Source reconstruction of mesial-temporal epileptiform activity: comparison of inverse techniques.

Purpose: To evaluate whether advanced source reconstruction such as current density reconstruction (CDR) provides additional hints for clinical presurgical evaluation, different source reconstruction techniques with idealized spherical as well as realistically shaped head models (boundary element method, BEM) were applied on interictal and ictal epileptiform activity in presurgical evaluated patients with temporal lobe epilepsy. It is discussed whether CDR and BEM give additional information for presurgical evaluation compared to "conventional" strategies, such as single moving, and spatio-temporal dipole modeling with spherical head models.

Methods: A variety of source reconstruction procedures were applied to the data of five patients with pharmacoresistent temporal lobe epilepsy with probable mesial origin: (1) single-moving dipole in a spherical head model and (2) in BEM, (3) spatio-temporal dipole modeling in a spherical head model and (4) in BEM; and (5) deconvolution with fixed locations and orientations and (6) with cortically constrained L1-norm CDR in BEM.

Adult myoclonic epilepsy: a distinct syndrome of idiopathic generalized epilepsy.

The authors present 11 cases of idiopathic generalized epilepsy that began in adulthood at a mean age of 39 years. All patients had myoclonic jerks, five had absence seizures, and nine had infrequent generalized tonic-clonic seizures. A majority had a family history of seizures.

Current clinical diagnosis in Creutzfeldt-Jakob disease: identification of uncommon variants.

According to the recently established molecular basis for phenotypic heterogeneity of sporadic Creutzfeldt-Jakob disease (CJD), six different phenotypes are characterized by the size of the protease-resistant fragment of the pathological prion protein (types 1 and 2) and homozygosity or heterozygosity for methionine or valine at codon 129 of the prion protein gene (designated by MM1, MM2, MV1, MV2, W1, and W2). In the present investigation, we analyzed the value of commonly used clinical tests (electroencephalogram [EEG], detection of 14-3-3 protein in cerebrospinal fluid [CSF], and hyperintensity of the basal ganglia in magnetic resonance imaging) for the clinical diagnosis in each CJD phenotype. The detection of periodic sharp and slow wave complexes in the EEG is reliable in the clinical diagnosis of MM1 and MV1 patients only.

Dipole-source analysis in a realistic head model in patients with focal epilepsy.

Purpose: By the use of three different head models in EEG dipole analysis, we tried to model the origin of interictal and ictal epileptic activity as precisely as possible. Further, as a control, a second evaluation was made by an independent group to control for interindividual reliability of the dipole source analysis. With the realistic head model (CURRY) considering cortex, skull, and skin segmentation, the spike source was located.

How to improve the clinical diagnosis of Creutzfeldt-Jakob disease.

This paper describes a prospective follow-up of 364 patients initially notified as suspected Creutzfeldt-Jakob disease to a Surveillance Unit in Göttingen, Germany. Six patients were diagnosed as having genetic prion disease by blood analysis and were excluded from the study. After examination and review of the remaining 358, 193 were classified as probable Creutzfeldt-Jakob disease.

Cisternal S100 protein and neuron-specific enolase are elevated and site-specific markers in intractable temporal lobe epilepsy.

In the brain, S100 protein and neuron-specific enolase (NSE) are mainly found in glial cells and neurons, respectively. We investigated concentrations of S100 protein and NSE in cisternal cerebrospinal fluid obtained during implantation of foramen ovale electrodes in eight patients with temporal lobe epilepsy (TLE). In addition, the meningeal markers cystatin-C and beta-trace as well as total protein were measured.

Kinetics of serum neuron-specific enolase and prolactin in patients after single epileptic seizures.

Purpose: To investigate and compare the temporal profile of serial levels of neuron-specific enolase (NSE) and prolactin in serum from patients after single epileptic seizures.

Methods: Measurement of NSE and prolactin by sensitive immunoassays in 21 patients with complex partial seizure (CPSs: n = 11) and secondarily generalized tonic-clonic seizures (SGTCSs; n = 10) during continuous video-EEG monitoring at four different time points (1, 3, 6, and 24 h after ictal event). Statistical analysis was performed by using a repeated-measures analysis of variance (ANOVA) model.

A new epileptic seizure classification based exclusively on ictal semiology.

Historically, seizure semiology was the main feature in the differential diagnosis of epileptic syndromes. With the development of clinical EEG, the definition of electroclinical complexes became an essential tool to define epileptic syndromes, particularly focal epileptic syndromes. Modern advances in diagnostic technology, particularly in neuroimaging and molecular biology, now permit better definitions of epileptic syndromes.

Hashimoto's encephalitis as a differential diagnosis of Creutzfeldt-Jakob disease.

Objectives: During an epidemiological study of Creutzfeldt-Jakob disease in Germany, Hashimoto's encephalitis was encountered as a differential diagnosis, which has not yet been described in this context.

Methods: The symptoms and findings of seven patients who fulfilled the criteria for "possible" Creutzfeldt-Jakob disease are presented.

Results: A Hashimoto's thyroiditis with antibodies against thyroglobulin or thyroid peroxidase, or both and a hypoechoic thyroid ultrasonogram were found in all cases.

The Heidenhain variant of Creutzfeldt-Jakob disease.

Objective: To investigate whether typical neuropathological and radiological findings can be identified in patients with the clinical diagnosis of the Heidenhain variant of Creutzfeldt-Jakob disease (CJD).

Design: Case study. The clinical symptoms, neuropathological findings, electroencephalograms, magnetic resonance images, and cerebrospinal fluid samples of 14 Heidenhain cases were evaluated.

Spatial-frequency-related efficacy of visual stabilisation of posture.

The present study investigates the efficacy of visual stabilisation of posture for different spatial frequencies of a visual stimulus. Circular sine wave gratings were used to analyse the correlation between perception of motion in depth and stabilisation of fore-aft sway by the mechanism of detecting changes in target size. Body sway was recorded by a force-measuring platform (series A) and, in addition, by simultaneous tracking of infrared markers fixed to the subject's body (series B).

[Elecroencephalographic charactistics of Creutzfeldt-Jakob disease and its differential diagnosis].

Although clinical electroencephalography is no longer as important as it used to be in differential diagnosis of a fair number of neurological and psychiatric diseases ever since imaging techniques have been making enormous strides, EEG is still an important diagnostic tool in dementias where specific morphological lesions are not immediately or not at all apparent which would otherwise be visible by imaging. Sporadic Creutzfeldt-Jakob disease is an important case in point. Although this is associated with some unspecific EEG findings, typical periodical sharp wave complexes (PSWC) become conspicuous in the course of the disease.

Semiological seizure classification.

We propose an epileptic seizure classification based exclusively on ictal semiology. In this semiological seizure classification (SSC), seizures are classified as follows: a. Auras are ictal manifestations having sensory, psychosensory, and experiential symptoms.

Surface EMG and myosonography in the detection of fasciculations: a comparative study.

Surface electromyography (EMG) and muscle sonography both facilitate the detection of fasciculations. This study was conducted to evaluate the prevalence of fasciculations in 10 lower extremity muscles in 58 subjects 47 +/- 18 years of age without and 54 patients 52 +/- 15 years of age with various neuromuscular diseases (3 with inflammatory myopathy, 15 with lower motor neuron disease, 22 with acquired and 11 with hereditary motor and sensory neuropathy (HMSN), and 3 with adrenomyeloneuropathy). When each muscle was screened by means of myosonography for 10 seconds, fasciculations were found in up to 8 muscles in 11 control subjects (19%) and in up to 10 muscles in 41 patients (76%).

Nonconvulsive status epilepticus in two patients receiving tiagabine treatment.

In the course of an open study on the add-on treatment of tiagabine (TGB) in patients with localization-related epilepsy syndromes, 2 of 9 patients developed nonconvulsive status epilepticus (NCSE) with electroclinical features consistent with those of atypical absence seizures. One patient had never had atypical absence seizures before. In both cases, immediate discontinuation of TGB was followed by complete and sustained electroclinical remission; we suggest a possible causative role of TGB.

Transcranial magnetic stimulation: its current role in epilepsy research.

This paper reviews the current role of transcranial magnetic stimulation (TMS) in epilepsy research. After a brief introduction to the technical principles, the physiology and the safety aspects of TMS, emphasis is put on how human cortex excitability can be assessed by TMS and how this may improve our understanding of pathophysiological mechanisms in epilepsy and the mode of action of antiepileptic drugs (AEDs). Also, potential therapeutical applications of TMS are reviewed.

[Juvenile myoclonic epilepsy (Janz syndrome). A well-known epilepsy syndrome?].

Juvenile myoclonic epilepsy (JME) was described electroclinically by Janz and Christian in 1957 and consists of myoclonic jerks in varying combination with the absence of seizures and generalized tonic-clonic seizures, a typical circadian distribution on awakening, sleep deprivation, alcohol consumption and flickering stimuli as characteristic precipitating factors, typical EEG abnormalities with generalized epileptiform discharges, normal physical and neuroradiological findings, and often a family history of epilepsy. Although the misdiagnosis of this well-classified and easily treatable syndrome is supposed to occur rarely in Germany, we retrospectively investigated how often the wrong initial classification had been made among patients who were finally treated in our outpatient epilepsy unit. Between 1993 and 1995, 30 patients with a typical JME-like symptom constellation were identified who had been misdiagnosed initially.

[Semiologic classification of epileptic seizures].

Recent advances in epileptology and epilepsy surgery require revision of the currently used International Classification of Epileptic Seizures, which was published 1981. We present a classification of epileptic seizures which is based purely on the clinical seizure semiology. The advantages of a semiological seizure classification are stressed.

Isolated ataxia as an idiosyncratic side-effect under gabapentin.

Gabapentin has been accepted worldwide as a novel antiepileptic drug with a favourable tolerability profile. However, movement disorders have been reported previously as rare side-effects in individual patients. We report on two patients who developed isolated severe ataxia under low-dose gabapentin which resolved abruptly after discontinuation of the drug.

The lateralizing value of ictal clinical symptoms in uniregional temporal lobe epilepsy.

In order to assess the lateralizing value of several ictal and postictal clinical symptoms in temporal lobe epilepsy (TLE), we analyzed 89 seizures of 20 left dominant patients with intractable left (n = 9) versus right (n = 11) TLE who had undergone successful anterior temporal lobectomy. In left TLE, movement arrest at seizure onset, postictal dysphasia > 120 s and postictal dyslexia > 180 s were the most typical findings and associated with a sensitivity of 94, 94, and 100%, respectively. The highest specificity of 100% each was evident for contralateral versions of eyes and head and dystonic posturing.