Semiologic aspects of epileptic seizures in 31 patients with hypothalamic hamartoma

Purpose: Characterization of seizure semiology in patients with hypothalamic hamartoma (HH) based on video–electroencephalography (EEG) monitoring (VEM). Methods: We retrospectively analyzed seizure semiology of 31 patients (20 male, mean age 23.5 years) who underwent VEM at the University Hospitals Freiburg or Heidelberg, Germany. Inclusion criteria were magnetic resonance evidence of an HH, no prior surgical or radiosurgical treatment, and at least two video‐documented seizures. A total of 263 seizures were included (mean number of seizures/patient 8.5, range 2–10). To analyze age‐dependent changes in seizure semiology, patients were grouped into “children” (3–11 years, n = 5), “adolescents” (12–17 years, n = 4), and “adults” (≥18 years, n = 22). Results: According to patient history, gelastic seizures had occurred in all patients, in 74% as the initial seizure type at epilepsy onset. In VEM, epileptic laughter varied from facial grinning to intense contractions of the diaphragm and body shaking. Unilateral motor signs were seen ipsi‐ and contralaterally to the HH. Tonic seizures were frequent and did not depend on the state of vigilance. Children, in contrast to adults and adolescents, did not show secondarily generalized tonic–clonic seizures, the gelastic component was the dominating and initial semiologic element, and seizures were significantly shorter. Conclusion: Seizure semiology is highly variable and age dependent. This may reflect network modulations with different propagation of ictal activity and/or secondary epileptogenesis. Detailed knowledge about such changes may contribute to both earlier recognition of seizures during childhood and better assignment of seizure types to a hypothalamic origin.     

Cognitive functions in juvenile and adult patients with gelastic epilepsy due to hypothalamic hamartoma

PURPOSE: To describe extend and severity of cognitive deficits in juvenile and adult patients with gelastic seizures and hypothalamic hamartoma (HH) and to analyze the impact of epilepsy-related variables on cognitive performance. METHODS: Thirteen juvenile and adult patients (mean age, 25 years; seven men) underwent comprehensive neuropsychological testing assessing intellectual performance, attention and executive functions, verbal and visual memory, and visuospatial abilities. RESULTS: Intellectual abilities ranged from moderate mental retardation to good average performance; 54% of the patients displayed below-average global intellectual abilities. Attentional and executive functions were impaired in 23% to 46% of the patients. Below-average visuospatial capabilities were observed in 39% of the cases. Memory functions were impaired regarding both visual (77%) and verbal learning (62%). Nonparametric correlation analysis revealed a significant relation between monthly partial seizure frequency and reduced cognitive flexibility and reduced performance in mental rotation. In addition, HH volume was significantly negatively correlated with cognitive flexibility, whereas age at onset and duration of epilepsy did not show significant correlation to cognitive performance. CONCLUSIONS: More than half of the adult patients with gelastic seizures and HH displayed deficits in a broad range of cognitive functions, expressed mostly in visual and verbal learning and memory. Some of the deficits could be shown to correlate with disease-related characteristics representing the severity of the epilepsy or the size of the underlying lesion. These findings prompt for a longitudinal investigation of the development of these cognitive deficits to analyze further the relevant factors contributing to this wide spectrum of cognitive impairments.     

Transcallosal resection of hypothalamic hamartoma for gelastic epilepsy

Introduction Hypothalamic hamartomas (HHs) are commonly associated with severe epilepsy resistant to anticonvulsant therapy. Historically, surgical resection of HHs resulted in considerable morbidity. Discussion Two series of patients who successfully underwent resection using a transcallosal approach have now been published; we report the first UK experience of this technique in a series of five patients with HHs and gelastic epilepsy resistant to anticonvulsant therapy. Patients were assessed pre- and postoperatively for seizure activity, endocrine function, ophthalmology, and neurocognitive function. Two patients had precocious puberty and all had evidence of developmental delay and behavioral problems. Postoperatively, all children experienced at least a 50% reduction in seizure frequency with abolition of major seizure types; one child remains seizure-free. One child developed a mild postoperative right hemiparesis and one developed transient diabetes insipidus. Conclusion There were no adverse developmental effects of surgery. Transcallosal resection of HHs ameliorates resistant epilepsy syndromes associated with HH.     

Cingulate epileptogenesis in hypothalamic hamartoma

Hypothalamic hamartoma (HH) is a relatively rare cause of epilepsy, mainly affecting children. Nearly all patients develop gelastic seizures, often followed by other focal seizure types. Our case illustrates the mechanisms of epileptogenesis in HH. The patient developed gelastic attacks as a baby, and secondarily generalized seizures and drop attacks at 9 years of age. Magnetic resonance imaging (MRI) confirmed the presence of a HH. Presurgical assessment with intracranial electroencephalography (EEG) monitoring recorded gelastic seizures with generalized epileptiform activity. Functional stimulation of the hamartoma provoked gelastic attacks. Single pulse electrical stimulation (SPES) was used to identify epileptogenic cortex. SPES of the left cingular cortex provoked generalized responses similar to the spontaneous generalized discharges. Our results suggest that long-standing history of epilepsy in patients with HH may be related to additional sources of epileptogenic activity. Electrical stimulation performed in this patient provided additional data to favor the hypothesis of secondarily epileptogenesis in the cingulate gyrus independently from the primary origin in the HH.     

Transcallosal resection of hypothalamic hamartoma for intractable epilepsy

PURPOSE: To present the results of transcallosal surgical resection of hypothalamic hamartoma (HH) in 26 patients with refractory epilepsy in a prospective outcome study. METHODS: Patients with refractory epilepsy symptomatic to HH were referred for surgical resection of their HH (mean age, 10.0 years; range, 2.1-24.2 years). A transcallosal, interforniceal approach was used to remove and/or disconnect the hamartoma. Volumetry was obtained on pre- and postoperative brain MRI scans to determine percentage of resection. Outcome assessment included determination of postoperative seizure frequencies in comparison to baseline and the incidence of postoperative complications. Postoperative changes in cognitive and behavioral functioning, in comparison to baseline, were elicited by parental report. RESULTS: The average postoperative follow-up interval was 20.3 months (range, 13-28 months). Fourteen (54%) patients were completely seizure free, and nine (35%) had at least a 90% improvement in total seizure frequency. Parents reported postoperative improvement in behavior in 23 (88%) patients and in cognition in 17 (65%) patients. Transient postoperative memory disturbance was seen in 15 (58%) patients, but persisted in only two (8%). Two (8%) patients had persisting endocrine disturbance requiring hormone replacement therapy (diabetes insipidus and hypothyroidism in one each). With univariate analysis, the likelihood of a seizure-free outcome correlated with younger age, shorter lifetime duration of epilepsy, smaller preoperative HH volume, and 100% HH resection. CONCLUSIONS: Refractory epilepsy associated with HH can be safely and effectively treated with surgical resection by a transcallosal, interforniceal approach. Short-term memory deficits appear to be transient for most patients, and family perception of the impact of surgery on cognitive and behavioral domains is favorable. Complete resection yields the best result.     

Gelastic seizures: Incidence,clinical and EEG features in adult patients undergoing video‐EEG telemetry

This study aimed to determine clinical features of adult patients with gelastic seizures recorded on video –electroencephalography (EEG) over a 5‐year period. We screened video‐EEG telemetry reports for the occurrence of the term “gelastic” seizures, and assessed the semiology, EEG features, and duration of those seizures. Gelastic seizures were identified in 19 (0.8%) of 2,446 admissions. The presumed epileptogenic zone was in the hypothalamus in one third of the cases, temporal lobe epilepsy was diagnosed in another third, and the remainder of the cases presenting with gelastic seizures were classified as frontal, parietal lobe epilepsy or remained undetermined or were multifocal. Gelastic seizures were embedded in a semiology, with part of the seizure showing features of automotor seizures. A small proportion of patients underwent epilepsy surgery. Outcome of epilepsy surgery was related to the underlying pathology; two patients with hippocampal sclerosis had good outcomes following temporal lobe resection and one of four patients with hypothalamic hamartomas undergoing gamma knife surgery had a good outcome.     

The clinical spectrum of epilepsy in children and adults with hypothalamic hamartoma

PURPOSE: Hamartomas of the hypothalamus (HH) cause an uncommon and unusual epilepsy syndrome. The condition is recognized to affect children, but the presentation in adults is not well understood. We present 19 children and adult patients with HH, including three patients whose epilepsy began in adult life. The patterns of clinical presentation, evolution of the epilepsy from childhood to adult life, and electroclinical diagnostic features are presented. METHODS: Nineteen patients, both children and adults with HH and epilepsy, were evaluated clinically, with EEG, video-EEG, and magnetic resonance imaging (MRI) scanning. Seven patients underwent surgical resection of the hamartoma. Stereotactic thermocoagulation of the hamartoma was performed in two patients. RESULTS: Gelastic seizures occurred at onset of epilepsy in 15 of 16 early-onset cases. Subsequently, multiple seizures types occurred, which then evolved to mainly partial epilepsy with tonic or complex partial seizures (five of eight adults), or became entrenched symptomatic generalized epilepsy with atypical absences, drop attacks, and secondarily generalized seizures, and cognitive impairment (three of eight adults). In the adult-onset patients, gelastic seizures were not prominent, the epilepsy was milder, and they functioned normally. Stereotactic thermocoagulation of the hamartoma resulted in improvement in seizure control in two patients. CONCLUSIONS: Gelastic seizures are not a prominent feature of epilepsy in adult patients with HH. The epilepsy associated with HH, although severe at onset, can evolve into a milder syndrome in later life. For less severely affected patients, minimally invasive alternatives to the traditionally difficult open surgical treatment should be considered.     

A review on the management of epilepsy associated with hypothalamic hamartomas

Introduction  Hypothalamic hamartomas are rare congenital malformations located in the region of the tuber cinereum and third ventricle. Patients may be asymptomatic, but the usual presentation is gelastic seizures, precocious puberty, and/or developmental delay. Clinical presentation  Without surgical intervention, the gelastic seizures, which are typically present in childhood, may progress to other seizure types, including generalized epilepsy, and are generally refractory to antiepileptic drugs. Summary  This review will discuss the clinical and electrophysiologic aspects of these lesions, as well as treatment options, including surgery, endoscopy, and radiosurgery.     

Functional brain mapping of ictal activity in gelastic epilepsy associated with hypothalamic hamartoma: A case report

Hypothalamic hamartomas (HHs) have been demonstrated as the cause of gelastic epilepsy, both by intracranial electrodes and functional imaging. The neocortex becomes secondarily involved, through poorly characterized propagation pathways. The detailed dynamics of seizure spread have not yet been demonstrated, owing to the limited spatial–temporal resolution of available functional mapping. We studied a patient with epilepsy associated with HH and gelastic epilepsy. Simultaneous electroencephalography (EEG) and functional magnetic resonance imaging (fMRI) of several seizure events were obtained, with blood oxygen level dependent (BOLD) activation of the hamartoma, and left hemisphere hypothalamus, hippocampus, parietal–occipital area, cingulate gyrus, and dorsal–lateral frontal area. Integration of regional BOLD kinetics and EEG power dynamics strongly suggests propagation of the epileptic activity from the HH through the left fornix to the temporal lobe, and later through the cingulate fasciculus to the left frontal lobe. The EEG/fMRI method has the spatial–temporal resolution to study the dynamics of seizure activity, with detailed demonstration of origin and propagation pathways.     

Clinical characteristics and long-term outcome of surgery for hypothalamic hamartoma in children with refractory epilepsy

Context:

Hypothalamic hamartomas (HH) are ectopic masses of neuronal and glial tissue most commonly presenting with medically refractory gelastic seizures with evolution to other seizure types. They are also associated with cognitive and behavioral problems to varying extent. Surgery has been found to improve quality of life in more than 50% of patients.

Aim:

To evaluate the clinical characteristics and long-term outcome of surgery in children with HH and refractory epilepsy.

Materials and Methods:

A retrospective analysis of presurgical, surgical, and postsurgical data of six children who underwent surgery for HH and had at least 3 years follow-up was performed.

Results:

Six children (male: female = 5:1) aged 3-16 years (at the time of surgery) underwent surgical resection of HH for refractory epilepsy. At last follow-up (range 3-9 years), three children were in Engel''s class I, two in Class II, and one in class III outcome. Significant improvement in behavior, quality of life was noted in four children; while the change in intelligence quotient (IQ) was marginal.

Conclusions:

Medically refractory epilepsy associated with behavioral and cognitive dysfunction is the most common presentation of HH. Open surgical resection is safe with favorable outcome of epilepsy in 50% with significant improvement in behavior and marginal change in cognitive functions.     

Clinical significance of nonhabitual seizures during intracranial EEG monitoring

This study sought to determine the frequency and clinical significance of nonhabitual seizures in 101 consecutive patients undergoing presurgical intracranial electroencephalography intracranial (icEEG) recording. We compared clinical data, recording details, and postsurgical outcome in patients with nonhabitual seizures to those without nonhabitual seizures during icEEG. In patients with nonhabitual seizures we compared icEEG recordings of nonhabitual seizures to recordings of habitual seizures. Nonhabitual seizures were recorded in 10% of patients. Those patients had a significantly higher rate of procedure‐related complications compared to patients without nonhabitual seizures. Ultimate seizure outcome did not differ between the groups. Nonhabitual seizures often occurred within the first 3 days of icEEG recording and had larger seizure‐onset zones compared to the patient's habitual seizures. Nonhabitual seizures have no effect on outcome of epilepsy surgery but may serve as important markers of procedure‐related complications during icEEG.     

长程视频脑电监测在下丘脑错构瘤诊断治疗中的应用(附5例报告)

目的探讨长程视频脑电监测在下丘脑错构瘤诊断治疗中的应用意义。方法回顾性分析5例下丘脑错构瘤长程视频脑电(VEEG)特征,VEEG结合磁共振成像(MRI)、发作间期正电子发射计算机断层扫描(PET)检查诊断定位。结果发作间期清醒平静状态脑电图表现:双侧波形均呈不对称表现,双侧存在广泛单发性棘慢波或者双侧广泛不规则θ或δ波,一侧波幅优势,主要为一侧额叶优势,2例左侧优势,3例右侧优势,优势侧别同MRI显示的错构瘤侧别一致;睡眠期脑电图表现:存在基本睡眠标志波形与睡眠周期,间有较多量棘慢波或多棘慢波,存在形式同间期清醒平静状态;5例均捕获临床发作过程,共计13次,其中痴笑发作8次、痴笑发作继发全身强直阵挛5次,发作期脑电图表现:3例为去同步化低电压数秒后EEG混合肌电干扰,2例以肌电伪差为主。MRI结果:下丘脑脚间池处部位可见占位性改变,位于左侧半球2例、右侧半球3例。PET结果:MRI所提示的占位性改变区域均显示低代谢。5例均手术彻底去除错构瘤,随访5例患者术后均无痴笑发作或继发全身强直阵挛。结论长程视频脑电监测结合MRI及PET检查对下丘脑错构瘤诊断定位准确性高,手术治疗下丘脑错构瘤是最佳选择。     

EEG dipole source localisation of interictal spikes acquired during routine clinical video-EEG monitoring

OBJECTIVE: We investigated the feasibility of electroencephalography (EEG) dipole source localisation of interictal epileptiform discharges from data acquired during routine clinical inpatient video-EEG monitoring (VEM) and compared a 19-channel 'routine montage' with a 29-channel 'surgical montage' that includes an additional row of 10 inferior temporal electrodes. METHODS: Twenty consecutive patients who had VEM for the presurgical evaluation of medically refractory partial epilepsy were screened. Thirteen of the patients had focal interictal spikes recorded, and in 11 (85%) these were technically satisfactory for source localisation. Fourteen spike foci were analysed as 3 patients had bilateral independent spikes. EEG data was acquired with 29 electrodes including an inferior temporal row (surgical montage). For comparison, the 10 additional electrodes were excluded from analysis (routine montage). Using NEUROSCAN Source 2.0 software, a computed dipole source localisation of averaged spikes was performed utilising a magnetic resonance imaging-based finite element model. Dipole localisation was compared with that of the Comprehensive Epilepsy Program (CEP) evaluation. RESULTS: Using the surgical montage dipole source localisation was consistent with the CEP spike localisation for 13/14 spikes (93%, P<0.005), compared with only 5/14 spikes (36%) using the routine montage. CONCLUSIONS: Data derived from routine clinical inpatient VEM using a routine montage can yield accurate EEG dipole source localisation, but significantly more accurate localisation is obtained using the surgical montage.     

Ictal bradycardia in a patient with a hypothalamic hamartoma: a stereo-EEG study

Little is known about bradycardia and cardiac asystole which occur during partial epileptic seizures, especially whether they relate to ictal involvement of well-defined cortical areas. Several reports based on simultaneous electrocardiographic and intracranial depth electroencephalographic monitoring have shown that either the fronto-orbital cortex or the amygdalohippocampal complex could be responsible for such cardiac variations. We performed stereo-EEG recordings in a patient with refractory localization-related epilepsy associated with a hypothalamic hamartoma. We found that other cortical areas, such as the frontocentral region and the temporal neocortex, can contribute to the genesis of ictal bradyarrhythmia. Second, the lesion per se, although located within the hypothalamus, is not involved with this phenomenon.     

Intensive EEG monitoring and psychological studies of patients with pseudoepileptic seizures

EEG/closed-circuit television long-term monitoring was used as a definitive diagnostic tool to identify and characterize 25 patients with pseudoepileptic seizures and a similar group of subjects with epilepsy, confirming the value of the procedure. The groups did not differ with respect to intelligence, neuropsychological impairment, or incidence of potential etiological factors for seizures. Scores on the Minnesota Multiphasic Personality Inventory (MMPI) and the Hypochondriasis, Hysteria, and Schizophrenia Scales were significantly higher for the pseudoepileptic group than for the other subjects. As a whole, the former patients exhibited an MMPI profile pattern frequently seen in the conversion form of hysteria. A set of three rules derived from the MMPI profiles was used to classify the patients correctly in 80-90% of cases. As evaluated by the Washington Psychosocial Seizure Inventory, psychosocial problems of patients with pseudoepileptic seizures were more severe in certain areas, and appeared to reflect early family background problems and inappropriate management of their disorders.     

Hyperventilation revisited: physiological effects and efficacy on focal seizure activation in the era of video-EEG monitoring

PURPOSE: Hyperventilation is an activation method that provokes physiological slowing of brain rhythms, interictal discharges, and seizures, especially in generalized idiopathic epilepsies. In this study we assessed its effectiveness in inducing focal seizures during video-EEG monitoring. METHODS: We analyzed the effects of hyperventilation (HV) during video-EEG monitoring (video-EEG) of patients with medically intractable focal epilepsies. We excluded children younger than 10 years, mentally retarded patients, and individuals with frequent seizures. RESULTS: We analyzed 97 patients; 24 had positive seizure activation (PSA), and 73 had negative seizure activation (NSA). No differences were found between groups regarding sex, age, age at epilepsy onset, duration of epilepsy, frequency of seizures, and etiology. Temporal lobe epilepsies were significantly more activated than frontal lobe epilepsies. Spontaneous and activated seizures did not differ in terms of their clinical characteristics, and the activation did not affect the performance of ictal single-photon emission computed tomography (SPECT). CONCLUSIONS: HV is a safe and effective method of seizure activation during monitoring. It does not modify any of the characteristics of the seizures and allows the obtaining of valuable ictal SPECTs. This observation is clinically relevant and suggests the effectiveness and the potential of HV in shortening the presurgical evaluation, especially of temporal lobe epilepsy patients, consequently reducing its costs and increasing the number of candidates for epilepsy surgery.     

Clinical and ictal characteristics of infantile seizures: EEG correlation via long-term video EEG monitoring

Purpose

The semiology of infantile seizures often shows different characteristics from that of adults. We performed this study to describe clinical and ictal characteristics of infantile seizures at less than two years of age.

Methods

A retrospective study was done for infants with epilepsy (ages: 1–24 months) who underwent long-term video electroencephalography (EEG) monitoring at Samsung medical center between November 1994 and February 2012. We analyzed the clinical and ictal characteristics of the 56 cases from 51 patients.

Results

In 69% of the patients, the seizure onset was before six months of age and the etiology was symptomatic in one third of the patients. Twelve seizure types were identified; spasms (24%), unilateral motor seizures (18%), and generalized tonic seizures (15%) were the three frequent types of seizure.All partial seizures were well correlated with the partial-onset ictal EEG, however 19.4% (7/36) of clinically generalized seizures revealed partial-onset ictal EEG. About one-thirds (4/11) of generalized tonic seizures had its ictal onset on unilateral or bilateral frontal areas and two out of seven generalized myoclonic seizures showed unilateral frontal rhythmic activities. Hypomotor seizures mainly arose from the temporal areas and hypermotor seizures from the frontal regions.

Conclusions

Even though most of the seizure semiology of infants is well correlated with ictal EEG, some of the generalized tonic seizures or myoclonic seizures revealed partial-onset ictal EEG suggesting localized epileptic focus. Accurate definition of seizures via video EEG monitoring is necessary for proper management of seizures in infancy, especially in some clinically generalized seizures.     

Hypothalamic hamartoma: the role of surgery in a series of eight patients

Hypothalamic hamartoma are rare lesions. We report a new series of eight patients treated for precocious puberty (six cases) or gelastic seizures (two cases). Surgical resection was total in four cases (three pediculated and one sessile). Precocious puberty was controlled by surgical treatment in all cases. Gelastic seizures were controlled by medical treatment, but the patients did not become seizure free. We observed no mortality and no endocrinological or visual morbidity. The fact that a vascular ”rete mirabilis” was observed on the surface of the lesion in our surgical material is an argument favoring a vascular mechanism in precocious puberty. Coagulation of this vascular structure can help control precocious puberty. Our series confirms that the hypothalamic hamartoma can be surgically treated when patients fail to respond to medical treatment, when the length of the treatment cannot be tolerated by the chidren and their families, and when there are uncontrolled gelastic seizures Received: 14 February 2000     

The value of long term EEG monitoring in children: A comparison of ambulatory EEG and video telemetry

PurposeOutpatient ambulatory EEG may be followed by inpatient video telemetry EEG when investigating children for possible seizures and for classification of epilepsy. We investigated the value of ambulatory EEG and subsequent video telemetry recording in our centre.MethodThe departmental EEG database was interrogated retrospectively for children undergoing ambulatory recording followed by inpatient video telemetry within an 18-month period.Results30 patients fitted these criteria, 21 females, 9 males, age range 3–16 years. The mean interval between studies was 9 months. For ambulatory recordings 93% of studies were undertaken to ascertain if behaviours were epileptic. 66% of ambulatory recordings studies captured an event of interest and 63% were able to answer the question asked of the test. In video telemetry recording 80% of studies were aimed at ascertaining if events were epileptic or not, 20% were undertaken for classification of seizure type. 70% of recordings captured an ictus and were considered helpful in addressing the clinical question. Pooled together 90% of patients had a paroxysmal event captured and the clinical question answered by the recording techniques. In patients for whom ambulatory recording failed to capture an attack or answer the clinical question, 70% went on to have a successful video telemetry recording.ConclusionBoth ambulatory EEG and inpatient video telemetry are effective tools for diagnosis of seizures. The majority of patients with failed ambulatory recordings go on to have successful video telemetry. Combining the two resources provides useful clinical information in nearly all instances.