders through clinical assessment. This study aims to assess inter-rater agreement among clinicians with experience in the diagnosis of various hyperkinetic jerky movements, including psychogenic jerks.

Methods: 60 patients with psychogenic jerks, myoclonus or tics were rated by international experts using a standardised survey resembling daily clinical practice.

The bereitschaftspotential in jerky movement disorders.

Objective: To assess the diagnostic value of the bereitschaftspotential (BP) in jerky movement disorders.

Methods: A cross-sectional case series of 48 patients with psychogenic jerks, Gilles de la Tourette syndrome (GTS) or myoclonus was investigated. We measured the BP prior to the spontaneous jerk and voluntary wrist extension.

A new familial syndrome with dystonia and lower limb action myoclonus.

Background: Myoclonus-dystonia (M-D) is genetic and clinically heterogeneous. Identification and description of rare M-D syndromes may contribute to gene identification.

Results: Here, we describe a new, autosomal dominant M-D syndrome in a 3-generation pedigree showing anticipation.

Axial jerks: a clinical spectrum ranging from propriospinal to psychogenic myoclonus.

Propriospinal myoclonus (PSM) is a rare disorder with repetitive flexor, arrhythmic jerks of the trunk, hips and knees. Its generation is presumed to relay in the spinal cord. We report a case series of 35 consecutive patients with jerks of the trunk referred as possible PSM to a tertiary referral center for movement disorders.

Decreased cortical inhibition and yet cerebellar pathology in 'familial cortical myoclonic tremor with epilepsy'.

Cortical hyperexcitability is a feature of "familial cortical myoclonic tremor with epilepsy" (FCMTE). However, neuropathological investigations in a single FCMTE patient showed isolated cerebellar pathology. Pathological investigations in a second FCMTE patient, reported here, confirmed cerebellar Purkinje cell degeneration and a normal sensorimotor cortex.