Psychogenic seizures and psychogenic movement disorders: are they the same patients?

Psychogenic nonepileptic seizures (PNES) and psychogenic movement disorders (PMD) are common and disabling problems with abnormal psychological profiles, and they may have common features that could aid in better understanding and management. Since PNES and PMD are investigated and reported separately, comparisons are lacking. Psychogenic nonepileptic seizure and psychogenic movement disorder patients completed demographic, clinical, and psychological inventories including the Short Form (SF)-12 Health Status Survey (Physical and Mental Health Summary Scores), the Brief Symptom Inventory (BSI)-18 (somatization, depression, and anxiety subscales), and the Lorig Self-Efficacy Scale. Psychogenic nonepileptic seizure and psychogenic movement disorder patients had similar psychological profiles with reduced SF-12 Physical Health and Mental Health Summary Scores and increased BSI somatization, depression, and anxiety ratings. They varied slightly in age and gender, but their main distinguishing features were the presenting signs. These similar profiles suggest that PNES and PMD may not be distinct or separate entities and that collaborative investigative efforts and management are warranted.

Practice parameter update: management issues for women with epilepsy--focus on pregnancy (an evidence-based review): vitamin K, folic acid, blood levels, and breastfeeding: report of the Quality Standards Subcommittee and Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology and American Epilepsy Society.

OBJECTIVE: To reassess the evidence for management issues related to the care of women with epilepsy (WWE) during pregnancy, including preconceptional folic acid use, prenatal vitamin K use, risk of hemorrhagic disease of the newborn, clinical implications of placental and breast milk transfer of antiepileptic drugs (AEDs), risks of breastfeeding, and change in AED levels during pregnancy. METHODS: A 20-member committee evaluated the available evidence based on a structured literature review and classification of relevant articles published between 1985 and October 2007. RESULTS: Preconceptional folic acid supplementation is possibly effective in preventing major congenital malformations in the newborns of WWE taking AEDs. There is inadequate evidence to determine if the newborns of WWE taking AEDs have a substantially increased risk of hemorrhagic complications. Primidone and levetiracetam probably transfer into breast milk in amounts that may be clinically important. Valproate, phenobarbital, phenytoin, and carbamazepine probably are not transferred into breast milk in clinically important amounts. Pregnancy probably causes an increase in the clearance and a decrease in the concentration of lamotrigine, phenytoin, and to a lesser extent carbamazepine, and possibly decreases the level of levetiracetam and the active oxcarbazepine metabolite, the monohydroxy derivative. RECOMMENDATIONS: Supplementing women with epilepsy with at least 0.4 mg of folic acid before they become pregnant may be considered (Level C). Monitoring of lamotrigine, carbamazepine, and phenytoin levels during pregnancy should be considered (Level B) and monitoring of levetiracetam and oxcarbazepine (as monohydroxy derivative) levels may be considered (Level C). A paucity of evidence limited the strength of many recommendations.

Practice parameter update: management issues for women with epilepsy--focus on pregnancy (an evidence-based review): obstetrical complications and change in seizure frequency: report of the Quality Standards Subcommittee and Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology and American Epilepsy Society.

OBJECTIVE: To reassess the evidence for management issues related to the care of women with epilepsy (WWE) during pregnancy, including the risk of pregnancy complications or other medical problems during pregnancy in WWE compared to other women, change in seizure frequency, the risk of status epilepticus, and the rate of remaining seizure-free during pregnancy. METHODS: A 20-member committee including general neurologists, epileptologists, and doctors in pharmacy evaluated the available evidence based on a structured literature review and classification of relevant articles published between 1985 and February 2008. RESULTS: For WWE taking antiepileptic drugs, there is probably no substantially increased risk (greater than two times expected) of cesarean delivery or late pregnancy bleeding, and probably no moderately increased risk (greater than 1.5 times expected) of premature contractions or premature labor and delivery. There is possibly a substantially increased risk of premature contractions and premature labor and delivery during pregnancy for WWE who smoke. Seizure freedom for at least 9 months prior to pregnancy is probably associated with a high likelihood (84%-92%) of remaining seizure-free during pregnancy. RECOMMENDATIONS: Women with epilepsy (WWE) should be counseled that seizure freedom for at least 9 months prior to pregnancy is probably associated with a high rate (84%-92%) of remaining seizure-free during pregnancy (Level B). However, WWE who smoke should be counseled that they possibly have a substantially increased risk of premature contractions and premature labor and delivery during pregnancy (Level C).

Practice parameter update: management issues for women with epilepsy--focus on pregnancy (an evidence-based review): teratogenesis and perinatal outcomes: report of the Quality Standards Subcommittee and Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology and American Epilepsy Society.

OBJECTIVE: To reassess the evidence for management issues related to the care of women with epilepsy (WWE) during pregnancy. METHODS: Systematic review of relevant articles published between January 1985 and June 2007. RESULTS: It is highly probable that intrauterine first-trimester valproate (VPA) exposure has higher risk of major congenital malformations (MCMs) compared to carbamazepine and possible compared to phenytoin or lamotrigine. Compared to untreated WWE, it is probable that VPA as part of polytherapy and possible that VPA as monotherapy contribute to the development of MCMs. It is probable that antiepileptic drug (AED) polytherapy as compared to monotherapy regimens contributes to the development of MCMs and to reduced cognitive outcomes. For monotherapy, intrauterine exposure to VPA probably reduces cognitive outcomes. Further, monotherapy exposure to phenytoin or phenobarbital possibly reduces cognitive outcomes. Neonates of WWE taking AEDs probably have an increased risk of being small for gestational age and possibly have an increased risk of a 1-minute Apgar score of <7. RECOMMENDATIONS: If possible, avoidance of valproate (VPA) and antiepileptic drug (AED) polytherapy during the first trimester of pregnancy should be considered to decrease the risk of major congenital malformations (Level B). If possible, avoidance of VPA and AED polytherapy throughout pregnancy should be considered to prevent reduced cognitive outcomes (Level B). If possible, avoidance of phenytoin and phenobarbital during pregnancy may be considered to prevent reduced cognitive outcomes (Level C). Pregnancy risk stratification should reflect that the offspring of women with epilepsy taking AEDs are probably at increased risk for being small for gestational age (Level B) and possibly at increased risk of 1-minute Apgar scores of <7 (Level C).

Practice Parameter: evaluating an apparent unprovoked first seizure in adults (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology and the American Epilepsy Society.

OBJECTIVE: The Quality Standards Subcommittee of the American Academy of Neurology develops practice parameters as strategies for patient care based on analysis of evidence. For this practice parameter the authors reviewed available evidence relevant to evaluating adults presenting with an apparent unprovoked first seizure. METHODS: Relevant questions were defined and addressed by multiple searches of medical literature. Each article was then reviewed, abstracted, and classified using an established evidence scoring system. Conclusions and recommendations were based on a standard three-tiered scheme of evidence classification. RESULTS: For adults presenting with a first seizure, a routine EEG revealed epileptiform abnormalities in approximately 23% of patients, and these were predictive of seizure recurrence. A brain imaging study (CT or MRI) was significantly abnormal in 10% of patients, indicating a possible seizure etiology. Laboratory tests such as blood counts, blood glucose, and electrolyte panels were abnormal in up to 15% of individuals, but abnormalities were minor and did not cause the seizure. Overt clinical signs of infection such as fever typically predicted significant CSF abnormalities on lumbar puncture. Toxicology screening studies were limited, but report some positive tests. RECOMMENDATIONS: EEG should be considered as part of the routine neurodiagnostic evaluation of adults presenting with an apparent unprovoked first seizure (Level B). Brain imaging with CT or MRI should be considered as part of the routine neurodiagnostic evaluation of adults presenting with an apparent unprovoked first seizure (Level B). Laboratory tests, such as blood counts, blood glucose, and electrolyte panels (particularly sodium), lumbar puncture, and toxicology screening may be helpful as determined by the specific clinical circumstances based on the history, physical, and neurologic examination, but there are insufficient data to support or refute recommending any of these tests for the routine evaluation of adults presenting with an apparent first unprovoked seizure (Level U).

Individual state driving restrictions for people with epilepsy in the US.

BACKGROUND: States in the United States vary widely in their approaches to restricting driving for patients with epilepsy. Many states have shortened seizure-free restrictions or have adopted flexible regulations that consider individual clinical factors in determining driving privileges. The authors summarized state driving restrictions for patients with seizures, particularly unpublished regulatory practices, and determined the role and liability of physicians in judging driving safety for patients with epilepsy. METHODS: The authors surveyed motor vehicle administration bureaus in the 50 states and the District of Columbia and compared the laws, regulations, and practices restricting driving for people with epilepsy. Key responses from a questionnaire were confirmed by state motor vehicle administrations with phone interviews and by a signed executive summary. RESULTS: Twenty-eight states, including the District of Columbia, have laws requiring patients with epilepsy to be free of seizures for single fixed periods, with a median restriction of six months (range, 3 to 12 months). Twenty-three states have adopted more flexible approaches to restricting driving, such as varying seizure-free restrictions based on individual clinical factors. Many states allow patients to drive after shorter seizure-free periods than stated in their laws. These practices, however, are usually unpublished and not easily accessible. Physicians helped determine when their patients may drive in 13 states and were not legally shielded for their assessments in six of these states. CONCLUSIONS: States vary widely in how they regulate driving for patients with seizures. These varied regulatory approaches present potentially valuable models to determine how driving might be best regulated to protect public and patient safety optimally while permitting patients with controlled seizures to drive.

"The scarlet E": the presentation of epilepsy in the English language print media.

OBJECTIVE: To examine the coverage of epilepsy in English language newspapers and magazines to determine how they portray the medical risks associated with epilepsy, whether they report research and treatment advances accurately, whether stigmatizing biases toward persons with epilepsy persist, and to examine the sources of errors in reporting about epilepsy. BACKGROUND: The print media reflect and shape current views about epilepsy and other neurologic conditions. They also have the potential to further misconceptions about neurologic issues and particular brain disorders. Persistent myths about epilepsy, such as the ancient belief that it is a demonic disorder, can result in discrimination, emotional difficulties, and reluctance to seek effective treatment. METHODS: A large commercial database was used to search for stories about epilepsy from approximately 2,000 English language newspapers and popular magazines. Two epileptologists independently classified story themes and main sources, and screened for the presence of gross errors in 210 stories about epilepsy or seizures. The authors analyzed the metaphors and terminology used to describe seizures and epilepsy. RESULTS: The majority of English language print stories about epilepsy were accurate depictions of social and medical issues regarding the disorder, most commonly depictions of persons overcoming epilepsy and announcements of new therapies and reports of scientific advances. Thirty-one percent of the stories, however, contained gross errors, most commonly scientific inaccuracy, exaggerated treatment claims, and overestimates of the risks of dying during a seizure. New drug therapies were often described inaccurately by physicians and pharmaceutical spokespersons as curative and without side effects. Patients and their families frequently overemphasized the risk of dying during a seizure and misstated medical issues. Most celebrities with recurring seizures denied having epilepsy. Seizures were described with demonic imagery in 6% of stories. United States epilepsy associations discourage labeling patients as "epileptics"; however, the term was used in 45% of stories. CONCLUSION: Physicians and reporters should be aware of both professional and popular biases that influence the print media's presentation of the causes and consequences of epilepsy.

Nonepileptic seizures: diagnosis and management.

Nonepileptic seizures are disorders that are mistaken for epilepsy. They may be caused by physiologic or psychological disturbances but, unlike true epilepsy, nonepileptic seizures are not the consequence of abnormal electrical discharges in the brain. Nonepileptic seizures related to psychological causes are termed "nonepileptic psychogenic seizures" and account for approximately 20% of all intractable seizure disorders. These seizures are often misdiagnosed as true epilepsy, resulting in inappropriate, ineffective, and costly treatment of many patients. Clinical observation has long been the basis for distinguishing nonepileptic from epileptic seizures. Recent advances in video-EEG monitoring have tremendously improved the ability of experienced epilepsy specialists to correctly distinguish nonepileptic seizures from epilepsy, but access to epilepsy experts and comprehensive epilepsy monitoring centers remains limited for many patients. Moreover, even after a correct diagnosis is made a high proportion of such patients continue to have seizures and serious disability. Recent evidence suggests that patients with nonepileptic seizures may benefit from structured treatment programs and extended support from epilepsy centers. As knowledge about the nature of psychogenic seizures and their associated psychopathology is gained, better treatment strategies can be developed that will improve the care and prognosis of these difficult and challenging patients.

Epidemiology and evidence for morbidity of nonconvulsive status epilepticus.

Convulsive status epilepticus (SE) is convincingly related to serious morbidity and mortality and well recognized as a medical emergency, but prompt diagnosis and treatment of patients with nonconvulsive status epilepticus (NCSE) is often not emphasized because its consequences are thought to be benign. Nonconvulsive status epilepticus has been considered a relatively benign entity because it does not produce the adverse systemic consequences of convulsive status epilepticus, such as hyperthermia, acidosis, hyperkalemia, pulmonary compromise, or cardiovascular collapse. However, recent reports indicate that NCSE is not so benign. There are two major forms of NCSE, absence status epilepticus and complex partial status epilepticus. Typical absence status epilepticus does not appear to have very serious consequences and may be a type of "inhibitory" seizure, but complex partial status epilepticus has been associated with serious morbidity and mortality. Despite not causing the systemic physiologic or metabolic derangements seen with convulsive SE, complex partial status epilepticus is still associated with the two other major factors correlated with poor outcomes in convulsive SE: 1) neuronal damage from abnormal electrical activity and 2) the interaction of acute neurologic disorders, such as stroke, that may precipitate SE. Other similar epileptiform encephalopathies such as "subclinical," "electroencephalographic," "nontonic-clonic," and "subtle" SE have not been as well studied as NCSE but pose similar issues. Early diagnosis and aggressive intervention have proven the best means of averting adverse outcomes in patients with convulsive SE. The diagnosis and treatment of NCSE, particularly complex partial status epilepticus, merit similar emphasis and attention.

Risk factors for seizure-related motor vehicle crashes in patients with epilepsy.

OBJECTIVE: We identified clinical risk factors for seizure-related motor vehicle crashes in patients with epilepsy. BACKGROUND: Current US laws permit epilepsy patients with controlled seizures to drive. These laws attempt to balance the important economic and social value of driving with the risk to public safety from seizure-related crashes. Various clinical factors are considered in these laws, particularly the seizure-free interval. Driving restrictions range from 3 to 18 months, however, and studies have not established how these various seizure-free intervals and other clinical factors influence the risk for seizure-related motor vehicle crashes. METHODS: We performed a retrospective case-control study to determine the influence of clinical risk factors associated with seizure-related motor vehicle crashes. Both "case" and "control" patients had epilepsy, drove, and were from the same clinic, but the cases differed in having had seizure-related crashes. RESULTS: Fifty patients with epilepsy who crashed during seizures and 50 matched control patients were compared. Factors that significantly decreased the odds of patients with epilepsy having motor vehicle crashes due to seizures were: long seizure-free intervals, reliable auras, few prior nonseizure-related accidents, and having had their antiepileptic drugs (AEDs) reduced or switched. For example, patients who had seizure-free intervals > or = 12 months had a 93% reduced odds for crashing compared to patients with shorter intervals. Other findings were: 25% of patients had more than one seizure-related crash and 20% had missed an AED dose just prior to their crash. The majority (54%) of patients who crashed were driving illegally, with seizure-free intervals shorter than legally permitted. CONCLUSION: Seizure-free intervals, the presence of reliable auras, AED therapy modifications, and a history of nonseizure-induced crashes should be considered when counseling patients with epilepsy on driving and when formulating driving regulatory policy. Case control studies of crashes due to seizures can help in assessing and monitoring such risks.

Effects of menopause on seizures in women with epilepsy.

PURPOSE: Although important associations between epilepsy and women's hormonal phases are described, the relation of menopause to epilepsy has received little attention. METHODS: By using a structured interview, we studied menopausal women with epilepsy seen at the University of Maryland Epilepsy Center over a 1-year period from 1994 to 1995. We analyzed the characteristics and temporal relation of the seizures to menopause and compared the frequency and severity of the seizures with those in a similar group of premenopausal women. RESULTS: We identified 61 menopausal women (46 who were postmenopausal and 15 perimenopausal) and compared them with 46 premenopausal women. No statistically significant differences were noted in either the frequency or the severity of seizures comparing all menopausal or only postmenopausal with premenopausal women. However, 12 (20%) of the 61 menopausal women noted that their seizures first began during or after menopause, with eight having no proven cause for their seizures. Many individual women described changes in their seizures with menopause. Among the 61 menopausal women, 49 had established epilepsy before the onset of menopause, and 20 (41%) reported worsening of their seizures with menopause, 13 (27%) noted improvement, and 16 (33%) described no changes. These observations were similar for peri- and postmenopausal women. Of the 15 menopausal women taking hormone replacement therapy, the six taking progestin were significantly less likely to report worsening of their seizures. CONCLUSIONS: These findings support the view that hormonal influences are important in women with seizures. Although, in aggregate, menopausal (combined perimenopausal and postmenopausal) and postmenopausal women's seizures were similar in frequency and severity to those of other women, menopause was associated with changes in seizures for some women. Moreover, menopause may be a previously unrecognized factor for some new-onset seizures. The relations between menopause and epilepsy deserve to be more fully investigated.

Nonepileptic posttraumatic seizures.

PURPOSE: Epileptic posttraumatic seizures (PTSs) are a well-recognized consequence of head injury (HI), but HI and nonepileptic seizures (NESs) have not been related. We describe a significant subset of patients with NESs who had their seizures attributed to HI. METHODS: We reviewed the records of all patients diagnosed with NES at the University of Maryland Medical Center over a 6-year period (1989-1995) and selected patients with seizures attributed to a head injury occurring < or =3 years before the onset of their seizures. RESULTS: Of 157 patients with video-EEG confirmed NES, 37 (24%) had the onset of their seizures attributed to an HI. Their average age was 34 years (range, 15-56 years); 68% were women. Nonepileptic PTS usually developed within the first year after HI (89%). Convulsive symptoms were present in 54%. Whereas epileptic PTSs characteristically follow severe HI, the majority (78%) of our patients with nonepileptic PTSs sustained only mild HI. Before their HI, 76% of our patients were employed, working in the home, or students, but only 11% could continue those activities after developing nonepileptic PTSs. CONCLUSIONS: Nonepileptic PTSs are frequently mistaken for epileptic PTSs and result in serious disability. The misdiagnosis of nonepileptic PTSs leads to ineffective and inappropriate treatment. Patients with intractable seizures after HIs, particularly mild HIs, should be carefully evaluated for NESs.

Antiphospholipid antibodies: an epiphenomenon in Tourette syndrome.

Antiphospholipids (aPLAs) have been previously identified in children with Tourette syndrome (TS), which has led to the speculation that these antibodies might have a pathophysiologic role in this disorder. Therefore, 21 healthy children and adolescents with TS, whose ages ranged from 7 to 17 years, underwent laboratory studies designed to diagnose the lupus anticoagulant, anticardiolipin (aCL) antibodies [immunoglobulin (Ig) G, IgA, and IgM], and antinuclear antibodies. Although five subjects had at least one value that differed from accepted laboratory standards, the changes were marginal in four of them. Lupus anticoagulant was identified in one patient, based on a minimal requirement of a prolonged dilute Russell viper venom time, clotting studies that did not correct after mixture with normal plasma, and an abnormal platelet neutralization procedure. A prolonged (but correctable) activated partial thromboplastin time was found in one individual, and aCL IgG was marginally increased in three subjects. Two (10%) of a control population of 20 same-age children also had low positive aCL IgG levels. There were no differences in tics (onset, type, frequency, severity, and family history) or comorbid features between children with normal or "abnormal" laboratory study results. Our data suggest that the presence of aPLAs in TS represents an epiphenomenon rather than a pathophysiologic mechanism.

Regulations prohibiting blood donation by individuals with seizures or epilepsy are not necessary.

Throughout the world people who have epilepsy and seizures are prohibited from donating blood. These restrictions are based on the assumption that they are prone to adverse donor reactions, specifically, syncope and convulsions. We describe a study evaluating whether that concern is warranted. During a two year period beginning in 1987, blood donors with a history of seizures were actively recruited by the American Red Cross in the state of Maryland, USA. According to accepted standards, adverse reactions were classified as "slight", for dizziness and nausea without loss of consciousness; "moderate", denoting syncope; and "severe", indicating convulsive syncope. We reviewed a total of 329,143 satisfactory blood donations, and 613 individuals reporting a history of seizures donated blood 723 times. Among donors with seizures, 186 (25.7%) were taking antiepileptic medication, and 61 (8.4%) had one or more seizures in the preceding year. Individuals with seizures had a low incidence of adverse reactions (3.34%). Although slightly higher than the entire population (2.24%), this difference was not statistically significant. In particular, the risk of syncope with or without convulsive activity was low for people with seizures (.21%) and not significantly increased as compared to other donors (.28%). Our study supports the view that individuals with seizures or epilepsy are not at greater risk for adverse reactions after blood donation. Major restrictions on individuals with epilepsy and seizures as blood donors are not warranted.

Complex partial seizure provocation by vasovagal syncope: video-EEG and intracranial electrode documentation.

Vasovagal syncope precipitating an epileptic seizure has only rarely been described. A patient with known intractable complex partial seizures being evaluated for a left anterior temporal lobectomy experienced a typical seizure with mesial temporal onset precipitated by an observed vasovagal episode. This is the first report of a partial epileptic seizure precipitated by vasovagal syncope and the first example of an epileptic seizure induced by syncope in an adult. Video and intracranial depth electrode and subdural grid recordings documented the event.

Radiologic appearance of the dysembryoplastic neuroepithelial tumor.

PURPOSE: To evaluate the computed tomographic (CT) and magnetic resonance (MR) imaging features of dysembryoplastic neuroepithelial tumor (DNT). MATERIALS AND METHODS: Six CT studies (four with contrast material enhancement) and 10 MR imaging studies (seven with gadolinium enhancement) obtained in 10 patients with a history of seizures and pathologically proved DNT were retrospectively reviewed. RESULTS: All tumors were intracortical or subcortical. CT showed a low-attenuation mass in all cases except one of mixed isoattenuation and low attenuation. The DNT had decreased signal intensity on T1-weighted MR images and well-demarcated increased signal intensity on T2-weighted images without peritumoral edema. Prominent MR imaging features were a gyriform configuration on T1- or T2-weighted images in 10 patients (100%), well-demarcated lobular tumor margins on T2-weighted images in eight (80%), and a high rate of bone remodeling of the adjacent calvaria on MR (60% [n = 6]) and CT (67% [n = 4]) images. CONCLUSION: Diagnosis of DNT with imaging modalities alone may be difficult, but these radiologic features may aid in differentiating DNT from other gliomas.

Panic attacks as ictal manifestations of parietal lobe seizures.

Although most panic attacks appear to be primary psychiatric disturbances, some evidence suggests a biologic basis for panic disorder, possibly associated with temporal lobe dysfunction. Fear is a common affective change associated with some complex partial seizures (CPS) originating from the right temporal lobe. We describe a previously unreported association between panic attacks and seizures originating from the parietal lobe in 2 patients with right parietal lobe tumors. Intracranial monitoring documented correlations between the symptoms of fear and restricted regional parietal cortical discharges. Surgical resections of the lesions (one total, one subtotal) resulted in complete recovery or improvement.

Complex partial status epilepticus accompanied by serious morbidity and mortality.

Nonconvulsive status epilepticus (NCSE) accounts for approximately 20% of all status epilepticus (SE). Although convulsive SE is recognized as a medical emergency, prompt diagnosis and treatment of patients with NCSE is often not emphasized because its consequences are thought to be benign. We report 10 patients with persistent neurologic deficits or death after well-documented NCSE in the form of complex partial status epilepticus (CPSE). All patients had prolonged CPSE lasting 36 hours or longer, as documented by clinical and EEG findings. Causes for CPSE were preexisting epilepsy with partial and secondarily generalized seizures (3 patients), vascular disease (2 patients), encephalitis (2 patients), and metabolic disease (1 patient); causes were unknown for two patients. Poor outcomes identified included persistent (lasting at least 3 months) or permanent cognitive or memory loss (5 patients), cognitive or memory loss plus motor and sensory dysfunction (3 patients), and death (3 patients). NCSE in the form of CPSE is not a benign entity. Serious morbidity and mortality may occur due to the adverse effects of prolonged seizures and as a result of acute brain disorders that precipitate the seizures.