Seizures are less frequent in progressive multifocal leukoencephalopathy (PML), because it is considered to be restricted to the white matter. In addition, types of seizures in patients with PML are mostly convulsive, and seizures are usually present at the time of diagnosis or early in the course of disease. We report a case of chronic PML with recurrent complex partial seizures in the absence of motor component.
Humans ; Leukoencephalopathy, Progressive Multifocal ; Seizures

Palinopsia means a visual image persisting for minutes to hours or reappearing episodically after the exciting stimulus has been removed. The anatomic correlation of palinopsia is not clear, whereas occipito-parietal and occipito-temporal lesions have been implicated. We describe a patient presenting palinopsia prior to motor seizures which was related to left posterior temporal lesion. A 36-year-old man had a generalized tonic clonic seizure after palinoptic positive visual afterimages. Brain MRI and Transfemoral cerebral angiography (TFCA) revealed an arteriovenous malformation of 1x1.5x2 cm3. We used oxcarbazepine for preventing recurrent seizures and planned gamma knife radiosurgery. Palinopsia is a very rare clinical manifestation as an aura of seizures. Palinopsia preceding clinical seizure as a localizing value, so that neuroimaging is mandatory in this clinical situation. This is the first reported case of palinopsia in Korea.
Afterimage ; Arteriovenous Malformations ; Brain ; Carbamazepine ; Cerebral Angiography ; Epilepsy ; Humans ; Korea ; Neuroimaging ; Radiosurgery ; Seizures ; Visual Perception

Oxcarbazepine (OXC) is generally assumed to be safe with regard to the risk for adverse hematopoietic effects. Recently, pancytopenia and leukopenia associated with the treatment of OXC have been reported. However, that serious adverse effect has never been reported in Korea. We describe a case of 47-year-old woman with temporal lobe epilepsy who developed reversible leukopenia following OXC monotherapy. She stopped to take OXC and WBC count was normalized completely.
Carbamazepine ; Epilepsy, Temporal Lobe ; Female ; Humans ; Korea ; Leukopenia ; Pancytopenia

Wolf-Hirschhorn syndrome is a well-recognized malformation syndrome with multiple congenital anomalies, resulting from partial deletion of the short arm of chromosome 4 (4p-). All affected individuals have intrauterine and postnatal growth retardation with marked feeding difficulties, developmental delay, and intellectual disability. Additionally, most of patients have seizures from early infancy. Although seizures are common with this syndrome, presenting with status epilepticus (SE) is rare. We report two cases of Wolf-Hirschhorn syndrome presenting with SE.
Arm ; Chromosome Deletion ; Chromosomes, Human, Pair 4 ; Humans ; Intellectual Disability ; Seizures ; Status Epilepticus ; Wolf-Hirschhorn Syndrome

PURPOSE: The aim of this study was to describe the clinical manifestations and radiologic characteristics of patients with hypothalamic hamartoma (HH) and to delineate various treatment modalities.METHODS: We retrospectively reviewed the medical records of 11 patients with HH at Samsung Seoul Hospital between 1997 and 2013.RESULTS: Mean age of diagnosis was 2.7 years. Seven patients had intractable epilepsy, seven exhibited precocious puberty, and four had both. Gelastic seizure was the most common seizure and was intractable to medications. The patients with the intrahypothalamic type (18.2%) had no precocious puberty, while those with the parahypothalamic type (36.4%) didn't have seizures. Patients (n=6) with intractable epilepsy underwent gamma-knife radiosurgery (GKS); five of these patients responded to this treatment.CONCLUSIONS: The most frequent presenting symptoms of HH were gelastic seizure and precocious puberty. The location and size of tumor defined by brain magnetic resonance imaging affected presenting symptoms and the selection of surgical treatment. GKS can be considered if the patients showed intractable epilepsy and the size of tumor is less than 2 cm in diameter.
Brain ; Epilepsy ; Hamartoma ; Humans ; Magnetic Resonance Imaging ; Medical Records ; Puberty, Precocious ; Radiosurgery ; Retrospective Studies ; Seizures

PURPOSE: Status epilepticus (SE) is a pathologic state where pharmacokinetic alterations can be more pronounced and more rapid than during the other epileptic states. The consequences of such changes can exert negative influences on the timely adequate treatments for stopping uncontrolled seizures during SE. Topiramte (TPM) is one of new antiepileptic drugs with high efficacy in epilepsy, which can also be effectively used in SE. The aim of this study was to evaluate the pharmacokinetic changes during the SE by an analysis of the therapeutic drug monitoring (TDM) of TPM in patients with SE.METHODS: We retrospectively analyzed 49 serum measurements of TPM from 22 subjects with SE. The serum concentrations of TPM were measured by HPLC-tandem mass spectrometry. TDM data were categorized into malignant status epilepticus (MSE), refractory status epilepticus (RSE), and non-status epilepticus (NSE) groups. We compared concentration-to-dose ratio (CDR) among those groups.RESULTS: Among 49 cases, 11 were in MSE, 19 in RSE, and 19 in NSE. The daily dose of TPM was higher in MSE (median, interquartile range: 600, 600-800 mg) than in RSE (300, 250-600 mg) and NSE (200, 150-400 mg). The daily dose adjusted for body weight was also higher in MSE (12.2, 10.4-13.9 mg/kg) than in RSE (4.5, 3.8-12.2 mg/kg) and NSE (4.1, 2.3-7.1 mg/kg) (p<0.01). Serum concentrations of TPM were less in MSE (5.8, 4.2-7.3 mg/L) and RSE (4.9, 2.9-6.0 mg/L) than in NSE (5.5, 3.3-9.0 mg/L), which were not significantly different among the groups (p>0.1). However, the concentration-to-dose ratio (CDR) was significantly lower in MSE (0.41, 0.35-0.59 kg/L) and RSE (0.85, 0.39-1.23 kg/L) than in NSE (1.72, 0.96-2.24 kg/L) (post hoc analysis, p<0.005, 0.05).CONCLUSIONS: The serum concentrations of TPM can be influenced by SE, particularly in MSE. The higher range of dose of TPM could be needed for an adequate treatment of SE.
Anticonvulsants ; Body Weight ; Drug Monitoring ; Epilepsy ; Fructose ; Humans ; Mass Spectrometry ; Retrospective Studies ; Seizures ; Status Epilepticus

Ring chromosome 14 syndrome is a rare cytogenetic disorder characterized by typical facial appearance, developmental delay, and intractable epilepsy. There have been about 50 reported cases in the world and one case in Korea. Epilepsy is the most common and serious neurologic comorbidity of the syndrome and it typically begins at early ages and frequently becomes intractable. We report a girl with ring chromosome 14 syndrome who showed early onset intractable epilepsy with repetitive episodes of clustering seizures. We describe the case and the result of long term follow-up for the epilepsy. The early suspicion of the syndrome and prompt management for seizures are necessary for the favorable prognosis.
Chromosomes, Human, Pair 14 ; Comorbidity ; Cytogenetics ; Epilepsy ; Follow-Up Studies ; Korea ; Prognosis ; Ring Chromosomes ; Seizures

Hiccups may be considered a form of myoclonus of diaphragm. Valproic acid has been considered the drug of choice in treatment of intractable hiccups; however, its various adverse events limit its use in the clinical basis, especially in elderly or complicated patients. Levetiracetam was known as a safe antiepileptic drug. However, the anti-hiccup property of the levetiracetam has been rarely reported. We report a 69-year old male patient who developed sudden persistent hiccups and was successfully treated with levetiracetam. Levetiracetam should be considered as alternative treatment option in selected patients with intractable hiccups.
Aged ; Diaphragm ; Hiccup ; Humans ; Male ; Myoclonus ; Piracetam ; Valproic Acid

Jeavons syndrome is one of the underrecognized epileptic syndromes, characterized by eyelid myoclonia with or without absence seizures, eye closure-induced seizures, electroencephalography (EEG) paroxysms, and photosensitivity. This syndrome is considered to be among idiopathic generalized epilepsies, but the underlying pathophysiology is unknown. Recent studies using functional MRI and EEG have suggested an important role of both thalamus and occipital cortex in the fundamental pathophysiology underlying Jeavons syndrome. We described here a patient with typical Jeavons syndrome, in whom SPCET studies performed ictally and interictally revealed ictal hyperperfusion mainly confined to the both occipital and parietal cortices and ictal hypoperfusion in the diffuse frontal and temporal cortices. Our SPECT findings of ictal hyperperfusion in occipital and parietal cortices and ictal hypoperfusion in widespread cortices are, to certain degree, in line with previous EEG and fMRI studies, suggesting that the interactions between occipital and other cortical areas might be implicated in generalized spike-waves generation and a photosensitivity in Jeavons syndrome.
Electroencephalography ; Epilepsy, Absence ; Epilepsy, Generalized ; Eye ; Eyelids ; Humans ; Magnetic Resonance Imaging ; Seizures ; Thalamus ; Tomography, Emission-Computed, Single-Photon

PURPOSE: Material-specific memory deficits (laterality effects) are frequently observed in patients with temporal lobe epilepsy (TLE), and yet the different functional roles of temporal cortical vs. mesial structures remain unclear. This study aimed to examine the lateralization and localization of memory and other neurocognitive functioning in patients with TLE.METHODS: Sixty-nine patients with lesional TLE participated. Subjects were categorized in terms of lesion laterality (left, n=34; right, n=35) and intratemporal location (mesial, n=50; lateral, n=19). Neurocognitive tests, including verbal memory, visual memory, general intelligence, attention, working memory and executive function, were tested.RESULTS: Left TLE patients showed significantly higher visual than verbal memory capacity, whereas right TLE patients showed significantly better verbal than visual memory capacity. There were no differences between mesial TLE and lateral TLE groups on any other memory tasks. Mesial TLE patients showed significantly lower working memory and executive function compared with lateral TLE patients.CONCLUSIONS: Selective verbal or visual memory deficits that are dependent on side of seizure foci were apparent in this study. The different functional roles of temporal cortical vs. mesial structures in memory process (i.e. aquisition/working memory vs. long-term consolidation) were not clear, but rather mesial temporal lobe damage was found to be associated with working memory deficit. It may be explained in part by epileptogenic activity propagating from temporolateral to mesial structures and vice versa.
Epilepsy, Temporal Lobe ; Executive Function ; Humans ; Intelligence ; Memory ; Memory Disorders ; Memory, Short-Term ; Seizures ; Temporal Lobe