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AHEAD OF PRINT
Late-onset, praxis-induced myoclonic epilepsy
Melanie Glenn, Enrique J Carrazana, Maria Raquel Lopez, Douglas M Wallace
been described in patients with juvenile myoclonic epilepsy. We report a patient presenting with praxis-induced myoclonic epilepsy at a late age. Ictal myoclonus was triggered by building a bird house and captured by video-polygraphic EEG recording. At 39 years old, the patient's age at onset of epilepsy was consistent with the syndrome of adult myoclonic epilepsy. Our case supports the notion of adult myoclonic epilepsy with possible occurrence of praxis-activation of seizures, as has been noted with the other idiopathic generalised epilepsies. [
<i>Published with videosequences</i>]
Gaucher disease: successful treatment of myoclonic status epilepticus with levetiracetam
Guadalupe Fernandez-Baca Vaca, Timothy Lenz, Elia M Pestana Knight, Ingrid Tuxhorn
epilepsy due to Gaucher disease. Under continuous video-EEG monitoring, the clinical myoclonic status and the electrographic ictal discharges resolved within 10 minutes after the infusion was initiated. The patient tolerated the treatment well without any reported side effects. This case suggests that levetiracetam may be a safe, effective, and well tolerated intravenous drug in patients with metabolic myoclonic status epilepticus such as Gaucher disease.
Topiramate and temporal lobe epilepsy: an open-label study
Angelo Labate, Antonio Siniscalchi, Laura Mumoli, Umberto Aguglia, Aldo Quattrone, Antonio Gambardella
We evaluated 41 patients (20 female, mean age 54+18 years) with temporal lobe epilepsy (TLE) referred to the Epilepsy Unit, University of Catanzaro, Italy. Patients received TPM as monotherapy directly or after having taken other antiepileptic drugs. Seizure frequency changes and adverse events were recorded. Follow-up was conducted for a period of at least two years after treatment.
<i>Results</i>. Patients received TPM, 50-600 mg/day,
<i>de novo</i> (
<i>n</i>=29) or initially as add-on therapy before the switch (
<i>n</i>=12). In total, 28 of 41 patients achieved seizure freedom, whereas 10 showed a ≥50% reduction of seizure frequency. Two patients did not respond well and one patient discontinued TPM due to adverse effects.
<i>Conclusions</i>. Our results confirm that TPM as either monotherapy or add-on therapy at doses of 50-600 mg/day effectively reduces seizure frequency in TLE. TPM is particular effective and very well tolerated in patients with mild TLE.
Late-onset Lennox-Gastaut syndrome as a phenotype of 15q11.1q13.3 duplication
João Rocha, Cátia Guerra, Renata Oliveira, Sofia Dória, Ricardo Rego, Maria José Rosas
cognitive and learning disabilities, autism spectrum disorder and refractory epilepsy. The late development of Lennox-Gastaut syndrome in patients with 15q11q13 duplication is a possibility that physicians should be aware of. We report the case of a 27-year-old man with a neurodevelopmental syndrome due to a 15q duplication, with intellectual disability, psychiatric disturbances, and an epileptic phenotype diagnosed as late-onset Lennox-Gastaut syndrome.
Late-onset temporal lobe epilepsy in a patient with juvenile myoclonic epilepsy
Octavian V Lie, Mark D Holmes
was an electrographic evolution from the typical generalised epileptiform patterns, characteristic of juvenile myoclonic epilepsy, to the novel appearance of interictal temporal spikes immediately preceding bisynchronous discharges, and subsequently to temporal intermittent rhythmic delta activity and temporal lobe-onset seizures. In this rare case of coexistent primary generalised epilepsy and focal epilepsy, the epileptic networks of the two forms of epilepsy appear to overlap.
Are proprioceptive-induced reflex seizures epileptically-enhanced stretch reflex manifestations?
Anna Szűcs, György Rásonyi, Péter Orbay, András Sólyom, András Holló, Zsuzsanna Arányi, József Janszky, Lóránd Erőss, Anita Kamondi
stretch reflexes. Proprioceptive reflex epilepsies are a good example of the so-called “system epilepsies”. We present three cases discussing the clinical features of such epilepsies. [
<i>Published with videosequences</i>]
Carbamazepine-induced non-epileptic myoclonus and tic-like movements
Adriana Magaudda, Gabriella Di Rosa
mechanism underlying carbamazepine-induced epileptic negative myoclonus has been proposed, a causative role of carbamazepine for positive myoclonus has not been fully identified. Here, we describe the video-documented case of an adult patient with non-epileptic myoclonus and tic-like movements persisting for 21 years, which appeared after he started carbamazepine treatment at 10 years of age. [
<i>Published with videosequences</i>]
Lamotrigine triggers the contact phase of coagulation
Thomas W Stief
Involuntary movements after correction of vitamin B12 deficiency: a video-case report
Caterina Zanus, Elena Alberini, Paola Costa, Franco Colonna, Floriana Zennaro, Marco Carrozzi
remain unclear due to a lack of video-EEG documentation making the electroclinical correlation difficult to ascertain. Here, we report video-EEG and neuroimaging findings of an 11-month-old girl with vitamin B12 deficiency, who acutely developed involuntary movements a few days after initiation of vitamin B12 treatment with normal vitamin plasmatic levels. Abnormal movements were a combination of tremor and myoclonus involving the face, mouth, and left arm, which disappeared after discontinuation of therapy. [
<i>Published with video sequences</i>]
Gelastic seizures in ring chromosome 20 syndrome: a case report with video illustration
Petia Dimova, Iliyana Boneva, Albena Todorova, Lorella Minotti, Philippe Kahane
the seizures manifesting with fear and hypermotor behaviour and episodes of non-convulsive status epilepticus (NCSE) are most typical, as well as cognitive impairment with behavioural problems and, sometimes, dysmorphic signs. We present a girl diagnosed at the age of 13 years who suffered from an atypical clinical presentation, with minimal cognitive problems, absence of dysmorphic symptoms, and hypermotor/gelastic seizures. [
<i>Published with video sequences</i>]
Psychogenic gelastic seizures in a patient with hypothalamic hamartoma
Timothy Scarella, Michael P Macken, Elizabeth Gerard, Stephan U Schuele
lesion prior to surgical intervention is challenging. Here, we report the case of a patient with a hypothalamic hamartoma who was diagnosed with psychogenic non-epileptic gelastic seizures using video-EEG monitoring. [
<i>Published with video sequences</i>]
