Hypergraphia and the diagnosis of psychogenic attacks.
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Publications and source records attributed to Selim R Benbadis.
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OBJECTIVE: The goal of this study was to estimate the frequency of psychogenic pseudosyncope in patients with "syncope of unknown origin." Twenty to thirty percent of patients referred to epilepsy centers for refractory seizures have psychogenic seizures. With syncope, about 20-30% of the cases remain unexplained after a complete evaluation, but, unlike in seizures, a psychogenic etiology is not usually investigated. METHODS: We prospectively evaluated patients referred to our epilepsy center for evaluation of recurrent syncope-like episodes, that is, limp, motionless fainting. All patients had a negative syncope workup. We performed EEG-video monitoring with activation by suggestion ("induction"), similar to what is used for diagnosis of psychogenic seizures. Activation was performed with patients standing or sitting up. The diagnosis of psychogenic pseudosyncope required: (1) an activation procedure that triggered the habitual event; (2) a clinical event of loss of postural tone and limp, motionless unresponsiveness with eyes closed; (3) normal EEG before, during, and after the clinical event, that is, no epileptiform abnormalities, a normal alpha rhythm during unresponsiveness, and no suppression of background or slowing as is typically seen in syncope. RESULTS: Ten patients were recruited over an 18-month period. Habitual syncope-like episodes were triggered in 9 of 10 (90%) patients, and all 9 were shown to have psychogenic pseudosyncope (eyes closed, motionless, unresponsive with normal EEG including normal alpha rhythm). In one patient, no episode was triggered, so a diagnosis could not be made. Among the 9 patients for whom episodes were recorded, age ranged from 21 to 60 (mean=36). Five were women. Duration of symptoms ranged from 6 months to 15 years (mean=4.2 years). Event frequency ranged from four per day to two per month. Prior evaluations for syncope included ECG in all patients, two-dimensional echocardiogram in three, Holter monitoring in two, and tilt-table test in five. Four patients had undergone cardiac catheterization, and one had received a pacemaker. Neurologic tests included CT of the head in seven and MRI of the brain in eight. CONCLUSION: Many patients with "syncope of unknown origin" may have psychogenic pseudosyncope, but most such patients do not undergo EEG-video monitoring, which is the only way to demonstrate a psychogenic etiology. Psychogenic pseudosyncope is not simply a diagnosis of exclusion, and can be firmly diagnosed. As is usually recommended for seizure-like events, patients with syncope-like events and a negative evaluation should undergo EEG-video monitoring with induction, specifically looking for a possible psychogenic etiology.
Psychogenic non-epileptic seizures (PNES) are common, but have not been well studied in older patients. We retrospectively reviewed patients with PNES who were 60 years of age and above at the time of "seizure" onset. Over a 27-month period, nine (9.6%) of 94 patients diagnosed with PNES had onset at age 60 or above. None had evidence for coexisting epilepsy. Eight were women. Characteristics of the group were comparable to younger patients with PNES. We conclude that PNES should be considered as a diagnostic possibility in older patients.
A thorough understanding of a normal EEG is critical in defining those patterns that are abnormal. Because EEG is unique in the ability to support a clinical diagnosis of epilepsy, epileptiform patterns merit careful consideration. Certain benign patterns maybe epileptiform, yet can occur in healthy individuals without epilepsy. Understanding normal EEG and the benign variants will help to minimize over-interpretation and possibly avoid overtreatment of patients during routine clinical practice.
Nonepileptic seizures are episodes that resemble seizures but are not epileptic. The importance of EEG in the diagnosis of NES is that misread (overread) EEGs are an important contributor to the misdiagnosis of epilepsy. About 20% to 30% of patients with refractory "seizures" seen at epilepsy centers have been misdiagnosed, and the vast majority have psychogenic nonepileptic seizures (PNES). Many such patients have had previous EEGs interpreted as epileptiform. These misdiagnoses based on EEG are easily perpetuated, complicate management, and adversely affect outcome. The reasons for the overinterpretation of EEGs include the common misconception that phase reversals indicate abnormalities and not applying strict criteria to make sharp transients epileptiform. The diagnosis of PNES typically begins with a clinical suspicion and then is confirmed with EEG-video monitoring. However, ictal EEG may be negative in some partial seizures and may be uninterpretable because of artifacts. Movements can generate rhythmic artifacts that mimic an electrographic seizure. Analysis of the ictal semiology (i.e., video) is at least as important as the ictal EEG. Provocative techniques, activation procedures, or "inductions" can also be useful for the diagnosis of PNES.
Psychogenic symptoms are common and pose an uncomfortable challenge. Among psychogenic symptoms, psychogenic nonepileptic seizures (PNES) are common and have been extensively studied. They are unique in that, unlike most other psychogenic symptoms, they can be diagnosed with near certainty. PNES can be used as a model, as almost everything that applies to PNES applies to other psychogenic symptoms. According to DSM-IV, somatic symptoms are the main manifestation of three groups of disorders: somatoform disorders, factitious disorder, and malingering. Treatment is challenging. Unfortunately, psychogenic symptoms tend to be neglected. For example, the American Psychiatric Association has abundant written patient education material available on diverse topics, but none on somatoform disorders. Psychogenic symptoms are also not the subject of much clinical research. A search of the journal Neurology for 1994-2003 for the word psychogenic in the title found 21 articles, only 4 of which on topics other than psychogenic seizures. A similar search for original articles in the New England Journal of Medicine found no articles with psychogenic in the title and two with psychogenic in the abstract. Thus, there seems to be a severe disconnect between the frequency of the problem and the amount of attention devoted to it.
The clinical suspicion for psychogenic nonepileptic seizures (PNES) is based on multiple features obtained in the history. We reviewed the records of all patients evaluated over 5 years in a single epilepsy clinic for refractory seizures who eventually underwent EEG/video monitoring. We designated two groups: (1) patients with a diagnosis of "fibromyalgia" or "chronic pain" and (2) patients who had a seizure during the visit, either in the waiting area or in the examining room. Of 36 patients with "fibromyalgia" or "chronic pain," 27 (75%) were found to have PNES. Of 13 patients who had a "seizure" during their clinic visit, 10 (75%) were found to have PNES. We conclude that each of these findings has a predictive value of 75%.
The idiopathic generalized epilepsies (IGE) are a group of epilepsies that are genetically determined, have no structural or anatomic cause, and usually begin early in life. Neurologic examination, intelligence, and imaging studies are normal, and EEG shows only epileptiform abnormalities (i.e., no abnormal slow activity or evidence for diffuse encephalopathy). In some IGE, the genetic substrate has been identified, whereas in most, it remains unknown. Depending on the age at onset and predominant seizure type, individual subtypes of IGE (syndromes) are defined. However, overall, there are more similarities than there are differences among the various subtypes, and the IGE are best viewed as a spectrum or continuum of conditions. In general, IGE respond to treatment, with 80-90% becoming fully controlled. However, not all antiepileptic drugs (AED) are equally effective in IGE. Some AED are ill advised because they either do not work or exacerbate seizure types other than generalized tonic-clonic (GTC) seizures, that is, absence and myoclonic seizures. These include carbamazepine, oxcarbazepine, phenytoin, gabapentin, and tiagabine. Their use is the main cause of "pseudo-intractability," and at least in the United States where PHT and CBZ are the most commonly used AEDs, patients with IGE are often on inadequate medications. For patients with clear IGE, the drug of choice is generally valproic acid because it effectively controls absence myoclonic seizures and GTC seizures. Second-line drugs (when first-line drugs fail or are not tolerated) may include benzodiazepines, but the use of second-line drugs is evolving rapidly. Some of the newer AEDs are considered broad spectrum, meaning that they work in IGE and focal epilepsies, although the evidence is largely preliminary at this point. These newer AEDs include lamotrigine, topiramate, levetiracetam, and zonisamide.
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Epilepsy is a condition of the central nervous system that is characterized by recurrent seizures. The goal of management is to make patients seizure free without intolerable adverse effects from treatment. Men and women differ in their physiologic makeup and therefore have different needs that must be considered when attempting to attain this goal. There are special concerns for women of child-bearing years with regard to contraception, pregnancy, and teratogenicity that should be considered during counseling and selection of appropriate treatment. There are also emerging concerns about the interaction of antiepileptic drugs and endocrine function that can affect ovarian function, induce polycystic ovary (PCO)-like syndrome, and threaten fertility. Systemic adverse effects can have a negative impact on weight, cosmetic appearance, sexual function, and bone health. Individualized treatment coupling antiepileptic drug use and the specific phase of impact of the reproductive cycle must be considered in treatment selection. Important concerns regarding long-term therapy are being raised as there are more treatment options to consider because of the plethora of new antiepileptic drugs that are available, often with more favorable pharmacokinetics and different adverse event profiles. Also, sex hormone fluctuations during maturation may exacerbate seizures at particular points during the life cycle for women, including menarche, during menses, during pregnancy, or later in the perimenopausal years, often presenting a uniquely challenging aspect to treatment. As the number of available treatment options for epilepsy increases, the optimal goal for primary care physicians is to work as a team with obstetricians, gynecologists, and neurologists in an effort to ensure the best treatment of women with epilepsy.
To investigate a possible relationship between seizure frequency and the lunar cycle, we reviewed the occurrence of seizures recorded in our epilepsy monitoring unit over a 3-year period. Analysis of the total number of seizures (epileptic plus nonepileptic) showed no significant association. A separate analysis revealed that for nonepileptic seizures, there was an increase at the full moon, and for epileptic seizures, an increase in the last quarter. We conclude that there is no "full moon" effect on seizures as a whole, although there is a possible effect on nonepileptic seizures.
PURPOSE: When seizures do not respond to medications, video-EEG monitoring is the best available diagnostic tool and is the principal activity of epilepsy centers. The purpose of this study was to analyze the eventual disposition of patients who undergo video-EEG monitoring at a typical referral epilepsy center. METHODS: We reviewed the diagnoses and dispositions of all patients (adults and children) who underwent inpatient video-EEG monitoring (> or = 24 h) at our center (University of South Florida-Tampa General Hospital) over a 1-year period (2002). RESULTS: In total, 251 inpatient video-EEG monitoring sessions were performed. Nonepileptic seizures were diagnosed in 75 (30%); 58 (23%) were found to be surgical candidates; seven were implanted with the vagus nerve stimulator. In 47 (19%) patients, seizures were recorded, and the diagnosis of epilepsy was confirmed and clarified (symptomatic/cryptogenic generalized epilepsy, seven; localization-related epilepsy, 35; idiopathic generalized epilepsy, five). CONCLUSIONS: The eventual outcome of video-EEG monitoring is diverse. The largest groups, as expected, are psychogenic nonepileptic seizures (30%), and surgery (23%).
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The authors reviewed 58 patients with EEG-confirmed idiopathic generalized epilepsy (IGE). When initially seen, 17 (29%) were on broad-spectrum (adequate) antiepileptic drugs (AED) only, 28 (48%) on ill-advised AED only, and 13 (22%) on a combination of both. Thus, a majority of patients with IGE initially receive ill-advised AED, which cause IGE to appear intractable.
RATIONALE: Epilepsy surgery is a standard of care in the treatment of medically intractable epilepsy, but is underutilised. We describe the results of epilepsy surgery and the referral patterns at a referral epilepsy programme. METHODS: We reviewed the outcome of epilepsy surgery performed at the University of South Florida and Tampa General Hospital epilepsy programme for the years 2000 and 2001. The typical presurgical evaluation included clinical evaluation, EEG-video monitoring, MRI with dedicated epilepsy protocol, PET, SPECT, neuropsychological testing and Wada testing. We used the Engel outcome classification, and focused on the referral information to determine how and when in the course of their illness patients arrive at a referral epilepsy centre. RESULTS: In the 2-year period (2000-2001), a total of 36 epilepsy surgeries were performed. Twenty-nine temporal lobectomies, six extratemporal resections and one corpus callosotomy. Ages varied from 17 to 65 years. Overall results were: 30 (83%) seizure-free [class I], 5 (17%) rare seizures or almost seizure-free [class II] and 1 no improvement. Of the 29 temporal lobectomies, 27 (93%) are completely seizure-free [class I] and 2 (7%) are >90% improved [class II]. Duration of seizures before being seen at the epilepsy centre averaged 18 years (range 2-58 years). Twenty-two (61%) were sent by their neurologists, while 14 (39%) came self-referred without having discussed surgery with their neurologists. Five (14%) were specifically advised by their neurologist to not consider surgery. Two had participated in clinical trials of antiepileptic drugs (AEDs) before being seen at the epilepsy centre. CONCLUSIONS: Epilepsy surgery has high efficacy and very low morbidity. Yet, there continues to be a long delay in the referral of patients to the epilepsy centre, suggesting that surgery for epilepsy is underutilised.
The overinterpretation of EEGs is a known problem that has not been reported specifically. The authors report a series of EEGs on patients who were diagnosed eventually with psychogenic nonepileptic seizures and who had an EEG read as epileptiform. Of the 15 actual records available for review, the overread patterns were wicket spikes (n = 1), hypnagogic hypersynchrony (n = 1), and hyperventilation-induced slowing (n = 1). In the other 12 records, the overread patterns were simple fluctuations of sharply contoured background rhythms or fragmented alpha activity. Rather than well-described normal variants, the overinterpreted patterns tend to be normal fluctuations of background activity.
Laughter is a complex human behavior that has an emotional and a physical component. We present a patient who suffered syncope as a result of intense laughter, and hypothesize that this is analogous to other types of Valsalva-induced syncope. We emphasize the clinical characteristics that differentiate gelastic syncope from cataplexy.