Evolution of interferon-beta binding antibodies in MS patients may predict development of neutralizing antibodies.
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Biomedical subjects
Publications and source records attributed to K Berek.
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Polyneuropathy developing in critically ill patients ("critical illness polyneuropathy"--CIP) is diagnosed with increasing frequency in intensive care units. CIP is an axonal polyneuropathy leading to difficulties in weaning from artificial ventilation and symmetrical flaccid tetraparesis. Pathogenetically CIP is considered to be part of the multiple organ dysfunction syndrome (MODS) in the course of sepsis or systemic inflammatory response syndrome (SIRS). The incidence of CIP in septic patients with MODS ranges from 50% to 70%. Electroneurography (ENG) and electromyography (EMG) are essential diagnostic procedures. The mortality in patients with CIP is higher than in patients without CIP. Depending on the severity of CIP, recovery of neurological function in survivors is usually relatively good.
Early determination of outcome after successful prehospital cardiopulmonary resuscitation (CPR) is a common problem with great ethical, economic, social, and legal consequences. We prospectively investigated 112 adult patients who had been resuscitated after out-of-hospital cardiac arrest (CA). The aim of our study was to determine whether coma rating by the mobile intensive care unit (MICU) is a useful tool for outcome prediction. For neurological assessment the Innsbruck Coma Scale (ICS) was used initially and after return of spontaneous circulation (ROSC) or 20-30 min after the start of CPR, before any sedating drugs were given. The duration of anoxia and CPR were determined with the automatically recorded emergency call protocol of the dispatch centre and the protocol of the MICU. For estimation of cerebral outcome at the time of discharge from hospital we used the Glasgow-Pittsburgh Cerebral Performance Categories (CPC). Restoration of spontaneous circulation was achieved in 42 patients (37%), and 15 (13%) were discharged from hospital. The first coma rating performed immediately at the time of arrival on scene had no significant prognostic value for prediction of neurological outcome (P = 0.204) and survival (P = 0.103). The second coma rating (performed after ROSC or 20-30 min after the start of CPR), however, demonstrated a significant correlation with neurological outcome (P = 0.0000) and survival (P = 0.0000), a correlation which was comparable to both duration of anoxia and duration of CPR. In patients with out-of-hospital cardiac arrest prognostic information could be obtained with the ICS as early as 20-30 min after the start of cardiopulmonary resuscitation.
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Early determination of outcome after out-of-hospital cardiopulmonary resuscitation is a common problem with great ethical, economic, social and legal consequences. Although there has been a fulminant development of emergency medicine during the last three decades, severe cerebral damage sometimes cannot be avoided. For neurological outcome prediction after cardiac arrest clinical neurological signs, electrophysiological examinations, neuroimaging tests, and laboratory parameters in serum and cerebrospinal fluid are used today, nevertheless, there still remains a considerable degree of uncertainty. However, although prognostic criteria which enable the clinician to stop treatment cannot be given at the present time, useful applications of early prognostication after cardiac arrest range from counseling of families, triage decisions, and do-not-resuscitate decisions to future clinical investigations of brain resuscitative measures.
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Hereditary factors play a major role in the etiology of idiopathic generalized epilepsies (IGEs). A trait locus (EBN1) for a rare subtype of IGEs, the benign neonatal familial convulsions, and a susceptibility gene (EEGV1) for the common human low-voltage electroencephalogram have been mapped close together with D20S19 to the chromosomal region 20q13.2. Both loci are potential candidates for the susceptibility to IGE spectra with age-related onset beyond the neonatal period. The present study tested the hypothesis that a putative susceptibility locus linked to D20S19 predisposes to spectra of IGEs with age-related onset from childhood to adolescence. Linkage analyses were conducted in 60 families ascertained through IGE patients with juvenile myoclonic epilepsy, juvenile absence epilepsy or childhood absence epilepsy. Our results provide evidence against linkage of a putative susceptibility gene for four hierarchically broadened IGE spectra with D20S19 assuming tentative single-locus genetic models. The extent of an "exclusion region" (lod scores below-2) varied from 0.5 cM up to 22 cM on either side of D20S19 depending on the trait assumed. These results are contrary to the expectation that a susceptibility gene in vicinity to D20S19 confers a common major gene effect to the expression of IGE spectra with age-related onset from childhood to adolescence.
OBJECTIVE: To determine the incidence, severity and course of polyneuropathies in patients with sepsis or systemic inflammatory response syndrome combined with multiple organ failure. DESIGN: Prospective study. SETTING: Division of Intensive Care Medicine of the Department of Anesthesiology and Intensive Care and Intensive Care Unit of the Department of Neurology, University Hospital Innsbruck, Austria. PATIENTS: Twenty-two patients between 23 and 77 years old with sepsis or systemic inflammatory response syndrome combined with multiple organ failure fulfilling strict inclusion and exclusion criteria. INTERVENTIONS: Clinical neurologic examination and electro myography/nerve conduction velocity measurements during the stay on the Intensive Care Unit and 2-3 months later. MEASUREMENTS AND RESULTS: In 9 of the 22 patients signs of polyneuropathy were found at the initial clinical investigation and in 7 patients at the follow-up investigation. Electrophysiologic investigation revealed signs of polyneuropathy in 18 patients initially and in 11 patients 2-3 months later. CONCLUSION: In our patient population the frequency of the development of polyneuropathy was high (81.8%). Electrophysiologic investigation is superior to clinical neurologic examination in the detection of polyneuropathies.
Linkage studies of families ascertained through patients with juvenile myoclonic epilepsy (JME) suggest that an HLA-linked susceptibility gene on chromosome 6, designated "EJM1," predisposes to a group of idiopathic generalized epilepsies (IGEs) comprising JME, juvenile absence epilepsy (JAE), childhood absence epilepsies (CAE), and epilepsies with generalized tonic-clonic seizures (GTCS). To explore the EJM1-related phenotypic spectrum, we conducted linkage studies with HLA-DQ alpha restriction fragment length polymorphisms in 44 families ascertained through patients with CAE or JAE. Our results for the entire group of families provide evidence against a major susceptibility locus for idiopathic absence epilepsies and broader spectra of IGEs in the HLA region. Lod scores less than -2 were obtained for a region from 10 cM up to 23 cM on either side of the HLA-DQ alpha locus, depending on the assumed trait model. Suggestive evidence for linkage was found only for a subgroup of families with JME patients assuming an autosomal dominant mode of inheritance with 70% penetrance. A maximum lod score was obtained when family members with JME, JAE, CAE, and idiopathic GTCS were included into the affection status. Our results demonstrate that (1) the genetic susceptibility to idiopathic absence epilepsies and broader spectra of IGEs is heterogeneous, (2) the gene effect of EJM1 depends on the familial genetic background, and (3) EJM1 confers genetic susceptibility to idiopathic absence epilepsies and broader spectra of IGEs in the presence of family members with JME.
BACKGROUND AND PURPOSE: Although there are various methods of determining neurological prognosis after cardiopulmonary resuscitation, the final outcome of patients often remains unclear for quite a long time. METHODS: We investigated 30 consecutively admitted patients who had been successfully resuscitated by the team of the local mobile intensive care unit after cardiac arrest. Determinations of the period of anoxia and of the cardiopulmonary resuscitation time, clinical investigation, echocardiography, electroencephalography, evoked potentials, magnetic resonance imaging, and magnetic resonance spectroscopy were performed. RESULTS: Demonstration of brain lactate in proton magnetic resonance spectroscopy (P < .01) and absent N20 waves in short-latency somatosensory evoked potentials (P < .01) proved to be significant in terms of a poor prognosis. Correlations between both duration of anoxia and cardiopulmonary resuscitation time and neurological outcome could be shown as well (both P < .05). CONCLUSIONS: Proton magnetic resonance spectroscopy and short-latency evoked potentials are of great benefit in the prognostic evaluation after cardiopulmonary resuscitation.
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We report a single-case study of a patient suffering from Marchiafava-Bignami disease (MBD), a rarely intra vitam diagnosed syndrome with focal demyelination in the corpus callosum, which is usually found in patients with chronic alcohol abuse. Our study explored a variety of motor, sensory and visual functions. Striking features were left apraxia caused by two lesions in the rostral area, and left hemidyslexia related to a large splenial lesion demonstrated by MRI. As symptoms of disconnection may remain undetected clinically, MRI is a most useful tool for the diagnosis of MBD.
Neurological manifestations of diabetes mellitus comprise structural changes in both central and peripheral nervous system, as well as functional cerebral disturbances. Macro- and microangiopathy are responsible for the development of cerebrovascular diseases; microangiopathic and metabolic disturbances are considered in the discussion to be responsible for the development of the various forms of diabetic polyneuropathy. Acute metabolic episodes with progressive impairment of consciousness have to be differentiated from structural neurological disorders. As a further point of interest the association between diabetes mellitus and special neurological diseases is mentioned.
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