PubMed Health⌕ Search

Biomedical subjects

Bernhard J Steinhoff

Publications and source records attributed to Bernhard J Steinhoff.

18 recordsLinked to original sources
PubMed2006

Optimizing therapy of seizures in patients with endocrine disorders.

Endocrinologic disorders, such as thyroid dysfunction or diabetes, may be comorbidities in patients with epilepsy. The choice of medication should address such comorbidities wherever possible. Enzyme-inducing antiepileptic drugs (AEDs) such as carbamazepine, phenytoin, barbiturates, and oxcarbazepine among the new AEDs, may reduce the levels of free and total thyroxin. However, clinically relevant thyroid dysfunction owing to AED treatment is rare. Nevertheless, there are now better alternative AEDs with similar efficacy to the classic first-line drugs but without their enzyme-inducing properties. Such drugs should be considered in cases of thyroid dysfunction to reduce the likelihood of iatrogenic adverse effects. Sufficient data on some of the new AEDs to support this hypothesis are, however, still lacking and urgently needed. Several reports have pointed out the possible negative impact of valproate on glucose and insulin metabolism and particularly on body weight. The increased risk for development of polycystic ovarian syndrome remains a matter of debate but is certainly another argument for the development of alternative agents. The group of new AEDs offers some potential candidates. Topiramate is the AED that is most likely to lead to weight loss; this may be perceived as a positive side effect in some patients. This review summarizes the data on the possible impact of AEDs in endocrinological disorders, concentrating on thyroid dysfunction, insulin/glucose metabolism, and body weight.

Anticonvulsants↗
PubMed2006

Neuropsychological and psychiatric impact of add-on titration of pregabalin versus levetiracetam: a comparative short-term study.

Cognitive and behavioral impairments are common in patients with epilepsy. Multiple factors may contribute to these difficulties; among them is antiepileptic drug (AED) treatment. We examined the short-term impact of two new add-on AEDs, pregabalin (PGB) and levetiracetam (LEV), on cognition and psychiatric states in 20 adult patients with medically refractory partial epilepsy, before and shortly after add-on titration. According to an open, prospective comparative trial, add-on PGB was titrated to 300 mg and add-on LEV to 1000 mg in 10 patients each. Patients were assessed before (T1) and 2 weeks after (T2) addition of the AED. During the trial, seizure frequency did not change significantly in either group. With PGB, patients manifested partly significant impairments in episodic memory of verbal and visual information. Psychiatric states were unchanged. With LEV treatment, we saw improvements in visual short-term memory performance and psychiatric states (i.e., interpersonal sensibility, depression, and anxiety). The comparison between PGB and LEV revealed a trend toward higher anxiety scores and higher variability in hostility scores with PGB that was significantly different from the trend with LEV. No significant differences were apparent in all other neuropsychological and psychiatric parameters investigated. This short-term study suggests that add-on LEV has a favorable neuropsychological and psychiatric impact. The negative neuropsychological effects of PGB may reflect temporary effects under titration. Still, the results did not confirm the promising effects on psychiatric comorbidity that have been emphasized by other reports.

Adult↗
PubMed2006

Clinical characteristics in focal cortical dysplasia: a retrospective evaluation in a series of 120 patients.

Focal cortical dysplasias (FCDs) are increasingly diagnosed as a cause of symptomatic focal epilepsy in paediatric and adult patients. However, little is known about the clinical characteristics of epilepsy in these patients. In order to elucidate the clinical characteristics of their epilepsy, 120 pharmacoresistant patients including children and adults with histologically proven FCD were studied retrospectively. Age at seizure onset was analysed in the total group and compared between subgroups with different localization and different histological subtypes of FCD. The role of febrile seizures with respect to dual pathology was investigated. Seizure semiology was analysed focusing on initial seizure type and change of seizure semiology during the course of disease. Finally, transient responsiveness to antiepileptic drug therapy was studied. In the majority of patients, epilepsy began in the first 5 years of life. However, onset of epilepsy could also occur in the second or third decade until the age of 60. Age at epilepsy onset was not significantly different between temporal, extratemporal and multilobar localization of FCD. Patients without cytoarchitectural abnormalities (mild malformations of cortical development, FCD 1a according to Palmini) had significantly later epilepsy onset (P= 0.001) compared with patients with cytoarchitectural abnormalities (FCD 1b, 2a and 2b according to Palmini). In patients with additional hippocampal sclerosis (dual pathology) febrile seizures were significantly more frequently reported (P = 0.02) than in patients without dual pathology. Moreover, patients with dual pathology and febrile seizures significantly more frequently presented with severe hippocampal sclerosis (Wyler Grade 3-4) as compared with patients with dual pathology in the absence of febrile seizures (P = 0.03). First observed seizures were mainly tonic or generalized tonic-clonic. A change of seizure semiology seemed to be age-dependent and occurred between the age of >1 and 14 years. About 15.8% of the patients presented with status epilepticus during the course of disease. About 17% of the patients showed transient responsiveness (> or =1 year seizure freedom) to antiepileptic drug therapy either after initial therapy (50%) or later in the course of epilepsy (50%). Patients with FCD represent a heterogeneous group. Different age at epilepsy onset and transient responsiveness to antiepileptic drugs in approximately 17% of patients may reflect different dynamics in epileptogenicity of the underlying FCD. Dual pathology may be associated with different pathomechanisms in patients with and without febrile seizures.

Adolescent↗
PubMed2006

Seizure anticipation by patients with focal and generalized epilepsy: a multicentre assessment of premonitory symptoms.

PURPOSE: To assess subjective seizure anticipation in patients with focal and generalized epilepsy. METHODS: Five hundred consecutively recruited out-patients (251 male, 249 female, mean age 38.1 year) from three German tertiary epilepsy referral centres filled out questionnaires regarding subjective anticipation of seizures by at least 30 min and to timing and semiologic characteristics of their premonitory symptoms versus those of ictal phenomena. Patients were not regarded as having prodromi if the semiology of symptoms reported long before a seizure was identical to auras. RESULTS: 6.2% of patients reported that they were able to anticipate seizures. Premonitory symptoms were classified as stereotyped in all but one patient. An intraindividual semiologic analysis showed that the majority of these patients had symptoms, which were distinct from ictal experiences during auras. Seizure anticipation was reported both by patients with focal and idiopathic generalized epilepsy. The median estimated time interval between occurrence of premonitory symptoms and seizure onset was 90 min. CONCLUSIONS: This study gives evidence that both patients with focal and idiopathic generalized epilepsy may subjectively anticipate the occurrence of epileptic seizures. Premonitory symptoms are distinct from auras in terms of semiology and time of occurrence. The lower percentage of patients regarded as having premonitory symptoms as compared to some earlier reports may be related to stricter criteria and to the exclusion of auras, which could directly evolve into seizures, and other ictal events. Premonitory symptoms occur at similar periods prior to seizures as anticipatory EEG-changes have been reported using methods from time series analysis.

Adult↗
PubMed2006

Remote memory in temporal lobe epilepsy.

PURPOSE: The present study aims at characterizing remote memory in patients with temporal lobe epilepsy (TLE); it also considers the impact of its most important variables (lateralization of the lesion, duration of epilepsy, age at onset, and seizure frequency) on remote memory. METHODS: We examined the performance of 38 patients with unilateral TLE (19 right TLE and 19 left TLE) and 35 healthy subjects on six remote memory tasks. Memory for personal events was assessed by using the Autobiographical Memory Interview and the Modified Crovitz Test. Memory for public events was evaluated by means of photographs of famous faces and famous scenes, questions about famous events, and the Dead/Alive Test. RESULTS: Both right-TLE and left-TLE groups had impaired memory for autobiographic episodes and public events relative to normal subjects. In contrast, personal semantic memory was preserved. In addition, an effect of laterality was recorded, with right-TLE patients obtaining significantly better scores than left-TLE patients on every test. Duration of epilepsy, age at onset, and seizure frequency did not influence performance on remote memory measures. CONCLUSIONS: The comprehensive neuropsychological study of 38 TLE patients showed that this neurologic condition affects remote memory systems differently. We discuss the different factors that could account for this pattern of performance on the bases of both functional brain organization and memory theories.

Adult↗
PubMed2006

How to replace lamotrigine with valproate.

If lamotrigine (LTG) has to be replaced with valproate (VPA), this exchange may be complicated by adverse events that result from the complex interaction of both drugs. We report on two cases in which such problems occurred in spite of a cautious switch considering the VPA induced LTG serum increase. The satisfying outcome after a sudden and complete withdrawal of LTG in both cases encouraged us to perform the switch from LTG to VPA systematically by discontinuing LTG abruptly and building up the VPA maintenance dosage very rapidly in the following five consecutive patients who required this exchange. We recommend our abrupt dosage change-over strategy as an easy, safe and cost-effective option.

Adolescent↗
PubMed2005

The LAM-SAFE Study: lamotrigine versus carbamazepine or valproic acid in newly diagnosed focal and generalised epilepsies in adolescents and adults.

OBJECTIVE: To investigate efficacy and safety of lamotrigine (LTG) versus carbamazepine (CBZ) or valproic acid (VPA) in newly diagnosed focal (FE) and idiopathic generalised (GE) epilepsies in adolescents and adults. METHODS: Open-label randomised comparative multicentre 24-week monotherapy trial in newly diagnosed epilepsy patients of >or=12 years of age. Patients with FE were treated with LTG or CBZ, those with GE received LTG or VPA. The primary efficacy variable was the number of seizure-free patients during study weeks 17 and 24. RESULTS: Two hundred and thirty-nine patients were included. One hundred and seventy-six patients suffered from FE and 63 from GE. In the FE group, 88 patients each were treated with CBZ or LTG. Ninety-four percent of the CBZ patients and 89% of the LTG patients became seizure-free according to an intent-to-treat analysis (not statistically different). The rate of patients discontinuing treatment due to adverse events or a lack of efficacy was 19% with CBZ compared to 9% with LTG (not statistically different). In the GE group, 30 patients received VPA and 33 LTG. During study weeks 17 and 24, 61% of the LTG patients and 84% of the VPA patients had become seizure-free (not statistically significant). The drop-out rate due to lack of efficacy or adverse events was 12% with LTG and 3% with VPA (not statistically different). CONCLUSIONS: This study indicates that the effectiveness of LTG in focal and generalised epilepsy syndromes as initial monotherapy in patients >or=12 years is in the range of standard first-line antiepileptic drugs.

Adolescent↗
PubMed2005

Levetiracetam in patients with refractory epilepsy: results of the SKATE trial in Austria, Germany and Switzerland.

PURPOSE: To further evaluate the safety, efficacy and optimal dose of levetiracetam (LEV) in daily clinical practice among patients with uncontrolled partial epilepsy with or without secondary generalization. METHODS: In this phase IV, open-label, 16-week community-based study, 178 at least 16-year-old patients with refractory focal epilepsy were treated with 1000, 2000 or 3000 mg levetiracetam as adjunctive therapy. All patients started with 500 mg LEV b.i.d. (1000 mg/day); the dose was adjusted in 2-week intervals up to 1500 b.i.d. (3000 mg/day) depending on seizure control and tolerability. The main objectives were the adverse events, the percentage reduction in partial and total seizure frequency per week from baseline and the retention rate, defined as the percentage of patients taking LEV at the end of the 16-week treatment period. RESULTS: Of the 178 patients who took at least one dose of LEV 151 completed the study. Thus, the retention rate (number of patients taking LEV at the end of the 16-week treatment period) was 84.8%. Most frequently reported adverse events were asthenia, dizziness, headache, nausea, somnolence and hostility; the majority of these events were of mild to moderate intensity. The seizure-free rate of the ITT population with focal seizures was 16.7%, for all seizures 16.6%; the median reduction of focal seizure frequency was 47.6%, and 46.5% for all seizures. The 50% responder rate was 46.6% for focal seizures and 45.1% for all seizures. CONCLUSION: Add-on treatment with LEV in patients with refractory partial epilepsy was safe and effective in this study.

Adolescent↗
PubMed2004

Focal cortical dysplasias: surgical outcome in 67 patients in relation to histological subtypes and dual pathology.

The purpose of this study was to assess whether the histological subtype of focal cortical dysplasia and dual pathology affect surgical outcome in patients with medically intractable epilepsy due to focal cortical dysplasia (FCD). We retrospectively analysed the outcome of 67 patients from 2 to 66 years of age at follow-up periods of 6 to 48 months after epilepsy surgery. Histological subtypes were classified according to Palmini and included a few cases with mild histological abnormalities corresponding to the definition of mild malformations of cortical development. The seizure outcome was classified according to Engel and evaluated at the last follow-up visit as well as at follow-up periods of 12 and 24 months after surgery. The outcome in patients with FCD and additional hippocampal pathology (dual pathology) was analysed separately. Distribution of histological subtypes differed in temporal and extratemporal localization, with a significantly higher extratemporal prevalence of FCD type 2. There was a tendency towards better postsurgical outcome related to the last follow-up visit in patients with more subtle abnormalities classified as mild malformations of cortical development (mMCD) (63% Engel Ia), FCD type 1a (67% Engel Ia) and FCD type 1b (55% Engel Ia) compared with patients with FCD type 2a (43% Engel Ia) and FCD type 2b (Taylor type) (50% Engel Ia). Considering the outcome at follow-up periods over 12 and 24 months, complete seizure-freedom was achieved significantly more often in patients with FCD type 1 and mMCD than with FCD type 2, and seizure reduction by less than 75% (Engel IV) occurred in more patients with FCD type 2a compared with the other subgroups. This tendency was seen in the whole patient group and in the extratemporal subgroup. Patients with dual pathology almost always had temporal lobe epilepsy; the outcome in this patient group was generally favourable (66% complete seizure-freedom at the last follow-up visit). The outcome remained almost constant with longer periods of follow-up. We conclude that patients with FCD type 1 and mMCD had a better outcome compared with those with more severe forms of cortical dysplasia. A higher incidence of FCD type 1 in temporal localization did not allow the effects of histological subtype and localization to be separated. A subanalysis of extratemporal FCDs, however, revealed a similar tendency for a better outcome with FCD type 1, suggesting that the histological subtype itself seems to be at least a relevant cofactor influencing postsurgical outcome.

Adolescent↗
PubMed2004

Analysis of hippocampal atrophy in alcoholic patients by a Kohonen feature map.

We investigated the correlation of hippocampal volume with homocysteine, folate, vitamin B12 and B6 in alcoholic patients and healthy controls applying a Kohonen feature map (KFM) and conventional statistics. Representation of subjects on the KFM suggested an inverse correlation of hippocampal volume with blood levels of homocysteine and correlation with folate and vitamin B6. In conventional statistical analyses (t-test) reduced folate and increased homocysteine was found in alcoholics compared to healthy controls (p < 0.01). In female alcoholics vitamin B6 was reduced significantly (p = 0.03). Multiple linear regression analyses showed a significant correlation between average hippocampal volume and homocysteine (p < 0.001). KFM proved to be a sensitive tool for visualisation of statistical correlations in data sets even if no further statistical information is available.

Adult↗
PubMed2004

Diagnostic value of periodic complexes in Creutzfeldt-Jakob disease.

In 1996, our group published objective electroencephalogram (EEG) criteria to define periodic sharp-wave complexes (PSWCs) suggestive for Creutzfeldt-Jakob disease (CJD). These criteria have since then been strictly applied in all cases reported to us as possible CJD in the course of the German CJD surveillance study. Furthermore, EEG analysis of the records was performed without any additional information on complementary clinical and laboratory data. In this study, we investigated sensitivity, specificity, and the predictive values of these EEG criteria exclusively in cases in which autopsy confirmed (n=150) or excluded (n=56) CJD. EEG criteria were positive in 64% (n=96) of the CJD cases and falsely positive in 9% (n=5) of other dementias. The resulting figures for sensitivity, specificity, and positive and negative predictive values were 64%, 91%, 95%, and 49%, respectively. In the falsely positive cases, Alzheimer's disease (n=4) and vascular dementia (n=1) were the underlying diseases. However, only in one of these five cases both clinical and EEG data would have led to the false-positive result to diagnose probable CJD. These data prove the high diagnostic value of our objective EEG criteria in CJD.

Adult↗
PubMed2003

Relationship between lamotrigine oral dose, serum level and its inhibitory effect on CNS: insights from transcranial magnetic stimulation.

The antiepileptic drug lamotrigine (LTG) is known to reduce cortical excitability evaluated by transcranial magnetic stimulation (TMS). We investigated the relationship between LTG oral dosages, serum levels and inhibitory effects on resting motor threshold (RMT), a parameter of motor system excitability assessed by TMS. In a randomized, placebo-controlled crossover study 16 male volunteers received 325 mg LTG as a single dose, as bi-hourly graded cumulative dose, or placebo. RMT and serum levels were measured before and after 2-8 h. With single dose, RMT elevation showed a poor but significant correlation to serum levels. With graded dose, serum levels as well as RMT increased dose-dependently with significant (P<0.0001) linear correlation. However, detailed comparison showed a high inter-individual variability in the relationship resembling a sigmoid correlation. Different mechanisms besides the sodium-channel blockage as the main mode of action of LTG are discussed to explain the diversity of individual dose-response relationships. Provided that the RMT elevation reflects the antiepileptic potential of LTG, TMS may be developed as a tool to monitor interindividual response of epilepsy patients to LTG treatment as well as to explore efficacy of other antiepileptic drugs with similar mode of action.

Administration, Oral↗
PubMed2002

Neuropsychological performance in frontal lobe epilepsy.

The search for a special neuropsychological profile of frontal lobe epilepsy subjects (FLE) has so far led to inconclusive results. In this paper we compared the preoperative neuropsychological performance of FLE and temporal lobe epilepsy (TLE) subjects. We further investigated whether frontal lobe lesions of epileptogenic cause produce the same type of cognitive dysfunction as do tumours of the frontal lobe. Sixteen FLE subjects were compared to 16 TLE subjects as well as to a group of 10 subjects after the removal of frontal lobe tumors (TUM) and a healthy control group. A set of neuropsychological test measures routinely used for presurgical evaluation, an emotional conceptualization task and two associative learning tasks were administered. We found that subjects with frontal lobe damage were significantly impaired relative to controls on a wide range of cognitive functions independent of neurological cause. FLE subjects could hardly be discriminated from TLE subjects as both groups showed a similarly reduced level of neuropsychological performance. Our results demonstrate the devastating effect that frontal lobe epilepsy can have on cognitive functioning. Routinely used neuropsychological test measures lack the specificity to distinguish between frontal and temporal lobe epilepsy. Highly specialized measures are necessary to reveal differences.

Adult↗
PubMed2002

Clinical findings in sporadic Creutzfeldt-Jakob disease correlate with thalamic pathology.

The pathogenesis underlying the typical findings in Creutzfeldt-Jakob disease (CJD) such as periodic EEG changes or myoclonus is not fully understood. The thalamus possesses a high density of inhibitory neurones and serves as a crucial pacemaker of rhythmic EEG activity. As inhibitory neurones expressing parvalbumin (PV) are reduced in the cerebral cortex and hippocampus in sporadic CJD (sCJD), we studied the distribution and number of PV-immunoreactive neurones in sCJD thalami in order to determine whether damage to them could account for certain clinical findings. Immuno histochemical analysis was performed on the thalami from 21 sCJD patients and five controls. The number of PV+ neurones was counted in the thalamic nuclei and compared with clinical and molecular findings. In sCJD patients, PV+ neurones were significantly reduced in the ventrolateral posterior (VLp), ventrolateral anterior (VLa), anteroventral (AV), lateral dorsal (LD), mediodorsal (MD) and reticular (Re) thalamic nuclei (P < 0.05). The VLp was especially damaged in sCJD patients with homozygosity for methionine at codon 129 and scrapie prion protein (PrP(Sc)) type 1. Patients with typical EEG changes [periodic sharp wave complexes (PSWCs)] and myoclonus had a predominant loss of PV+ cells in the reticular thalamic nucleus. In conclusion, our data support the hypothesis that the damage to PV-immunoreactive neurones determines the generation of certain typical clinical features of CJD, i.e. PSWCs associated with myoclonus.

Action Potentials↗
PubMed2002

Sonographic imaging of foramen ovale electrodes.

OBJECTIVE: In patients with medically intractable partial epilepsy of mesiotemporal origin, video electroencephalographic monitoring with foramen ovale electrodes is necessary to plan neurosurgical interventions. Imaging of these electrodes after implantation hitherto required conventional radiography, magnetic resonance imaging, or computed tomography of the skull. These methods are expensive. Therefore, the aim of our work was to show the capability of more cost-effective transcranial B-mode sonography for visualization of the electrodes. METHODS: In this pilot study, a 42-year-old female patient with implanted foramen ovale electrodes was examined transtemporally with a 2-MHz sector transducer to visualize the intracranially implanted electroencephalographic recording device. RESULTS: Foramen ovale electrodes could be detected easily in the patient, and bedside monitoring of explantation was possible. CONCLUSIONS: We were able to show the applicability of transcranial B-mode sonography for visualization of foramen ovale electrodes in preoperative electroencephalographic monitoring of patients with epilepsy. Further evaluation of this method in additional patients will follow.

Adult↗
PubMedDate not supplied

Trigeminal evoked potentials and sensory deficits in atypical facial pain--a comparison with results in trigeminal neuralgia.

Trigeminal evoked potentials (TEPs) and sensory deficits in eighty-three patients admitted for first surgical treatment of facial pain were retrospectively analysed. Thirty-seven patients suffered from trigeminal neuralgia (TN), 10 from symptomatic TN (sTN), and 36 from atypical facial pain (AFP). Eighteen percent of the TN patients reported sensory deficits on the pain side, but 35% had delayed ipsilateral N13 waves. Of the sTN patients, 60% had either sensory deficits or a pathological corneal reflex and 62.5% a pathological N13. Of the AFP patients, 61% complained of sensory deficits, but only 31% had a pathological N13. The percentage of pathological P19 waves was slightly lower (20%, 50%, and 11%, respectively), but showed a similar trend. Normal TEPs were found even in the presence of a sensory deficit (reported only in the AFP group). These findings may add weight to the hypothesis of underlying psychiatric disorders in AFP.

Blinking↗