Edge-Enhancing Gradient-Echo MP2RAGE for Clinical Epilepsy Imaging at 7T.

The 3D edge-enhancing gradient-echo (EDGE) MR imaging sequence offers superior contrast-to-noise ratio in the detection of focal cortical dysplasia. EDGE could benefit from 7T MR imaging but also faces challenges such as image inhomogeneity and low acquisition efficiency. We propose an EDGE-MP2RAGE sequence that can provide both EDGE and T1-weighted contrast, simultaneously, improving data-acquisition efficiency. We demonstrate that with sequence optimization, EDGE images with sufficient uniformity and T1-weighted images with high gray-to-white matter contrast can be achieved.

Brain Abnormalities and Epilepsy in Patients with Parry-Romberg Syndrome.

BACKGROUND AND PURPOSE: Parry-Romberg syndrome is a rare disorder characterized by progressive hemifacial atrophy. Concomitant brain abnormalities have been reported, frequently resulting in epilepsy, but the frequency and spectrum of brain involvement are not well-established. This study aimed to characterize brain abnormalities in Parry-Romberg syndrome and their association with epilepsy. MATERIALS AND METHODS: This is a single-center, retrospective review of patients with a clinical diagnosis of Parry-Romberg syndrome and brain MR imaging. The degree of unilateral hemispheric atrophy, white matter disease, microhemorrhage, and leptomeningeal enhancement was graded as none, mild, moderate, or severe. Other abnormalities were qualitatively reported. Findings were considered potentially Parry-Romberg syndrome-related when occurring asymmetrically on the side affected by Parry-Romberg syndrome. RESULTS: Of 80 patients, 48 (60%) had brain abnormalities identified on MR imaging, with 26 (32%) having abnormalities localized to the side of the hemifacial atrophy. Sixteen (20%) had epilepsy. MR imaging brain abnormalities were more common in the epilepsy group (100% versus 48%, P < .001) and were more frequently present ipsilateral to the hemifacial atrophy in patients with epilepsy (81% versus 20%, P < .001). Asymmetric white matter disease was the predominant finding in patients with (88%) and without (23%) epilepsy. White matter disease and hemispheric atrophy had a higher frequency and severity in patients with epilepsy (P < .001). Microhemorrhage was also more frequent in the epilepsy group (P = .015). CONCLUSIONS: Ipsilateral MR imaging brain abnormalities are common in patients with Parry-Romberg syndrome, with a higher frequency and greater severity in those with epilepsy. The most common findings in both groups are white matter disease and hemispheric atrophy, both presenting with greater severity in patients with epilepsy.

Electroencephalogram findings in patients with posterior cortical atrophy.

AIM OF THE STUDY: The aim of this study is to evaluate standard scalp EEG findings in patients with posterior cortical atrophy (PCA), an atypical variant of Alzheimer's disease (AD). CLINICAL RATIONALE: PCA is a topographically selective variant of AD. Patients with typical AD have an increased likelihood of seizures, which may negatively impact overall functional performance and cognition. It is currently unknown what the typical EEG findings are for patients with PCA. MATERIALS AND METHODS: A retrospective chart review was performed on patients identified either with autopsy confirmed (n=13) or clinically (n=126) as PCA. RESULTS: 139 patients were included though only 23 (16.5%) had undergone EEG recording. The EEG was normal in 6 (26%), while an abnormal EEG was present in 17 (74%). Interictal epileptic discharges (IEDs) were found in 2 of the 23 patients (9%). CONCLUSIONS: This study of limited sample size suggests that there may be an increased predilection to find IEDs within PCA when compared to typical AD. Larger cohorts are required to determine frequency of abnormal EEGs in PCA, roles of AEDs in therapy, and in the selection of preferred AED. CLINICAL IMPLICATIONS: Patients with PCA would potentially benefit from an EEG for assessment of IEDs which may provide the clinician with a therapeutic opportunity.

Clinical utility of EEG in diagnosing and monitoring epilepsy in adults.

Electroencephalography (EEG) remains an essential diagnostic tool for people with epilepsy (PWE). The International Federation of Clinical Neurophysiology produces new guidelines as an educational service for clinicians to address gaps in knowledge in clinical neurophysiology. The current guideline was prepared in response to gaps present in epilepsy-related neurophysiological assessment and is not intended to replace sound clinical judgement in the care of PWE. Furthermore, addressing specific pathophysiological conditions of the brain that produce epilepsy is of primary importance though is beyond the scope of this guideline. Instead, our goal is to summarize the scientific evidence for the utility of EEG when diagnosing and monitoring PWE.

Nonconvulsive status with metronidazole.

OBJECTIVE: To expand the adverse events associated with metronidazole to include nonconvulsive status epilepticus (NCSE). DESIGN: Observational single case report of a rare association. SETTING: Hospitalized lung transplant recipient treated with metronidazole for prevention of infection. PATIENT: A 56-year-old man with systemic symptoms, peripheral neuropathy, generalized seizure, and a subsequent acute deterioration of mental status due to NCSE. INTERVENTIONS: Administration of midazolam was successful in terminating status epilepticus. MAIN OUTCOME MEASURES: Abrupt termination of NCSE was evident on continuous bedside electroencephalogam associated with clinical resolution of mental status. RESULTS: Recovery occurred from NCSE eventually deteriorating to a fatal outcome. CONCLUSIONS: Metronidazole may be associated with successfully treated NCSE.

Outcome and complications of chronically implanted subdural electrodes for the treatment of medically resistant epilepsy.

BACKGROUND: Surgery for medically resistant epilepsy is safe and effective. However, when noninvasive techniques are insufficient, then consideration is given to invasive electrocorticography (EcoG). OBJECTIVE: The aim of the study was to analyze results and complications of subdural electrodes placement in the treatment of intractable epilepsy. METHODS: Ninety-one consecutive patients who underwent placement of subdural electrodes (1999-2010) were considered for this study. All patients underwent a standardized pre-operative evaluation. Invasive subdural electrode placement was considered when there were inadequate ictal recordings, there was discordance between EEG and neuroimaging or the epileptogenic zone was localized near eloquent cortex. RESULTS: Resective epilepsy surgery was performed in 70/91 patients (76.9%). Twenty-four out of seventy (34.3%) who underwent surgical resection were seizure-free (CL-I) at last follow-up. A statistical evaluation revealed a very strong trend for patients with positive lesional pre-operative MRI to have improved outcomes compared to normal brain MRI population (p=.028). There were 10 surgical related complications (11%), but no mortality or permanent morbidity. Statistical analysis demonstrated that placement of a subdural grid in any combination was statistically significant (p=.01) for surgical complications. CONCLUSIONS: Invasive monitoring is a useful and necessary technique for the surgical treatment of intractable epilepsy. Careful surveillance is required during the monitoring period especially when the patient has undergone large subdural grid placement. A good working hypothesis can minimize complications and achieve better outcomes.

Evidence that temporal lobe is a default area in absence epilepsy.

Correctly classifying seizures is essential for appropriate epilepsy management. Focal and generalized epilepsy rarely occur independently in the same patient. Cases of focal ictal evolution during seizures that are generalized in onset have been reported though these have included a small heterogeneous series of patients with generalized epilepsy and features on the EEG. We wish to report two patients with absence epilepsy that were noted on video-EEG monitoring to manifest a focal temporal electroclinical transformation from a typical absence seizure. Defining the electroclinical spectrum of absence seizures could add to our understanding of the selective cortical and subcortical networks that are involved in patients with "prototypic" generalized and focal seizures.

Disobedience and driving in patients with epilepsy.

OBJECTIVE: Motor vehicle accidents direct legislators to ensure pubic safety. We attempted to characterize and quantify driving risk in patients with seizures (PWS). METHODS: We delivered 12-question surveys to 287 consecutive PWS at an epilepsy clinic in Florida. Illegal and disobedient driving practices were analyzed. RESULTS: Eighty-three of 236 (35.2%) PWS were eligible to drive and 62.3% were ineligible with a seizure in <6 months (P<0.001, 95% CI: 0.57-0.70). Among the ineligible responders, 23.8% (35/147) of ineligible responders were illegally driving (14.83% of cohort); 11.86% (28/236) of PWS were disobedient refusing to obey the law, and 8.9% (21/236) of PWS were defiant and knew the law. Sadness (75/236, 31.8%) was the most common reaction to restriction, but disobedient PWS were angry (10/28, 35.7%). CONCLUSION: Overall, a small number of PWS are disobedient and illegally driving. A targeted approach to high-risk drivers with repeated verbal and supplemental driving information may help avoid unnecessary universal physician reporting for PWS.

Crying with left temporal lobe seizures and Wada testing.

Dacrystic seizures are characterized by crying and are rare in patients with epilepsy. The crying that occurs during complex partial seizures is expressed as an unconscious behavior unassociated with sadness or depressive quality. It has been reported to occur with focal seizures that are localized to the frontotemporal regions and is frequently lateralized to the nondominant hemisphere. We describe a woman with left temporal lobe epilepsy and recurrent complex partial seizures with crying behavior that was reproduced during left carotid methohexital anesthesia, suggesting functional inactivation by two different mechanisms. Following left amygdalohippocampectomy she became seizure free for >2years without perioperative mood destabilization. Localized functional networks that involve limbic structures may be involved in patients with dacrystic seizures with a favorable outcome following epilepsy surgery.

What constitutes high quality of care for adults with epilepsy?

BACKGROUND: Providers are increasingly being held accountable for the quality of care provided. While quality indicators have been used to benchmark the quality of care for a number of other disease states, no such measures are available for evaluating the quality of care provided to adults with epilepsy. In order to assess and improve quality of care, it is critical to develop valid quality indicators. Our objective is to describe the development of quality indicators for evaluating care of adults with epilepsy. As most care is provided in primary and general neurology care, we focused our assessment of quality on care within primary care and general neurology clinics. METHODS: We reviewed existing national clinical guidelines and systematic reviews of the literature to develop an initial list of quality indicators; supplemented the list with indicators derived from patient focus groups; and convened a 10-member expert panel to rate the appropriateness, reliability, and necessity of each quality indicator. RESULTS: From the original 37 evidence-based and 10 patient-based quality indicators, the panel identified 24 evidence-based and 5 patient-based indicators as appropriate indicators of quality. Of these, the panel identified 9 that were not necessary for high quality care. CONCLUSION: There is, at best, a poor understanding of the quality of care provided for adults with epilepsy. These indicators, developed based on published evidence, expert opinion, and patient perceptions, provide a basis to assess and improve the quality of care for this population.

Short-term outpatient EEG video with induction in the diagnosis of psychogenic seizures.

To analyze the yield of short-term outpatient EEG video monitoring, the authors reviewed data on all patients who underwent this procedure at their center. All patients were suspected of having psychogenic nonepileptic seizures (PNES) on clinical grounds. The total number of cases of short-term outpatient EEG video monitoring was 74. In 49 (66%) cases, the suspected diagnosis of PNES could be confirmed, thereby obviating the need for prolonged inpatient EEG video monitoring.

Diffusion-weighted imaging and status epilepticus during vagus nerve stimulation.

PURPOSE: Transient abnormalities have been reported on diffusion-weighted imaging (DWI) during status epilepticus. Vagus nerve stimulation (VNS) is a therapy for epilepsy that has previously demonstrated alteration in regional cerebral blood flow on functional neuroimaging. We describe the peri-ictal DWI abnormalities in a patient with status epilepticus. METHODS: A 21-year-old woman with pharmacoresistant localization-related epilepsy was treated with VNS and underwent brain magnetic resonance imaging (MRI) with DWI for clinical purposes. RESULTS: Transient and reversible hyperintense signal abnormalities were noted on DWI at the site of seizure onset, in addition to the thalamus and midbrain bilaterally. A concomitant decrease in the apparent diffusion coefficient mimicked ischemia, yet complete clinical, and electrographic resolution occurred following successful termination of status. CONCLUSIONS: High-energy brain MRI sequences using DWI were safely performed in our epilepsy patient with a vagus nerve stimulator who experienced status epilepticus. This case highlights the bilateral and robust involvement of subcortical structures present immediately following status epilepticus. Additionally, bilateral abnormalities in the thalamus and midbrain in addition to the region of seizure origin, were observed in our patient implanted with a vagus nerve stimulator. Modulation of regional cerebral blood flow is one potential mechanism of action for VNS in humans; therefore, these regions of involvement could reflect the effects of status epilepticus, activation or facilitation by VNS, or both.

Vagus nerve stimulation for pharmacoresistant epilepsy: clinical symptoms with end of service.

PURPOSE: Limited capability exists to predict when vagus nerve stimulation (VNS) battery deterioration becomes significant. Initial models last 2-5 years. We evaluated the first 18 patients with pharmacoresistent epilepsy after reimplantation to examine the clinical course observed during VNS end of service (EOS). METHODS: Of 72 patients with VNS, 18 patients had generator replacement. EOS was estimated based on duration of use and stimulus parameters in accordance with manufacturer guidelines. Eight males and ten females had pharmacoresistent epilepsy for a mean of 17.9 years. Thirteen with localization-related epilepsy (LRE) and 5 nonverbal patients with symptomatic generalized epilepsy (SGE) failed a mean of 11.1 antiepileptic drugs (AEDs) over 21.5 years. Seven had intracranial evaluations and five failed epilepsy surgery. Reimplantation was performed after a mean of 34.4 months. Symptoms at end of service (EOS) were addressed by postoperative survey submitted at initial reprogramming within 2 weeks of reimplantation. Stimulus parameters were compared before and after surgery. RESULTS: Nine of thirteen (69.2%) verbal patients and 11 of 18 (61.1%) total patients had signs or symptoms prior to replacement, suggesting clinical EOS, and 4 of 18 (22.2%) failed interrogation denoting battery failure without symptoms; however, this did not reach significance (chi2=0.359,p=0.54). Increased seizures were the most frequent sign in 8 of 18 (44.4%), with intensification in 7 of 18 (38.9%). Irregular stimulation was detected in 5 of 18 (27.7%), with less intense stimulation in 4 of 18 (22.2%). Painful stimulation and behavioral worsening each occurred in 2 of 18 (11.1%). A subjective improvement in function after reimplantation was noted in 12 of 13 (92.3%) verbal patients, with greater intensity and consistency. Maximally tolerated reimplant current averaged -0.56 mA less. All but one (94.4%) felt surgery should be performed before clinical EOS occurred. CONCLUSIONS: We conclude that clinical signs and symptoms may arise during VNS EOS and following replacement. Seizure increase or a change in seizure pattern was most frequently observed. The tolerated reimplant current was less than the preoperative output current in most cases. Battery replacement before EOS appears desirable from a patient perspective.

MRI of the brain is safe in patients implanted with the vagus nerve stimulator.

Metallic devices generally represent a contra-indication for MRI scanning. Based on laboratory testing, the neuro cybernetic prosthesis (NCP) is labelled MRI compatible when used with a send and receive head coil. However, there are no published clinical data to support the safety of brain MRI in patients with the NCP. Our objective was to report clinical experience with such a population. We questioned 40 centres that had implanted the NCP system as of 10/1/99. If MRI had been performed on any vagus nerve stimulator patients, we collected information on these patients, the MRI technique used, any events noted during the scan, including both subjective reports (by the patient ), and observable (objective) changes noted by the staff. Twelve centres (30%) responded. Over a time period of 3 years, there were a total of 27 MRI scans performed in 25 patients. All scanners were 1.5 T. A head coil was used in 26 scans, and a body coil in one. The indications for the scans were diverse. Seven were related to the epilepsy, including aetiology or pre-surgical evaluation. Others were unrelated, including brain tumours, cerebral haematoma, vasculitis, headaches, and head trauma. Three scans were performed with the stimulator on, while 24 were performed with the stimulator off. One patient had a mild objective voice change for several minutes. No other objective changes were noted in any of the patients. One 11-year old reported chest pain while experiencing severe claustrophobia. Twenty-five patients denied any discomfort around the lead or the generator. We conclude that this clinical series supports the safety of routine brain MRI using a send and receive head coil in patients implanted with the NCP System.

Long-term EEG monitoring: a clinical approach to electrophysiology.

Long-term electroencephalographic monitoring (LTM) is the capability of recording the EEG over long periods of time and not a specific duration. Prolonged EEG recording is used primarily for epilepsy monitoring, but LTM is also used in the intensive care unit, the operating room, and in the emergency department. The purpose of LTM is to expand the limited time sampling associated with shorter "routine" EEG recording. Audiovisual monitoring may also be used in conjunction with LTM to evaluate simultaneously a specific clinical behavior that may or may not be associated with EEG alteration. This is typically performed in a hospital setting for safety and ancillary testing purposes. LTM is used most frequently in the diagnosis and management of seizures and "spells," but has also gained wider application in the evaluation of sleep disorders, cerebrovascular disease, psychiatric conditions, and movement disorders. Computer-assisted LTM systems that process, analyze, compress, and store data digitally have become widely available in clinical practice both in the hospital as well as outside the hospital when the patient is ambulatory.

Postmarketing experience with topiramate and cognition.

Ideal antiepileptic drugs (AEDs) are designed to stop seizures with limited central nervous system (CNS) side effects. However, CNS-related treatment-emergent adverse events (TEAEs) often occur in patients receiving AEDs. Topiramate (TPM) is an AED proven to be safe and effective as adjunctive treatment for epilepsy patients with partial seizures. Double-blind, placebo-controlled, multicenter trials demonstrated potential effects on cognition. The P.A.D.S. (post-marketing antiepileptic drug survey) group, a cooperative group of 14 epilepsy centers that collaborate on obtaining data about new AEDs and devices, prospectively collected standardized data forms before and during treatment with TPM for epilepsy, and analyzed the postmarketing experience of CNS TEAEs with TPM. Our results from 701 treated patients show that cognitive complaints were the most common reason to discontinue TPM. The presence of complaints did have predictive value if the patient would discontinue TPM, although was not specific as to when discontinuation would occur. The spectrum of complaints in our open-label prospective multicenter postmarketing study was similar to those observed in controlled clinical trials. We were unable to demonstrate a specific population, dose titration, or concomitant AED that was at risk to discontinue treatment. We conclude that most patients treated with TPM will continue therapy beyond 6 months. Cognitive complaints and not efficacy reflect the primary reason for discontinuing therapy. Psychomotor slowing was the most common complaint, yet most patients elect to continue treatment, with "better" or "much better" ratings of both seizure and global improvement during treatment.

How many patients with psychogenic nonepileptic seizures also have epilepsy?

The proportion of patients with psychogenic nonepileptic seizures (PNES) who also have epilepsy has been reported to vary from 10% to over 50%. The authors reviewed all 32 patients diagnosed with PNES in our EEG-video monitoring unit over a period of 1 year, and only 3 (9.4%) had interictal epileptiform discharges to support a coexisting diagnosis of epilepsy. Thus, the authors believe that only a small proportion of patients with PNES have coexisting epilepsy.

Advances in the treatment of epilepsy.

Significant advances have been made in the diagnosis and treatment of epilepsy over the past decade. With the advent of electroencephalographic video monitoring, physicians are now able to reliably differentiate epilepsy from other conditions that can mimic it, such as pseudoseizures. In addition, neuroimaging has changed the way treatment for difficult epilepsy is approached. As a result, the classification systems that have been in use since the early 1980s are currently being revised. A broader range of treatment options for epilepsy is now available. Many new antiepileptic drugs have become available in recent years, including felbamate, gabapentin, lamotrigine, topiramate, tiagabine, levetiracetam, oxcarbazepine and zonisamide. These medications offer options for patients with epilepsy whose seizures cannot be controlled using the classic agents. Several classic antiepileptic drugs have been modified and reformulated. The ketogenic diet has resurfaced as a treatment option in certain types of epilepsy. The vagus nerve stimulator, approved in 1997, represents a completely new treatment modality for patients with seizures not controlled by medications. Epilepsy surgery is now a well-documented and effective treatment for some patients with intractable epilepsy.

Outpatient seizure identification: results of 502 patients using computer-assisted ambulatory EEG.

Patients with epilepsy may not always be able to identify their seizures. Epilepsy management relies on patient reporting to validate whether seizures occur during treatment. The goal of this study was to assess the frequency of unreported seizures recorded during routine outpatient ambulatory EEG recording. The authors reviewed 552 records from 502 patients who underwent outpatient 16-channel computer-assisted ambulatory EEG monitoring (CAA-EEG). Seizure identification was evaluated by assessing push-button activation. Partial seizures were seen most commonly. A total of 47 of 552 records (8.5%) had partial seizures recorded on CAA-EEG, with 29 of 47 (61.7%) with electroclinical seizures identified by push-button activation. Seizures on EEG without push-button activation were analyzed separately and compared with a self-reported written diary to verify lack of recognition. A total of 18 of 47 records (38.3%) had some partial seizures that were unrecognized by the patient, and 11 of 47 records (23.4%) had seizures recognized only by the computer. The authors conclude that patients frequently have seizures outside of the hospital that go unrecognized. Underreporting of seizure frequency occurs in the outpatient setting and impacts optimal diagnosis and treatment for patients with epilepsy.