Ioannis Karakis

A quick and reliable EEG montage for the detection of seizures in the critical care setting.

Obtaining an emergent EEG for the diagnosis of nonconvulsive status epilepticus and conconvulsive seizures in the intensive care unit raises logistic problems in most hospitals. Previous studies have looked into the hairline EEG for a broader population than the critically ill, with controversial conclusions. The authors created a montage sufficiently simple to be performed and interpreted by residents and rapidly achievable to meet the time constraints of a busy on-call schedule. Seven electrodes (Fp1, Fp2, T3, T4, O1, O2, and Cz), easily applied without the need for tape measure by using only anatomic landmarks (pupils, ears, vertex, and inion), were used to configure three different montages: double diamond, circumferential, and Cz referential. EEG records obtained with the full 10-20 system in critically ill patients were reformatted into these montages and reviewed retrospectively independently by neurology attending physicians with expertise in EEG interpretation and senior neurology residents. A comparison was done with the previously studied hairline EEG. The average sensitivity of the study montage for seizure detection was 92.5%, whereas the average specificity was 93.5%. These results suggest that the seven-electrode montage could potentially be a quick and reliable EEG montage for the detection of seizures in the intensive care unit, when technical support is not available. Further prospective studies are required to validate these promising results in a larger population sample.

Association of Serum Vitamin D with the Risk of Incident Dementia and Subclinical Indices of Brain Aging: The Framingham Heart Study.

BACKGROUND Identifying nutrition- and lifestyle-based risk factors for cognitive impairment and dementia may aid future primary prevention efforts. OBJECTIVE We aimed to examine the association of serum vitamin D levels with incident all-cause dementia, clinically characterized Alzheimers disease (AD), MRI markers of brain aging, and neuropsychological function. METHODS Framingham Heart Study participants had baseline serum 25-hydroxyvitamin D (25(OH)D) concentrations measured between 1986 and 2001. Vitamin D status was considered both as a continuous variable and dichotomized as deficient (<10 ng/mL), or at the cohort-specific 20th and 80th percentiles. Vitamin D was related to the 9-year risk of incident dementia (n = 1663), multiple neuropsychological tests (n = 1291) and MRI markers of brain volume, white matter hyperintensities and silent cerebral infarcts (n = 1139). RESULTS In adjusted models, participants with vitamin D deficiency (n = 104, 8% of the cognitive sample) displayed poorer performance on Trail Making B-A (β= -0.03 to -0.05±0.02) and the Hooper Visual Organization Test (β= -0.09 to -0.12±0.05), indicating poorer executive function, processing speed, and visuo-perceptual skills. These associations remained when vitamin D was examined as a continuous variable or dichotomized at the cohort specific 20th percentile. Vitamin D deficiency was also associated with lower hippocampal volumes (β= -0.01±0.01) but not total brain volume, white matter hyperintensities, or silent brain infarcts. No association was found between vitamin D deficiency and incident all-cause dementia or clinically characterized AD. CONCLUSIONS In this large community-based sample, low 25(OH)D concentrations were associated with smaller hippocampal volume and poorer neuropsychological function.

Patient and caregiver quality of life in psychogenic non-epileptic seizures compared to epileptic seizures

PURPOSE Little is known about the effect of psychogenic non epileptic seizures (PNES) to caregiver quality of life (QOL), particularly as it compares to epileptic seizures (ES). We sought to characterize this effect and identify its determinants. METHODS The study population comprised of 126 ES and 33 PNES patients who underwent video EEG monitoring along with 48 and 18 caregivers respectively who accompanied them to their investigations. Patients completed questionnaires providing demographic, disease-related, cognitive, psychiatric, sleep and QOL information on admission, prior to their diagnosis being clarified. Their caregivers completed questionnaires providing demographic, disease burden and generic QOL information. Paraclinical data were also gathered. Regression analysis was used to identify patient and caregiver related determinants of patient and caregiver QOL. RESULTS QOL scores were significantly worse for PNES than ES patients and were mainly linked to depression levels. PNES and ES caregivers had comparable demographic characteristics and QOL scores. ES caregiver QOL was better in employed caregivers with lower burden scores for the physical component summary (PCS) and worse in female caregivers of depressed patients with higher burden scores for the mental component summary (MCS). Caregiver burden score was the strongest correlate of PNES caregiver MCS QOL score. CONCLUSION Caregiver QOL in PNES does not differ from caregiver QOL in ES, while patient QOL is worse in PNES. Caregiver burden emerges as a consistent correlate of caregiver QOL both in ES and PNES. These findings advocate for consideration of caregiver burden and QOL in PNES in clinical practice and for future research paradigms.

Mutation of SCARB2 in a Patient With Progressive Myoclonus Epilepsy and Demyelinating Peripheral Neuropathy

OBJECTIVE To report the detection of mutations in the SCARB2 gene in a previously described patient with progressive myoclonus epilepsy (PME) and demyelinating peripheral neuropathy. DESIGN Case report. SETTING Epilepsy Genetics Research Laboratory and Epilepsy Service in a tertiary care center. PATIENT A 27-year old male patient with PME with preserved intellect and peripheral neuropathy. RESULTS We have solved a previously reported case of PME, preserved intellect, and demyelinating peripheral neuropathy. The patient is a compound heterozygote for 2 mutations in the SCARB2 gene, which has recently been found to be a cause of PME. CONCLUSIONS Demyelinating neuropathy is a clinical clue to the presence of SCARB2 mutations in PME.

Caregiver Burden in Epilepsy: Determinants and Impact

Aim. Caregiver burden (CB) in epilepsy constitutes an understudied area. Here we attempt to identify the magnitude of this burden, the factors associated with it, and its impact to caregiver quality of life (QOL). Methods. 48 persons with epilepsy (PWE) underwent video-EEG monitoring and their caregivers completed questionnaires providing demographic, disease-related, psychiatric, cognitive, sleep, QOL, and burden information. Results. On regression analysis, higher number of antiepileptic drugs, poorer patient neuropsychological performance, lower patient QOL score, and lower caregiver education level were associated with higher CB. Time allocated to patient care approximated but did not attain statistical significance. A moderate inverse correlation between CB and caregiver QOL physical component summary score and a stronger inverse correlation between CB and caregiver QOL mental component summary score were seen. Conclusion. In a selected cohort of PWE undergoing video-EEG monitoring, we identified modest degree of CB, comparable to that reported in the literature for other chronic neurological conditions. It is associated with specific patient and caregiver characteristics and has a negative effect on caregiver QOL.

The effect of epilepsy surgery on caregiver quality of life

PURPOSE Epilepsy surgery has been shown to improve patient quality of life (QOL). Little is known about its effect on caregiver QOL. METHODS The study population comprised of 26 persons with epilepsy (PWE) who underwent long term video EEG monitoring at Massachusetts General Hospital for presurgical evaluation along with 16 caregivers. The PWE completed epilepsy directed QOL (QOLIE-31) and psychological (Beck depression-BDI and anxiety inventory-BAI) questionnaires before and after surgery. Their participating caregivers completed generic health related QOL (SF36v2) and disease burden (Zarit caregiver burden inventory-ZCBI) questionnaires before and after surgery. Demographic data for all participants and disease/surgery related data for the PWE were collected. Statistical analysis was performed to compare PWE and caregiver QOL before and after surgery. RESULTS Mean patient age was 37 years. Most (77%) suffered from symptomatic partial epilepsy for approximately 18 years prior to surgery, averaging 4 seizures per month and 2.2 antiepileptic drugs (AEDs). 78% of them underwent an anterior temporal lobectomy and the rest extra-temporal resections. On follow up at approximately 9 months, 69% had a surgical outcome of Engel class I, 23% of class II and 8% class IV. Postoperatively, the PWE remained on average on 1.9 AEDs. There was a statistically significant improvement for both the aggregate QOLIE-31 score and all its subscales (except for medication effects) as well as the BAI scores. 96% of the PWE felt that the decision to go through surgery was worthwhile. Mean caregivers age was 47 years. Half of them were spouses to the PWE and the majority of the rest their parents. 50% of them stated that their overall time devoted to patients care decreased after surgery and 50% that it remained unchanged. The mental component scale (SF36v2, MCS) of caregiver QOL showed statistically significant improvement. ZCBI score and the physical component scale of their QOL (SF36v2, PCS) did not significantly vary before and after surgery. 75% of caregivers deemed their QOL better post surgery vs 19% similar. 94% of the caregivers felt that the decision to go through surgery was worthwhile. CONCLUSIONS Successful epilepsy surgery has a positive impact not only to patient QOL but also to their caregiver. To the best of our knowledge, this is the first pilot study to systematically address the impact of epilepsy surgery on caregivers providing additional support to epilepsy surgery as the optimal treatment modality in carefully selected patients. These findings call for further investigation on the caregiver quality of life in epilepsy and for its inclusion in the treatment plan and quality indicators for epilepsy surgery.

Clinical correlates of Charcot-Marie-Tooth disease in patients with pes cavus deformities.

Introduction: Given its association with Charcot–Marie–Tooth disease (CMT), pes cavus is a common reason for referral to a neurologist. We investigated clinical features that may predict CMT in children with pes cavus. Methods: In this study we retrospectively reviewed pes cavus patients referred to Boston Childrens Hospital in the past 20 years. Patients were categorized as idiopathic or CMT, based on EMG/genetic testing, and their clinical features were compared. Results: Of the 70 patients studied, 33 had idiopathic pes cavus, and 37 had genetically confirmed CMT. Symptoms of weakness, unsteady gait, family history of pes cavus and CMT, and signs of sensory deficits, distal atrophy and weakness, absent ankle jerks, and gait abnormalities were associated with CMT. Conclusions: In children with pes cavus, certain clinical features can predict CMT and assist in selection of patients for further, potentially uncomfortable (EMG) and expensive (genetic) confirmatory investigations. Muscle Nerve, 2013

Referral and diagnostic trends in pediatric electromyography in the molecular era

Introduction: Major advances in genetic analysis and neuroimaging have modified the traditional diagnostic approach for neuromuscular disorders. The purpose of this study was to investigate the role of electromyography (EMG) in the molecular era. Methods: We retrospectively surveyed reports of all EMG studies performed at Boston Childrens Hospital from 2001 through 2011. Data were collected on study numbers, patient ages, referring provider subspecialty, study indication, electrophysiological diagnosis, and study utility. Results: A total of 2100 studies were performed. The volume increased from ∽160 to ∽250 studies/year. There was a trend toward studying older children. Neurologists, including neuromuscular specialists, constituted the major referral pool, whereas referrals from orthopedics increased steadily. Polyneuropathies followed by mononeuropathies were the most common indications and diagnoses. Fifty‐seven percent of studies were normal. EMG provided meaningful information in 94% of cases. Conclusion: EMG continues to play a cardinal role in the diagnosis of pediatric neuromuscular disorders, although its practice paradigm is evolving. Muscle Nerve 50:244–249, 2014

The utility of routine EEG in the diagnosis of sleep disordered breathing.

Summary: Sleep disordered breathing (SDB) is a common medical condition. Its manifestations of snoring, nocturnal choking, arousals, and sleep fragmentation can lead to excessive daytime sleepiness, neuropsychological slowing, lapses of consciousness, and accidents that can be misinterpreted as epileptic phenomena. Moreover, patients with documented epilepsy commonly exhibit similar symptomatology because of the undiagnosed coexistence of sleep apnea. Therefore, a large proportion of patients referred to the electroencephalogram (EEG) laboratory primarily to confirm or refute the diagnosis of epilepsy could suffer from latent sleep apnea and the routine EEG has the potential to divulge it. We retrospectively evaluated the reporting of sleep apnea symptomatology (snoring, choking, gasping/deep breath, apnea, desaturation, excessive drowsiness) in routine inpatient and outpatient adult EEG studies performed in our institution over the past 12 years (39,130 studies, approximately half of which recorded at least early stages of sleep). Comparisons were performed with the medical records to ascertain the coexistence of objectively diagnosed SDB with polysomnography before or after the EEG study and the importance of reporting variations in assisting with the diagnosis. Two illustrative examples are provided. Sixty-nine EEG studies were identified, performed primarily to confirm, or refute the diagnosis of epilepsy. The mean age of the subjects at EEG was 64 years (range 30–89), and 55 (80%) were male. 36% of them suffered from known epilepsy. Snoring was the most commonly reported sign in 48 (70%) of the studies, followed by arousals in 29 (42%), apnea in 16 (23%), excessive drowsiness in 13 (19%), gasping/deep breath in 9 (13%), and desaturation in 7 (10%). A sleep disorder was suggested in 25 (36%) of the interpretations and a direct recommendation for a sleep study was made in 22 of them (32%). This interpretation was included in the impression of the report in 21 (30%) of the cases, in the detail in 20 (30%) of the cases and in both in 28 (40%). Only 14 (20%) patients underwent polysomnography, and all of them were formally diagnosed with SDB. Seven (50%) of them were diagnosed with obstructive sleep apnea, 2 (14%) with central sleep apnea, 3 (22%) with both, 1 (7%) with upper airways resistance syndrome, and 1 (7%) with primary snoring. From these 14 patients, 9 (64%) were diagnosed with a sleep study performed after the EEG, 4 (29%) before the EEG interpretation, and 1 (7%) had a repeat study after the EEG. In the logistic regression model applied, with the exception of the presence of arousals (odds ratio = 4.63, P = 0.033), none of the aforementioned symptomatology or the reporting of suspicion for SDB or the location (impression vs. detail) of the reporting showed a statistically significant association with the completion of a sleep study. Routine EEG offers a unique opportunity of direct clinical observation along with electrophysiologic and cardiorespiratory monitoring. When sleep is recorded, it can help identify clinical and electrographic features of sleep apnea and prompt confirmation with a polysomnogram in the appropriate clinical context. It can therefore serve as a valuable, adjunctive tool for the diagnosis of SDB. Our data highlight that potential but unveil its decreased use in the neurology community. Increased awareness is required by the EEG technologists, interpreting neurologists, and referring physicians, regarding reporting and using sleep apnea features on the EEG.

Intra-stimulation discharges: an overlooked cortical electrographic entity triggered by direct electrical stimulation.

OBJECTIVE Intra-stimulation discharges (IDs) can occur during language mapping, are largely unrecognized, and may precede the occurrence of after-discharges (ADs) and seizures. This study aimed to identify predictors of ID occurrence and determine whether IDs increase the probability of triggered ADs. METHODS A total of 332 stimulation events performed during language mapping were analyzed in 3 patients who underwent intracranial EEG recordings during evaluations for epilepsy surgery. IDs were identified in 76 stimulation events. The relationships between IDs and the stimulus current intensity, stimulation duration, and proximity to regions of abnormal cortical excitability [characterized by the presence of baseline epileptiform discharges (BEDs)] were determined using regression analysis. RESULTS The presence of BEDs in close proximity to stimulation, an increase in stimulus intensity by 1 mA, and an increase in stimulation duration by 1s independently increased the odds of triggering IDs by 7.40, 1.37, and 1.39 times, respectively. All IDs were triggered during stimulations in the temporal lobe. The occurrence of IDs increased the odds of triggering ADs 5-fold. CONCLUSIONS Longer stimulations, higher currents, and the presence of BEDs at the stimulation site increase the probability of ID occurrence, which in turn increases the probability of triggering ADs. SIGNIFICANCE Attention to IDs may improve the safety and precision of neurophysiologic mapping.