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Review article
Temporal lobe epilepsy in children: overview of clinical semiology
Amit Ray, Prakash Kotagal
epilepsy syndromes, tumors and malformations of cortical development are more commonly seen in children. Differences in seizure semiology are also apparent. Temporal lobe epilepsy in very young infants may exhibit prominent motor manifestations reminiscent of extra-temporal seizures. These motor manifestations however decrease with increasing age and are less abundant in adults. Automatisms, which are commonly seen in temporal lobe epilepsy in all age groups, are simple at a younger age and become increasingly more complex and discrete with age. Several case studies, illustrated on the video are included in this review. They highlight the differences in temporal lobe seizure semiology between children and adults. [Published with video sequences]
Original article
A proposal for a five‐dimensional patient‐oriented epilepsy classification
Tobias Loddenkemper, Christoph Kellinghaus, Elaine Wyllie, Imad M. Najm, Ajay Gupta, Felix Rosenow, Hans O. Lüders
according to various, poorly defined parameters. The resulting syndromes have frequently no biological significance, with overlap among different syndromes and syndromes changing with age. Additionally, only a minority of patients can be classified syndromatically, and the axes of this classification system convey redundant information. We propose a five‐dimensional, patient‐oriented approach to classifying epilepsies. This approach shifts from the syndrome‐oriented approach to a standard, neurological, methodological, patient‐oriented approach, using independent criteria in each of the five dimensions. Similar to general neurology, the first step in each patient‐physician encounter in epileptology is to take a history of the presenting symptoms and generate a hypothesis regarding the localization and etiology of the symptom within the nervous system. Therefore, the main dimensions of this classification consist of: 1) localization of the epileptogenic zone, 2) seizure semiology classified according to the semiological seizure classification, 3) etiology, 4) seizure frequency, and 5) related medical conditions. These dimensions characterize all of the information necessary for patient management, are independent parameters, and include more pertinent information with regards to patient management than the ILAE axes. All patients can be classified according to this five‐dimensional system even at the initial patient encounter when no detailed test results are available. Information from clinical tests, such as MRI and EEG, are translated into the best possible working hypothesis at the time of classification, allowing for increasing precision of the classification as additional information becomes available. This patient‐oriented classification envisions an epileptic seizure as an independent symptom of a central nervous system dysfunction due to different causes, with various cortical localizations, occurring at various frequencies, and in conjunction with other diseases and clinical symptoms.
Classification is not EZ (Invited Editorial Comment)
Jerome Engel
Developmental outcome of epilepsy surgery in tuberous sclerosis complex
Charles M Zaroff, Chris Morrison, Nina Ferraris, Howard L Weiner, Daniel K Miles, Orrin Devinsky
is increasingly recognized as a therapeutic option given the often medication-resistant nature of the disease. Seven subjects with medically refractory epilepsy associated with TSC, who underwent surgery at a tertiary care epilepsy center and in whom both preoperative and postoperative neuropsychological data were available, were examined. The Vineland Adaptive Behavior Scales, and in one case, the WISC-III were utilized. Postoperatively, the composite standard scores declined in six of the seven subjects, although for the most part this decline was quite modest (8 points or less in 5/6 subjects). The mean overall developmental/intellectual quotients were comparable across assessments (preoperative M = 55, SD = 20.3; postoperative M = 49 SD = 16.6). Good outcomes appeared to be related to seizure relief. Age estimates of developmental level indicated developmental progress in the majority of subjects in the current sample, and may yield greater clinical information for individuals with developmental delay than do standard scores.
Tonic status epilepticus in patients with idiopathic generalized epilepsy
Eliane Kobayashi, Pierre Thomas, Frederick Andermann
patients with IGE. Methods. We retrospectively reviewed the clinical and EEG evaluation of three IGE patients who presented TSE. Results. The three patients had mainly clinical features of IGE, but had developed, in addition, focal discharges, diffuse EEG abnormalities and some focal or diffuse neuropsychological dysfunction. The tonic attacks eventually responded to treatment, but were not completely controlled in any of the patients. Discussion. The continuum between IGE and secondary generalized epilepsy is demonstrated in these patients. Most of their clinical and EEG features are however, in keeping with an idiopathic generalized epileptic process with additional focal and diffuse components. Recognition of the significance of TSE in such patients has important therapeutic and prognostic implications. [Published with video sequences]
Ictal video-polysomnography and EEG spectral analysis in a child with severe Panayiotopoulos syndrome
Pasquale Parisi, Raffaele Ferri, Jacopo Pagani, Manuela Cecili, Enza Montemitro, Maria Pia Villa
single, prolonged, nocturnal, focal seizures comprising an unusual constellation of autonomic symptoms (malaise, nausea, pallor, tachycardia, vomiting) and unilateral deviation of the eyes at the onset of seizures. These clinical, ictal manifestations are rarely followed by post-ictal headache. In the literature, there is little information on the ictal EEG characteristics of Panayiotopoulos syndrome and, in particular, on certain autonomic manifestations, such as tachycardia, as the sole ictal phenomena at the onset of seizures.
<b>Methods and results.</b> One, all-night videopolysomnography, during which one seizure was recorded. Video-EEG data were evaluated visually and by means of quantitative spectral analysis. The spectral analysis of the recorded seizure showed a complex ictal pattern of cortical involvement with focal onset in the right occipital area followed by the recruitment of widespread extra-occipital cortical regions.
<b>Conclusions.</b> This is the first such analysis of this peculiar epileptic condition. Most of the symptoms were consistent with a diagnosis of severe Panayiotopoulos syndrome, although the patient also presented “atypical findings”: a relatively high frequency of seizures, post-ictal headache, no spontaneous remission of seizures with age, and late onset of visual hallucinations; this last finding is more frequent in "Gastaut-type childhood occipital epilepsy", in which onset typically occurs later than in Panayiotopoulos syndrome. [Published with video sequences]
Late-onset, “Gastaut Type”, childhood occipital epilepsy: an unusual evolution
Roberto Horacio Caraballo, Ricardo Oscar Cersósimo, Natalio Fejerman
unusual evolution. Neurological examination and brain imaging were normal in all three. At the age of 7.5 years, eight years and ten years respectively, the three children presented with episodes of visual symptoms when awake, and in one of them, the seizures were occasionally followed by oculocephalic deviation. The interictal EEG showed bilateral occipital spike-wave activated by eye closing. In two patients, the occipital seizures had been immediately followed by typical absences, since onset; in the other patient, five months after onset. The ictal EEG showed irregular bilateral occipital spike-wave discharges during the visual symptoms, followed by generalized spike-wave activity during the typical absences. The typical absences were activated by hyperventilation; the EEG did not show continuous spikes and waves during slow sleep. These three patients, with typical electroclinical features of “Gastaut type”, childhood occipital epilepsy, demonstrated an evolution which, to our knowledge, has not been previously described. We investigated whether this unusual, age-dependent evolution was due to secondary bilateral synchrony or if these electroclinical features represent two types of idiopathic epileptic syndromes in the same patients.
Subjective signs in premotor epilepsy: confirmation by stereo-electroencephalography
Katell Beauvais, Arnaud Biraben, Eric Seigneuret, Stephan Saïkali, Jean-Marie Scarabin
premotor epilepsy. Six of them had focal cortical dysplasia. Ictal symptomatology was analysed to establish electroclinical correlations. The localisation of the epileptogenic zone was assessed by stereoelectroencephalographic studies. Subjective manifestations were described in all cases, more frequently in a sensory rather than an emotional or psychological fashion. Focal seizures limited to subjective features were recorded in two patients. In one, psychological illusions and visual hallucinations were related to the superior frontal sulcus. Another presented isolated paraesthesia in the left arm with the implication of the supplementary motor area. Electrical stimulation of an electrode located in the premotor area evoked isolated subjective manifestations in three other patients. One patient reported sensory manifestations and another, ideational manifestations. Cephalic sensations and emotional manifestations were associated in one case. Subjective manifestations were observed in all patients, and were proved to be related to a discharge restricted to the premotor area in five. These were non-specific signs, but were always the same in a given patient. Spontaneous, isolated sensations and stimulation data tended to be contradictory. This illustrates the complexity of analyzing subjective signs, as well as the complexity of the neuronal networks participating in the propagation of discharges arising in the premotor frontal area.
Spike frequency is dependent on epilepsy duration and seizure frequency in temporal lobe epilepsy
Jozsef Janszky, M Hoppe, Z Clemens, I Janszky, C Gyimesi, R Schulz, A Ebner
Methods. We included all patients who consecutively underwent the adult presurgical evaluation program at our center and who had intractable, medial TLE with complex partial seizures due to unilateral medial temporal lobe lesions. The interictal EEG samples were automatically recorded and stored on computer. The location and frequency of interictal epileptiform discharges were assessed by visual analysis of interictal EEG samples of 2-minute duration every hour. Results. There were 303 patients (aged 16-63) who met the inclusion criteria. The median interictal epileptiform discharge frequency was 15 IED/h, the median seizure frequency was 4 seizures/month. According to univariate analyses, we found that age at monitoring, epilepsy duration, and higher seizure frequency were associated with higher interictal epileptiform discharge frequency. In the logistic regression analysis, we found that higher seizure frequency (p < 0.001) and longer epilepsy duration (p = 0.007) were independently associated with higher spike frequency, while the age at monitoring was not. Conclusions. Seizure frequency and epilepsy duration (years of patient’s life with seizure activity) were independently associated with IED frequency, suggesting that IED are modulated by seizures.
Familial rectal pain: a familial autonomic disorder as a cause of paroxysmal attacks in the newborn baby
Nathalie Bednarek, Anne-Sophie Arbuès, Jacques Motte, Pascal Sabouraud, Perrine Plouin, Patrice Morville
dysautonomy called “familial rectal pain”. These events were recurrently triggered by emotion, diaper changes or wiping of the perineal areas or eating. Sometimes they occurred spontaneously. Carbamazepine had an excellent effect on the fainting. At four years of age, the child had normal psychomotor development with only minimal symptoms, and very rare paroxysmal attacks. The father of the child has minimal symptoms of this entity, with essentially ocular manifestations. Familial rectal pain is a very rare entity that must always be considered as a possible aetiology of any life-threatening event in an infant because of the availability of a very effective treatment. The existence of minimal forms of familial rectal pain is possible, and it is likely that this entity is underdiagnosed. [Published with video sequences].
A comparative study of mismatch negativity (MMN) in epilepsy and non-epileptic seizures
Nuri Gene-Cos, Richard Pottinger, Geoff Barrett, Michael R Trimble, Howard A Ring
(ERPs) were obtained from 14 patients with NES, 15 patients with epilepsy and 16 healthy control subjects. A conventional MMN procedure was used with a random sequence of 12% deviant tones (922 Hz) and 88% standard tones (1000 Hz). Subjects were instructed to ignore the tones delivered through headphones whilst reading a book. Significant differences in distribution of the mismatch negativity (MMN) in patients with NES compared to controls were obtained (F3, p ≤ 0.019; Cz, p ≤ 0.044) and longer MMN duration in patients with epilepsy compared with patients with NES (p ≤ 0.039) was observed. The change that has been analyzed is one of relative (or scaled) amplitude rather than absolute amplitude. These differences observed at Cz/F3 suggest an increase in emphasis of the MMN in the frontocentral region in patients with NES compared to healthy controls, suggesting that the MMN is generated in a different way in NES compared with controls. This could indicate that one of the normal MMN generator areas does not function normally in NES. Increased absolute amplitude of the MMN has previously been observed in anxiety disorders particularly in post-traumatic stress disorder (PTSD). We discuss similarities between NES and PTSD, suggesting that the increased relative amplitude obtained in this study may be related to mechanisms of generation of NES. The prolonged duration of the MMN in epilepsy could be related to difficulties in processes associated with novelty discrimination (closure of MMN generating mechanism). This information processing dysfunction could be associated with the concentration and memory difficulties that are observed in some patients with epilepsy. This study provides electrophysiological evidence of abnormal processing of auditory stimuli in both clinical conditions when compared to healthy controls, and interictal differences between a group of patients with epilepsy and a group of patients with non-epileptic seizures, as measured by the MMN.
Typical childhood absence seizures are associated with thalamic activation
A Labate, RS Briellmann, DF Abbott, AB Waites, Graeme D Jackson
IGE, performed prior to the start of antiepileptic medication. The 7-years-old girl had very frequent absences, associated with eyelid myoclonia. Her EEG showed frequent, typical 3/sec discharges. Functional MRI was performed with a 3T scanner using whole brain gradient echo-planar imaging, and the EEG was recorded with 18, non-metallic, scalp electrodes. Ten bursts of generalized discharges were captured during 30 minutes fMRI/EEG acquisition. The bursts lasted 3.4 (SD ± 0.6) seconds. Event-related analysis was performed with SPM2 and iBrain
<sup>TM</sup> software. Functional MRI showed prominent, bilateral thalamic activation, and less pronounced areas of cortical activation and deactivation. This study demonstrates thalamic activation in typical, untreated childhood absence epilepsy. The cortical signal change may be related to a thalamo-cortical circuit.
Video case report
Partial seizures triggering infantile spasms in the presence of a basal ganglia glioma
Rajesh RamachandranNair, Ayako Ochi, Tomoyuki Akiyama, David J Buckley, Teesta B Soman, Shelly K Weiss, Hiroshi Otsubo
age group. We report the case of a female infant with a low-grade glioma in the right basal ganglia, spreading to the cortical area. She presented at the age of five months with left hemiparesis and partial seizures. She developed infantile spasms at the age of 12 months. This is the first video clip report of partial seizures triggering symmetrical spasms in series, secondary to a basal ganglia glioma extending to the cortex. [Published with video sequences]
