Paroxysmal kinesigenic dyskinesia (PKD) is a rare disease characterized by involuntary movements triggered by a sudden initiation of voluntary movement. No EEG abnormalities are observed both during and between PKD attacks. Clinically, the involuntary movements are readily controlled by sodium channel-related antiepileptic drugs such as carbamazepine  and phenytoin . Two hypotheses have been proposed for the pathophysiology of PKD; kinesthetic reflex epilepsy  and basal ganglia functional abnormality , but no consensus has been reached. We report a patient with amygdala enlargement who manifested complex partial seizure (CPS) following PKD attack.
The day of September 15, 2011 will be remembered in Europe as a historical date for all people engaged in the fight against epilepsy. On that date, the Parliament of the European Union (EU) approved the Written Declaration on Epilepsy, a landmark document which calls for the political establishment to improve the lives of people with epilepsy by promoting public awareness, quality of care and scientific research. The Declaration has resulted already in several positive initiatives in a number of countries, and its impact extends beyond the boundaries of the EU. The purpose of this article is to explain the background that motivated the drafting of the Declaration, to describe the processes that led to its approval, and to discuss future developments in the implementation of the plan set out by the Declaration. It is hoped that the activities described in this report will provide an inspiration to further actions in other parts of the world to mobilize stakeholders, address diagnostic and treatment gaps in epilepsy care, and stimulate greater allocation to resources to epilepsy research.
Purpose: To investigate the internal tempo of passage of time in patients with temporal lobe epilepsy (TLE), and to assess possible dependence on the laterality of interictal epileptiform discharges (IEDs). Methods: A verbal estimation task was administered to 30 right-handed patients with TLE in whom unilateral IEDs were repeatedly observed on interictal scalp electroencephalogram. Results: Patients with IEDs on the right side showed a significantly longer subjective duration of one minute than did patients with IEDs on the left. Discussion: IEDs on the non-dominant side may interfere with the activities of the supratemporal gyrus on the ipsilateral side during mental counting. However, the locus and nature of the internal clock that generates internal tempo remain unknown.
Objective: We used three-dimensional stereotactic surface projection (3D-SSP) technique to analyze 123I-iomazenil single-photon emission computed tomographic (IMZ-SPECT) images in temporal lobe epilepsy (mTLE) resulting from hippocampal sclerosis, to assess the usefulness of 3D-SPP in identifying epileptic focus and demonstrating the relationship between IMZ uptake distribution and clinical profile. Methods: We compared the 3D-SSP images with the original IMZ-SPECT visual images in 26 cases. We assessed IMZ uptake quantitatively in each brain region in all patients and investigated the influence of laterality (involved or non-involved side), a past history of secondary generalized seizure (SGS), and treatment with benzodiazepine on IMZ uptake in each region. Results: 3D-SSP showed significantly decreased IMZ uptake in the amygdale-hippocampus region on the involved side in 23 of 26 cases, while visual inspection by epileptologists identified correct focus laterality in 17 of 26 cases. The IMZ uptake in the temporal and frontal cortex as well as the amygdala on the non-involved side was significantly greater than that on the involved side. A past history of SGS and benzodiazepines treatment had no apparent effect on IMZ uptake in any region. Discussion: 3D-SSP was more useful than visual inspection in identifying epileptic focus and showing the distribution of IMZ uptake objectively and accurately. IMZ-SPECT using 3D-SSP not only facilitates detection of epileptic focus but also elucidation of the spread of pathological BZD receptors in the whole brain.
Purpose: To evaluate the efficacy and safety of lamotrigine monotherapy in patients with newly diagnosed partial seizures including secondarily generalized seizures or generalized tonic-clonic seizures, and those with recurrent seizures in Japan and South Korea. Methods: The study was a multi-center, open-label, evaluation of lamotrigine monotherapy in patients with newly diagnosed epilepsy or epilepsy with recurrent seizures. The primary endpoint was the seizure-free rate in the maintenance phase. Results: A total of 67 patients were enrolled; 52 patients completed 6 weeks of treatment (the escalation phase) and 42 patients completed 30 weeks of treatment (the escalation phase and the maintenance phase). The seizure-free rate in the maintenance phase for all seizure types was 43.1%. Adverse events (AEs) were reported in 82% (53/65) of patients. The most common AEs were headache (14/65, 22%), nasopharyngitis (12/65, 18%) and rash (7/65, 11%). Conclusion: The seizure-free rate with lamotrigine monotherapy in the maintenance phase was 43.1% for all seizure types. Lamotrigine monotherapy appears to be effective in patients with newly diagnosed epilepsy and those with recurrent seizures in Japan and South Korea. The safety profile of lamotrigine in this monotherapy study was similar to that observed in patients with adult bipolar disorder treated with lamotrigine monotherapy.
Cerebral microbleeds (CMBs) usually produce no symptoms. We encountered a patient who developed cognitive decline and psychotic symptoms associated with nonconvulsive status epilepticus (NCSE), with presumptive epileptogenic focus possibly caused by a CMB. A 70-year-old man developed progressive cognitive disturbances including disorientation and hallucinations two months after a mild head injury. He was admitted to our hospital three months after the trauma, because of progression of symptoms. The first positron emission tomography (PET) with [18F]fluoro-2-deoxy-d-glucose (FDG) demonstrated intense FDG uptake in the left occipitoparietal region, in which a CMB was detected by T2∗-weighted magnetic resonance imaging (MRI). Electroencephalography showed continuous slow waves in the left occipital and parietal areas. After anticonvulsive therapy, his symptoms completely disappeared, accompanied by change in FDG uptake. Our case suggests that CMBs may be an epileptogenic focus of NCSE, and that FDG-PET is useful for the diagnosis of NCSE and assessment of therapeutic efficacy.
A male infant who presented with neonatal asphyxia began to exhibit infantile spasms at three months of age. His seizures were refractory to conventional antiepileptic drugs including valproic acid, pyridoxal phosphate, zonisamide, clonazepam and adrenocorticotrophic hormone (ACTH)-Zn. Immediately following re-administration of valproic acid, infantile spasms were exacerbated and novel complex partial seizures with oral automatism and pedaling behaviors appeared. An electroencephalogram showed more severe hypsarrhythmia and novel focal polyspike bursts in the left mid-temporal region. High levels of glycine were detected in the cerebrospinal fluid (CSF) and plasma, and the activity of the glycine cleavage system was partially reduced to 18.0 % (-1.5 SD) in a [1-(13) C] glycine breath test. Therefore, non-ketotic hyperglycinemia was diagnosed. Administration of valproic acid exacerbated the basal infantile spasms and induced novel complex partial seizures, suggesting that the patienthad distinctive clinical seizures due to non-ketotic hyperglycinemia.
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