A description of variant transthyretin amyloidosis (ATTRv) stage 1 patients and asymptomatic carriers in Spain: the EMPATIa study.

Background: Variant transthyretin amyloidosis (ATTRv) is a rare multisystemic disorder caused by mutations in the transthyretin (TTR) gene. The aim of the present work was to describe the clinical profile of asymptomatic carriers (AC) and Coutinho stage 1 ATTRv patients in Spain.

Methods: National, multicentre, cross-sectional study that included 86 AC and 19 patients diagnosed in the previous 12 months to enrolment.

Psychiatric Comorbidity and Emotional Dysregulation in Chronic Tension-Type Headache: A Case-Control Study.

Background: Chronic tension-type headache (CTTH) is frequently associated with a psychiatric comorbidity of depression and anxiety. Most studies focus their attention on this association, and only few link CTTH with psycho-affective emotional regulation disorders.

Objective: To evaluate the association of CTTH with anxiety, depression, positive and negative affectivity, and emotional management in CTTH patients with neither a previous diagnosis of psychiatric disorder nor use of psychoactive drugs or abuse of analgesics.

Sensory Thresholds and Peripheral Nerve Responses in Chronic Tension-Type Headache and Neuropsychological Correlation.

Chronic tension-type headache (CTTH) is a common disease with no fully defined pathophysiological processes. We designed a study to value electrophysiological responses in these patients and their correlation with possible psychopathological manifestations in order to deepen understanding of central and peripheral mechanisms of CTTH. In 40 patients with CTTH and 40 healthy controls, we used electrical stimulation to determine sensory threshold (SPT) and pain perception threshold (PPT) and the characteristics of the electrophysiological sensory nerve action potential (SNAP): initial sensory response (ISR) and supramaximal response (SMR).

Forced normalization: case series from a Spanish epilepsy unit.

Introduction: Forced normalization is a clinical situation of singular relationship betweenepilepsy and psychosis, in which a patient shows behavioural and psychiatric symptoms coinciding with a reduction or termination of seizures and a total or partial normalization ofelectroencephalogram. Behavioural symptoms (frequently psychosis) or mood disturbances may appear. More information about this phenomenon, risk factors and prognostic variables is needed.

Epidemiological study of mortality in epilepsy in a Spanish population.

Purpose: Studies concerning mortality in epilepsy have been performed primarily in Northern-Central Europe and US. The aim of this study was to provide information about mortality in people with epilepsy in Southern European countries.

Method: We studied a Spanish prevalence and incidence cohort of 2309 patients aged ≥14 years with epilepsy who were treated in an outpatient epilepsy clinic between 2000 and 2013.

[Control of epilepsy in adult patients with tuberous sclerosis].

Introduction: Tuberous sclerosis is a genetic disease, the main manifestations of which are the formation of neuroectodermal tumours, which are very often associated to secondary epilepsy.

Aim: To describe the epileptic profile, control, frequency of seizures and effectiveness of treatment in adult patients with tuberous sclerosis.

Patients And Methods: The study was descriptive and included adult patients (over 14 years of age) with a confirmed diagnosis of tuberous sclerosis.

[Healthcare management of an epilepsy clinic: factors involved in the demand for health care and clinical situation of patients].

Introduction: Epilepsy is a chronic illness that requires a long-term periodic follow-up of the patient and this means that as time goes by the number of patients attended increases, with the ensuing added cost for the healthcare system.

Aim: To determine the factors involved in the time until an epileptic patient's next visit.

Patients And Methods: Our sample consisted of a selection of patients who visited the epilepsy clinic at our hospital consecutively during one year.

Prevalence and clinical characteristics of epilepsy in the South of Spain.

Purpose: Epilepsy is a common neurological disorder found in all societies. There are extensive epidemiologic studies of different European areas. However, not much information about the South-West of Europe exists.

[Psychogenic pathology in the neurology unit. A review of the hospital admissions over the last 10 years in a third-level service].

Introduction: Psychogenic disorders are frequently seen in neurological practice and sometimes make it necessary to hospitalise the patient in order to rule out a potentially severe organic pathology.

Aim: To determine the profile of patients with a discharge diagnosis of psychogenic disorder that are admitted to the neurology unit of a Spanish hospital.

Patients And Methods: The neurology unit discharge abstracts from the last 10 years were reviewed and the patients who were diagnosed with 'non-organicity' were selected; a total of 28 demographic, epidemiological and clinical variables were also collected.

[Does Jeavons syndrome exist? A report of a series of 10 cases].

Introduction: Jeavons syndrome (JS) or eyelid myoclonia (EM) with or without absences is an epileptic syndrome characterised by EM on closing the eyes, in bright environments, which coincides on the electroencephalogram with brief generalised polyspike and polyspike-wave discharges at 3-6 Hz. Photoparoxysmal response is associated in photostimulation. The ILAE recognises EM as a special type of myoclonic seizures, yet it still does not include JS as a separate condition in its classification of epileptic syndromes.

Calretinin interneurons are early targets of extracellular amyloid-beta pathology in PS1/AbetaPP Alzheimer mice hippocampus.

Specific neuronal networks are preferentially affected in the early stages of Alzheimer's disease (AD). The distinct subpopulations of hippocampal inhibitory GABAergic system have been shown to display differential vulnerability to neurodegeneration in AD. We have previously reported a substantial loss of SOM/NPY interneurons, whereas those expressing parvalbumin were unaltered, in the hippocampus of 6 month-old PS1/AbetaPP transgenic mice.

Extracellular amyloid-beta and cytotoxic glial activation induce significant entorhinal neuron loss in young PS1(M146L)/APP(751SL) mice.

Here we demonstrated that extracellular, not intracellular, amyloid-beta (Abeta) and the associated cytotoxic glial neuroinflammatory response are major contributors to early neuronal loss in a PS1xAPP model. A significant loss of principal (27%) and SOM/NPY (56-46%) neurons was found in the entorhinal cortex at 6 months of age. Loss of principal cells occurred selectively in deep layers (primarily layer V) whereas SOM/NPY cell loss was evenly distributed along the cortical column.