When Drugs Do Not Work: Alternatives to Antiseizure Medications.

PURPOSE OF REVIEW: Despite the increase in the number of novel antiseizure medications over the past 20 years, approximately one-third of patients will not have adequate seizure control on medications. For these patients, additional options need to be considered, including dietary, device, and surgical treatments. In addition, many complementary therapies can be considered as adjunctive treatment, with the intent of improving quality of life for persons with epilepsy and potentially allowing for improvement in seizure control. RECENT FINDINGS: This review outlines established and developing treatments for drug-resistant epilepsy. Surgical treatments, including resective surgery and vagus nerve stimulation, have been routine care for several decades. In the last several years, new neurostimulation options have been approved (responsive neurostimulation and deep brain stimulation) or are under development (continuous subthreshold cortical stimulation). For patients with lesion or well-defined seizure-onset zones, less invasive options including laser ablation and ultrasound therapy provide the potential for faster recovery times and less morbidity. Not all therapies are in the pharmacological or surgical arenas. This review also explores the evidence for complementary treatments, including relaxation and meditation techniques, and art and music therapy. Despite the range of antiseizure medications available, they still provide inadequate for a large number of patients with epilepsy, either due to ongoing seizures or intolerable side effects. Complementary therapies, including diet, meditation techniques, and music therapy, provide compelling treatment options to improve quality of life while potentially improving seizure control. In appropriate patients, stimulation devices or surgical resection can offer options for significant seizure reduction or even cure. The full range of therapeutics should be considered for each patient with epilepsy when they are struggling with inadequate seizure control or side effects with traditional pharmacological treatment.

Usage of EpiFinder clinical decision support in the assessment of epilepsy.

BACKGROUND: The diagnosis of epilepsy is at times elusive for both neurologists and nonneurologists, resulting in delays in diagnosis and therapy. The development of screening methods has been identified as a priority in response to this diagnostic and therapeutic gap. EpiFinder is a novel clinical decision support tool designed to enhance the process of information gathering and integration of patient/proxy respondent data. It is designed specifically to take key terms from a patient's history and incorporate them into a heuristic algorithm that dynamically produces differential diagnoses of epilepsy syndromes. OBJECTIVE: The objective of this study was to test the usability and diagnostic accuracy of the clinical decision support application EpiFinder in an adult population. METHODS: Fifty-seven patients were prospectively identified upon admission to the Epilepsy Monitoring Unit (EMU) for episode classification from January through June of 2017. Based on semiologic input, the application generates a list of epilepsy syndromes. The EpiFinder-generated diagnosis for each subject was compared to the final diagnosis obtained via continuous video electroencephalogram (cVEEG) monitoring. RESULTS: Fifty-three patients had habitual events recorded during their EMU stay. A diagnosis of epilepsy was confirmed (with cVEEG monitoring) in 26 patients while 27 patients were found to have a diagnosis other than epilepsy. The algorithm appropriately predicted differentiation between the presence of an epilepsy syndrome and an alternative diagnosis with 86.8% (46/53 participants) accuracy. EpiFinder correctly identified the presence of epilepsy with a sensitivity of 86.4% (95% confidence interval [CI]: 65.0-97.1) and specificity of 85.1% (95% CI: 70.2-96.4). CONCLUSION: The initial testing of the EpiFinder algorithm suggests possible utility in differentiating between an epilepsy syndrome and an alternative diagnosis in adult patients.

Diagnostic utility of the Minnesota Multiphasic Personality Inventory-2 Restructured Form in the epilepsy monitoring unit: Considering sex differences.

Psychological assessment measures are frequently used to evaluate patients in epilepsy monitoring units. One goal of that assessment is to contribute information that may help with differential diagnosis between epilepsy and psychogenic nonepileptic seizures (PNES). The Minnesota Multiphasic Personality Inventory-2 Restructured Form (MMPI-2-RF) is one such measure. Del Bene et al. (2017) recently published an analysis that was the first to compare MMPI-2-RF scale elevations between diagnostic groups stratified by sex. The purpose of the present study was to replicate that analysis in a larger sample. Similar to previous work, we found that both men and women with PNES were more likely than men and women with epilepsy to report high levels of somatic complaints (2 to 5 times greater odds of somatic symptom reporting) and a variety of types of complaints. Mood disturbance scales were not significantly elevated in our PNES sample. Results contribute to the small body of research on sex differences in patients with PNES and suggest that somatization is a key characterization across sexes.

Quality of life in psychogenic nonepileptic seizures and epilepsy: the role of somatization and alexithymia.

It is clear that many individuals with psychogenic nonepileptic seizures (PNESs) often present with poorer quality of life compared with those with epileptic seizures (ESs). However, the mechanisms linking seizure diagnosis to quality-of-life outcomes are much less clear. Alexithymia and somatization are emotional markers of psychological functioning that may explain these differences in quality of life. In the current study, patients from an epilepsy monitoring unit with vEEG-confirmed diagnosis of PNESs or ESs were compared on measures of alexithymia, somatization, quality of life, and a variety of demographic and medical variables. Two models using alexithymia and somatization individually as mediators of the relations between diagnosis and quality of life were tested. Results indicated that patients with PNESs had significantly poorer quality of life compared with those with ESs. Alexithymia was associated with poor quality of life in both groups but did not differentiate between diagnostic groups. Further, alexithymia did not mediate the relationship between diagnosis and quality of life. Somatization was associated with poor quality of life, and patients with PNESs reported greater somatization compared with patients with ESs. Somatization also significantly mediated the relationship between diagnosis and quality of life. In conclusion, somatization may be one mechanism affecting poor quality of life among patients with PNESs compared with ESs and should be a target of comprehensive treatments for PNESs. Alexithymia proved to be an important factor impacting quality of life in both groups and should also be targeted in treatment for patients with PNESs and patients with ESs.

Fall prevention and bathroom safety in the epilepsy monitoring unit.

Falls are one of the most common adverse events occurring in the epilepsy monitoring unit (EMU) and can result in significant injury. Protocols and procedures to reduce falls vary significantly between institutions as it is not yet known what interventions are effective in the EMU setting. This study retrospectively examined the frequency of falls and the impact of serial changes in fall prevention strategies utilized in the EMU between 2001 and 2014 at a single institution. Overall fall rate was 2.81 per 1000 patient days and varied annually from 0 to 9.02 per 1000 patient days. Both seizures and psychogenic nonepileptic events occurring in the bathroom were more likely to result in falls compared with events occurring elsewhere in the room. With initiation of increased patient education, hourly nurse rounding, nocturnal bed alarms, having two persons assisting for high fall risk patients when out of bed, and immediate postfall team review between 2001 and 2013, there was a trend of decreasing fall frequency; however, no specific intervention could be identified as having a particular high impact. In late 2013, a ceiling lift system extending into the bathroom was put in place for use in all EMU patients when out of bed. In the subsequent 15 months, there have been zero falls. The results reinforce both the need for diligent safety standards to prevent falls in the EMU as well as the challenges in identifying the most effective practices to achieve this goal.

Electroencephalography in survivors of cardiac arrest: comparing pre- and post-therapeutic hypothermia eras.

Electroencephalography in the setting of hypothermia and anoxia has been studied in humans since the 1950s. Specific patterns after cardiac arrest have been associated with prognosis since the 1960s, with several prognostic rating scales developed in the second half of the twentieth century. In 2002, two pivotal clinical trials were published, demonstrating improved neurologic outcomes in patients treated with therapeutic hypothermia (TH) after cardiac arrest of shockable rhythms. In the following years, TH became the standard of care in these patients. During the same time period, the use of continuous EEG monitoring in critically ill patients increased, which led to the recognition of subclinical seizures occurring in patients after cardiac arrest. As a result of these changes, greater amounts of EEG data are being collected, and the significance of specific patterns is being re-explored. We review the current role of EEG for the identification of seizures and the estimation of prognosis after cardiac resuscitation.

Migralepsy: a borderland of wavy lines.

The special relationship between migraine and epilepsy has been recognized for centuries and was formally acknowledged by Gowers in his 1906 lecture "Borderland of Epilepsy." The term migralepsy was introduced by Lennox and Lennox in 1960, with multiple cases described in the literature since that time. In the ensuing years, the relationship between migraine and epilepsy has proven complex. The 2 conditions have been found to be comorbid with each other, suggesting a common underlying mechanism or genetic tendency. Specific diseases with both phenotypes provide further evidence of a common pathophysiology, and as the mechanism of migraine has been further elucidated, commonalities with seizure have been recognized. The terms "hemicrania epileptica" and "migraine triggered seizure" were defined by the International Headache Society, formalizing the concept that one can lead to the other. However, case reports and case series in the literature reveal that distinguishing between the 2 entities can be challenging. The concept of migralepsy is likely to evolve as greater understanding of both conditions is gained.

Comparison of electroencephalography and cerebral oximetry to determine the need for in-line arterial shunting in patients undergoing carotid endarterectomy.

OBJECTIVE: To compare cerebral near-infrared regional spectroscopy (NIRS) with the 12-lead electroencephalogram for the detection of ischemia during carotid artery clamping for carotid endarterectomy (CEA). DESIGN: Prospective, observational. SETTING: Single, tertiary care center. PARTICIPANTS: Ninety patients older than 18 undergoing elective, unilateral CEA. INTERVENTIONS: In addition to EEG monitoring, all patients underwent continuous blinded NIRS monitoring with sensors placed bilaterally above the supraorbital ridge. MEASUREMENTS AND MAIN RESULTS: Seventeen patients were excluded, leaving 73 patients available for evaluation. Four patients (5.5%) required shunting based on EEG findings. Changes in cerebral oxygen saturation (rSO2) were assessed on the operative side using the average value for the 1 minute prior to cross-clamp and the lowest rSO2 value the first 5 minutes postclamp. Each 1% absolute decrease and each 1% relative decrease from baseline conferred a 50% increase in the need for shunt placement (OR 1.5; 95% CI (1.03-2.26); p = 0.03 and OR 1.4; 95% CI (1.02-1.81); p = 0.04 respectively). Sensitivity, specificity, and positive and negative predictive values were determined using significant cutoffs of≥5% absolute change or≥10% relative change. Positive predictive value was low (<25%) for both absolute and relative changes. CONCLUSIONS: A decrease in rSO2 during carotid cross-clamping for CEA is associated with EEG-determined need for shunting, but the positive predictive value is low. Using the above cutoffs in the current series would have resulted in an increase in the shunt rate by approximately 20% when it was not indicated by EEG.

Does Initiation of Prophylactic Antiseizure Medication Improve Neurological Outcomes in Patients With Acute Intracerebral Hemorrhage?: A Critically Appraised Topic.

OBJECTIVE: The objective of this study was to critically assess current evidence regarding the role of prophylactic antiseizure medication in patients presenting with acute intracerebral hemorrhage (ICH). METHODS: The objective was addressed through the development of a structured critically appraised topic. This included a clinical scenario with a clinical question, literature search strategy, critical appraisal, results, evidence summary, commentary, and bottom-line conclusions. Participants included resident neurologists, a medical librarian, and content experts in the fields of epilepsy, stroke neurology, neurohospitalist medicine, and neurocritical care. RESULTS: A randomized clinical trial was selected for critical appraisal. The trial assessed whether prophylactic levetiracetam (LEV) use reduced the risk of acute seizures in patients with ICH, as defined by clinical or electrographic seizure, captured by continuous electroencephalogram 72 hours after enrollment. A total of 42 patients were included in the final analysis (19 in the LEV group and 23 in the placebo group). There was a significantly higher occurrence of seizures in the placebo versus LEV group (LEV 16% vs placebo 43%, P = 0.043). There were no differences in functional outcomes between the groups at 3, 6, or 12 months (P > 0.1). CONCLUSIONS: The role of prophylactic treatment with antiseizure medication in ICH remains unclear.

Brain responsive neurostimulation device safety and effectiveness in patients with drug-resistant autoimmune-associated epilepsy.

PURPOSE: This study examines the safety and effectiveness of brain-responsive neurostimulation (RNS System) therapy in patients with refractory autoimmune-associated epilepsy (AAE). METHODS: We retrospectively reviewed 85 medical records of patients who were treated with the RNS System at the three Mayo Clinic sites to identify patients with AAE. We collected clinical data including demographics, epilepsy history, prior evaluations and treatment, RNS implantation and lead information, long term ambulatory electrocorticography (ECoG) data, and patient-reported seizure details. Inclusion criteria included: (1) confirmed neural antibodies, or absent/negative autoimmune panel with Antibody Prevalence in Epilepsy (APE2) score ≥ 4 and (2) at least 6 months of follow up after RNS implantation. The primary outcomes measured were patient-reported seizure frequencies at last follow-up as compared to baseline, ECoG long-episode frequencies, and adverse events following RNS implantation. RESULTS: Of the 85 patients reviewed, nine (11 %) met the inclusion criteria. Three patients had GAD65 antibodies (mean serum titer = 816 ug/dl), one had Rasmussen's encephalitis (biopsy proven), and five had absent/negative neuronal antibody panel but APE scores were ≥ 4. Six out of nine patients (67 %) reported improvement in clinical seizure frequency, all reported improvements in seizure duration and intensity. Four of 9 patients (44 %) showed trends of decreasing frequency of prolonged periods of epileptiform activity over time. One patient (11 %) developed a superficial wound infection at the implant site. CONCLUSION: The findings from our study suggest that adjunctive treatment with the RNS System may be a safe option for patients with refractory AAE.

Safety and Management of Implanted Epilepsy Devices for Imaging and Surgery.

Permanently implanted devices that deliver electrical stimulation are increasingly used to treat patients with drug-resistant epilepsy. Primary care physicians, neurologists, and epilepsy clinicians may encounter patients with a variety of implanted neuromodulation devices in the course of clinical care. Due to the rapidly changing landscape of available epilepsy-related neurostimulators, there may be uncertainty related to how these devices should be handled during imaging procedures and perioperative care. We review the safety and management of epilepsy-related implanted neurostimulators that may be encountered during imaging and surgery. We provide a summary of approved device labeling and recommendations for the practical management of these devices to help guide clinicians as they care for patients treated with bioelectronic medicine.

Anticipating Postoperative Delirium During Burst Suppression Using Electroencephalography.

OBJECTIVE: This study develops an electro-encephalography-based method for predicting postoperative delirium early during cardiac surgeries involving deep hypothermia circulatory arrest (DHCA), potentially providing an opportunity to intervene and minimize poor surgical outcome. DHCA is a surgical technique used during cardiac surgeries to facilitate repairs. Deep hypothermia is induced and supplemented by perfusion techniques to protect the brain during circulatory arrest, but concern for cerebral injury still remains. METHODS: This research studies whether or not monitoring burst suppression, an electrophysiological phenomenon observed during patient cooling and warming, helps in predicting postoperative delirium, a correlate of poor prognosis. A metric called the burst suppression duty cycle (BSDC), akin to burst suppression ratio, is formulated to characterize this electrophysiological activity. RESULTS: Assuming no complications occur prior to circulatory arrest, delirium diagnoses are correlated with the time elapsed until suppression activity ceases since resuming cardiopulmonary bypass. By comparing against a benchmark the times when BSDC reaches 100%, 15 of 16 cases can be correctly predicted. Similar accuracy can be achieved when querying BSDC progress earlier during warming. CONCLUSION: Our results show that our BSDC metric is a promising candidate for early detection of postoperative delirium, and motivates further analysis of the causal relationship between postoperative delirium and the procedure transitioning out of circulatory arrest. SIGNIFICANCE: The developed methodology anticipates incidences of postoperative delirium during rewarming, which potentially provides an opportunity to intervene and avert it.

Treatment of Status Epilepticus: What Are the Guidelines and Are We Following Them?

OBJECTIVES: Status epilepticus (SE) is associated with high morbidity and mortality. The American Epilepsy Society (AES) has established guidelines regarding proper dosing of antiepileptic drugs (AEDs). There are also recommendations for appropriate weaning off anesthetic agents in SE. Our aim was to determine the frequency with which guidelines were being followed during routine clinical care. METHODS: A retrospective chart review was completed for patients with a diagnosis of SE within the last 10 years. All patients with SE on anesthetic agents were included when analyzing AED dosing. Patients with anoxic brain injury were excluded when reviewing anesthetic wean times. RESULTS: Seventy-six patients were identified on anesthetic agents. Five patients had anoxic brain injury and were excluded from the anesthetic weaning analysis. Medications were under dosed as follows: lorazepam 0.05 mg/kg (N=51), midazolam 0.07 mg/kg (N=14), fosphenytoin 17.9 mg/kg (N=47), levetiracetam 21.7 mg/kg (N=42), valproate sodium 16.7 mg/kg (N=12), phenobarbital 4.1 mg/kg (N=4), lacosamide 215 mg (N=13), and topiramate 333 mg (N=3). Anesthetic agents were weaned as follows: <24 hours 32/71 (45%), 24 to 48 hours 11/71 (15%), 48 to 72 hours 12/71 (17%), and >72 hours 11/71 (15%). Seizure recurrence rates were: 8/32 (25%) <24 hours, 2/11 (18%) 24 to 48 hours, 1/12 (8.3%) 48 to 72 hours, and 1/11 (9%) at >72 hours. CONCLUSIONS: This retrospective review demonstrates how guidelines and recommendations for SE are not consistently followed. Under dosing of AEDs and aggressive weaning of anesthetic agents may result in higher morbidity and mortality.

EEG in WNV Neuroinvasive Disease.

PURPOSE: Neuroinvasive West Nile virus (WNV) is rare, occurring in less than 1% of those infected, and may manifest as meningitis, encephalitis, and/or acute flaccid paralysis. Patients may present initially with nonspecific symptoms including fevers. Although rare, neuroinvasive WNV is associated with significant morbidity and mortality. The mainstay of treatment is supportive care. Electroencephalography (EEG) allows for identification of nonconvulsive status epilepticus and other epileptiform and nonepileptiform patterns suggestive of underlying cognitive dysfunction. Our aim was to describe specific EEG patterns observed in WNV neuroinvasive disease. METHODS: A retrospective chart review was conducted. West Nile virus was confirmed with serum and/or cerebrospinal fluid markers. Patients with a history of abnormal EEG were excluded. Electroencephalography reports were classified into categories based on the presence of epileptiform activity, focal slowing, generalized periodic discharges with triphasic morphology, and frontally predominant generalized rhythmic delta activity. RESULTS: In our cohort of 34 patients, 60% of focal EEG abnormalities were anterior-predominant, seen as epileptiform discharges, focal slowing, or frontally predominant generalized rhythmic delta activity. Nonepileptiform EEG patterns included nonspecific slowing and generalized periodic discharges with triphasic morphology. Two patients had electrographic seizures, one arising from the frontocentral head region. CONCLUSIONS: EEGs are important in the evaluation of WNV infection to rule out seizures or alternative causes of encephalopathy, and because of the risk of nonconvulsive seizures or status epilepticus in encephalitis. Although an anterior predominance of EEG abnormalities was seen in our cohort, this most likely is more correlative to encephalopathy than WNV itself. Although a specific correlative EEG pattern may not accompany all cases of WNV neuroinvasive disease, WNV should be considered as a possible etiology in patients presenting with an encephalitic or meningitic syndrome in the presence of abnormal EEG findings including encephalopathic patterns, particularly those with anterior predominant EEG changes.

Neuroendocrine aspects of improving sleep in epilepsy.

Sleep plays an intricate role in epilepsy and can affect the frequency and occurrence of seizures. With nearly 35% of U.S. adults failing to obtain the recommended 7 h of sleep every night, understanding the complex relationship between sleep and epilepsy is of utmost relevance. Sleep deprivation is a common trigger of seizures in many persons with epilepsy and sleep patterns play a role in the occurrence of seizures. Some patients have their first seizure or repeated seizures after an "all-nighter" at college or after a long period of chronic sleep deprivation. The strength of the relationship between sleep and seizures varies between patients, but improving sleep and optimizing seizure control can have significant positive effects on the quality of life for all these patients. Research has shown that the changes in the brain's electrical and hormonal activity occurring during normal sleep-wake cycles can be linked to both sleep and seizure patterns. Many questions remain to be answered about sleep and epilepsy. How can sleep deprivation trigger an epileptic seizure? How do circadian and hormonal changes influence sleep pattern and seizure occurrence? Can hormones or sleeping pills help with sleep in epilepsy? In this article we discuss these and many other questions on sleep in epilepsy, with an emphasis on sleep architecture, hormone changes, mechanistic factors, and possible prevention strategies.

Is Adjunctive Progesterone Effective in Reducing Seizure Frequency in Patients With Intractable Catamenial Epilepsy? A Critically Appraised Topic.

BACKGROUND: Catamenial epilepsy refers to cyclic seizure exacerbation in relation to the menstrual cycle. Three distinct patterns have been described: C1-perimenstrual, C2-periovulatory, and C3-inadequate luteal. There is experimental and clinical evidence that gonadal steroid hormones affect neuronal excitability with estrogens being mainly proconvulsant and progesterone anticonvulsant. If reproductive steroids have a role in seizure occurrence, they may also have a role in treatment. OBJECTIVE: The objective of this study was to critically assess current evidence regarding the efficacy of progesterone as adjunctive therapy in women with intractable catamenial epilepsy. METHODS: The objective was addressed through the development of a structured critically appraised topic. This included a clinical scenario with a clinical question, literature search strategy, critical appraisal, results, evidence summary, commentary, and bottom line conclusions. Participants included consultant and resident neurologists, medical librarian, and content experts in the fields of epilepsy and gynecology. RESULTS: A randomized, placebo-controlled clinical trial was selected for critical appraisal. This trial compared the efficacy of adjunctive cyclic natural progesterone therapy versus placebo for seizures in women with intractable partial epilepsy, stratified by catamenial and noncatamenial status. There was no significant difference in proportions of responders between progesterone and placebo in the catamenial and noncatamenial strata. Prespecified secondary analysis showed that the level of perimenstrual seizure exacerbation is a significant predictor of the responder rate for progesterone therapy. CONCLUSIONS: Cyclic natural progesterone is not superior to placebo in reducing seizure frequency in women with intractable partial epilepsy. Posthoc findings suggest that progesterone may benefit a subset of women with perimenstrually exacerbated seizures.

Laser ablation for mesial temporal epilepsy: a multi-site, single institutional series.

OBJECTIVEAlthough it is still early in its application, laser interstitial thermal therapy (LiTT) has increasingly been employed as a surgical option for patients with mesial temporal lobe epilepsy. This study aimed to describe mesial temporal lobe ablation volumes and seizure outcomes following LiTT across the Mayo Clinic's 3 epilepsy surgery centers.METHODSThis was a multi-site, single-institution, retrospective review of seizure outcomes and ablation volumes following LiTT for medically intractable mesial temporal lobe epilepsy between October 2011 and October 2015. Pre-ablation and post-ablation follow-up volumes of the hippocampus were measured using FreeSurfer, and the volume of ablated tissue was also measured on intraoperative MRI using a supervised spline-based edge detection algorithm. To determine seizure outcomes, results were compared between those patients who were seizure free and those who continued to experience seizures.RESULTSThere were 23 patients who underwent mesial temporal LiTT within the study period. Fifteen patients (65%) had left-sided procedures. The median follow-up was 34 months (range 12-70 months). The mean ablation volume was 6888 mm3. Median hippocampal ablation was 65%, with a median amygdala ablation of 43%. At last follow-up, 11 (48%) of these patients were seizure free. There was no correlation between ablation volume and seizure freedom (p = 0.69). There was also no correlation between percent ablation of the amygdala (p = 0.28) or hippocampus (p = 0.82) and seizure outcomes. Twelve patients underwent formal testing with computational visual fields. Visual field changes were seen in 67% of patients who underwent testing. Comparing the 5 patients with clinically noticeable visual field deficits to the rest of the cohort showed no significant difference in ablation volume between those patients with visual field deficits and those without (p = 0.94). There were 11 patients with follow-up neuropsychological testing. Within this group, verbal learning retention was 76% in the patients with left-sided procedures and 89% in those with right-sided procedures.CONCLUSIONSIn this study, there was no significant correlation between the ablation volume after LiTT and seizure outcomes. Visual field deficits were common in formally tested patients, much as in patients treated with open temporal lobectomy. Further studies are required to determine the role of amygdalohippocampal ablation.

Management of Adult Onset Seizures.

Epilepsy is a common yet heterogeneous disease. As a result, management often requires complex decision making. The ultimate goal of seizure management is for the patient to have no seizures and no considerable adverse effects from the treatment. Antiepileptic drugs are the mainstay of therapy, with more than 20 medications currently approved in the United States. Antiepileptic drug selection requires an understanding of the patient's epilepsy, along with consideration of comorbidities and potential for adverse events. After a patient has failed at least 2 appropriate antiepileptic drugs, they are determined to be medically refractory. At this time, additional therapy, including dietary, device, or surgical treatments, need to be considered, typically at a certified epilepsy center. All these treatments require consideration of the potential for seizure freedom, balanced against potential adverse effects, and can have a positive effect on seizure control and quality of life. This review article discussed the treatment options available for adults with epilepsy, including medical, surgical, dietary, and device therapies.

Spectrum of Autonomic Nervous System Impairment in Sjögren Syndrome.

OBJECTIVE: To describe the spectrum of autonomic dysfunction in a uniformly evaluated cohort of patients with Sjögren syndrome. METHODS: A series of 13 patients underwent a comprehensive evaluation for suspected autonomic impairment, including a neurological examination, autonomic testing, and laboratory studies. A diagnosis of Sjögren syndrome was established as the cause of autonomic dysfunction in all. Clinical features, findings on autonomic testing, and laboratory results are described. RESULTS: All patients in this series reported postural lightheadedness and syncope or near-syncope. Autonomic testing confirmed the presence of orthostatic hypotension on tilt-table testing in 5 patients and an excessive postural tachycardia and/or hypertensive response in 8 patients. Only 2 of the patients with orthostatic hypotension had a significant sensory neuropathy. Symptoms suggestive of gastrointestinal and genitourinary impairment were seen in nearly all patients, with abnormal motility testing (most frequently esophageal dysmotility) in 5 of 6 patients who underwent formal testing. Patients in this series treated with immune-modulating therapy experienced significant improvement. CONCLUSIONS: A diagnosis of Sjögren syndrome should be aggressively pursued in patients with signs and symptoms suggestive of autonomic nervous system impairment. Although the spectrum of adrenergic failure is variable, ranging from orthostatic hypotension to an excessive postural tachycardia, most patients do have symptoms of more generalized autonomic failure. Patients who were treated with immune-modulating therapy did improve.