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Original article
Seizure-related automatic locomotion triggered by intracerebral electrical stimulation
Elena Gardella, Guido Rubboli, Stefano Francione, Laura Tassi, Giorgio Lo Russo, Sten Grillner, Carlo Alberto Tassinari
cortical dysplasia and who became seizure-free after epilepsy surgery. The unexpected finding of the investigations was that intracerebral, high frequency (50 Hz) electrical stimulation (HFS) triggered the emergence of automatic and involuntary forward-backward locomotion during a focal seizure while the boy was standing. This clinical manifestation was different from the chaotic motor activity described during epileptic wanderings. The stimulation of the same fronto-lateral region, while the patient was lying, produced only the subjective sensation that the legs were moving, although there was no physical manifestation of this. Human locomotion is an innate motor behavior that is normally due to the activation of the spinal network for locomotion (central pattern generator). The emergence of different stereotyped motor behaviors during focal epileptic seizures or sleep disorders has recently been interpreted as a release of subcortical central pattern generators (Tassinari et al. 2005). In view of this, we hypothesize that the involuntary and robot-like locomotion of our patient could be the ictal expression of the release of subcortical locomotor CPGs. [Published with video sequences]
Refractory and lethal status epilepticus in a patient with ring chromosome 20 syndrome
Julia Jacobs, Geneviève Bernard, Eva Andermann, François Dubeau, Frederick Andermann
Patients suffer from severe seizures with accompanying cognitive decline and frequent episodes of non-convulsive status epilepticus (SE). Other features of this rare disorder, such as dysmorphic changes, mental retardation and behavioral disturbances are variable. Because of the variability of the clinical presentation, some patients with r(20) undergo invasive investigations before being diagnosed. Case study. We present the case of a young boy with no dysmorphic traits, who was only diagnosed with r (20) syndrome at the age of 13. His first seizure occurred at the age of four. Later seizures were of various types including non-convulsive SE, with deterioration of the background EEG and severe cognitive decline. Despite multiple trials of anti-epileptic medications, his seizures remained highly refractory, and he died as the result of an uncontrollable, prolonged SE, shortly after the diagnosis was made. Discussion. Non-convulsive SE is common in patients with r(20) syndrome and may be caused by a dysfunction in dopaminergic neurotransmission. However, until now, no case of lethal status epilepticus has been reported. This case report suggests that patients with unexplained refractory seizures and episodes of non-convulsive SE should undergo genetic testing early in their disease, even in the absence of any morphologic features or dysmorphic traits suggestive of a chromosomal disease. [Published with video sequences]
Early seizure propagation from the occipital lobe to medial temporal structures and its surgical implication
Naotaka Usui, Tadahiro Mihara, Koichi Baba, Kazumi Matsuda, Takayasu Tottori, Shuichi Umeoka, Fumihiro Nakamura, Kiyohito Terada, Keiko Usui, Yushi Inoue
with electrodes implanted in the medial temporal lobe in patients who underwent occipital lobe surgery. Four patients with occipital lesions, who underwent intracranial EEG monitoring with intracerebral electrodes implanted in the medial temporal lobe prior to occipital lobe surgery, were studied. Subdural electrodes were placed over the occipital lobe and adjacent areas. Intracerebral electrodes were implanted into bilateral hippocampi and the amygdala in three patients, and in the hippocampus and amygdala ipsilateral to the lesion in one. In light of the intracranial EEG findings, the occipital lobe was resected but the medial temporal lobe was spared in all patients. The follow-up period ranged from six to 16 years, and seizure outcome was Engel Class I in all patients. Sixty six seizures were analyzed. The majority of the seizures originated from the occipital lobe. In complex partial seizures, ictal discharges propagated to the medial temporal lobe. No seizures originating from the temporal lobe were documented. In some seizures, the ictal-onset zone could not be identified. In these seizures, very early propagation to the medial temporal lobe was observed. Interictal spikes were recorded in the medial temporal lobe in all cases. Intracranial EEG revealed very early involvement of the medial temporal lobe in some seizures. Seizure control was achieved without resection of the medial temporal structures.
Pathways of seizure propagation from the temporal to the occipital lobe
Julia Jacobs, François Dubeau, André Olivier, Frederick Andermann
the reverse direction of spread has not been emphasized. We describe two patients who experienced ictal propagation from temporal to occipital regions. One case presented with amaurosis during a seizure with temporal onset and temporal-occipital spread. In the second, temporal-occipital spread was documented during a seizure, which continued in the occipital lobe for six minutes. Depth electrode studies suggested the temporal ictal onset of seizures in both patients. Propagation from temporal to occipital lobe structures must be considered in the assessment of patients who have seizures with both temporal and occipital features. The propagation may have predictive value for their surgical outcome. The underlying anatomical structure might be the inferior longitudinal fasciculus.
Familial Creutzfeldt-Jakob disease presenting as epilepsia partialis continua
Max R Lowden, Kevin Scott, Milind J Kothari
symptoms start there is a progressive neurological decline. Clinical features include dementia, ataxia and myoclonus (startle), among others. We report a biopsy-proven case of familial CJD (fCJD) presenting with continuous focal seizures, epilepsia partialis continua (EPC), as the initial presentation. CJD is an unusual neurological disorder with an incidence of approximately one case per million population (Prusiner 2001). The disorder is due to neuronal degeneration resulting from the accumulation of a pathological isoform (PrP) of the prion protein (PrPc). Patients with fCJD have mutations in the gene encoding PrPc (PRNP) (Vercueil 2006, Collins et al. 2004). This fCJD represents 10-15% of CJD cases making the sporadic form more common 85-95% (Parry et al. 2001). During the course of the disease myoclonus has been reported in 88% of cases, and epileptic seizures (partial seizures, generalized status epilepticus) in 8% (Vercueil 2006). Periodic sharp wave complexes (PSWC) are uncommon in fCJD and occur in about 10% of patients (Wieser et al. 2006).
Accidents and injuries in people with epilepsy attending a tertiary care center in India
CS Sapna, SL Sheeba, J John, SV Thomas
are known to influence this risk. The risk of accidental injury to people with epilepsy may vary in different geographical areas, according to their life style, leisure activities and other pursuits. The objective was to ascertain the accidents and injuries experienced by a cohort of people with epilepsy in India, and to ascertain any possible predictors. The study was carried out in the Epilepsy Clinic of Sree Chitra Tirunal Institute for Medical Sciences and Technology between January and June 2007. Using a structured questionnaire, we interviewed all consenting adults attending this tertiary epilepsy center who had had active epilepsy for one or more years. Two hundred and fifty five patients completed the study. The diagnoses included localization-related epilepsy for 62.6% of the patients and generalized epilepsy for the remainder. During the 12 months prior to the interview, 44.8% had had at least one injury; 7.8% had sustained an accident. Road traffic accidents (3.1%), burns (2.5%), electric shocks (0.8%) and near drowning (0.4%) were the commonly reported accidents. Age, sex, type of epilepsy and type of medication had no significant association with the occurrence of injuries or accidents. However, we found that a quarter of the people with epilepsy studied had experienced serious injuries.
Psychosocial impact of epilepsy in women of childbearing age in India
Sureshbabu Sachin, Madakasira V Padma, Rohit Bhatia, Kameshwar Prasad, C Gureshkumar, Manjari Tripathi
women. One hundred women with epilepsy, 50 with migraine and 100 healthy, pregnant women were enrolled over a two-year period. The three groups were assessed using questionnaires for quality of life (QOL), coping strategies and caregiver burden. The influence of demographic and seizure variables on these psychosocial outcomes were also assessed.It was found that quality of life was least, and the burden experienced by the caregiver was significantly more in patients with epilepsy (p < 0.001). Women with epilepsy relied more on religion/faith as a coping method (p = 0.021), and less on problem solving strategies (p < 0.001) when compared to those with migraine. When compared to healthy, pregnant women, they more frequently employed religious methods of coping and denial (p < 0.001), with significantly less use of problem solving techniques, acceptance, and positive and negative distraction(p < 0.001). Less frequent seizures, better education and remission sustained for at least six months, were associated with better QOL. Educational status, frequency of seizures and time elapsed since last seizure emerged as significant determinants of coping behaviour. Low educational status and monthly income of the family contributed significantly to caregiver burden.This study helped to identify the different areas of psychosocial impairment in patients with epilepsy, as well as the contributing factors. Women with epilepsy rarely used constructive coping strategies, and this was found to contribute to their poor psychosocial status and adjustment within the family and society at large.
Pre-surgery expectations and post-surgery life-changing validation process
Priscila Camile Barioni Salgado, Paula Teixeira Fernandes, Fernando Cendes
yes/no questions and the life changes questionnaire 25 yes/no questions, which includes the 18 questions of the expectations questionnaire plus seven negative questions, considering that the surgery could have a negative effect on the patient quality of life. There were also questions asking the patients to state if they consider the surgery to be a success or a failure, and about the frequency of seizures and their intensity. Patients were interviewed in three different phases: pre-surgery, post-surgery 1 (six months after surgery) and post-surgery 2 (12 months after surgery). The internal consistency of the instruments showed a general Kuder-Richardson coefficient of 0.855 (pre-surgery), 0.833 (post-surgery 1), 0.756 (post-surgery 2), showing that both instruments have a satisfactory content validity and high internal consistency. In this context, the answers obtained in this study are important because they represent the development of a complete set of categorical instruments to evaluate pre-surgery epilepsy expectations and post-surgery life changes.
Effect of levetiracetam on cognitive functions and quality of life: a one-year follow-up study
Mariana López-Góngora, Alejandro Martínez-Domeño, Carmen García, Antonio Escartín
therapy. Methods. Thirty-two patients (16 women; 16 men) who received LEV as an add-on treatment were included, and 27 completed the one-year follow-up period. Extensive neuropsychological assessments, together with a quality-of-life questionnaire were administered at baseline and at one, three, six and twelve months after beginning the add-on treatment. Patients received LEV starting with 500 mg/day in the first week, increasing by a further 500 mg/day per week until a target dose of 2 000 mg/day was reached by the end of the first month. Results. At the one-year follow-up, a significant improvement was observed in measurements of prospective memory, working memory, motor functions, verbal fluency, attention and quality of life. Performance for neuropsychological and quality-of-life tests was not affected by external variables such as seizure reduction or changes in previous anti-epileptic treatment. Slight changes between patients were observed, but these were not clinically significant.The limited sample size and the lack of a control group should be mentioned as limitations of the study. No control group was evaluated as in our clinical practice it was difficult to establish a comparable group of patients. Changes in the different variables were assessed by comparing baseline information with follow-up results.Despite the study limitations, we consider that the one-year treatment period provides valuable information regarding the drug’s long-term effects in this setting. Conclusions. Results of the present study suggest that long-term LEV treatment as add-on therapy does not interfere with cognitive function and improves quality of life.
Clinical commentary
A patient with two episodes of epilepsia partialis continua of the abdominal muscles caused by cortical dysplasia
F Irsel Tezer, Ozlem Celebi, Burce Ozgen, Serap Saygi
muscles may be affected. Rarely, EPC may also present in association with cortical dysplasia. In this report, we describe the clinical, neuroimaging and ictal electroencephalographic findings of a young woman presenting with persistent myoclonic twitches of the abdominal muscles that were considered to represent a rare manifestation of EPC due to cortical dysplasia. To the best of our knowledge, this is the first report of malformation of cortical development causing abdominal myoclonus. [Published with video sequences]
Late-onset epilepsy in a surgically-treated Sturge-Weber patient
Julia Jacobs, Pierre LeVan, André Olivier, Frederick Andermann, François Dubeau
childhood and the severity of the epilepsy correlates with the patient’s neurological outcome. The patient reported here remained asymptomatic until age 24, when he started to experience migraine accompanied by visual scotoma and hemiparesis. Ten years later, he developed severe, refractory epilepsy, with prolonged postictal neurological deficits and ictal as well as post-ictal headaches. It became increasingly difficult to distinguish between migraines and seizures as both could last for several hours, as demonstrated in the two video EEGs. Both the epilepsy and the migraine may therefore have contributed to the patient’s severe, neurological deterioration, probably by accelerating the progressive neuronal damage. Surgery improved the situation, but lesional areas were too extensive for complete removal. Late-onset symptoms in Sturge-Weber syndrome may thus result in a severe course for the disease. Early intervention and surgical treatment options are discussed. [Published with video sequences]
Rasmussen’s encephalitis and Behcet’s disease: autoimmune disorders in first degree relatives
Demet Kinay, Nerses Bebek, Ebru Vanli, Candan Gurses, Ayşen Gokyigit, Frederick Andermann
His father had Behcet’s disease, considered to be an autoimmune disorder. Recent reports have directly implicated the role of cytotoxic T lymphocytes in the pathogenesis of both Rasmussen’s encephalitis and Behcet’s disease. The occurrence of Behcet’s disease and Rasmussen’s encephalitis in the same family suggests involvement of common genetic factors such as HLA haplotypes in both autoimmune disorders. It is possible that members of this family are genetically susceptible to developing autoimmune conditions that have been precipitated by separate environmental triggers.
Catatonic psychosis related to forced normalization in a girl with Dravet’s syndrome
Giuseppe Gobbi, Simona Giovannini, Antonella Boni, Paola Visconti, Massimiliano Beghi, Cesare Maria Cornaggia
both generalised and focal epilepsies. On the basis of the evaluation of a video recording, we found that forced normalization was concomitant with catatonic psychosis in a patient with epilepsy, intellectual disability and pervasive developmental disorder. Catatonic psychosis accompanying forced normalization has not been previously reported. As the psychotic symptoms and quality of life worsen seizure control improves, we believe it may be better for the patient to tolerate some seizures, thus preserving their capacity to interact socially. [Published with video sequences]
Anatomo-electro-clinical correlations
MRI-negative prefrontal epilepsy due to cortical dysplasia explored by stereoelectroencephalography (SEEG)
Aileen McGonigal, Martine Gavaret, Agnès Trébuchon Da Fonseca, Maxime Guye, Didier Scavarda, Nathalie Villeneuve, Jean Régis, Fabrice Bartolomei, Patrick Chauvel
automatisms. There was no visible lesion on MRI, but following localisation using stereoelectroencephalography (SEEG), surgery revealed an underlying dysplastic lesion. [Published with video sequences]
Medically intractable seizures originating from the primary somatosensory hand area
Mohamad Z Koubeissi, Robert J Maciunas, Adriana Tanner, Hans O Lüders
left hand since the age of 13 years. Occasionally, her seizures progressed to left arm posturing followed by secondary generalization. Scalp EEG revealed interictal epileptiform discharges in the right posterior quadrant, but with no ictal EEG correlates. Brain MRI showed a right temporal encephalomalacia, sparing mesial temporal structures, suggestive of a perinatal vascular insult. Ictal electrocorticogram, electrical stimulation mapping, and somatosensory evoked potentials localized the ictal onset to the hand area of the postcentral gyrus. Resection of that area resulted in total resolution of seizures with no significant lasting deficits. Potential complications of resecting the primary somatosensory hand area can be severe, as proprioceptive sensory loss may be permanent, resulting in significant disability. Such deficits may be temporary however, and the literature continues to report conflicting results regarding postsurgical outcome. Cortical plasticity may explain recovery of sensory deficits after partial resection of the primary somatosensory hand area. Multiple subpial transections of that area are sometimes performed to minimize functional deficits, but seizure control may be less optimal than with cortical resection.
MRI-negative frontal lobe epilepsy with ipsilateral akinesia and reflex activation
Stephen Malone, Ian Miller, Prasanna Jakayar, Trevor Resnick, Sanjiv Bhatia, Michael Duchowny
MRI-negative, frontal lobe epilepsy is often challenging owing to the semiologic expression being unusual or non-lateralizing. Localization based on functional tests may be non-convergent, further compounding the difficulty of surgical planning. We describe a patient with seizures presenting with early, subjective akinesia of the dominant hand that were at times triggered by repetitive motor tasks. Subdural EEG monitoring revealed seizure-origin in the ipsilateral dorsolateral frontal lobe. After resective surgery the patient has had a seizure free period of 9 months, and is free of deficits. This is the first report of frontal lobe seizure semiology consisting of ipsilateral ictal akinesia. [Published with video sequences]
Early-onset symptomatic focal epilepsy: a dilemma in the timing of surgery
Neely Desai, Ronit M Pressler, Nicola Jolleff, Maria Clark, Brian Neville, Christin Eltze, William Harkness, J Helen Cross
and left focal seizures. An MR scan at two months was suggestive of a right parietal cortical dysplasia, although this was less apparent on repeat scan at 11 months. The initial response to anti-epileptic medications was good; surgery was therefore deferred at that time. Subsequently, seizure control fluctuated and developmental progress was, on the whole, good. However, ultimately seizures increased despite changing the AED, and he began showing developmental problems. Surgery was reconsidered. Again, a repeat MR scan did not define the lesion well. Following full further evaluation, including functional imaging that still implicated the right parietal cortex, subdural grid and depth electrode monitoring were undertaken at 6.5 years, which located the ictal onset zone deep within the lesion. This enabled a right inferior parietal lobe resection to be performed. Four years post-surgery he remains seizure-free and has shown progress in development.
Multifocal epilepsy: the role of palliative resection - intractable frontal and occipital lobe epilepsy secondary to radiotherapy for acute lymphoblastic leukaemia
Ashalatha Radhakrishnan , Pasiri Sithinamsuwan , A Simon Harvey, Danny Flanagan, Gregory Fitt, Sam Berlangieri, Graeme D Jackson, Samuel F Berkovic, Ingrid E Scheffer
We report an adolescent with intractable frontal and occipital lobe seizures, secondary to complications of treatment for acute lymphoblastic leukaemia as a young child. Chemotherapy and radiotherapy were complicated by bilateral, posterior leukoencephalopathy and later an acquired frontal cerebral cavernous malformation (CCM). Detailed electro-clinical and imaging studies showed multiple, frontal lobe seizures per day with less frequent and non-debilitating, simple, occipital lobe seizures. Focal resection of the frontal CCM abolished the socially-disabling seizures with resultant marked improvement in the patient’s quality of life at 12 months. Careful analysis of the type and impact of focal seizures in the setting of multifocal epilepsy may demonstrate that one seizure type is more deleterious to quality of life and may be amenable to surgery. In this situation, the patient may benefit significantly from surgery to resect the more active epileptic focus. [Published with video sequences]
Letter to the Editor
Auditory manifestations during focal seizures
Laura Tassi
1st International Epilepsy Colloquium Abstracts
The mesial temporal lobe epilepsies
A
syndromes best characterized by clinical and basic science research.The 1
<sup>st</sup> International Epilepsy Colloquium was the first of a series of annual meetings that will be hosted in alternating years in Marburg (Germany), IDEE-Lyon (France) and UH Cleveland (USA). The Marburg Colloquium, directed by Prs. Rosenow, Hamer and Knake covered all aspects of the mesial temporal lobe epilepsies including the influence of genetics, psychosocial sequelae, novel imaging techniques and alternative surgical strategies used. The extensive body of knowledge that has accumulated over the recent years was made available to interested adult and pediatric epileptologists, neurologists, neurosurgeons and neuropsychologists. The Proceedings of the 1
<sup>st</sup> International Epilepsy Colloquium will soon be published in the format of a book dedicated to Temporal Lobe Epilepsies.Abstracts of the posters presented for discussion are published in the present issue of
<i>Epileptic Disorders</i>.
