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Electroclinical Features in Epilepsy Surgery Candidates With Epileptic Spasms
Summary: Electroclinical features in surgical candidates with epileptic spasms differ significantly from the other focal epilepsy phenotypes. EEG findings tend to be more diffuse and less localizing in children with epileptic spasms. These are illustrated with five case studies to highlight three different categories of findings on interictal and ictal EEG: lateralizing, nonlateralizing, and false lateralizing. Hemihypsarrhythmia on interictal EEG is the most striking lateralizing abnormality that occurs in a minority of surgical candidates. Persistent focal epileptiform discharges in one region or asymmetric physiolog...
Source: Journal of Clinical Neurophysiology - November 1, 2022 Category: Neurology Tags: Invited Review Source Type: research

Utility of Functional MRI and Magnetoencephalography in the Diagnosis of Infantile Spasms and Hypsarrhythmia
Summary: Neuroimaging and neurophysiology techniques can add a significant contribution to the comprehension of infantile spasms (IS) and hypsarrhythmia. Functional MRI and magnetoencephalography (MEG) are two noninvasive tools that can be used in young children with IS. In the past two decades, interesting data about IS have emerged from functional MRI and MEG studies. Regarding their clinical utility, MEG has supported the concept that epileptic spasms can have a focal origin. Moreover, MEG might contribute to the localization of the epileptogenic zone in children with IS under investigation for epilepsy surgery. Fun...
Source: Journal of Clinical Neurophysiology - November 1, 2022 Category: Neurology Tags: Invited Review Source Type: research

Scoring Systems for the Evaluation of Hypsarrhythmia
Summary: Hypsarrhythmia is a well-recognized EEG pattern and it has been long known as a feature of one of the more severe forms of epilepsy early in life with adverse consequences if not recognized and treated promptly. Yet, it has been difficult to objectively quantify its varied manifestations and link them to the clinical severity of epilepsy, implications for treatment, or the overall seizure and developmental outcome of young patients. This is not for want of effort, for there have been several approaches to score hypsarrhythmia ever since its initial recognition as a unique EEG pattern. In this article, we revie...
Source: Journal of Clinical Neurophysiology - November 1, 2022 Category: Neurology Tags: Invited Review Source Type: research

Animal Models in Epileptic Spasms and the Development of Novel Treatment Options
Summary: The infantile spasms (IS) syndrome is a catastrophic developmental epileptic encephalopathy syndrome characterized by an age-specific expression of epileptic spasms that are associated with extremely abnormal, oftentimes described as chaotic, interictal EEG pattern known as hypsarrhythmia. Patients with IS generally have poor neurodevelopmental outcomes, in large part because of the frequent epileptic spasms and interictal EEG abnormalities. Current first-line treatments such as adrenocorticotropic hormone or vigabatrin are often ineffective and are associated with major toxic side effects. There is therefore ...
Source: Journal of Clinical Neurophysiology - November 1, 2022 Category: Neurology Tags: Invited Review Source Type: research

Historical Overview of Hypsarrhythmia and Its Association to Epileptic Spasms: A Review of the Medical Literature From 1952 to 1982
This article will provide a historical review of knowledge developments about hypsarrhythmia and infantile spasms, emphasizing the period 1952 to 1982. The goal of the article was to highlight clinical elements that were discovered then and remain clinically relevant today. (Source: Journal of Clinical Neurophysiology)
Source: Journal of Clinical Neurophysiology - November 1, 2022 Category: Neurology Tags: Invited Review Source Type: research

Hypsarrhythmia and Epileptic Spasms: A Look at One Old Epilepsy in the Modern Era
No abstract available (Source: Journal of Clinical Neurophysiology)
Source: Journal of Clinical Neurophysiology - November 1, 2022 Category: Neurology Tags: GUEST EDITORIAL Source Type: research

Success Rate of Obtaining Baseline Somatosensory and Motor Evoked Potentials in 695 Consecutive Cranial and Spine Surgeries
Conclusions: The success rates of obtaining adequate baseline SSEP and MEP data are overall higher than previously reported. Preoperative diagnosis like spinal infection or trauma may predict lower success rates for acquiring adequate baseline SSEPs and MEPs. (Source: Journal of Clinical Neurophysiology)
Source: Journal of Clinical Neurophysiology - September 1, 2022 Category: Neurology Tags: Original Research Source Type: research

Evoked Potentials in Patients With Wilson Disease
Conclusions: The data showed that VEP and BAEP are more frequently abnormal in WD than previously reported. The abnormal VEP and BAEP even without clinical signs and brain MRI abnormalities point to subclinical involvement of visual and auditory pathways caused by copper toxicity. Because VEP and BAEP are noninvasive and widely available, they should be performed in all patents with WD. (Source: Journal of Clinical Neurophysiology)
Source: Journal of Clinical Neurophysiology - September 1, 2022 Category: Neurology Tags: Original Research Source Type: research

Multimodal Evoked Potential Profiles in Woodhouse–Sakati Syndrome
Conclusions: Multiple EP abnormalities are observed in Woodhouse–Sakati syndrome patients, mainly in pattern reversal visual EPs and somatosensory EPs. These findings indicate potential myelin dysfunction that has a role in the underlying pathophysiology, disease course, and phenotypic variability. (Source: Journal of Clinical Neurophysiology)
Source: Journal of Clinical Neurophysiology - September 1, 2022 Category: Neurology Tags: Original Research Source Type: research

Effect of Motor State on Postactivation Depression of the Soleus H-Reflex in Parkinson's Disease During Deep Brain Stimulation and Dopaminergic Medication Treatment: A Pilot Study
Purpose: Postactivation depression of the Hoffmann reflex is reduced in Parkinson's disease (PD), but how the recovery is influenced by the state of the muscle is unknown. The present pilot study examined postactivation depression in PD at rest and during a voluntary contraction while patients were off treatment and while receiving medications and/or deep brain stimulation. Methods: The authors recruited nine patients with PD treated with implanted deep brain stimulation and examined postactivation depression under four treatment conditions. Paired pulses were delivered 25 to 300 ms apart, and soleus Hoffmann re...
Source: Journal of Clinical Neurophysiology - September 1, 2022 Category: Neurology Tags: Original Research Source Type: research

Does Essential Tremor Alter the Axonal Excitability Properties of Lower Motor Neurons?
This study aimed to identify alterations in the excitability of distal motor axons in subjects with ET, with the intention of clarifying whether a trans-synaptic mechanism or LMN adaptation for tremor affects the LMNs of subjects with ET. Methods: Twenty-one consecutive patients diagnosed with ET underwent a clinical and electrophysiological evaluation. For the enrolled cases and 45 age- and gender-matched healthy controls, automated nerve excitability testing with threshold tracking techniques (QTRACS software with TRONDF multiple-excitability protocol) was used to evaluate multiple nerve excitability indices in di...
Source: Journal of Clinical Neurophysiology - September 1, 2022 Category: Neurology Tags: Original Research Source Type: research

Reduced Occipital Cortex Excitability in Amyotrophic Lateral Sclerosis
Conclusions: Visual cortex is affected, and the occipital excitability is reduced in ALS, without any relation to motor cortical excitability changes, providing another clue suggestive of sensory involvement in ALS. (Source: Journal of Clinical Neurophysiology)
Source: Journal of Clinical Neurophysiology - September 1, 2022 Category: Neurology Tags: Original Research Source Type: research

Tongue as a Wire? Glossokinetic Artifact and Insights From Intracranial EEG
Conclusions: The traditional view of the tongue as a dipole generator of scalp GKA is simplistic and does not account for the findings reported herein. The tongue most probably shunts other scalp and soft-tissue currents. Knowledge of tongue potentials is of interest in the education and the design of tongue–computer interfaces. (Source: Journal of Clinical Neurophysiology)
Source: Journal of Clinical Neurophysiology - September 1, 2022 Category: Neurology Tags: Original Research Source Type: research

Stereotactic EEG Practices: A Survey of United States Tertiary Referral Epilepsy Centers
Conclusions: Stereotactic EEG has become the principal method for intracranial EEG monitoring in the majority of epilepsy surgery centers in the United States. Most report similar indications for use of SEEG, though significant variability exists in the utilization of concurrent cardiopulmonary monitoring as well as several technical and patient care practices. There is significant variability in level of background training in SEEG among practitioners. The study highlights the need for consensus statements and guidelines to benchmark SEEG practice and develop uniform standards in the United States. (Source: Journal of...
Source: Journal of Clinical Neurophysiology - September 1, 2022 Category: Neurology Tags: Original Research Source Type: research

Source Analysis of Triphasic Waves Using Quantitative Neuroimaging
The objective of the current study was to evaluate the source of TWs using EEG source imaging and computerized tomography. Methods: Twenty-eight patients with TWs were investigated. Source analysis was performed on the averaged TWs for each individual, and source maps were extracted. Normalization and automatic segmentation of gray matter were performed on computerized tomography scans before analysis. Finally, voxelwise correlation analyses were conducted between EEG source maps and gray matter volumes. Results: Source analyses showed that the anterior cingulate cortex was mainly involved in TWs (16/28 patie...
Source: Journal of Clinical Neurophysiology - September 1, 2022 Category: Neurology Tags: Original Research Source Type: research