Non-Hyperammonaemic Valproate encephalopathy after 20years of treatment
We present the case of a 60-year lady with a generalized seizure disorder, treated with phenytoin, valproate, lamotrigine and clonazepam. Following withdrawal of phenytoin she developed an akinetic-rigid syndrome, with ataxia and marked cognitive impairment. Extensive investigation failed to identify a cause. Serum ammonia and valproate levels were normal. Hypothesizing this might be valproate encephalopathy, valproate was rapidly substituted with levetiracetam. Her severe motor symptoms resolved within two weeks and cognitive impairment markedly improved. Valproate-induced encephalopathy, with or without hyperammonaemia a...
Source: Epilepsy and Behavior Case Reports - April 21, 2017 Category: Neurology Source Type: research

Six Adult Patients with Septo-Optic Dysplasia and Drug-Resistant Epilepsy: Clinical Findings and Course
We report six SOD patients with associated malformation of cortical development (MCD) and drug-resistant epilepsy who underwent video-EEG telemetry at our centre between 1998 and 2016 for drug-resistant epilepsy. ‬Three then underwent surgery; Right temporal neocortical resection, right functional hemispherectomy and placement of a vagus nerve stimulator. Clinical findings and the patients' ultimate courses are discussed. (Source: Epilepsy and Behavior Case Reports)
Source: Epilepsy and Behavior Case Reports - April 19, 2017 Category: Neurology Source Type: research

Generalized Epilepsy in Baraitser –Winter Cerebrofrontofacial Syndrome
Publication date: Available online 18 March 2017 Source:Epilepsy & Behavior Case Reports Author(s): Seth Andrew Climans, Seyed M. Mirsattari (Source: Epilepsy and Behavior Case Reports)
Source: Epilepsy and Behavior Case Reports - March 17, 2017 Category: Neurology Source Type: research

West syndrome in three patients with brain injury and a benign epileptic course
Publication date: Available online 15 March 2017 Source:Epilepsy & Behavior Case Reports Author(s): Angelo Russo, Michael Duchowny, Antonella Boni, Melania Giannotta, Melissa Filippini, Giuseppe Gobbi Infants with West Syndrome and underlying structural pathology typically experience persistent symptomatic partial seizures and intellectual disability. We performed a retrospective case review of 84 patients with West Syndrome evaluated at one institution between 1990 and 2013 who were followed for a mean of 179months. From this group we identified three patients with West syndrome and congenital hemiplegia w...
Source: Epilepsy and Behavior Case Reports - March 16, 2017 Category: Neurology Source Type: research

Evolution of Epilepsy in Hemimegalencephaly from Infancy to Adulthood: Case Report and Review of the Literature
Publication date: Available online 1 March 2017 Source:Epilepsy & Behavior Case Reports Author(s): Kristin M. Ikeda, Seyed M. Mirsattari Hemimegalencephaly (HME) is a rare disorder of cortical development with overgrowth of one cerebral hemisphere. Patients have intellectual delay, hemiparesis and severe epilepsy. Drug-resistant epilepsy is often treated with a hemispherectomy. We review the literature on HME natural history and report a 26-year-old man with HME who did not undergo hemispherectomy in childhood with recurrent focal convulsive or non-convulsive status epilepticus. Few patients with HME have been ...
Source: Epilepsy and Behavior Case Reports - February 28, 2017 Category: Neurology Source Type: research

Epileptic Angina
Conclusion Pain is a rare manifestation of epilepsy observed in less than 1% of patients. When present, it is usually accompanied by other focal features. This rare occurrence of epileptic seizures masquerading as angina is a novel observation. (Source: Epilepsy and Behavior Case Reports)
Source: Epilepsy and Behavior Case Reports - February 21, 2017 Category: Neurology Source Type: research

Influenza-associated seizures in healthy adults: Report of 3 cases
Conclusion Seizures can occur in healthy adults with influenza infection without evidence of encephalitis, a possibility to be borne in mind to avoid potentially harmful therapeutic and diagnostic procedures. (Source: Epilepsy and Behavior Case Reports)
Source: Epilepsy and Behavior Case Reports - February 6, 2017 Category: Neurology Source Type: research

Compound heterozygosity with PRRT2: Pushing the phenotypic envelope in genetic epilepsies
We describe a patient with compound heterozygous variants, infantile epilepsy with status epilepticus, paroxysmal dyskinesia and episodic ataxia. Testing revealed a pathogenic PRRT2 duplication (c.649dupC), and a likely pathogenic missense variant (c916G>A). His presentation meets the severe phenotypic category with a combination of at least 3 neurological symptoms: seizures and status epilepticus, prolonged episodic ataxia, and paroxysmal dyskinesia. This further expands the clinical findings related to PRRT2, and suggests that compound heterozygous variants could confer a severe phenotype. (Source: Epilepsy and Behavior Case Reports)
Source: Epilepsy and Behavior Case Reports - February 1, 2017 Category: Neurology Source Type: research

Clinical and Neuropsychological Changes after the Disappearance of Seizures in a Case of Transient Epileptic Amnesia
We describe the memory function of the patient both before and after the administration of antiepileptic medication. After the patient's seizures were controlled with anticonvulsant therapy, her neuropsychological performance scores showed improvement. We presumed that the disappearance of epileptic seizures was associated with a decrease in the accelerated rate of forgetting. However, her lost memories were not recovered after the seizures were controlled by antiepileptic medication. (Source: Epilepsy and Behavior Case Reports)
Source: Epilepsy and Behavior Case Reports - January 28, 2017 Category: Neurology Source Type: research

Ezogabine skin discoloration is reversible after discontinuation
We present a case of ezogabine-induced skin discoloration that resolved completely after discontinuation. A 55-year-old woman started ezogabine 400mg three times a day at age 41. Bluish pigmentation over the toe nails, finger nails, around eyes and over and around lips was first noted after 5years of treatment. Ezogabine was discontinued eight years after initiation. Skin discoloration improved within 6months and completely resolved within 6years of discontinuation. This case suggests that ezogabine-induced discoloration is reversible after discontinuation of treatment. Graphical abstract (Source: Epilepsy and Behavior Case Reports)
Source: Epilepsy and Behavior Case Reports - January 28, 2017 Category: Neurology Source Type: research

Delayed diagnosis of shunt overdrainage following functional hemispherotomy and ventriculoperitoneal shunt placement in a hemimegalencephaly patient
Publication date: Available online 24 January 2017 Source:Epilepsy & Behavior Case Reports Author(s): Yasunori Nagahama, David Peters, Sho Kumonda, Adam Vesole, Charuta Joshi, Brian Dlouhy, Hiroto Kawasaki Shunt overdrainage represents a nebulous condition of variable clinical and imaging presentations, where the diagnosis is primarily clinical. The condition presents a diagnostic challenge particularly in patients with cognitive impairment and developmental delays. Here we present a 3-year-old boy who previously underwent functional hemispherotomy for hemimegalencephaly followed by ventriculoperitoneal sh...
Source: Epilepsy and Behavior Case Reports - January 23, 2017 Category: Neurology Source Type: research

Preoperative Visual Field Deficits in Temporal Lobe Epilepsy
We describe a patient with quadrantanopsia discovered as part of our recently modified protocol to workup patients prior to epilepsy surgery. This field cut was subtle and not detected on routine neurological examination. While we understand this is a single case, we advocate for more judicious preoperative visual field examinations to truly characterize the incidence of postoperative visual field lesions. (Source: Epilepsy and Behavior Case Reports)
Source: Epilepsy and Behavior Case Reports - January 20, 2017 Category: Neurology Source Type: research

Unilateral predominance of abnormal movements: a characteristic feature of the pediatric anti-NMDA receptor encephalitis?
This report illustrates previous descriptions of asymmetric presentation of abnormal movements in pediatric anti-NMDA receptor encephalitis and emphasises the importance of video-EEG interpreted within the overall clinical context, to differenciate epileptic from non-epileptic abnormal movements in patients with autoimmune encephalitis. (Source: Epilepsy and Behavior Case Reports)
Source: Epilepsy and Behavior Case Reports - January 17, 2017 Category: Neurology Source Type: research

Lamotrigine-related pseudolymphoma presenting as cervical lymphadenopathy
We describe the case of a 24year-old woman who developed lamotrigine-related bilateral cervical lymphadenopathy (pseudolymphoma) fifteen months following therapy initiation. This is the second such case reported in the medical literature. (Source: Epilepsy and Behavior Case Reports)
Source: Epilepsy and Behavior Case Reports - January 17, 2017 Category: Neurology Source Type: research

Vagus nerve stimulation for generalized epilepsy with febrile seizures plus (GEFS+) accompanying seizures with impaired consciousness
Publication date: 2017 Source:Epilepsy & Behavior Case Reports, Volume 7 Author(s): Ryosuke Hanaya, Fajar H Niantiarno, Yumi Kashida, Hiroshi Hosoyama, Shinsuke Maruyama, Sei Sugata, Toshiaki Otsubo, Kazumi Tanaka, Atsushi Ishii, Shinichi Hirose, Kazunori Arita (Source: Epilepsy and Behavior Case Reports)
Source: Epilepsy and Behavior Case Reports - January 5, 2017 Category: Neurology Source Type: research