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Myoclonic encephalopathy caused by chronic bismuth abuse (Published with video sequence.)
Michael Teepker, Hajo M. Hamer, Susanne Knake, Oliver Bandmann, Wolfgang H. Oertel, Felix Rosenow
even though it can cause severe myoclonic encephalopathy if ingested chronically in high doses. We report a 49 year-old woman with chronic gastric ulcers and 5 years of Bi abuse who developed the typical clinical course of Bi encephalopathy. She presented with progressive dementia, dysarthria and myoclonic jerks one week after increasing the Bi dosage. The EEG showed generalized spike-wave complexes suggesting that the myoclonus was epileptic in nature. Bi intake was stopped and valproate was given, which decreased the frequency of the myoclonic jerks. Administration of the metal chelator D,L-2,3-dimercaptopropane- 1-sulfonic acid (DMPS) led to increased urine excretion of Bi, but was accompanied by a clinical deterioration which resulted in it being discontinued. The subsequent clinical recovery of the patient was documented over 40 days by EEG, video and neuropsychological testing. A time lag of two weeks was observed between falling plasma levels and clinical improvement. In conclusion, Bi-induced encephalopathy is a differential diagnosis for myoclonic encephalopathies. Treatment with metal chelators may aggravate the encephalopathy. The over-the-counter availabily of medications containing Bi should be questioned.
(Published with video sequence.)
Fear, anger and compulsive behavior during seizure: involvement of large scale fronto-temporal neural networks
Fabrice Bartolomei, Maxime Guye, Fabrice Wendling, Martine Gavaret, Jean Régis, Patrick Chauvel
In this report, we studied a patient in whom seizures initially involved the medial temporal region before involving the frontal cortex. Seizure semiology of the second part of the seizure included marked emotional disturbances (dominated by intense fear and anger) and compulsive behavior to bite into something. This patient underwent presurgical evaluation including intracerebral electroencephalographic recordings (SEEG, stereoelectroencephalography).
Methods In addition to SEEG examination, we used coherence analysis of signals as a means of studying functional coupling between different regions of the brain. Two seizures were studied. Coherence values from different periods of interest were compared to identify the neural structures involved at the onset of seizure activity as well as during the emotional behavioral changes.
Results A first network of neural structures was identified within the right anterior temporal regions (amygdala, temporal pole, hippocampus, temporal neocortex). At the time of intense affective and compulsive changes, and by comparison with the first ictal period, a second network was identified characterized by significant functional coupling between the amygdala, the orbito-frontal structures and the frontal opercular region, while a decrease in functional coupling between these regions and the dorsolateral region and the cingulate gyrus was apparent.
Conclusion This study show that the emergence of an intense affective and behavioral state during a temporal lobe seizure could be related to the involvement of a network of structures including the anterior temporal lobe and the orbito-frontal cortex. The decrease of coupling between these regions and the lateral prefrontal and cingulate regions could also participate in these phenomena.
Long-term neuropsychological follow-up and nosological considerations in five patients with Continuous Spikes and Waves during Slow Sleep
Pierangelo Veggiotti, Cristiano Termine, Elisa Granocchio, Stefania Bova, Grazia Papalia, Giovanni Lanzi
follow-up and nosological considerations of five patients who developed CSWSS during childhood. All five of our patients presented CSWSS, although the duration and severity of this pattern varied. The outcome was of three basic types: acquired frontal dementia, language deficits and normal. Four of our patients were initially diagnosed with Landau-Kleffner syndrome but have had markedly diverse outcomes in terms of the severity and type of compromise. Our data suggest that the initial diagnosis, according to current nosological categories, has almost no prognostic significance, while the length and the age of onset of CSWSS, the site of epileptiform activity and the individual neuropsychological profile are more useful for identifying the long-term outcome of patients with CSWSS.
Epilepsy in children with congenital hemiplegia: correlation between clinical, EEG and neuroimaging findings
Christos Panteliadis, Gert Jacobi, Athanasios Covanis, Maria Tzitiridou, Urania Kotzaeridou, Georgios Arsos, Panagiotis Kardaras
in children with congenital hemiplegia (CH).
Patients and methods: two hundred and three children with CH were assessed by history, neurological and developmental examination.Electroencephalogram (EEG) and CT/MRI brain imaging were performed in 150 of them (81/150 had an MRI and 69/150 had a CT scan).Patients were re-evaluated every six months for, at least, a two-year follow-up period (range 2-14 yrs).
Results: the EEG was abnormal in 76% of patients; epileptic seizures developed in 38.9% of them. The frequency of epilepsy paralleled the degree of EEG abnormality, approaching 85% in patients with severe EEG abnormalities and was also closely related to the extent of neuroimaging findings (up to 79% in patients with cerebral malformations).The prevalence of epilepsy in 12/62 patients (19.4%) with mild hemiplegia was significantly lower as compared to 67/141 (47.5%) of patients with moderate or severe hemiplegia. 36.7% of the children had their first seizure between the 1st and the 5th year of life, and 26.5% during the first year of life.
Conclusions: epileptic seizures developed in more than one third of patients with CH, although EEG abnormalities were evident in the majority of them.The prevalence of epilepsy is closely related to the severity of hemiparesis, the extent of neuroimaging findings and the degree of EEG abnormalities.The absence of EEG abnormalities and/or normal (or minor) neuroimaging findings was negatively related to the occurrence of epilepsy.
Tolerability of tiagabine: a prospective open-label study
Jürgen Bauer, Arnfin Bergmann, Markus Reuber, Stefan Rudolf Günther Stodieck, Pierre Genton
patients (299 men and 275 women, mean age 38.1 years), with refractory partial seizures, were enrolled in this prospective, open-label study. Tiagabine was added to one (44.1%) or more (up to nine) antiepileptic drugs. The median daily dose of tiagabine was 30 mg (mean 29.1, SD 12.0). The mean duration of follow-up was 94.9 ± 42.7 days. 12.3% of patients were completely seizure-free at the end of the observation period. Median total seizure frequency decreased from 4.5 to 2.0 seizures/4 weeks. Adverse events occurred in 78 patients (13.6%). Tiagabine proved to be a well-tolerated AED.
Compte-rendu de cas
Ictal bradycardia followed by cardiac asystole: a case report
Karl Mondon, Bernard Charbonnier, Caroline Hommet, Philippe Corcia, Alain Autret, Bertrand de Toffol
and analyzed. Clinical features and therapeutic considerations are discussed.
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