Epileptic encephalopathies in early infancy with suppression-burst.

Early infantile epileptic encephalopathy with suppression-burst, or Ohtahara syndrome (OS), and early myoclonic encephalopathy (EME) are epileptic encephalopathies with onset of frequent seizures in the neonatal and early infancy period and with a characteristic EEG pattern, namely, suppression-burst, in which higher-voltage bursts of slow waves mixed with multifocal spikes alternate with isoelectric suppression phase. Their nosologic independence is now widely accepted, although some controversy initially occurred because of their common characteristics such as age of onset, EEG features, seizure intractability, and poor prognosis. Major differences between the two syndromes include (1) tonic spasms in OS versus partial seizures and erratic myoclonias in EME, (2) continuous suppression-burst pattern in both waking and sleeping states in OS versus this EEG pattern almost limited to sleep in EME, and (3) static structural brain damage in OS versus genetic or metabolic disorders in EME. The most important differentiating point is their evolutional pattern with age, which may reflect their pathophysiologic difference. Ohtahara syndrome evolves to West syndrome and further to Lennox-Gastaut syndrome with age, but EME demonstrates no unique evolution; namely, it continues as such for a long time or changes into partial epilepsy or severe epilepsy with multiple independent spike foci.     

Epileptic encephalopathies: Optimizing seizure control and developmental outcome

Cognitive and developmental outcomes in patients with epileptic encephalopathy are hypothesized to result from an interplay between the underlying epileptic pathologic substrate and the acquired consequences of frequent and repetitive seizures and epileptiform discharges that often straddle the interictal and ictal boundaries. This article briefly reviews the evidence related to this assumption, presents critical questions that need to be answered to clarify this relationship, and advances a set of concrete steps that may help improve developmental patient outcomes.     

Epileptic encephalopathies (including severe epilepsy syndromes)

Epileptic encephalopathies represent a group of devastating epileptic disorders that appear early in life and are characterized by pharmacoresistant generalized or focal seizures, persistent severe electroencephalography (EEG) abnormalities, and cognitive dysfunction or decline. The ictal and interictal epileptic discharges are age-specific and are the main etiologic factors causing cognitive deterioration. This is most obvious in the idiopathic group. In the symptomatic group, the most common causes are structural, congenital, or acquired and rarely some metabolic disorders. In certain cases, clinical and EEG abnormalities persist and may evolve from one type to another as the child grows older. Various factors trigger and sustain the underlying pathophysiologic process and the ongoing epileptic and epileptiform activity during the most critical periods of brain maturation, perpetuating their deleterious effect on the brain. Immune-mediated mechanisms may have a role, suggested by certain encephalopathies responding to immune-modulating treatments and by the finding of various autoimmune antibodies. The chance of a better cognitive outcome improves with early diagnosis and treatment that is appropriate and effective. Current antiepileptic drugs are, in general, not effective: we urgently need new trials in this very special epileptic category. This article briefly reviews the most common epileptic encephalopathies and analyzes the most important clinical issues.     

Journalists and trauma: a brief overview

In the past decade, journalism experts realized what those in the fields of emergency response and management have known for much longer: That journalists could be psychologically affected by the traumatic events they covered. Although a fledging field of study, groups such as the Dart Center for Journalism and Trauma, based at the University of Washington, in Seattle, WA, have dedicated themselves to advocating the ethical and thorough reporting of trauma, educating working journalists about trauma and serving as a forum for journalists to discuss topics related to covering traumatic events. The three major studies conducted on the subject--focusing on print journalists, photojournalists, and war journalists--have discovered that journalists can suffer sleeplessness, flashbacks, and in most extreme cases, posttraumatic stress disorder. According to Dr. Roger Simpson, director of the Dart Center, there is much work still to be done on the subject.     

Neuromuscular junction channelopathies: a brief overview

The neuromuscular junction lacks the protection of the blood-nerve barrier and is vulnerable to antibody-mediated disorders. In myasthenia gravis (MG), 85% of patients have IgG antibodies to acetylcholine receptors (AChRs). About half the remaining patients have IgG antibodies to Muscle Specific Kinase (MuSK), an AChR-associated transmembrane post-synaptic protein concerned in AChR aggregation. Bulbar weakness is typically predominant in this form of MG, and females are more often affected. The Lambert-Eaton Myasthenic Syndrome (LEMS) can occur in a paraneoplastic form (P-LEMS) usually with small cell lung cancer, or in a non-paraneoplastic form (NP-LEMS). In both, IgG antibodies to nerve terminal voltage-gated calcium channels (VGCCs), detectable in over 90% of patients, lead to VGCC loss and impaired quantal release of transmitter and may be implicated in the occasionally associated cerebellar ataxia. Neuromyotonia (NMT) and Cramp-Fasciculation syndrome (C-FS) are manifestations of peripheral nerve hyperexcitability and share some clinical and electromyographic features. Antibodies to voltage-gated potassium channels (VGKCs) are present in about 40% of NMT patients, but less frequently in C-FS, and appear to cause loss of functional VGKCs. They may also be implicated in the Maladie de Morvan and limbic encephalitis that can associate with NMT: The antibodies described here provide valuable aids to diagnosis and management. The Congenital Myasthenic Syndromes are a group of genetically determined heterogeneous disorders, usually recessively inherited. The commonest mutation sites appear to be the acetylcholine receptor epsilon-subunit and rapsyn.     

On the neuronal basis for multisensory convergence: a brief overview

For multisensory stimulation to effect perceptual and behavioral responses, information from the different sensory systems must converge on individual neurons. A great deal is already known regarding processing within the separate sensory systems, as well as about many of the integrative and perceptual/behavioral effects of multisensory processing. However, virtually nothing is known about the functional architecture that underlies multisensory convergence even though it is an integral step to this processing sequence. This paper seeks to summarize the findings pertinent to multisensory convergence, and to initiate the identification of specific convergence patterns that may underlie different multisensory perceptual and behavioral effects.     

Challenges in molecular imaging of Parkinson's disease: a brief overview

Positron emission tomography (PET) is a sensitive and specific non-invasive molecular imaging technique providing quantitative images and data on biochemical pathways, molecular interactions, drug pharmacokinetics and pharmacodynamics. The PET measurement of regional biochemical functions requires a rational design of the radiolabelled probes employed. When choosing a drug to radiolabel there are many considerations, the most important being the potential of the drug to provide the information that is required in vivo. For example, when developing a radiolabelled probe for measuring the regional concentration of a neurotransmitter receptor the affinity and selectivity of the drug need to be considered. Other factors which influence a decision about which drug to radiolabel include the ability of the drug to reach its target, drug lipophilicity and metabolism, toxicity and the amenability to radiolabelling. PET has been applied in studies of Parkinson's disease (PD) and has provided important information on disease on-set and progression. This brief overview aims to outline the challenges and capabilities of PET and the more commonly used molecular probes in PD studies.     

Female sexual offenders in the educational system: a brief overview

Female sexual offenders comprise the minority of sexual offenders in the criminal justice system. However, empirical research reveals that sexual offenses against adolescents by females are a bigger problem than previously thought, particularly in the educational system. The authors review some of the data in the criminal justice system as well as in empirical research studies about female sexual offenders, with a specific focus on females who commit sexual crimes against students who are minors.     

Indolealkylamine and phenalkylamine hallucinogens: A brief overview

Various indolealkylamine and phenalkylamine derivatives are hallucinogenic in man and/or are behaviorally active in animals. This overview is divided into two parts. The first part attempts to bring together information concerning the activity of indolealkylamines (i.e., tryptamines, α-methyltryptamines, N,N-dimethyltryptamines, N-alkyltryptamines, lysergic acid derivatives and β-carbolines) and phenalkylamines (i.e., phenethylamines, phenylisopropylamines) along with major key references, and with emphasis on those agents not recently reviewed. The latter portion of this overview describes some of the work being conducted in our laboratories in an effort to elucidate the role of the neurotransmitter serotonin in the mechanism of action of various indolealkylamine and phenalkylamine hallucinogens.     

Indolealkylamine and phenalkylamine hallucinogens: A brief overview

Various indolealkylamine and phenalkylamine derivatives are hallucinogenic in man and/or are behaviorally active in animals. This overview is divided into two parts. The first part attempts to bring together information concerning the activity of indolealkylamines (i.e., tryptamines, α-methyltryptamines, N,N-dimethyltryptamines, N-alkyltryptamines, lysergic acid derivatives and β-carbolines) and phenalkylamines (i.e., phenethylamines, phenylisopropylamines) along with major key references, and with emphasis on those agents not recently reviewed. The latter portion of this overview describes some of the work being conducted in our laboratories in an effort to elucidate the role of the neurotransmitter serotonin in the mechanism of action of various indolealkylamine and phenalkylamine hallucinogens.     

Miller Fisher syndrome: brief overview and update with a focus on electrophysiological findings

Miller Fisher syndrome (MFS), a variant of the Guillain–Barré syndrome (GBS), is characterized by ophthalmoplegia, ataxia, and areflexia. The annual incidence is around one patient per one million population. The antiganglioside anti‐GQ1b IgG antibody has a role in the pathogenesis of the syndrome, especially of ophthalmoplegia. The presence of this antibody in the serum can be identified in over 80% of the patients, peaking in the first week, whereas albuminocytological dissociation in the cerebrospinal fluid (CSF) appears later. The most consistent electrophysiological findings in MFS are reduced sensory nerve action potentials and absent H reflexes. More variability is seen with F waves and various investigations involving cranial structures. Although there are usually no abnormalities in MFS by routine neuroimaging, in a few cases, contrast enhancement of nerve roots and signs of central nervous system involvement were described supporting the hypothesis of an anti‐GQ1b‐syndrome, a continuum involving GBS, MFS, and Bickerstaff’s brainstem encephalitis. Owing to the lack of randomized trials, treatments used for GBS (intravenous immunoglobulin and plasmapheresis) are usually applied, although from retrospective analyses, the outcome was similar between treated and untreated subjects. The outcome of MFS is usually good with case fatality of < 5%. In the few autopsy cases, macroscopic abnormalities were generally not seen in the nervous system. Microscopic examination of the peripheral nervous system (including cranial nerves) showed segmental demyelination with minimal perivascular infiltration with normal spinal cord and brain stem.     

A brief overview of Social Role Valorization

Social roles dominate people's lives, and people largely perceive themselves and each other in terms of their roles. The value people attribute to various social roles tends to decisively shape their behavior toward persons whom they see in valued or devalued roles. Those in valued roles tend to be treated well and those in devalued roles, ill. The most current and recently revised version of the Social Role Valorization (SRV) schema is presented in condensed form, showing how social role theory can be recruited for designing very powerful practical measures to pursue valued roles for mentally retarded and other persons or classes at risk of social or even societal devaluation, to upgrade the perceived value of the roles such persons already occupy, and/or to extricate such persons from devalued roles.     

The age-dependent epileptic encephalopathies.

The most severe epilepsies that affect neonates, infants, and children include Ohtahara, West, and Lennox-Gastaut syndromes. These three syndromes display considerable similarities and transitional features in their clinical symptoms, seizure phenomena, and electroencephalographic abnormalities. This review examines the similarities and differences between these three syndromes and the other severe epilepsies of infancy and childhood, and discusses the hypothesis that the three disorders form a continuum of epileptic encephalopathies that have a predictable age-related evolution.