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Special article
Non-invasive alternatives to the Wada test in the presurgical evaluation of language and memory functions in epilepsy patients
Isabelle Pelletier, Hannelore C Sauerwein, Franco Lepore, Dave Saint-Amour, Maryse Lassonde
be spared by the neurosurgeon. Traditionally, presurgical evaluation of epileptic patients has been achieved by means of the intracarotid amobarbital test assisted by neuropsychological measures. However, the advent of neuroimaging techniques has provided new ways of assessing these functions by means of non-invasive or minimally invasive methods, such as anatomical and functional magnetic resonance imaging, positron emission tomography, single-photon emission computed tomography, transcranial magnetic stimulation, functional transcranial Doppler monitoring, magnetoencephalography and near infrared spectroscopy. This paper aims at comparing and evaluating the traditional and recent preoperative approaches from a neuropsychological perspective.
Original article
Presurgical evaluation in refractory epilepsy secondary to meningitis or encephalitis: bilateral memory deficits often preclude
Antonio Donaire, Mar Carreno, Rolando Agudo, Pilar Delgado, Nuria Bargalló, Xavier Setoaín, Teresa Boget, Toni Raspall, Merce Falip, Jordi Rumiá, Luis Pintor, Iratxe Maestro
evaluation. Six patients had a history of meningitis and 11 patients had a history of encephalitis. Median age at infection was three years (40 days-40 years). Time to seizure onset was shorter in the encephalitis group (median of 0.9 years versus 5.9 years in the meningitis group). MRI showed unilateral mesial temporal sclerosis (MTS) in all but one patient with meningitis (5/6). MRI in the encephalitis group showed unilateral MTS (four patients), bilateral MTS (three), porencephalic cysts (one) or no significant findings (three). Seizure semiology, following analysis of 127 seizures, included automotor seizures, complex motor/hypermotor seizures, dialeptic seizures and bilateral asymmetric tonic seizures. Neuropsychological assessment in patients with MTS frequently showed bilateral memory impairment (7 out of 12 MTS-patients), even in 4 patients with unilateral MTS, precluding epilepsy surgery. Six patients (two meningitis and four encephalitis patients) underwent a temporal lobe resection. All patients are either seizure-free (Class 1a) or are having only auras after surgery. One patient from the meningitis group underwent functional hemispherectomy and he is also seizure-free. In our series, MTS was the most common finding in refractory epilepsy after CNS infections. Bilateral memory deficits were often encountered in patients with MTS, even when unilateral, these deficits being a limiting factor for surgery. Good surgical outcome can be expected in selected patients with unilateral MTS and congruent memory deficits.
Complex partial status epilepticus is an unrecognised feature in SESA syndrome: new insights into its pathophysiology
José L Fernández-Torre, José L Hernández-Hernández, Julio Jiménez-Bonilla, Andrés González-Mandly, Oscar García-Regata
significantly to the alteration of the mental state. Our case suggests that the occurrence of episodes of complex partial status epilepticus (CPSE) may contribute greatly to the origin and pathophysiology of the confusional state in this rare, epileptic entity.
Atypical case of Aicardi-Goutières syndrome with late-onset myoclonic status
Andrea Berger, Christiane Schroeter, Adelheid Wiemer-Kruel, Karl Strobl, Georg F Hoffmann, Dietz Rating, Pierre Lebon, Jan-Peter Ernst, Nicole I Wolf
investigations for the common prenatal infections. The clinical profile is characterised by acquired microcephaly, mild to severe cognitive delay and dystonia. Epilepsy is usually not prominent. We report on a 19-year-old patient with an atypical clinical course, characterized by a relatively benign presentation at onset. Epilepsy with complex-focal seizures, possibly with a visual aura and sometimes with secondary generalization, started at the age of nine years. Clinical deterioration occurred later, and at the age of 17 years he experienced severe, generalized, myoclonic attacks lasting hours, which were partly controlled by the administration of piracetam.[Published with video sequences]
Temporal lobe dual pathology in malignant migrating partial seizures in infancy
Giangennaro Coppola, Francesca Felicia Operto, Gianfranca Auricchio, Alessandra D’Amico, Delia Fortunato, Antonio Pascotto
were drug-resistant, with recurring episodes of status epilepticus. The child developed microcephaly with arrest of psychomotor development. Focal brain lesions, in the context of migrating partial seizures, have not been previously reported.[Published with video sequences]
Focal motor seizure with automatisms in a newborn
Marilena Vecchi, Agnese Suppiej, Massimo Mastrangelo, Clementina Boniver
seizures. Recently, complex behaviours and motor automatisms in newborns have been reported as being epileptic in nature. However, the debate on physiopathology (cortical or brainstem release phenomena) is ongoing. We present the synchronized video-EEG documentation of epileptic complex motor behaviours in a newborn male. Our case contributes to the discussion regarding the semiological classification of the neonatal seizures.[Published with video sequences]
“Tonic-absence seizures”: an unusual seizure phenotype, but not necessarily in that order
Krishna B Das, Stewart G Boyd
seconds, abduction of the arms and a brief vocal utterance (“ugh”). Each episode lasts around 30 seconds, 3-4 times/day despite treatment. The EEG showed generalized 3-4Hz spike-wave discharges during the “absence” period followed immediately by a run of fast polyspikes typical of a tonic seizure, terminating in a run of 1-2 Hz sharp-slow wave complexes. Although tonic-absence seizures have been reported rarely, the clinical sequence appears to be reversed in this patient, with the occurrence of “absence-tonic” attacks. We present video documentation of such attacks and discuss their nosology.[Published with video sequences]
Magnetoencephalographic studies of focal epileptic activity in three patients with epilepsy suggestive of Lennox-Gastaut syndro
Kotaro Sakurai, Naoaki Tanaka, Kyousuke Kamada, Fumiya Takeuchi, Youji Takeda, Tsukasa Koyama
syndrome (LGS).MethodsMEG and simultaneous electroencephalography (EEG) were recorded from three adult patients using a 204-channel, whole-head MEG system. Equivalent current dipoles (ECDs) were calculated for epileptic spikes on MEG according to the single dipole model.ResultsIn two patients, MEG showed epileptiform discharges restricted to the unilateral temporal area, corresponding to the EEG spikes. The ECDs calculated from these MEG spikes were clustered in the unilateral temporal lobe. In our third patient, MEG spikes appeared in the right centroparietal area; ECDs were located to the right parietal lobe.ConclusionsThe sources of epileptiform discharges that were detected in a restricted area were localized to specific parts of the brain cortex. Despite certain limitations (small number of patients; atypical late-onset epilepsy in one) our study suggests that MEG may prove to be a useful tool for investigating electromagnetic features of localized epileptic discharges in patients with LGS. Based on these preliminary results, further studies performed in patients with typical LGS features are justified.
Chronic PLEDs with transitional rhythmic discharges (PLEDs-plus) in remote stroke
José F Téllez-Zenteno, Sylaja N Pillai, Michael D Hill, Neelan Pillay
transiently in acute lesions, but a few reports have described persistent PLEDs in chronic lesions. Case report. An 86-year-old female was admitted, in 1999, with a left MCA stroke associated with right hand focal motor seizures. The first EEG in February of 2002 showed PLEDs over the left hemisphere associated with rhythmic discharges (PLEDs-plus). The patient was admitted on a second occasion in 2003 because of three sequential seizures and the EEG showed a similar pattern. Finally in 2006, the patient was admitted again because of sequential complex partial seizures and an EEG showed the same PLEDS-plus pattern as the EEGs of 2002 and 2003. Discussion. We report an unusual case of chronic PLEDs associated with rhythmic discharges in a patient with recurrent seizures and remote stroke.
Over-interpretation of electroclinical and neuroimaging findings in syncopes misdiagnosed as epileptic seizures
Jinane Fattouch, Carlo Di Bonaventura, Stefano Strano, Nicola Vanacore, Mario Manfredi, Massimiliano Prencipe, Anna Teresa Giallonardo
epileptic seizure and consequently treated with antiepileptic drugs. In this study, we identified 57 patients with syncope (diagnosis based on accepted criteria) who had come to our unit with a previous diagnosis of definite epilepsy in 30 cases (syncope misdiagnosed as epileptic seizures, SMS), or suspected epilepsy in the remaining 27 cases (unrecognized syncope, US). We attempted to identify factors underlying misdiagnosis by reviewing clinical findings, particularly potentially confounding features, and EEG/neuroimaging data. Finally, we compared these two groups of patients to search for crucial elements that had led to misdiagnosis. Although some clinical elements were found to be confounding in both groups, it was the interpretation of the EEG and MRI findings, particularly when combined with the confounding clinical features that constituted the main reasons for misdiagnosis.
Clinical commentary
Video-EEG in syncopal attack due to ocular compression in an adolescent mistreated for epilepsy
Philippe Gelisse, Anna Serafini, Jean Marc Davy, Marc Balbe, Philippe Coubes, Arielle Crespel
produced a typical syncope. Before this recording, the patient had been treated for five years with sodium valproate because of a misdiagnosis of epilepsy. The diagnosis of neurally-mediated syncope was not difficult on the basis of the clinical history, but the reproduction of syncope reinforced the correct diagnosis. Ocular compression is now only indicated in exceptional circumstances and should be performed with precautions. The video of this episode in an adolescent should be of considerable educational value to those who are confronted to diagnostic work-up of transient loss of consciousness.[Published with video sequences]
Unrecognized paroxysmal ventricular standstill masquerading as epilepsy: a Stokes-Adams attack
Chi-Fang You, Chee-Fah Chong, Tzong-Luen Wang, Tzu-Yao Hung, Chien-Chih Chen
puzzle before it was found that he had paroxysmal ventricular standstill with complete atrioventricular block: he made a complete recovery after temporary pacemaker insertion. The main lessons from this case were (1) a convulsive seizure of only seconds duration and with an abrupt return of consciousness suggests syncope not epilepsy, (2) repeated, convulsive syncopes without provocation suggest cardiac syncope, (3) a 12-lead ECG should be recorded as soon as possible after such a series of episodes and should not be discontinued until an event is captured, and (4) Emergency Department clinicians should be familiar with any automatic gain on their ECG machine, lest fast, atrial activity be mistaken for narrow complex tachycardia. In summary, a good clinical history is of prime importance in differentiating convulsive syncope from epilepsy, and a simple, non-invasive cardiovascular evaluation may help to diagnose the condition as cardiac syncope.
Spinal algetic-tonic seizures manifesting as paroxysmal “positive” Brown-Séquard syndrome
Anne Kirchner, Annkathrin Poepel, Matthias Hantsch, Horst Urbach, Christian E Elger, Christian G Bien
inflammatory-demyelinating in origin, was identified as the morphological correlate of these attacks. Their pathogenesis is discussed in the light of similar case reports from the literature. For the first time, this rare type of seizure is published with a video documentation. It may deserve consideration in the differential diagnosis of otherwise unexplained paroxysmal events that present in an “epileptic” manner.[Published with video sequences]
Status gelasticus associated with levetiracetam as add-on treatment
Giuseppina Pustorino, Maria Spano, Domenica Lucia Sgro, Gabriella Di Rosa, Giovanni Tricomi, Dorotea Bellantone, Gaetano Tortorella
as add-on treatment to oxcarbazepine and diazepam. Gelastic seizures were documented by video-EEG and were responsive to i.v. administration of diazepam. A possible causative role of levetiracetam is suggested. Specific susceptibility to some AEDs is also discussed, as this patient, at the age of four years, had presented an episode of non-convulsive status epilepticus, following introduction of tiagabine, in association with vigabatrin and nitrazepam.[Published with video sequences]
A case of congenital bilateral perisylvian syndrome due to bilateral schizencephaly
K Puthenveettil Vinayan, Kiyohito Terada
triad of CBPS, which included congenital facio-masticatory diplegia, epilepsy and only mild mental retardation, despite the presence of bilateral, open-lip clefts in the perisylvian region. We hypothesize that the minimal loss of cortical tissue, along with the possible sparing of vital white matter association fibers and neuronal plasticity might have contributed to the better functional outcome in this patient.
Conference report
Genetics of epilepsy : Epilepsy Research Foundation workshop report
Sanjay Sisodiya, J. Helen Cross, Ingmar Blümcke, David Chadwick, John Craig, Peter B. Crino, Paul Debenham, Norman Delanty, Frances Elmslie, Mark Gardiner, Jeffrey Golden, David Goldstein, David A. Greenberg, Renzo Guerrini, Michael Hanna, John Harris, Paul Harrison, Michael R. Johnson, George Kirov, Dimitri M. Kullman, Andrew Makoff, Carla Marini, Rima Nabbout, Lina Nashef, Jeffrey L. Noebels, Ruth Ottman, Munir Pirmohamed, Asla Pitkänen, Ingrid Scheffer, Simon Shorvon, Graeme Sills, Nicholas Wood, Sameer Zuberi
to consider progress, issues and strategies for harnessing genetics to improve the understanding and treatment of the epilepsies. General principles were considered, including the fundamental importance of clear study design, adequate patient numbers, defi ned phenotypes, robust statistical data handling, and follow-up of genetic discoveries. Topics where some progress had been made were considered including chromosomal abnormalities, neurodevelopment, hippocampal sclerosis, juvenile myoclonic epilepsy, focal cortical dysplasia and pharmacogenetics. The ethical aspects of epilepsy genetics were reviewed. Principles and limitations of collaboration were discussed. Presentations and their matched discussions are produced here. There was optimism that further genetic research in epilepsy was not only feasible, but might lead to improvements in the lives of people with epilepsy.
