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Biomedical subjects

B J Steinhoff

Publications and source records attributed to B J Steinhoff.

At least 37 records · Page 2Linked to original sources
PubMed1999

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. RESULTS: Mean+/-SD values for NSE levels (ng/ml) were 12.5 +/-4.4 (1 h), 10.8+/-3.8 (3 h), 11.1+/-4.9 (6 h), and 8.2+/-1.9 (24 h). The corresponding prolactin levels (mU/L) were 1,311+/-1,034, 232+/-158, 237+/-175, and 251+/-98. There was a significant decrease of NSE and prolactin levels over time (p < 0.001). The pair-wise comparison of NSE levels showed significant differences between the time points 1 vs. 24 h (p < 0.001), 3 vs. 24 h (p = 0.007), and 6 vs. 24 h (p = 0.009). In contrast, serum prolactin levels showed a significant difference between 1 vs. 3 h (p < 0.001) only. Most of the NSE levels remained normal after CPSs and SGTCSs. At 1 h after the seizure, only 33% of the subjects had increased NSE, whereas abnormal prolactin levels occurred with a sensitivity of 80%. CONCLUSIONS: In contrast to prolactin, serum NSE is not a sensitive marker of individual seizures. Only some individuals showed an increase of NSE beyond the prolactin-sensitive time frame after a single seizure, and mean NSE levels were not significantly increased compared with those of normal controls.

Adult↗
PubMed1999

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. At the same time detailed studies showed that there does not necessarily exist a one-to-one relationship between epileptic seizures or electroclinical complexes and epileptic syndromes. These developments call for the reintroduction of an epileptic seizure classification based exclusively on clinical semiology, similar to the seizure classifications which were used by neurologists before the introduction of the modern diagnostic methods. This classification of epileptic seizures should always be complemented by an epileptic syndrome classification based on all the available clinical information (clinical history, neurological exam, ictal semiology, EEG, anatomical and functional neuroimaging, etc.). Such an approach is more consistent with mainstream clinical neurology and would avoid the current confusion between the classification of epileptic seizures (which in the International Seizure Classification is actually a classification of electroclinical complexes) and the classification of epileptic syndromes.

Electroencephalography↗
PubMed1999

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. Analysis of CSF disclosed an increased leucocyte count in three patients, and a raised CSF:serum concentration ratio of albumin (QA1b) in four patients. The 14-3-3 protein, typical of Creutzfeldt-Jakob disease, could not be detected in any of our patients. No periodic sharp wave complexes, which are typical of Creutzfeldt-Jakob disease, were detected on EEG in any of the cases. By contrast with Creutzfeldt-Jakob disease, which leads to death within a few months, the patients with Hashimoto's encephalitis often recover quickly when treated adequately. All the patients improved after administration of corticosteroids. CONCLUSION: The clinical symptomatology of both diseases may be very similar: dementia, myoclonus, ataxia, and personality change or psychotic phenomena are characteristic symptoms.

Adult↗
PubMed1998

[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.

Brain Mapping↗
PubMed1998

[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. In all patients, localization-related epilepsy had been classified, although regional EEG abnormalities were rare (n = 5). All patients reported the typical circadian distribution, abnormal neuroradiological findings were absent, and typical EEG abnormalities were present in all cases. Under treatment with appropriate antiepileptic drugs 97% of the patients became almost completely seizure-free. Even 40 years after the first description of the syndrome, JME is an underdiagnosed illness. The generally positive course underlines the importance of its correct identification.

Adolescent↗
PubMed1998

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). Mean velocity and amplitude (RMS) of body sway were calculated in both sagittal (a-p) and lateral (l-r) planes. Sagittal sway was of least magnitude when viewing contrast gratings with lowest thresholds, whereas higher thresholds resulted in increasing sway parameters. As intended by the design of the stimuli, sagittal sway was correlated closer with the stabilising effect exerted by the different stimuli than was lateral sway. Sway velocity was reduced more efficiently, however, with a lower correlation with the psychophysical transfer function, than was RMS sway. Since sway velocity measured by the platform is suggested to depend to a greater extent on dynamic muscle forces generated at each individual body site the results indicate that visual information can be used to reduce and thereby optimise dynamic muscle action (sway velocity) even though static body sway is either not or less reduced. A comparable economisation of sway velocity but not of RMS sway was also seen at the end of posture investigations, indicative of positive training effects.

Adult↗
PubMed1998

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. For all aspects of this paper, a clear distinction was made between single-/paired-pulse TMS and repetitive TMS, since these two techniques have fundamentally different scopes and applications.

Anticonvulsants↗
PubMed1998

[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. If these are meticulously studied and particular attention is paid to their periodicity, a sensitivity of 67% and a specificity of 86% are attained. With the exception of one familial variant of Creutzfeldt-Jakob disease PSWC ar usually absent all other human prion diseases. Hence, it is not likely that they are linked to the aetiology of sporadic Creutzfeldt-Jakob disease. We present a patho-physiological hypothesis on the development of PSWC basing on the assumption that the specific periodicity of PSWC results from a still functionally active but greatly impaired subcortical-cortical circuit of neuronal excitability. This specific pattern of neuronal degeneration may obviously arise--albeit very rarely--also in other diseases independent of their aetiology, so that the EEG patterns appear identical. For this reason it is imperative to make complementary use of EEG and of recent clinical and laboratory data of Creutzfeldt-Jakob disease before PSWC and be considered a relevant diagnostic criterion. Conversely, clinical diagnosis of Creutzfeldt-Jakob disease should be reconsidered if repeated EEG recordings fail to reveal PSWC even under technically adequate conditions.

Creutzfeldt-Jakob Syndrome↗
PubMed1998

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. This observation may be consistent with a paradoxical effect of TGB in selected cases. Possible risk factors and a pathophysiological hypothesis are discussed.

Adult↗
PubMed1998

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. b. Autonomic seizures are seizures in which the main ictal manifestations are objectively documented autonomic alterations. c. "Dialeptic" seizures have as their main ictal manifestations an alteration of consciousness that is independent of ictal EEG manifestations. The new term "dialeptic" seizure has been coined to differentiate this concept from absence seizures (dialeptic seizures with a generalized ictal EEG) and complex partial seizures (dialeptic seizures with a focal ictal EEG). d. Motor seizures are characterized mainly by motor symptoms and are subclassified as simple or complex. Simple motor seizures are characterized by simple, unnatural movements that can be elicited by electrical stimulation of the primary and supplementary motor area (myoclonic, tonic, clonic and tonic-clonic, versive). Complex motor seizures are characterized by complex motor movements that resemble natural movements but that occur in an inappropriate setting ("automatisms"). e. Special seizures include seizures characterized by "negative" features (atonic, astatic, hypomotor, akinetic, and aphasic seizures). The SSC identifies in detail the somatotopic distribution of the ictal semiology as well as the seizure evolution. The advantages of a pure SSC, as opposed to the current classification of the International League Against Epilepsy (ILAE), which is actually a classification of electroclinical syndromes, are discussed.

Electroencephalography↗
PubMed1998

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%). Within the same recording period surface EMG revealed fasciculations in 5 control subjects (9%) and 30 patients (56%), whereas during a recording time of 20 minutes fasciculations were detected in 55 (95%) control subjects and all patients. An amplitude of 400 microV proved to be the optimum cutoff between fasciculations for healthy subjects and patients with neuromuscular disease (accuracy, 74%). Myosonography allowed differentiation of both groups with an accuracy of 79%. Surface EMG was more liable to artifacts than myosonography. The average interval between subsequent fasciculations cannot be used to differentiate patients with acquired and hereditary polyneuropathy and with lower motor neuron disease. Long-term surface EMG recording indicates fasciculations to occur in almost all patients with neuromuscular disease and the vast majority of healthy subjects. Muscle ultrasonography was more convenient and reliable than surface EMG in differentiating patients and healthy subjects.

Adult↗
PubMed1998

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. In right TLE, the highest sensitivity was seen for whole-body movements at seizure onset, postictal dysphasia < 120 s and postictal dyslexia < 180 s with figures of 82, 87, and 93%, respectively. As compared to left TLE, contralateral version and dystonic posturing, ictal speech, and postictal dyslexia < 180 s each had a specificity of 100%. The careful combined analysis of certain ictal clinical signs combined with consistent findings of interictal EEG and neuroimaging studies may be often sufficient to proceed with epilepsy surgery without invasive recordings even if ictal scalp EEG is not unambiguous.

Adolescent↗
PubMed1997

[Topiramate--an effective new anticonvulsant. An open prospective study].

Topiramate is a new antiepileptic drug, which is not yet marketed in Germany. The anticonvulsive activity is probably mediated by sodium-channel blockade combined with gabaergic and weak antiglutamatergic properties. We investigated 23 patients with partial seizures in this open prospective study. The efficacy was analysed under stable concurrent antiepileptic drugs. Two of the patients became seizure free. Ten patients had a reduction of seizure frequency of at least 50%. The responder rate was 57%. The maximum daily dosages were 400 to 850 mg. Side-effects without relationship to dose were nervousness and aggression. In two patients an psychotic episode occurred. Dose-related side-effects were ataxia, dizziness, somnolence and dysarthria. This study is underlining that topiramate is efficacious in the treatment of partial onset seizures but also associated with a narrow therapeutic width.

Adult↗