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Jan Kassubek

Publications and source records attributed to Jan Kassubek.

14 recordsLinked to original sources
PubMed2003

Quantification of brain atrophy in patients with myotonic dystrophy and proximal myotonic myopathy: a controlled 3-dimensional magnetic resonance imaging study.

Myotonic dystrophy (DM1) and proximal myotonic myopathy (PROMM or DM2) are two distinct muscular disorders with multisystemic involvement. Both have previously been reported to be associated with cognitive impairment and white matter lesions detected by cerebral magnetic resonance imaging (MRI). In this study, the extent of brain atrophy was investigated in vivo in ten DM1 and nine PROMM patients in comparison to age-matched healthy controls for each group. The diagnosis was confirmed by DNA analysis of all patients. As a quantitative marker, the ratio of brain parenchymal to intracranial volume, called brain parenchymal fraction (BPF), was calculated from 3-dimensional MRI data using an automated analysis technique. Compared to age-matched healthy controls (mean BPF 0.852 +/- 0.032), the BPF in DM1 patients (0.713 +/- 0.031) was highly significantly decreased (P < 0.001). In contrast, the PROMM patients (mean BPF 0.792 +/- 0.029) showed only slightly decreased BPF values (P < 0.05). BPF was not significantly correlated to any of the clinical or genetic parameters in both diseases (disease duration, motor score, educational level, and number of CTG repeats in the expanded allele). In summary, global brain atrophy was demonstrated to occur in both diseases, but was more severely manifestated in DM1 patients.

Adolescent↗
PubMed2003

Activation of a residual cortical network during painful stimulation in long-term postanoxic vegetative state: a 15O-H2O PET study.

Survivors of prolonged cerebral anoxia often remain in the persistent vegetative state (PVS). In this study, long-term PVS patients were investigated by 15O-H(2)O PET to analyze their central processing of pain. The study was approved by the local Ethics Committee, the experiments were performed in accordance with the Helsinki Declaration of 2000. Seven patients remaining in PVS of anoxic origin for a mean of 1.6 years (range 0.25-4 years) were investigated. We performed functional PET of the brain using 15O-labelled water during electrical nociceptive stimulation. Additionally, a brain metabolism study using 18F-fluorodeoxyglucose (FDG) PET and multi-sequence MRI (including a 3-D data set) were acquired in all patients. PET data were analyzed by means of Statistical Parametric Mapping (SPM99) and coregistered to a study-specific brain template. MRI and FDG PET showed severe cortical impairment at the structural and the functional level, that is, general atrophy of various degrees and a widespread significant hypometabolism, respectively. Pain-induced activation (hyperperfusion) was found in the posterior insula/secondary somatosensory cortex (SII), postcentral gyrus/primary somatosensory cortex (SI), and the cingulate cortex contralateral to the stimulus and in the posterior insula ipsilateral to the stimulus (P<0.05, small-volume-corrected). No additional areas of the complex pain-processing matrix were significantly activated. In conclusion, the regional activity found at the cortical level indicates that a residual pain-related cerebral network remains active in long-term PVS patients.

Adult↗
PubMed2003

Age-related brain parenchymal fraction is significantly decreased in young multiple sclerosis patients: a quantitative MRI study.

The extent of brain atrophy was determined in 33 patients with multiple sclerosis (MS) and in 60 healthy subjects (21-76 years) by calculating brain parenchymal fractions (BPF, the ratio of brain parenchymal to intracranial volume) from 3D MRI. Within the normal data base, subjects at higher ages showed significantly lower BPF values. In younger MS patients, BPF was significantly decreased compared with age-matched controls (20-29 years, p= 0.0022; 30-39 years, =p 0.0001; 40-49 years,p = 0.0444) and was significantly correlated with disease duration and disease severity, but not with the number of detectable MS lesions. Determination of age-related BPF demonstrated significant brain atrophy in early MS and can be considered as a useful biological marker for monitoring MS.

Adult↗
PubMed2002

Cerebral regional hypometabolism caused by propofol-induced sedation in children with severe myoclonic epilepsy: a study using fluorodeoxyglucose positron emission tomography and statistical parametric mapping.

Cerebral positron emission tomography (PET) in children often requires sedation. This study evaluated sedation-associated effects on cerebral glucose metabolism in 30 children with severe myoclonic epilepsy as investigated by cerebral (18)F-fluorodeoxyglucose (FDG)-PET. Prior to the PET acquisition, 24 children underwent propofol sedation. Pixel-based t-statistics were calculated using statistical parametric mapping (SPM99) for comparisons of the patients' PET scans with both a healthy adult control group and an age-matched child intra-group control. In both analyses, statistically significant hypometabolic areas were found in the medial parieto-occipital cortex bilaterally, including the lingual gyrus, cuneus, posterior cingulate and middle occipital gyrus in all sedated children. All these localizations correlated in a covariate analysis with the injected dose of propofol (P<0.01, corrected). The bilateral parieto-occipital hypometabolism is likely to be a sedation-specific effect and should be taken into account when evaluating cerebral FDG-PET scans in sedated children.

Adolescent↗
PubMed2002

[Clinical symptomatology, diagnostics, and treatment in patients with tuberculous meningoencephalitis].

BACKGROUND: Tuberculous meningoencephalitis (TBM) is still associated with a high mortality. The relative rareness of TBM in Western European countries and the accompanying heterogeneous and unspecific clinical symptoms often result in a delayed diagnosis. PATIENTS AND METHODS: We present six HIV-negative patients (age 37-72 years) with a laboratory-confirmed or clinically probable diagnosis of TBM. The diagnosis could be confirmed in three patients by culture of the cerebrospinal fluid (CSF), in one patient by positive tracheal aspirate culture. In the cases with probable TBM, the diagnosis was confirmed by the combination of clinical symptoms, CSF analysis, and magnetic resonance imaging (MRI). DISCUSSION: The diagnostic and therapeutic problems in TBM are discussed. Moreover, the neurologic complications are presented which developed in all patients during the clinical course despite immediate antituberculous therapy.

Adult↗
PubMed2002

Unusually quick resorption of an intracerebral hemorrhage in congenital afibrinogenemia.

Neuroimaging diagnostics of cerebral hemorrhage in congenitally afibrinogenic patients may be compromised by different pitfalls. We describe the case of a 28-year-old patient with the diagnosis of congenital afibrinogenemia who suffered a large intracerebral hemorrhage. Initial cerebral computed tomography showed typical bleeding, but follow-up cerebral CT scans 1 and 2 weeks later revealed an unusually quick and subtotal resorption of hemorrhage with only a small hypodense lesion within the former bleeding area left. Imaging findings and differential diagnosis are discussed with respect to previous reports about intracerebral hemorrhage in afibrinogenemia.

Adult↗
PubMed2002

Thalamic gray matter changes in unilateral Parkinsonian resting tremor: a voxel-based morphometric analysis of 3-dimensional magnetic resonance imaging.

The thalamus is assumed to be involved in the generation of Parkinsonian tremor. Ten patients with tremor-dominant idiopathic Parkinson's disease (IPD) and strictly unilateral resting tremor were investigated by cerebral high-resolution 3-dimensional magnetic resonance imaging (MRI). MRI data were analyzed by an observer-independent morphometric technique, voxel-based morphometry (VBM). For VBM, MRI data were automatically normalized and segmented, then gray matter volumes were analyzed on a voxel-by-voxel basis in comparison to an age-matched control group using Statistical Parametric Mapping (SPM99). Highly significant structural changes, i.e. locally increased gray matter concentrations (P<0.001), were found in the nucleus ventralis intermedius (VIM) of the thalamus contralateral to the tremor side and were significantly covariant with tremor amplitudes. On the one hand, these changes were localized in close vicinity to a thalamic focal hypermetabolism as revealed by a previous positron emission tomography study in unilateral Parkinsonian tremor patients. On the other hand, the localization of the focal structural changes in VIM corresponds with the generally accepted target area of tremor surgery in IPD.

Aged↗
PubMed2002

SMART-PET: multimodality white matter imaging and display without loss of quantitative information.

PURPOSE: To improve analysis of cerebral white matter (WM) in fluoro-deoxy-glucose positron emission tomography (PET) images. MATERIALS AND METHODS: A multimodality analysis technique (segmented MRI and registered Talairach-transformed PET [SMART-PET]) was used for quantitative assessment of WM metabolism. Data processing included Talairach transformation of three-dimensional magnetic resonance imaging (MRI) and subsequent automated segmentation and coregistration to normalized PET images. Color model transformations were used for combined display: the hue saturation value color model was regarded as a three-dimensional data matrix, integrating quantitative voxel data of both modalities. The technique was applied in normal subjects and in patients suffering from different WM diseases. Regional analysis was performed to classify metabolic impairment on a five-point scale. RESULTS: Using SMART-PET, a considerable gain in image contrast for WM was achieved in all cases. In the normal subjects, WM metabolism was shown to be homogeneously unimpaired. Sum scores of regional analysis revealed metabolic WM changes in all patients. Extent of WM hypometabolism exceeded the extent of the lesions as delineated by MRI signal changes. CONCLUSION: The potential of the method for further elucidation of the role of WM diseases in brain dysfunction in patients is discussed.

Brain↗
PubMed2002

Transcranial color-coded sonography in basilar artery stenting.

Basilar artery (BA) stenting is an emerging technique with promising results in revascularization of severe arteriosclerotic stenoses. Because of the limitations associated with other noninvasive techniques, we applied transcranial color-coded sonography (TCCS) in the follow-up. Successful stent application in two cases of acute basilar occlusion caused by thrombosis on pre-existing severe midbasilar stenoses was initially confirmed by angiography. Later recanalization was controlled noninvasively by magnetic resonance angiography (MRA), computed tomographic angiography (CTA) and TCCS. Ultrasound (US) was performed through the transtemporal bone window; a contrast-enhancing agent had to be used in one case. TCCS showed an orthograde flow in the distal BA in both patients without signs of severe stenoses. Transtemporal TCCS was demonstrated to be a noninvasive bedside technique in monitoring the patency of the BA after stenting.

Basilar Artery↗
PubMed2002

Spatial imagery in deductive reasoning: a functional MRI study.

Various cognitive theories aim to explain human deductive reasoning: (1) mental logic theories claim syntactic language-based proofs of derivation, (2) the mental model theory proposes cognitive processes of constructing and manipulating spatially organized mental models, and (3) imagery theories postulate that such abilities are based on visual mental images. To explore the neural substrates of human deductive reasoning, we examined BOLD (blood oxygen level dependent) contrasts of twelve healthy participants during relational and conditional reasoning with whole-brain functional magnetic resonance imaging (fMRI). The results indicate that, in the absence of any correlated visual input, reasoning activated an occipitoparietal-frontal network, including parts of the prefrontal cortex (Brodmann's area, BA, 6, 9) and the cingulate gyrus (BA 32), the superior and inferior parietal cortex (BA 7, 40), the precuneus (BA 7), and the visual association cortex (BA 19). In the discussion, we first focus on the activated occipito-parietal pathway that is well known to be involved in spatial perception and spatial working memory. Second, we briefly relate the activation in the prefrontal cortical areas and in the anterior cingulate gyrus to other imaging studies on higher cognitive functions. Finally, we draw some general conclusions and argue that reasoners envisage and inspect spatially organized mental models to solve deductive inference problems.

Adult↗
PubMed2002

On the role of quantitative brain imaging in the differential diagnosis of speech disorders.

We present the case of a 71-year-old woman with an 11-year history of slowly progressive decline of motor speech. Normal clinical investigations including routine magnetic resonance imaging (MRI) at 7, 8 and 10 years after the onset of speech dysfunction led to the suggestion of a psychogenic disorder. Extensive clinical, neuropsychological and neuroimaging investigations including 18F-desoxyglucose-positron emission tomography (PET), quantitative MRI and MR spectroscopy were performed to look for subtle brain pathology. Quantitative assessment of 3D-MRI, F-desoxyglucose-PET and magnetic resonance spectroscopy all demonstrated clear evidence of multifocal frontotemporal brain pathology that had not been picked up on routine MRI investigations on previous admissions. This is the longest benign history of slowly progressive anarthria reported so far. It demonstrates a possible role of quantitative neuroimaging techniques in the diagnosis of complex neuropsychiatric disorders.

Aged↗
PubMed2002

Detection and localization of focal cortical dysplasia by voxel-based 3-D MRI analysis.

PURPOSE: Focal cortical dysplasia (FCD) is a frequent cause of partial epilepsy. Its diagnosis by visual evaluation of magnetic resonance images (MRIs) remains difficult. The purpose of this study was to apply a novel automated and observer-independent voxel-based technique for the analysis of 3-dimensional (3-D) MRI to detect and localize FCD. METHODS: The technique was based on algorithms of the SPM99 software and included the spatial normalization of 3-D MRI data sets to a common stereotaxic space and the segmentation of cortical grey matter. The resulting data sets represented grey-matter density maps where each voxel encoded the grey-matter concentration at the corresponding position in the original MRI. A normal database was set up by calculating and averaging the grey-matter density maps of 30 healthy volunteers. The MRI data sets of seven epilepsy patients with FCD were evaluated retrospectively for dysplastic lesions by voxelwise subtraction of the mean grey-matter density map of the normal database and searching automatically for local and global maxima in the resulting data set. RESULTS: In all patients, the results of voxel-based 3-D MRI analysis corresponded both to the location of the dysplastic lesions in conventional MRI and to seizure semiology and EEG findings. In one case, surgery was performed, and the diagnosis FCD was supported by histology. CONCLUSIONS: The technique of voxel-based 3-D MRI analysis and comparison with a normal database seems to provide a valuable additional screening tool for the detection of FCD.

Adolescent↗