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.

Peri-ictal SPECT and surgical treatment for intractable epilepsy related to schizencephaly.

The authors evaluated four patients with schizencephaly who underwent subtraction ictal SPECT coregistered to MRI (SISCOM) prior to epilepsy surgery. Three patients had a SISCOM alteration that was concordant with the epileptic brain tissue. Two of these patients were rendered seizure-free and one individual experienced a significant reduction in seizures. The patient with an indeterminate SISCOM had an unfavorable outcome. SISCOM is useful in evaluating patients with schizencephaly for epilepsy surgery.

Is there a neurologist on this flight?

OBJECTIVE: To analyze the frequency of neurologic events during commercial airline flights and to assess whether onboard emergency medical kits are adequate for in-flight neurologic emergencies. METHODS: Collaboration of the Mayo Clinic's Departments of Emergency Medicine and Medical Transportation Service and the Division of Aerospace Medicine to provide real-time in-flight consultation to a major US airline that flies approximately 10% of all US passengers. We analyzed all medical events reported from 1995 to 2000 in a database that catalogs the air-to-ground medical consultations. All cases with potential neurologic symptoms were reviewed and classified into various neurologic symptom categories. The cost of diversion for each neurologic symptom was calculated and then extrapolated to assess the cost of neurologic symptoms to the US airline industry. RESULTS: A total of 2,042 medical incidents led to 312 diversions. Neurologic symptoms were the single largest category of medical incidents, prompting 626 air-to-ground medical calls (31%). They caused 34% of all diversions. Dizziness/vertigo was the most common neurologic symptom followed by seizures, headaches, pain, and cerebrovascular symptoms. Whereas seizures and dizziness/vertigo were the most common reasons for diversion, loss of consciousness/syncope was the complaint most likely to lead to a diversion. The estimated annual cost of diversions due to neurologic events is almost 9,000,000 dollars. CONCLUSION: Neurologic symptoms are the most common medical complaint requiring air-to-ground medical support and are second only to cardiovascular problems for emergency diversions and their resultant costs to the US airline industry. Adding antiepileptic drugs to the onboard medical kit and greater emergency medical training for in-flight personnel could potentially reduce the number of diversions for in-flight neurologic incidents.

Early versus late antiepileptic drug withdrawal for people with epilepsy in remission.

BACKGROUND: Antiepileptic drugs ( AEDs) are used to prevent seizures but are associated with both short and long term adverse effects. When epilepsy is in remission, it may be in the patient's best interest to discontinue medication. However, the optimal timing of AED discontinuation is not known. OBJECTIVES: To quantify seizure relapse risk after early (less than two seizure free years) versus late (more than two seizure free years) AED withdrawal in adult and pediatric epilepsy patients. To assess which variables modify the risk of seizure recurrence. SEARCH STRATEGY: We searched the Cochrane Epilepsy Group trials register, the Cochrane Controlled trials register (Cochrane Library Issue 4, 2000), MEDLINE (January 1996 to January 2001), EMBASE, Index Medicus, CINAHL, as well as hand-searching of journals. SELECTION CRITERIA: Randomized controlled trials that evaluate withdrawal of AEDs after varying periods of seizure remission in adult and pediatric epilepsy patients with or without blinding were included. Included studies compared an early versus late AED discontinuation. DATA COLLECTION AND ANALYSIS: Two reviewers independently extracted data and assessed trial quality. Relative risks with 95% confidence intervals were calculated for each trial. Summary relative risks and 95% confidence intervals for dichotomous data were calculated using a random effects model. A test of statistical heterogeneity was conducted for each pooled relative risk calculation. MAIN RESULTS: Seven eligible controlled trials were included in the analysis representing 924 randomized pediatric patients. There were no eligible trials evaluating adult seizure free patients. The pooled relative risk for seizure relapse in early versus late AED withdrawal was 1.32 (95% confidence interval 1.02 to 1.70). On the basis of this estimate, the number needed to harm, that is expose an individual to a higher risk of seizure relapse because of early withdrawal of AED, is 10. Early discontinuation was associated with greater relapse rates in patients with partial seizures[pooled RR = 1.52; 95% confidence interval 0.95 to 2.41] or an abnormal EEG [pooled RR=1.67; 95% confidence interval 0.93 to 3.00]. REVIEWER'S CONCLUSIONS: There is evidence to support waiting for at least two or more seizure free years before discontinuing AEDs in children, particularly if individuals have an abnormal EEG and partial seizures. There is insufficient evidence to establish when to withdraw AEDs in pediatric patients with generalized seizures. There is no evidence to guide the timing of withdrawal of AEDs in adult seizure free patients. Further blinded randomized controlled trials are needed to identify the optimal timing of AED withdrawal and risk factors predictive of relapse.

Musical hallucinations associated with seizures originating from an intracranial aneurysm.

Hallucinations are defined as sensory phenomena in the absence of external sensory stimuli. Auditory hallucinations have been shown to arise from many different intracranial lesions, but seizures manifesting as musical hallucinations triggered by unruptured intracranial aneurysms are rare. We present a case of persistent, episodic musical hallucinations associated with seizures that led to the discovery of 2 small intracranial aneurysms. Typical electroencephalographic findings for seizure activity were observed but resolved after surgical clipping of the aneurysms. Concomitantly, the patient's hallucinations resolved. The literature on musical hallucinations is reviewed.

Acute and chronic seizures in patients older than 60 years.

As the number of older adults continues to increase, the population at risk for neurologic illnesses also rises. Acute and chronic seizures are commonly seen in adults older than 60 years; however, no consensus exists on the best way to diagnose and treat seizures and epilepsy in this age group. Several new medical and surgical therapies are now available for all epilepsy patients, some of which may be especially helpful for older patients. This article reviews the current literature on the diagnosis and management of seizures in older adults. Unique aspects of the epidemiology, etiology, diagnosis, and medical and surgical treatments of acute and chronic seizures in patients older than 60 years are discussed.

Temporal lobectomy outcome in older versus younger adults.

A total of 340 patients age 50 years and older were compared with 30 patients younger than 50 years, all of whom had anterior temporal lobectomy for refractory epilepsy. Seizure outcome, neuropsychological test scores, and change in driving status were analyzed. Age and duration of epilepsy were related independently to outcome, but laterality of interictal sharp waves (an early epilepsy risk factor) and presence of tumor were not. Sixteen patients (52%) in the older group and 257 patients (75.6%) in the younger group (p < 0.008) were seizure free. Postoperative neuropsychological outcome and driving status were similar in older and younger patients.

Vagus nerve stimulation therapy for epilepsy in older adults.

The authors assessed the efficacy, safety, and tolerability of vagus nerve stimulation (VNS) for refractory epilepsy in 45 adults 50 years of age and older. They determined seizure frequency, adverse effects, and quality of life. At 3 months, 12 patients had a >50% decrease in seizure frequency; at 1 year, 21 of 31 studied individuals had a >50% seizure decrease. Side effects were mild and transient. Quality of life scores improved significantly with time.

Physical activity and epilepsy: what are the rules?

Exercise has important benefits for patients who have epilepsy, but several factors must be considered when making activity recommendations. Seizures during sports activity are rare, and exercise may have anti-epileptic effects acutely, but physicians, coaches, and parents should know what to do if a seizure occurs. Many sports activities are permissible as long as patients avoid overexertion, dehydration, and hypoglycemia. Some antiepileptic drugs may adversely affect sports performance, and exercise in turn may reduce serum drug levels by increasing circulating liver enzymes.

Psychogenic nonepileptic seizures: theoretic and clinical considerations.

OBJECTIVE: The psychogenic nonepileptic seizure (PNES) is a common problem that occurs in 10% to 20% of children and 10% to 58% of adults referred to epilepsy centers. Despite the high prevalence of this condition, the magnitude of this public health problem and its impact on quality of life are not appreciated. Moreover, there is no clear consensus regarding classification, diagnosis, or management of PNES. Therefore, this article reviews the current literature on the clinical and theoretical aspects of PNES. DESIGN/METHOD: The medical literature was reviewed for all articles pertaining to pathophysiology, diagnosis, and treatment of PNES. RESULTS: Epidemiology, etiology, and diagnosis, and the use of diagnostic tests (including electroencephalogram and laboratory tests) are discussed. Current therapies and their efficacies are presented as well. CONCLUSIONS: Psychogenic nonepileptic seizures are much more common than previously known. There are several clinical and laboratory tools that may aid in confirming the diagnosis of PNES. Despite the advances in the diagnosis of PNES, there is no clear agreement as to the best treatment plan for PNES patients.

Epilepsy in older adults: causes, consequences and treatment.

OBJECTIVES: Seizures and epilepsy are common problems in older adults. Although, the highest incidence of seizures and epilepsy occurs in individuals more than 65 years of age, the magnitude of this public health problem, and its consequences on the quality of life of older adults, are not appreciated. Moreover, there is no consensus on the most appropriate way to diagnose or manage epilepsy in this population. This report reviews the current literature on all aspects of epilepsy in older people. DESIGN/METHODS: The medical literature was reviewed for all articles pertaining to pathophysiology, diagnosis, and treatment of epilepsy in older people. RESULTS: Epidemiology, etiology, diagnosis, use of diagnostic tests including EEG and imaging, new medical and surgical treatments, and psychosocial issues as they relate to older epilepsy patient are discussed. Several questions that merit future systematic investigation are presented. CONCLUSION: Seizures and epilepsy in older people are much more common than is generally known. There are unique issues related to this population with regard to diagnosis and treatment. Several new medical and surgical therapies are now available for all epilepsy patients, some of which may be helpful for the older epilepsy patient.

Outcome after temporal lobectomy in bilateral temporal lobe epilepsy.

We determined how noninvasive presurgical data relate to prognosis after temporal lobectomy in patients with independent bilateral temporal lobe (IBTL) complex partial seizures on the intracranial electroencephalogram (EEG). Between 1986 and 1994, 28 patients had IBTL seizures on intracranial EEG. Fifteen of these 28 patients underwent temporal lobectomy and 13 were not offered surgery. Of the 15 patients who had surgery, 10 patients became seizure-free. Magnetic resonance imaging (MRI) and the Wada test were the only variables associated with a seizure-free outcome. Seven of 10 seizure-free patients had a lateralized Wada result or the presence of unilateral hippocampal sclerosis, whereas none of the patients with persistent seizures had either of these findings. Variables not found to be predictive of a seizure-free outcome included location of scalp interictal spikes, degree of seizure-onset laterality, presence of early epilepsy risk factor, duration of epilepsy, and full-scale intelligence quotient. We conclude that MRI and the Wada test provide information of prognostic value in patients with bilateral temporal seizures independent of intracranial EEG data.

New antiepileptic drugs. Overcoming the limitations of traditional therapy.

Several new antiepileptic drugs have become available recently. Since seizures and epilepsy are common, primary care physicians are likely to encounter a patient who is taking one of these new medications. Successful medical management of epilepsy requires a proper understanding of medication half-life, indications, and side effects. Felbamate has a broad spectrum of efficacy but is limited by side effects and idiosyncratic reactions. Fosphenytoin has the efficacy of phenytoin and offers the advantage of intramuscular and intravenous dosing without the significant adverse effects associated with intravenous phenytoin; however, it is expensive. Gabapentin has minimal side effects and drug interactions yet has limited efficacy for seizures. Lamotrigine has broad seizure efficacy but requires a slow adjustment to therapeutic levels. Topiramate has minimal drug interactions, but therapy must be initiated slowly to avoid side effects. All of the new antiepileptics hold great promise in the management of patients with recurrent seizures.

Seizures in temporal lobe epilepsy: I. Reliability of scalp/sphenoidal ictal recording.

We determined both (1) the reliability (reproducibility) of seizure onset location in patients with temporal lobe recorded with extracranial EEG and (2) if interictal spike (IIS) location influences that reliability. EEGs were recorded with the 10-20 system and sphenoidal electrodes. Between 1985 and 1993, 166 patients with suspected temporal lobe epilepsy were eligible for inclusion with a total of 734 seizures recorded. Each seizure onset was classified as either localized, lateralized, or nonlocalized. Individual patients were then grouped according to interictal spike location (i.e., unilateral, bilateral, or none) as well as by ictal onset reproducibility patterns (i.e., concordant, semiconcordant, conflicting, or nonlocalized) based on each patient's monitoring session. Seizure onset location was reproducible in 68% of the cases and variable patterns of seizure onset were seen in 32% of patients. Patients with unilateral IIS (68%) were more likely to have consistent ictal onset patterns than patients with bilateral IIS (27%). The first seizure more often predicted subsequent seizure onset patterns when it was localized than when it was nonlocalized. Of 18 patients with conflicting temporal lobe seizures, up to four seizures were necessary to detect independent bilateral temporal lobe seizures.

Significance of simple partial seizures in temporal lobe epilepsy.

We determined how localization of simple partial seizures (SPS) correlated with localization of complex partial seizure (CPS) in scalp/sphenoidal EEG and assessed prognosis after temporal lobe resective surgery in patients with an ictal correlate of SPS in scalp/sphenoidal EEG recordings. EEGs were recorded with the 10-20 system of electrode placement and supplemented with sphenoidal electrodes. Between 1985 and 1992, 183 patients with temporal lobe epilepsy (TLE) reported an aura (SPS) during inpatient monitoring; all were eligible for inclusion in our study. The EEGs during SPS showed ictal changes in 51 patients (28%, 117 SPS). Forty-four patients had unilateral temporal interictal spikes (IIS), and SPS and CPS always arose from the same region. Seven patients had bitemporal interictal spikes; SPS colocalized with CPS in 4 patients (57%), SPS were contralateral to CPS in 2 patients, and 1 patient had bilateral independent CPS but unilateral SPS. SPS accompanied by EEG ictal changes conveyed a favorable prognosis in patients who underwent epilepsy surgery. Scalp/sphenoidal recorded IIS but were less reliable in identifying the location of CPS onset in patients with bitemporal spikes.

Localization of subclinical ictal activity by functional magnetic resonance imaging: correlation with invasive monitoring.

Functional magnetic resonance imaging (fMRI) with susceptibility-based contrast was used to detect focal changes in cerebral blood flow and metabolism in a patient with focal epilepsy. The patient presented with frequent partial motor seizures involving his right lower face that spread to produce speech arrest and occasionally right arm jerking. Consciousness was never impaired during these events. A multislice echoplanar technique was used to acquire 16 contiguous axial slices every 4 seconds for 11 minutes. Although no overt seizures were observed or reported by the patient during the scanning, a time series analysis of the functional data revealed focal signal-intensity changes in the posterior left frontal lobe, which correlated well both in duration and spatial localization with ictal activity subsequently recorded by invasive electrophysiological monitoring. The spatial localization of fMRI was more accurate than electroencephalography recorded from a subdural grid in predicting the site of successful surgical therapy. These results illustrate the potential of functional MRI for localizing seizure foci with high spatial and temporal resolution. Such studies can be readily combined with high-resolution anatomical imaging, task-activation studies, and other magnetic resonance techniques.