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Review article
Therapy of encephalopathy with status epilepticus during sleep (ESES/CSWS syndrome): an update
Pierangelo Veggiotti, Maria Carmela Pera, Federica Teutonico, Daniela Brazzo, Umberto Balottin, Carlo Alberto Tassinari
seizure types, global or selective neuropsychological regression, motor impairment, and a typical EEG pattern of continuous epileptiform activity for more than 85% of non-rapid eye movement (NREM) sleep. Although the first description of ESES/CSWS dates back to 1971, an agreement about the optimal treatment for this condition is still lacking. ESES/CSWS is rare (incidence is 0.2-0.5% of all childhood epilepsies) and no controlled clinical trials have been conducted to establish the efficacy of different antiepileptic drugs; only uncontrolled studies and case reports are reported in the literature. Treatment options for ESES/CSWS include some antiepileptic drugs (valproic acid, ethosuximide, levetiracetam, and benzodiazepines), steroids, immunoglobulins, the ketogenic diet, and surgery (multiple subpial transections). In this study, the comparative value of each of these treatments is reviewed and a personal therapeutic approach is proposed.
Progress in Epileptic Disorders
Starting at the beginning: the neuropsychological status of children with new-onset epilepsies
Bruce P Hermann, Jana E Jones, Daren C Jackson, Michael Seidenberg
epilepsies, attention turns to cognitive, psychiatric and social correlates of childhood epilepsies reported in population-based and tertiary care studies. The focus then becomes the neurobehavioural status of children with new-onset epilepsy; a point in time not confounded by the effects of years of recurrent seizures, medications, and social reactions to epilepsy. Recent research shows that abnormalities in cognition, brain structure and behaviour are present at or near the time of diagnosis. Further, careful history taking indicates that neurobehavioural problems may be present in advance of the first seizure suggesting the potential influence of epileptogenesis, antecedent neurodevelopmental abnormalities, genetic and environmental susceptibilities, and other risk factors. This becomes the substrate upon which to characterise the effects of epilepsy and its treatment on subsequent neurodevelopment. The review concludes with suggestions for future clinical care and research.
Original article
Parietal lobe epilepsy: the great imitator among focal epilepsies
Aleksandar J Ristić, Andreas V Alexopoulos, Norman So, Chong Wong, Imad M Najm
that such widespread projections are responsible for producing inaccurate localisation readings on scalp EEG and clinical semiology in patients with parietal lobe epilepsies, as opposed to frontal or temporal lobe epilepsies.
<i><b> </b>Methods.</i> Our study included 50 patients with pharmacoresistant focal epilepsy, who were subsequently rendered seizure-free for ≥12 months (median: 23 months) following resections limited to the frontal (
<i>n</i>=17), temporal (
<i>n</i>=17), or parietal (
<i>n</i>=16) lobes. Interictal and ictal EEG data with accompanying seizure video recordings were extracted from archived files of scalp video-EEG monitoring. Two blinded raters independently reviewed the EEG according to predetermined criteria. Videos of seizures were then observed, as raters formulated their final electroclinical impression (ECI), identifying patients’ abnormal neuronal activities with parietal, temporal, and frontal lobe epilepsy, or unspecified localisation.
<i> Results.</i> Groups did not differ significantly in demographics, age at epilepsy onset, or presence of MRI abnormalities. Interictal discharges in parietal lobe epilepsy showed the greatest magnitude of scatter outside the lobe of origin; the majority of patients with parietal lobe epilepsy had more than one spike population (
<i>p</i><0.045). Localised ictal EEG recognition was most frequent in temporal, followed by frontal and parietal lobe epilepsy cases (
<i>p</i>=0.024). Whenever raters confidently limited their ECI to one lobar subtype, overall accuracy was excellent. Lobar classifications by ECI were highly accurate for temporal lobe epilepsy, vacillating in frontal lobe epilepsy, and least accurate in parietal lobe epilepsy subjects.
<i>Conclusion</i>. Scalp EEG readings of parietal lobe epilepsy patients showed a more variable scatter of interictal discharges and a lower localisation value of ictal recordings compared to temporal and frontal lobe epilepsy subjects, suggesting an increased likelihood of misidentification and mislocalisation of parietal lobe epilepsy. Combining seizure semiology with scalp interictal and ictal EEG readings facilitates a more accurate lobar classification in patients with temporal and frontal, but not parietal, lobe epilepsy.
Clinical patterns and pathophysiology of hypermotor seizures: an ictal SPECT study
Hiroshi Masuda, Erum Shariff, John Tohyama, Hiroatsu Murakami, Shigeki Kameyama
of seizure onset, similar semiologic features during seizures may exist. Ictal single-photon emission tomography (SPECT) apparently reflects not only the origin of epileptic discharge but also the spread to adjacent cortical areas. Taking this benefit of SPECT studies into account, preoperative SPECT results from 13 patients with HMS who underwent epilepsy surgery were analysed. The radioisotope 99mTc-ECD was injected in all patients within five seconds after seizure onset. Group analysis was performed with statistical parametric mapping (SPM) of paired ictal-interictal SPECTs in order to identify regions of significant ictal hyperperfusion. Hyperperfused regions with a corrected cluster-level significance
<i>p</i>-value of < 0.002 were considered significant. Seizure onset at implanted subdural electrodes was defined as the epileptic focus in 12 of 13 patients. Two patterns were recognized: HMS-1 with marked agitation and HMS-2 with mild agitation. Ictal hyperperfusion images revealed significant hyperperfusion in the anterior cingulate cortex, orbito-frontal gyrus, lentiform nucleus, midbrain and pons. These hyperperfused areas represent the symptomatogenic zone which was different from the epileptogenic zone, as confirmed by the favourable outcomes after surgical resection. The present findings suggest that a network, including frontal and possibly extrafrontal brainstem and limbic structures, is involved in the genesis of the complex epileptic manifestations of HMS. Moreover, ictal SPECT analysed by SPM is a useful method for studying the neural networks of different types of seizures.
Ambulatory non-convulsive status epilepticus evolving into a malignant form
José L Fernández-Torre, Peter W Kaplan, Mariano Rebollo, Agustín Gutiérrez, Miguel A Hernández-Hernández, José L Vázquez-Higuera
case series with ambulatory non-convulsive status epilepticus (NCSE) which evolved into a malignant form (mNCSE). mNCSE was defined as NCSE that continues or recurs five days or more after the onset of general anaesthesia, including those cases where NCSE recurs upon reduction or withdrawal of anaesthetic therapy. Four women and two men were studied. The mean age was 42.8 years (range: 19-63 years). Two of six patients had a previous diagnosis of epilepsy. Four patients died. Two patients had a good outcome with return to consciousness and activities of daily life, and one of these subjects developed temporal lobe epilepsy. In four patients, the cause of mNCSE was encephalitis. Clinical presentation was complex partial status epilepticus (CPSE) in three patients and generalised NCSE (GNCSE) in the other three. Two of the latter had encephalitis and one was diagnosed with progressive myoclonus epilepsy (PME) of Lafora type. The mean duration of the NCSE episode was 47.5 days (range: 9-139 days; SD: 53.1). In three patients, continuous or rhythmic focal epileptiform discharges were the EEG pattern at onset. In the other three, continuous rhythmic generalised spike-wave, polyspike-wave or sharp slow-wave complexes were observed. Antiepileptic treatment was heterogeneous and patients were treated with an average of 6.0 AEDs (range: 3-10; SD: 3.0). In summary, mNCSE is a severe epileptic condition that occurs often in the context of encephalitis or pre-existing epilepsy. Both CPSE and GNCSE may occur. Although high mortality is associated with mNCSE, some patients may survive with normal or good recovery.
Seizure-related adverse events during video-electroencephalography monitoring
Jianming Liu, Fanxin Meng, Zhiliang Liu
Our study evaluated adverse events in 230 epilepsy patients during vEEG monitoring while patients were awake and asleep.</p><p>
<i>Results.</i> A total of 588 seizures were recorded and 231 adverse events were identified including electrode displacement (14.5%), aspiration risk (8.5%), urinary incontinence (7.5%), postictal psychosis (5.8%), tongue biting (5.3%), and patient falls (0.5%). No severe types of adverse events were observed, such as choking or aspiration pneumonia. Of the seizures recorded, 39.1% occurred while patients were sleeping and 38.5% of adverse events occurred during this time, which included electrode displacement (48.2%), aspiration risk (28.0%), tongue biting (60.0%), postictal psychosis (16.7%), patient falls (66.7%), and urinary incontinence (38.6%).</p><p>
<i>Conclusion.</i> The occurrence of seizure-related adverse events during vEEG monitoring is mild. vEEG is an acceptably safe procedure for epilepsy research and precautions should be put in place to prevent these events.</p>
Patients with generalised epilepsy have a higher white blood cell count than patients with focal epilepsy
Rani A Sarkis, Lara Jehi, Diosely Silveira, Damir Janigro, Imad Najm
on leukocyte count in the absence of seizures.
<i>Methods</i>. We performed a retrospective chart review of adult epilepsy patients presenting for evaluation over a four-month period. Demographics, epilepsy type and characteristics, number and type of antiepileptic drugs, as well as medical co-morbidities were noted.
<i>Results</i>. A total of 241 patients fulfilled study criteria. Variables correlating with leukocyte count were identified using univariate analysis. Based on multivariate analysis, only the correlation with type of epilepsy and use of more than two antiepileptic drugs remained statistically significant. Patients with generalised epilepsy had a higher leukocyte count (7.21 k/μL) compared to those with focal epilepsy (6.53 k/μL); the main difference was due to the number of monocytes.
<i>Conclusion</i>. These findings raise the possibility that there are different neuro-immune profiles between patients with generalised and focal epilepsy.
Clinical commentary
Upward gaze and head deviation with frontal eye field stimulation
Kitti Kaiboriboon, Hans O Lüders, Jonathan P Miller, R John Leigh
only thought possible by stimulating both frontal eye fields simultaneously. If stimulation was started when the subject looked laterally, the initial eye movement was back to the midline, followed by upward deviation. Our finding challenges current view of topological organisation in the human FEF and may have general implications for concepts of topological organisation of the motor cortex, since sustained stimulation also induced upward head movements as a component of the vertical gaze shift. [
<i>Published with video sequences</i>]
Frontal lobe epilepsy with atypical seizure semiology resembling shuddering attacks or wet dog shake seizures
Alena Jahodova, Pavel Krsek, Vladimir Komarek, Martin Kudr, Martin Kyncl, Josef Zamecnik, Michal Tichy
after a short psychic aura and face grimacing. Consciousness was fully preserved. The seizures resembled “wet dog shake” seizures described in rat models of epilepsy or shuddering attacks in infants. EEG findings were inconclusive, however, MRI showed a clear dysplastic lesion in the right frontal mesial and polar structures. The patient underwent an extended lesionectomy guided by neuronavigation and intraoperative electrocorticography. Focal cortical dysplasia type Ib was histologically confirmed and the patient has been seizure-free for the three years following resection. [
<i>Published with video sequences</i>]
Pilomotor seizures: a video case report
Mohamad Ayman Haykal, Bassel Abou-Khalil
with left temporal lobe epilepsy and remote herpes zoster encephalitis. In general, piloerection is most often localised to the temporal lobe. Unilateral piloerection is usually associated with an ipsilateral ictal onset. Bilateral piloerection has a less certain lateralising value, although earlier reports suggest left hemispheric predominance
<i> [Published with videosequences].</i>
Bálint-like syndrome as an unusual representation of non-convulsive status epilepticus
Aleksandar J Ristić, Ivan Marjanović, Leposava Brajković, Barbara R Wolgamuth, Strahinja Odalović, Slobodan Lavrnić, Nikola Vojvodić, Slavko Janković, Vladimir Baščarević, Dragoslav Sokić
Therefore, focal non-convulsive status epilepticus emerging from the posterior cortex, and especially the parietal lobes, can be fairly under-recognised. We report a 66-year-old patient with focal non-convulsive status epilepticus presenting as isolated Bálint-like syndrome, successfully treated to full clinical and electrophysiological recovery. The diagnostic and pathophysiological features are discussed. Focal non-convulsive status epilepticus can be associated with negative phenomena such as neuropsychological deficits mimicking those detected more often in degenerative and vascular brain diseases.
<i>[Published with video sequences]</i>
Transient periodic lateralised epileptiform discharges (PLEDs) following internal carotid artery stenting
Daniela Marino, Giampaolo Vatti, Alessandra Rufa, Alessandro Malandrini, Raffaele Rocchi, Sandra Bracco, Rosaria Buccoliero, Antonio Federico
epileptiform discharges at a rate of 1-2 Hz. PLEDs may be triggered by acute brain injuries or systemic metabolic changes such as fever, hyperglycaemia or electrolyte imbalance and may result in disturbance of consciousness and/or neurological deficits.
<i>Case report.</i>
<b> </b>A 58-year-old female with a history of focal epilepsy and deep brain haematoma presented with acute change in awareness,
<i> </i>associated with EEG evidence of PLEDs, three days after a left internal carotid artery stenting procedure. Clinical examination, laboratory testing and MRI were unchanged with respect to pre-stenting investigations.
<i>Conclusion.</i> In this patient, PLEDs may have been triggered by local haemodynamic changes due to reperfusion after stenting in a previously damaged brain area.
PET follow-up in a case of anti-NMDAR encephalitis: arguments for cingulate limbic encephalitis
Jean-Baptiste Chanson, Mihaela Diaconu, Jérôme Honnorat, Thierry Martin, Jérôme De Seze, Izzie-Jacques Namer, Edouard Hirsch
could be useful to identify brain dysfunction associated with psychiatric symptoms, but few precise data are available up to now.
<i>Case study.</i> A 27-year-old woman was referred for recent behavioural changes and jerks of the right foot. Serial left fronto-temporal seizures were recorded. Identification of anti-NMDA receptor antibodies in CSF indicated a diagnosis of anti-NMDA receptor encephalitis. Two foci of hypermetabolism, in the left prefrontal cortex and the anterior cingulate cortex, were identified using 18-fluorodeoxyglucose PET and both disappeared after treatment. Brain MRI was normal, except for a mild left prefrontal hypersignal.
<i>Conclusions.</i> The increase in marker uptake in motor and premotor regions in our case probably corresponds to epileptic activity. Our data suggest that the anterior cingulate cortex could play an important role in psychiatric symptoms. Other studies are needed to better understand the pathophysiology of anti-NMDA receptor encephalitis.
Presentation of an unusual patient with Lafora disease
Selim Gökdemir, Hande Çağlayan, Meral Kızıltan, Naci Karaağaç, Cem Leblebici, S Naz Yeni
who presented with generalised tonic-clonic seizures at the age of 30 is discussed. Until the age of 48, the patient did not have myoclonic jerks or ataxia clinically, but had well controlled seizures. He developed dementia and late extrapyramidal signs. Axillary skin biopsy revealed typical Lafora inclusion bodies. Genetic analysis showed a mutation in the
<i>EMP2B</i> gene. To our knowledge, this is the first description of a patient suffering from a Lafora disease without disabling myoclonus and ataxia but rather rare seizures, extrapyramidal signs, and dementia.
Possible induction of West syndrome by oxcarbazepine therapy in a patient with complex partial seizures
Aravindhan Veerapandiyan, Piyush Singh, Mohamad A Mikati
and spasms. Here, we report a one-year, six-month-old girl with complex partial seizures who developed infantile spasms, developmental regression, and hypsarrhythmia during the two weeks directly following initiation of oxcarbazepine (14 mg/kg/day). All of these resolved within a few days after discontinuation of this medication. Although we cannot rule out that the above association may have been coincidental, or that the improvement may have been due to concurrent therapy, this case raises the possibility that oxcarbazepine, like carbamazepine, may precipitate infantile spasms and West syndrome.
