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Non-epileptic myoclonic attacks in infancy: three cases

A. Vignoli, et al.

Volume 16, Number 4, December 2014

Keywords : Unknown, DROP ATTACKS, MYOCLONUS (NON-EPILEPTIC) , Nonepileptic paroxysmal event, Not applicable, Non epileptic paroxysmal disorder

has been increasingly confirmed in the literature. Non-epileptic attacks mimic epileptic paroxysms in clinical presentation, but they have a typically benign course and are unresponsive to pharmacological treatment. An evident feature of the syndrome is its extreme variability in clinical manifestation. Here, we describe three normal infants with two similar forms of non-epileptic paroxysms. Electroclinical manifestations and profile of evolution were investigated. Ictal video-EEG polygraphic recordings were obtained for each patient. The increasing number of such reported clinical cases in the literature may contribute to high quality systematic reviews and the development of useful guidelines in the future. The clinical heterogeneity of non-epileptic attacks, together with the relative rarity of the condition, may make differential diagnosis with epileptic attacks very challenging. [ Published with video sequences]

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Non-epileptic myoclonic attacks in infancy: three cases

A. Vignoli, et al.

Volume 16, Number 4, December 2014

Keywords : Unknown, DROP ATTACKS, MYOCLONUS (NON-EPILEPTIC) , Nonepileptic paroxysmal event, Not applicable, Non epileptic paroxysmal disorder

has been increasingly confirmed in the literature. Non-epileptic attacks mimic epileptic paroxysms in clinical presentation, but they have a typically benign course and are unresponsive to pharmacological treatment. An evident feature of the syndrome is its extreme variability in clinical manifestation. Here, we describe three normal infants with two similar forms of non-epileptic paroxysms. Electroclinical manifestations and profile of evolution were investigated. Ictal video-EEG polygraphic recordings were obtained for each patient. The increasing number of such reported clinical cases in the literature may contribute to high quality systematic reviews and the development of useful guidelines in the future. The clinical heterogeneity of non-epileptic attacks, together with the relative rarity of the condition, may make differential diagnosis with epileptic attacks very challenging. [ Published with video sequences]

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Non-epileptic myoclonic attacks in infancy: three cases

A. Vignoli, et al.

Volume 16, Number 4, December 2014

Keywords : Unknown, DROP ATTACKS, MYOCLONUS (NON-EPILEPTIC) , Nonepileptic paroxysmal event, Not applicable, Non epileptic paroxysmal disorder

has been increasingly confirmed in the literature. Non-epileptic attacks mimic epileptic paroxysms in clinical presentation, but they have a typically benign course and are unresponsive to pharmacological treatment. An evident feature of the syndrome is its extreme variability in clinical manifestation. Here, we describe three normal infants with two similar forms of non-epileptic paroxysms. Electroclinical manifestations and profile of evolution were investigated. Ictal video-EEG polygraphic recordings were obtained for each patient. The increasing number of such reported clinical cases in the literature may contribute to high quality systematic reviews and the development of useful guidelines in the future. The clinical heterogeneity of non-epileptic attacks, together with the relative rarity of the condition, may make differential diagnosis with epileptic attacks very challenging. [ Published with video sequences]

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Adult absence semiology misinterpreted as mesial temporal lobe epilepsy

R. Hurst, et al.

Volume 16, Number 4, December 2014

Keywords : Genetic predisposition, AUTOMATISMS, Lip smacking, Staring, Unkown, Juvenile absence epilepsy (JAE)

occur in the treatment of generalized epilepsy and may aggravate some seizure types, including absence seizures, potentially leading to pseudo-drug resistance. Fortunately, a correct diagnosis of absence seizures is usually not difficult, though rarely demonstrates electroclinical overlap with focal seizures. EEG can be especially misleading when secondary bilateral synchronous discharges occur in patients with focal seizures. However, the semiology of focal seizures associated with mesial temporal lobe epilepsy has a characteristic and consistent semiology that is the mark of this common epilepsy syndrome in adulthood. We recently encountered a 53-year-old female with refractory seizures and a semiology strongly suggesting mesial temporal lobe epilepsy. Instead of focal seizures, prolonged absence seizures were validated by video-EEG monitoring and she became seizure-free after a change to broad-spectrum antiepileptic drugs. This case further expands our understanding of the complexity of semiology in electroclinical classification and the spectrum that may occur in adult absence seizures. It serves to underscore the need for ictal EEG recordings and the importance of concordance with the clinical course during the pre-surgical evaluation of patients with lesions and drug-resistant epilepsy. [ Published with video sequences]

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Musicogenic and spontaneous seizures: EEG analyses with hippocampal depth electrodes

F. I. Tezer, et al.

Volume 16, Number 4, December 2014

Keywords : Unknown, AUTOMATISMS, CRYING, Reflex seizures, Temporal lobe (bilateral), Focal non-idiopathic temporal (TLE)

were obtained using subdural arrays, as well as hippocampal depth electrodes. Interestingly, this patient had both spontaneous seizures and musicogenic seizures, and they originated from different hippocampi. Due to bilateral independent musicogenic seizures and spontaneous seizures, our patient was not eligible for surgery, but vagal nerve stimulation treatment was almost successful. [ Published with video sequence]</p>

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Focal EEG slowing and chorea: electroclinical clues to the diagnosis of anti-NMDAR encephalitis

A. Osei-Lah, et al.

Volume 16, Number 4, December 2014

Keywords : Encephalitis (anti-NMDA receptor), Nonepileptic paroxysmal event, Not applicable, Not applicable

Most patients have an EEG study performed early in the course of their illness. Although not specific, there may be clues in the electroclinical features that should alert clinicians and electroencephalographers to the possibility of this diagnosis. This case is a reminder that anti- anti-N-methyl-D-aspartate receptor encephalitis may present initially with a movement disorder as the sole symptom, without features of an encephalopathy. In addition, it adds to the growing body of evidence that recognition of certain electroclinical clues may shorten the time to diagnosis. [ Published with video sequence]

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Myoclonic status epilepticus as a presentation of caspr2 antibody-associated autoimmune encephalitis

S. Ramanathan, et al.

Volume 16, Number 4, December 2014

Keywords : Encephalitis, Myoclonic seizure, MYOCLONUS (NON-EPILEPTIC) , Reflex seizures, Status epilepticus (convulsive), Central motor, Multifocal, Epileptic encephalopathy not otherwise classified, Not applicable

the presentation with myoclonic status epilepticus and the prolonged clinical course of refractory seizures, which are demonstrated in the accompanying videos, and not previously associated with this condition. Treatment with prednisone, intravenous immunoglobulin, plasma exchange, rituximab, cyclophosphamide, and mycophenolate mofetil resulted in significant functional improvement. Historically, myoclonic status epilepticus is associated with a grave prognosis and minimal chance of meaningful recovery. This case demonstrates that autoimmune encephalitis remains an important differential diagnosis in patients with such a presentation, and that early recognition and the appropriate institution of immunotherapy can result in seizure control and functional recovery. [ Published with video sequences]

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