Neurophysiology of Juvenile and Progressive Myoclonic Epilepsy.

SUMMARY: Myoclonus can be epileptic or nonepileptic. Epileptic myoclonus has been defined in clinical, neurophysiological, and neuroanatomical terms. Juvenile myoclonic epilepsy (JME) is typically considered to be an adolescent-onset idiopathic generalized epilepsy with a combination of myoclonic, generalized tonic-clonic, and absence seizures and normal cognitive status that responds well to anti-seizure medications but requires lifelong treatment. EEG shows generalized epileptiform discharges and photosensitivity. Recent observations indicate that the clinical picture of JME is heterogeneous and a number of neuropsychological and imaging studies have shown structural and functional abnormalities in the frontal lobes and thalamus. Advances in neurophysiology and imaging suggest that JME may not be a truly generalized epilepsy, in that restricted cortical and subcortical networks appear to be involved rather than the entire brain. Some patients with JME may be refractory to anti-seizure medications and attempts have been made to identify neurophysiological biomarkers predicting resistance. Progressive myoclonic epilepsy is a syndrome with multiple specific causes. It is distinct from JME because of the occurrence of progressive neurologic dysfunction in addition to myoclonus and generalized tonic-clonic seizures but may sometimes be difficult to distinguish from JME or misdiagnosed as drug-resistant JME. This article provides an overview of progressive myoclonic epilepsy and focuses on the clinical and neurophysiological findings in the two most commonly recognized forms of progressive myoclonic epilepsy-Unverricht-Lundborg disease (EPM1) and Lafora disease (EPM2). A variety of neurophysiological tests can be used to distinguish between JME and progressive myoclonic epilepsy and between EPM1 and EPM2.

Visually sensitive seizures: An updated review by the Epilepsy Foundation.

Light flashes, patterns, or color changes can provoke seizures in up to 1 in 4000 persons. Prevalence may be higher because of selection bias. The Epilepsy Foundation reviewed light-induced seizures in 2005. Since then, images on social media, virtual reality, three-dimensional (3D) movies, and the Internet have proliferated. Hundreds of studies have explored the mechanisms and presentations of photosensitive seizures, justifying an updated review. This literature summary derives from a nonsystematic literature review via PubMed using the terms "photosensitive" and "epilepsy." The photoparoxysmal response (PPR) is an electroencephalography (EEG) phenomenon, and photosensitive seizures (PS) are seizures provoked by visual stimulation. Photosensitivity is more common in the young and in specific forms of generalized epilepsy. PS can coexist with spontaneous seizures. PS are hereditable and linked to recently identified genes. Brain imaging usually is normal, but special studies imaging white matter tracts demonstrate abnormal connectivity. Occipital cortex and connected regions are hyperexcitable in subjects with light-provoked seizures. Mechanisms remain unclear. Video games, social media clips, occasional movies, and natural stimuli can provoke PS. Virtual reality and 3D images so far appear benign unless they contain specific provocative content, for example, flashes. Images with flashes brighter than 20 candelas/m2 at 3-60 (particularly 15-20) Hz occupying at least 10 to 25% of the visual field are a risk, as are red color flashes or oscillating stripes. Equipment to assay for these characteristics is probably underutilized. Prevention of seizures includes avoiding provocative stimuli, covering one eye, wearing dark glasses, sitting at least two meters from screens, reducing contrast, and taking certain antiseizure drugs. Measurement of PPR suppression in a photosensitivity model can screen putative antiseizure drugs. Some countries regulate media to reduce risk. Visually-induced seizures remain significant public health hazards so they warrant ongoing scientific and regulatory efforts and public education.

Encephalopathy after unintentional intrathecal gadolinium: A letter to the editor.

Objective: Raise awareness of gadolinium encephalopathy, a rare cause of neurological symptoms. Setting: An L5-S1 interlaminar epidural steroid injection (IL-ESI) was performed with a gadolinium-based contrast agent (GBCA) due to the patient's history of allergic reaction to iodine-based contrast agents. Discussion: Several hours after administration of GBCA, the patient had nausea and vomiting with altered mental status. Patient was treated with dexamethasone IV, and was discharged on day 2. Patient had no residual deficits at follow-up two weeks later. Current literature shows that caution should be used to prevent inadvertent intrathecal GBCA, and doses >2.0 mmols are associated with serious adverse effects, including death. Conclusions: Intrathecal administration of GBCAs should be limited to less than 0.5 mmol. If adverse effects are experienced, IV steroids should be administered as soon as possible, and a CSF drain should be considered.

The Impact of COVID-19 on Epilepsy Care: A Survey of the American Epilepsy Society Membership.

The COVID-19 pandemic has impacted the delivery of care to people with epilepsy (PWE) in multiple ways including limitations on in-person contact and restrictions on neurophysiological procedures. To better study the effect of the pandemic on PWE, members of the American Epilepsy Society were surveyed between April 30 and June 14, 2020. There were 366 initial responses (9% response rate) and 337 respondents remained for analysis after screening out noncompleters and those not directly involved with clinical care; the majority were physicians from the United States. About a third (30%) of respondents stated that they had patients with COVID-19 and reported no significant change in seizure frequency. Conversely, one-third of respondents reported new onset seizures in patients with COVID-19 who had no prior history of seizures. The majority of respondents felt that there were at least some barriers for PWE in receiving appropriate clinical care, neurophysiologic procedures, and elective surgery. Medication shortages were noted by approximately 30% of respondents, with no clear pattern in types of medication involved. Telehealth was overwhelmingly found to have value. Among the limitation of the survey was that it was administered at a single point in time in a rapidly changing pandemic. The survey showed that almost all respondents were affected by the pandemic in a variety of ways.

Overview of EEG Montages and Principles of Localization.

Montages are logical, orderly arrangements of electroencephalographic derivations or channels that are created to display activity over the entire head and to provide lateralizing and localizing information. Most often, bipolar and referential montages are used for routine electroencephalographic recordings. Common average and Laplacian montages can also be helpful in some situations. Because each type of montage has certain strengths and limitations, the ACNS guidelines recommend the use of multiple classes of montages for each electroencephalographic recording. A variety of factors need to be considered for localization by scalp electroencephalogram, but in clinical practice, a three-step approach can be used to localize an interictal epileptiform discharge by visual inspection using a standard set of scalp electrodes and conventional montages. The ACNS guideline provides a number of standard and suggested montages, but, depending on the clinical situation, additional montages can be designed using the electrodes within the 10-20 system or by placing additional electrodes.

American Clinical Neurophysiology Society: EEG Guidelines Introduction.

This revision to the EEG Guidelines is an update incorporating current EEG technology and practice. "Standards of practice in clinical electroencephalography" (previously Guideline 4) has been removed. It is currently undergoing revision through collaboration among multiple medical societies and will become part of "Qualifications and Responsibilities of Personnel Performing and Interpreting Clinical Neurophysiology Procedures." The remaining guidelines are reordered and renumbered.

American Clinical Neurophysiology Society Guideline 2: Guidelines for Standard Electrode Position Nomenclature.

This revision to the EEG Guidelines is an update incorporating current electroencephalography technology and practice and was previously published as Guideline 5. While the 10-10 system of electrode position nomenclature has been accepted internationally for almost two decades, it has not been used universally. The reasons for this and clinical scenarios when the 10-10 system provides additional localizing information are discussed in this revision. In addition, situations in which AF1/2, AF5/6, PO1/2 and PO5/6 electrode positions may be utilized for EEG recording are discussed.

American Clinical Neurophysiology Society Guideline 3: A Proposal for Standard Montages to Be Used in Clinical EEG.

This revision to the EEG Guidelines is an update incorporating current electroencephalography technology and practice and was previously published as Guideline 6. A discussion of methodology for the appropriate selection of reference electrodes is added. In addition, montages are added to assist with localization of abnormal activity in mesial frontal and anterior temporal regions.

American Clinical Neurophysiology Society: EEG Guidelines Introduction.

This revision to the EEG Guidelines is an update incorporating current EEG technology and practice. "Standards of practice in clinical electroencephalography" (previously Guideline 4) has been removed. It is currently undergoing revision through collaboration among multiple medical societies and will become part of "Qualifications and Responsibilities of Personnel Performing and Interpreting Clinical Neurophysiology Procedures." The remaining guidelines are reordered and renumbered.

American Clinical Neurophysiology Society Guideline 3: A Proposal for Standard Montages to Be Used in Clinical EEG.

This revision to the EEG Guidelines is an update incorporating current electroencephalography technology and practice and was previously published as Guideline 6. A discussion of methodology for the appropriate selection of reference electrodes is added. In addition, montages are added to assist with localization of abnormal activity in mesial frontal and anterior temporal regions.

American Clinical Neurophysiology Society Guideline 2: Guidelines for Standard Electrode Position Nomenclature.

This revision to the EEG Guidelines is an update incorporating current electroencephalography technology and practice and was previously published as Guideline 5. While the 10-10 system of electrode position nomenclature has been accepted internationally for almost two decades, it has not been used universally. The reasons for this and clinical scenarios when the 10-10 system provides additional localizing information are discussed in this revision. In addition, situations in which AF1/2, AF5/6, PO1/2 and PO5/6 electrode positions may be utilized for EEG recording are discussed.

Epilepsy in the elderly: Special considerations and challenges.

The elderly are generally defined as those over 60 or 65 years old, but they are a heterogeneous group and may be subdivided into categories based on age and health status. The incidence of epilepsy is highest in the elderly. With a progressive increase in life expectancy, this is the fastest growing segment of patients with epilepsy. Older patients most often have focal seizures, with less prominent auras and automatisms, and longer duration of postictal confusion compared to younger patients. Status epilepticus is common and has a high mortality. The most common specific etiology is cerebrovascular disease, but the cause remains unknown in many patients. Diagnosis can be challenging because of several patient-related, physician-related and investigation-related factors. Over-diagnosis and under-diagnosis are common. Treatment is complicated by the presence of physiological changes related to aging, co-morbidities and cognitive problems as well as concerns regarding drug interactions and medication adherence. Seizures can be controlled in most patients with low doses of a single anti-epileptic drug (AED). Tolerability is an important factor in selection of an AED, as elderly patients tend to be highly sensitive to side effects. Drug-resistant epilepsy is uncommon. Epilepsy surgery, especially temporal lobectomy, can be performed in older patients with good results. More studies addressing the pathophysiological mechanisms of epilepsy in this age group, and greater inclusion of the elderly in clinical trials, as well as development of comprehensive care models are needed to provide optimal care to these patients.