Semiology of hypermotor (hyperkinetic) seizures.

BACKGROUND: Hypermotor seizures (HMSs) consist of complex movements involving proximal segments of the limbs and trunk that appear violent and inappropriate for the situation. METHODS: We analyzed hypermotor seizure videos in seizure-free patients (Engel class I) following resective epilepsy surgery. After completion of video analysis, we reviewed EEG and neuroimaging data. RESULTS: Search of our epilepsy surgery database yielded 116 patients classified as having hypermotor seizures between 1996 and 2013. From this subset, 17/31 (55%) patients had been seizure-free for >6months (mean follow-up: 3.3years). Mean seizure duration was 35s (range: 6-91s), of which the HM phase lasted a mean of 22s (range: 3-53s). In 16 patients (95%), hypermotor activity was seen at or within 10s of clinical seizure onset. Type I semiology occurred in 6 patients, type II semiology in 10 patients, and 1 patient exhibited features of both. Type I and type II semiologies were noted in patients who had frontal lobe as well as extrafrontal resections. Nonversive head and body turning occurred in 10 patients (ranging from 90° to 270°) which was ipsilateral to the side of resection in all patients and seen both in frontal and extrafrontal resections. Six out of eleven patients with abnormal MRI and 4/6 patients with nonlesional MRI underwent invasive EEG evaluation. Eight patients (47%) had frontal lobe resection, 4/17 (23%) patients had temporal lobe resection, and one patient each had parietal lobe, insular, temporoparietooccipital, or motor sparing resection; 1 patient had functional hemispherectomy. CONCLUSION: Hypermotor semiology typically occurs at or within 10s after seizure onset. Ipsilateral head/body turning appears to be of lateralizing value whereas asymmetry of limb movement was not lateralizing. Hypermotor semiology is most often seen in frontal lobe epilepsy but may occur in seizures arising from other locations.

Calcium-sensitive potassium channelopathy in human epilepsy and paroxysmal movement disorder.

The large conductance calcium-sensitive potassium (BK) channel is widely expressed in many organs and tissues, but its in vivo physiological functions have not been fully defined. Here we report a genetic locus associated with a human syndrome of coexistent generalized epilepsy and paroxysmal dyskinesia on chromosome 10q22 and show that a mutation of the alpha subunit of the BK channel causes this syndrome. The mutant BK channel had a markedly greater macroscopic current. Single-channel recordings showed an increase in open-channel probability due to a three- to fivefold increase in Ca(2+) sensitivity. We propose that enhancement of BK channels in vivo leads to increased excitability by inducing rapid repolarization of action potentials, resulting in generalized epilepsy and paroxysmal dyskinesia by allowing neurons to fire at a faster rate. These results identify a gene that is mutated in generalized epilepsy and paroxysmal dyskinesia and have implications for the pathogenesis of human epilepsy, the neurophysiology of paroxysmal movement disorders and the role of BK channels in neurological disease.

Long-term functional outcomes and their predictors after hemispherectomy in 115 children.

PURPOSE: To examine the long-term functional outcomes and their predictors using a patient/family centered approach in a cohort of children who had hemispherectomy. Functional outcome measures studied were the following: ambulation ability, visual symptoms, spoken language, reading skills, and behavioral problems. METHODS: We reviewed 186 consecutive children who underwent hemispherectomy between 1997 and 2009 at our center. Preoperative clinical, electroencephalography (EEG), imaging, and surgical data were collected. One hundred twenty-five families completed a structured questionnaire to assess the functional status and seizure outcome. Prognostic predictors were examined using a multivariate regression analysis. KEY FINDINGS: At a mean follow-up of 6.05 years after hemispherectomy, 70 patients (56%) were seizure-free and 45 (36%) had seizure recurrence; 10 patients (8%) were free of their preoperative seizures but had new-onset nonepileptic spells and were excluded from further analysis. Of 115, at follow-up (mean age at follow-up 12.7 years, range 2-28 years), 96 patients (83%) walked independently, 10 (8.7%) walked with assistance, and 9 (7.8%) were unable to walk. New visual symptoms that were not present preoperatively were reported only in 28 patients (24%). Eighty patients (70%) had satisfactory spoken language skills but only 44 (42%) of the 105 children older than 6 years had satisfactory reading skills. Significant behavioral problems were reported in 30 patients (27%). Only five (6.2%) of the 81 children aged between 6 and 18 years attended mainstream school without assistance; 48 (59%) were in mainstream school with assistance and the rest were in special school for disabled or home cared. Five (21%) of the 24 patients older than 18 years of age were gainfully employed. Multivariate logistic regression analysis identified the following factors as independently associated with poor functional outcome. (1) Seizure recurrence negatively affected all functional domains--ambulation ability, spoken language and reading skills, and behavior (p < 0.05). (2) Abnormalities in the unoperated hemisphere on magnetic resonance imaging (MRI) (p < 0.05) and preexisting quadriparesis (p < 0.01) correlated with poor motor outcome. (3) Multilobar MRI abnormalities in the contralateral hemisphere (odds ratio [OR] = 13.9, p = 0.001) and young age (indeterminate preoperative language status) at hemispherectomy (OR = 11.1, p = 0.01) also correlated with poor language outcome. (4) Younger age at epilepsy onset correlated with poor reading skills (p = 0.01) but not with spoken language skills. SIGNIFICANCE: This study highlights the long-term functional status of patients after hemispherectomy. The majority of patients were ambulant independently; however, impairments in reading and spoken language were frequent. Seizure recurrence after hemispherectomy and contralateral hemisphere abnormalities on MRI were the major predictors of poor outcome in ambulation, spoken language, and reading abilities. This study will assist in presurgical counseling using simple understandable functional outcome measures and may help in planning early interventions after hemispherectomy to improve functional outcome.

Surgically proven coexistence of focal and generalized epilepsy: a case report.

The coexistence of focal and idiopathic generalized epilepsy (IGE) is rarely observed, and the mechanism underlying this situation remains unknown. We report a 13-year-old girl with well-controlled generalized epilepsy and medically-refractory left temporal lobe epilepsy. She underwent intracranial EEG recording, which demonstrated two characteristic seizure patterns of generalized ictal onset and left mesial temporal onset. In addition, two types of interictal spike distribution, including the left mesial temporal region and generalized spikes, were also supportive of the coexistence of left mesial temporal lobe epilepsy and IGE. Thereafter, a left anterior temporal lobectomy and post-surgical medication significantly improved her seizure outcome. This case illustrates the importance of considering surgical management for patients with medically-intractable focal epilepsy coexisting with generalized epilepsy.

Imag(in)ing seizure propagation: MEG-guided interpretation of epileptic activity from a deep source.

Identification and accurate localization of seizure foci is vital in patients with medically-intractable focal epilepsy, who may be candidates for potentially curative resective epilepsy surgery. We present a patient with difficult-to-control seizures associated with an occult focal cortical dysplasia residing within the deeper left parietal operculum and underlying posterior insula, which was not detected by conventional MRI analysis. Propagated activities from this deeper generator produced misleading EEG patterns both on surface and subdural electrode recordings suggesting initial activation of the perirolandic and mesial frontal regions. However, careful spatio-temporal analysis of stereotyped interictal activities recorded during MEG, using sequential dipole modeling, revealed a consistent pattern of epileptic propagation originating from the deeper source and propagating within few milliseconds to the dorsal convexity. In this instance, careful dissection of noninvasive investigations (interictal MEG along with ictal SPECT findings) allowed clinicians to dismiss the inaccurate and misleading findings of the traditional "gold-standard" intracranial EEG. In fact, this multimodal noninvasive approach uncovered a subtle dysplastic lesion, resection of which rendered the patient seizure-free. This case highlights the potential benefits of dynamic analysis of interictal MEG in the appropriate clinical context. Pathways of interictal spike propagation may help elucidate essential neural networks underlying focal epilepsy.

Seizure outcomes following multilobar epilepsy surgery.

PURPOSE: Outcomes following unilobar surgeries for refractory epilepsy have been well described. However, little is known about long-term seizure outcomes following multilobar resections. The aim of the current study was to identify long-term seizure control and predictors of seizure recurrence in this patient population. METHODS: Records of patients who underwent multilobar epilepsy surgery at the Cleveland Clinic between 1994 and 2010 were retrospectively reviewed. A postoperative follow-up of at least 6 months was required. Patients were classified as seizure free if they achieved an Engel class I at last follow-up. Long-term chances of seizure freedom were illustrated using a survival analysis, and predictors of recurrence were identified using Cox proportional hazard modeling. KEY FINDINGS: Sixty-three patients with medically intractable epilepsy underwent multilobar surgical resections during the study period (mean follow-up of 4.6 years). Predominant resection types included extended occipital (temporoparietooccipital, parietooccipital, temporooccipital: 57%), frontotemporal (21%), and temporoparietal (17%). Mean age at surgery was 21.4 years and mean age at seizure onset was 10.1 years. Fifty-six percent of the patients underwent extraoperative invasive electroencephalography (EEG) evaluations. At 6 postoperative months, 71% (95% confidence interval (CI) 65-77) were seizure-free (SF), 64% (CI 58-70) were SF at 1 year, 52% (CI 46-59) were SF at 5 years, and 41% (CI 32-50) remained SF at 10 years. Forty-one patients had at least one breakthrough seizure after surgery (median timing of recurrence 6.1 months), with an Engel class 1 achieved again by last follow-up in 12 of these 41 cases. Nine patients required a reoperation. Patients who underwent extended occipital/posterior quadrant resections had more favorable outcomes as compared to the other groups. With multivariate analysis, the type of resection (p = 0.03), preoperative auras (p = 0.03), an incomplete resection (0.03), and the presence of postoperative spikes (p = 0.0003) correlated with seizure recurrence. The risk of seizure recurrence for an incomplete resection was 2.3 (CI 1.53-3.36), preoperative aura 2.3 (CI 1.34-3.87), and postoperative spikes on surface EEG 2.5 (CI 1.29-4.71). SIGNIFICANCE: A favorable outcome can be achieved in 41% of patients undergoing multilobar resections for epilepsy surgery at 10 years of follow-up. Close to one-third of patients who have breakthrough seizures after surgery are able to regain seizure freedom by last follow-up. Predictors of recurrence include resection type (frontotemporal and parietotemporal resections did worse), presence of preoperative aura, an incomplete surgical resection, and the presence of postoperative interictal discharges on EEG.

Long-term seizure outcome after resective surgery in patients evaluated with intracranial electrodes.

PURPOSE: Despite advances in "noninvasive" localization techniques, many patients with medically intractable epilepsy require the placement of subdural (subdural grid electrode, SDE) and/or depth electrodes for the identification and definition of extent of the epileptic region. This study investigates the trends in longitudinal seizure outcome and its predictors in this group. METHODS: We reviewed the medical records, and electroencephalography (EEG) data of 414 consecutive patients who underwent intracranial electrode placement (SDE and/or depth electrodes) at Cleveland Clinic Epilepsy Center between 1998 and 2008. A favorable outcome was defined as complete seizure freedom, discounting any auras or seizures that occurred within the first postoperative week. Survival curves were constructed, and Cox proportional hazard modeling was used to identify outcome predictors. KEY FINDINGS: The estimated probability of complete seizure freedom was 61% (95% confidence interval [CI] 58-64%) at one postoperative year, 47% (95% CI 44-50%) at 3 years, 42% (95% CI 39-45%) at 5 years, and 33% (95% CI 28-38%) at 10 years. Half of all seizure recurrences occurred within the first two postoperative months. Subsequently, the rate of seizure freedom declined by 4-5% every 2-3 years. After multivariate analysis, two independent predictors of seizure recurrence were identified: (1) prior resective surgery (p ≤ 0.002), mostly in patients with temporal lobe resections, and (2) sublobar or multilobar resection (p ≤ 0.02), mostly in patients following frontal lobe resections. SIGNIFICANCE: Favorable seizure outcomes are possible in the complex epilepsy population requiring invasive EEG studies. We propose that mislocalization of the epileptogenic zone or its incomplete resection account for early postoperative recurrences, whereas epileptogenesis may lead to later relapses.

Epilepsy with Eyelid myoclonias - A diagnosis concealed in other genetic generalized epilepsies with photoparoxysmal response.

OBJECTIVE: Epilepsy with eyelid myoclonias(EMA) is a genetic generalized epilepsy (GGE) characterized by eyelid myoclonia, eye-closure sensitivity and photosensitivity. Data on EMA patients who specifically present with photoparoxysmal response on EEG is lacking. EMA is an under-recognized syndrome which is frequently misclassified as another GGE. The main objective of our research is to describe the occurrence of EMA versus other GGEs among patients with photoparoxysmal response and evaluate their distinguishing features. METHODS: We retrospectively identified all patients who had photoparoxysmal response on EEGs performed at Cleveland clinic between 01/01/2012 and 12/31/2019. Initial epilepsy diagnosis and clinical data were collected. EEGs were reviewed for eyelid myoclonia and eye-closure-sensitivity which were used as main diagnostic clues for EMA. If clinical criteria was met, diagnosis was revised as EMA. RESULTS: Of 249 patients with photoparoxysmal response, 70(28.1%) met EMA criteria. Sixty-two (88.6%) were females. Mean age of onset of epilepsy was 7 years (+7.9) and 120(48.2%) had other GGEs. Fifty-four (77.1%) patients with EMA were initially classified as another epilepsy. Initial diagnosis included CAE or JME in 40(57.1%) patients with EMA so we compared EMA with these syndromes. Female preponderance, drug refractoriness, older age of onset and generalized myoclonia were more common in EMA than CAE. Earlier age of onset, absence seizures, and lack of generalized myoclonic jerks were more common EMA than JME. SIGNIFICANCE: Our study demonstrates that EMA is under-recognized among GGE patients with photoparoxysmal response. It highlights distinguishing clinical and electrographic features which separate EMA from other GGEs. It emphasizes the diverse treatments utilized and the need for therapeutic options for patients with refractory EMA.

Exacerbation of eyelid myoclonia in patients with epilepsy and eyelid myoclonia receiving cannabidiol.

Epilepsy with eyelid myoclonia or Jeavons Syndrome (JS) is a childhood genetic generalized epilepsy. Its clinical features include eyelid myoclonia (hallmark) with or without brief absences, eyelid closure-induced seizures and/or electroencephalographic (EEG) paroxysms (generalized polyspikes and/or generalized spike-wave activity at 3-6 Hz, elicited by closure of eyelid) and photosensitivity. Broad-spectrum anti-seizure medications are often utilized for the management of JS patients. A wide variety of medications may be utilized especially in refractory cases. Efficacy or safety of cannabidiol (CBD) for JS has not been studied. We describe two cases of exacerbation of eyelid myoclonia in JS which correlated with CBD use and resolved after CBD discontinuation. These cases highlight that caution should be practiced when using CBD for JS as it can potentially worsen eyelid myoclonia.

Surgical candidates in children with epileptic spasms can be selected without invasive monitoring: A report of 70 cases.

OBJECTIVE: Prior surgical series in children with drug-resistant epileptic spasms have reported use of intracranial EEG monitoring in up to two-third of patients. We report outcome after epilepsy surgery for drug-resistant epileptic spasms in a cohort of children without the use of intracranial EEG monitoring in any of the patients. METHODS: Medical records of all consecutive children aged 5 years or under who had epilepsy surgery for epileptic spasms at Cleveland Clinic between 2000 and 2018 were reviewed. Post-operative seizure outcome and predictors of prognosis of seizure outcome were analyzed. RESULTS: Seventy children with active epileptic spasms underwent surgical resections during the study period. Mean age at seizure onset was 6.8 (+9.31) months and median age at surgery was 18.5 months. An epileptogenic lesion was identified on brain MRI in all patients; 17 (24%) had bilateral abnormalities. Etiologies included malformations of cortical development (58%), perinatal infarct/encephalomalacia (39%), and tumor (3%). None of the patients had intracranial EEG. Surgical procedures included hemispherectomy (44%), lobectomy/ lesionectomy (33%), and multilobar resections (23%). Twelve children needed repeat surgery; six (50%) became seizure free after the second surgery. At six months follow-up, 73% (51/70) were seizure-free since surgery. At a mean follow-up of 4.7 years, 60% (42/70) had Engel 1 outcome. In those with seizure recurrence, 17 (60%) reported improvement. Shorter epilepsy duration (p = 0.05) and lobar or sub-lobar epileptogenic lesions (p = 0.02) predicted favorable seizure outcome at 6 months after surgery. For long term outcome, patients with bilateral abnormalities on MRI (p = 0.001), and multilobar extent on MRI (p = 0.02) were at higher risk for recurrence. SIGNIFICANCE: Children with drug-resistant epileptic spasms secondary to an epileptogenic lesion detected on MRI could be selected for epilepsy surgery without undergoing intracranial EEG monitoring. A surgical selection paradigm without intracranial monitoring may allow early surgery without the risks of invasive monitoring.

When is a postoperative seizure equivalent to "epilepsy recurrence" after epilepsy surgery?

PURPOSE: Up to one-half of epilepsy surgery patients will have at least one seizure after surgery. We aim to characterize the prognosis following a first postoperative seizure, and provide criteria allowing early identification of recurrent refractory epilepsy. METHODS: Analyzing 915 epilepsy surgery patients operated on between 1990 and 2007, we studied 276 who had >or=1 seizure beyond the immediate postoperative period. The probability of subsequent seizures was calculated using survival analysis. Patients were divided into seizure-free (no seizures for >or=1 year) and refractory (persistent seizures) and analyzed using multivariate regression analysis. RESULTS: After a first seizure, 50% had a recurrence within 1 month and 77% within a year before the risk slowed down to additional 2-3% increments every two subsequent years. After a second seizure, 50% had a recurrence within 2 weeks, 78% within 2 months, and 83% within 6 months. Having both the first and second seizures within six postoperative months [odds ratio (OR) 4.04; 95% confidence interval (CI) 2.05-8.40; p = 0.0001], an unprovoked initial recurrence (OR 3.92; 95% CI 2.13-7.30; p < 0.0001), and ipsilateral spikes on a 6-months postoperative electroencephalography (EEG) (OR 2.05; 95% CI 1.10-3.88; p = 0.025) predicted a poorer outcome, with 95% of patients who had all three risk factors becoming refractory. All patients with cryptogenic epilepsy and recurrent seizures developed refractoriness. DISCUSSION: Seizures will recur in most patients who present with their first postoperative event, with one-third eventually regaining seizure-freedom. Etiology and early and unprovoked postoperative seizures with epileptiform activity on EEG at six postoperative months may predict recurrent medical refractoriness.

Contralateral MRI abnormalities in candidates for hemispherectomy for refractory epilepsy.

PURPOSE: To assess the impact of contralateral magnetic resonance imaging (MRI) findings on seizure outcome after hemispherectomy for refractory epilepsy. METHODS: We retrospectively reviewed 110 children, 0.4-18 (median 5.9) years of age, who underwent hemispherectomy for severe refractory epilepsy at Cleveland Clinic Children's Hospital. In children with contralateral (as well as ipsilateral) MRI findings appreciated preoperatively, the decision to proceed to surgery was based on other features concordant with the side with the most severe MRI abnormality, including ipsilateral epileptiform discharges, lateralizing seizure semiology, and side of hemiparesis. RESULTS: We retrospectively observed contralateral MRI abnormalities (predominantly small hemisphere, white matter loss or abnormal signal, or sulcation abnormalities) in 81 patients (74%), including 31 of 43 (72%) with malformations of cortical development (MCD), 31 of 42 (73%) with perinatal injury from infarction or hypoxia, and 15 of 25 (60%) with Rasmussen's encephalitis, Sturge-Weber syndrome, or posttraumatic encephalomalacia. Among 84 children (76%) with lesions that were congenital or acquired pre- or perinatally, 67 (83%) had contralateral MRI abnormalities (p = 0.02). Contralateral findings were subjectively judged to be mild or moderate in 70 (86%). At follow-up 12-84 (median 24) months after surgery, 79% of patients with contralateral MRI abnormalities were seizure-free compared to 83% of patients without contralateral MRI findings, with no differences based on etiology group or type or severity of contralateral MRI abnormality. DISCUSSION: MRI abnormalities, usually mild to moderate in severity, were seen in the contralateral hemisphere in the majority of children who underwent hemispherectomy for refractory epilepsy due to various etiologies, especially those that were congenital or early acquired. The contralateral MRI findings, always much less prominent than those in the ipsilateral hemisphere, did not correlate with seizure outcome and may not contraindicate hemispherectomy in otherwise favorable candidates.

Patient management conference: Correlating pre-surgical data.

The epilepsy surgery patient management conference is a fundamental part of the presurgical evaluation. This article reviews the literature and describes a single center's approach. Case examples highlight how patient management conferences may influence clinical care.

Investigation of networks underlying hyperkinetic seizures utilizing ictal SPECT.

OBJECTIVE: To study neural networks involved in hyperkinetic seizures (HKS) using ictal SPECT. METHODS: We retrospectively identified 18 patients with HKS evaluated at the Cleveland Clinic between 2005 and 2015 with video-EEG monitoring and ictal SPECT. Semiology was confirmed by the consensus of 2 epileptologists' independent reviews and classified as type 1, 2, or 3 HKS. SPECT data were analyzed by 2 independent physicians using a z score of 1.5. Ictal hyperperfusion patterns for each group were analyzed visually and with SPM. Spatial normalization to Montreal Neurological Institute space for each patient's data was performed, followed by flipping of data from patients with left-sided ictal onset to the right side. Finally, an average z score map for each group was calculated. RESULTS: Visual analysis and SPM identified different patterns of ictal hyperperfusion in the 3 subtypes of HKS. Type 1 seizures showed hyperperfusion in a more anteriorly located network involving the anterior insula, orbitofrontal cortex, cingulate, and anterior perisylvian region and rostral midbrain. Type 2 seizures were associated with hyperperfusion in a more caudally located network involving the orbitofrontal cortex, cingulate (middle and posterior), basal ganglia, thalami, and cerebellum. Type 3 seizures showed a mixed pattern of SPECT hyperperfusion involving the temporal pole and anterior perisylvian region. CONCLUSIONS: Each of the 3 different semiologic subtypes of HKS is associated with distinct patterns of hyperperfusion, providing further insight into the neural networks involved. This knowledge may inform placement of invasive EEG electrodes in patients with HKS semiology undergoing presurgical evaluation.

Don't Just Stand There: Do Something! The Case for Peri-Ictal Intervention.

Hypoxemia Following Generalized Convulsive Seizures: Risk Factors and Effect of Oxygen Therapy Rheims S, Alvarez BM, Alexandre V, Curot J, Maillard L, Bartolomei F, et al; the REPO2MSE Study Group. Hypoxemia following generalized convulsive seizures: risk factors and effect of oxygen therapy . Neurology. 2019;92(3):e183-e193 Objective: To analyze the factors that determine the occurrence or severity of postictal hypoxemia in the immediate aftermath of a generalized convulsive seizure (GCS). METHODS: We reviewed the video-electroencephalogram (EEG) recordings of 1006 patients with drug-resistant focal epilepsy included in the REPO2MSE study to identify those with ≥1 GCS and pulse oximetry (SpO2) measurement. Factors determining recovery of SpO2 ≥90% were investigated using Cox proportional hazards models. Association between SpO2 nadir and person- or seizure-specific variables was analyzed after correction for individual effects and the varying number of seizures. RESULTS: A total of 107 GCS in 73 patients were analyzed. A transient hypoxemia was observed in 92 (86%) GCS. Rate of GCS with SpO2 <70% dropped from 40% to 21% when oxygen was administered early ( P = .046). Early recovery of SpO2 ≥90% was associated with early administration of oxygen ( P = .004), absence of postictal generalized EEG suppression (PGES; P = .014), and extratemporal lobe epilepsy ( P = .001). Lack of early administration of O2 ( P = .003), occurrence of PGES ( P = .018), and occurrence of ictal hypoxemia during the focal phase ( P = .022) were associated with lower SpO2 nadir. CONCLUSION: Postictal hypoxemia was observed in the immediate aftermath of nearly all GCS, but administration of oxygen had a strong preventive effect. Severity of postictal hypoxemia was greater in temporal lobe epilepsy and when hypoxemia was already observed before the onset of secondary GCS.

Ictal biting injuries in the epilepsy monitoring unit, a cohort study of incidence and semiological significance.

PURPOSE: Oral biting injuries are widely recognized complications of seizures. Value in differentiating epileptic vs paroxysmal non-epileptic seizures is well demonstrated, but frequency and semiological value are poorly described. We aimed to evaluate frequency in an epilepsy monitoring unit setting as well as semiological value. MATERIAL AND METHODS: Systematic evaluation of ictal oral injuries was provided by the nursing staff on the Epilepsy Monitoring Unit. Cases were identified by chart review. Demographic, diagnostic and seizure-related data were collected from medical records and video-EEG recording, and correlated with location of tongue laceration. Lateralized oral lacerations while recumbent were further analyzed to eliminate contribution of body position. RESULTS: 89 events were reported in 52 individuals (5.6% of all admissions or 8.3% of patients with epileptic seizures). Lacerations occurred in 88 epileptic seizures (80 generalized, three focal, five undetermined) and one non-epileptic seizure. 87/88 had a tonic component and 86/88 had a clonic component. Lateralized tongue laceration occurred in 30 patients. This correlated with body position during the event but not with handedness or seizure characteristics. CONCLUSIONS: Oral biting is a common complication in the epilepsy monitoring unit. Its presence suggests a tonic or clonic component but is of no help in localizing seizure onset.

Perampanel-induced hair curling in a patient with epilepsy associated with Pitt Hopkins syndrome.

Antiepileptic drug side effects are frequent, 42% of them corresponding to cosmetic changes. The most frequent effects are weight gain, gingival hyperplasia, and hair loss. Hair changes in texture or colour are rarely reported in the literature. We present a case of hair curling after the introduction of perampanel. A 13-year-old girl with genetically confirmed Pitt-Hopkins syndrome with uncontrolled seizures, while on treatment with levetiracetam and valproic acid, was started on perampanel, reaching seizure control. After a few weeks of the introduction of the new antiepileptic drug, she developed hair curling. Hair curling is a rare cosmetic side effect, reported mainly in patients under valproic acid treatment. Perampanel is a recently introduced pharmaceutical molecule with no prior reports of hair changes as a side effect. There is no clear explanation for this side effect, but it should be discussed with patients taking valproate whenever perampanel is added to the treatment.

The relationship between sleep and epilepsy.

The occurrence of seizures in the sleep state is observed in nearly one third of patients. This is caused by an intimate relationship between the physiological state of sleep and the pathological process underlying epileptic seizures. Both sleep and sleep deprivation influence the frequency of epileptiform discharges on electroencephalograms as well as the occurrence of clinical seizures, typically during nonrapid eye movement sleep. The relationship of epileptiform activity to nonrapid eye movement sleep is vividly shown in the syndrome of continuous spikes in slow-wave sleep and the Landau-Kleffner syndrome. Seizure semiology can also be influenced by sleep and sleep deprivation. Sleep disorders may influence seizure control, and effective treatment of sleep disorders can improve seizure control. Seizures, antiepileptic drugs, ketogenic diet, and vagus nerve stimulation all influence sleep quality, daytime alertness, and neurocognitive function.

Metabolic testing in the pediatric epilepsy unit.

Unexplained mental retardation is noted in up to 3% of the general population, and upwards of 30% of these patients manifest epilepsy. There is no standardized approach to metabolic testing in these patients. In a first step toward a standardized approach for our center, we performed a retrospective evaluation of testing of our patients in the past. Records of 429 children admitted to our pediatric epilepsy-monitoring center in 2005 were reviewed. We noted whether they had developmental delay, their type of epilepsy, and the extent of metabolic testing. Ninety percent of our patients had developmental delay, and 20% received some form of metabolic testing. Abnormal results suggesting mitochondrial dysfunction were found in 28% of our patients without a previous underlying diagnosis. Metabolic abnormalities were found in 75% of those with multifocal interictal discharges. Secondary carnitine deficiency was identified in 22%; these patients were taking valproic acid. Primary or secondary metabolic abnormalities likely represent a prominent finding in these patients. Differential diagnoses involving mitochondrial dysfunction should receive serious consideration. Findings such as carnitine deficiency can be identified and treated. A systematic approach for such testing is needed. A prospective evaluation at our institution is planned.