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Biomedical subjects

Jan Kassubek

Publications and source records attributed to Jan Kassubek.

At least 19 recordsLinked to original sources
PubMed2006

Amygdala size in amyotrophic lateral sclerosis without dementia: an in vivo study using MRI volumetry.

BACKGROUND: Evidence for extra-motor involvement in non-demented patients with amyotrophic lateral sclerosis (ALS) has been provided by multiple studies, in particular neuropathological studies have demonstrated neuronal loss in the amygdala. The aim of this study was to investigate possible alterations of amygdala volumes in vivo. METHODS: Twenty-two moderately disabled patients with definite ALS without cognitive or behavioural deficits and 22 age-matched healthy controls were included. Amygdala and total brain volumes were measured by region-of-interest-based volumetry in 3-D MRI. RESULTS: A trend was observed with reduced amygdala size in the ALS group, since mean absolute and brain size-corrected amygdala volumes were 6.9% and 7.6% lower in the patient group compared to those in normal controls (P = 0.086 and P = 0.110), respectively. CONCLUSION: Volumetrically identifiable alterations of the amygdala can be mapped in vivo and may be associated with psychopathological findings in later stages of ALS.

Amygdala↗
PubMed2006

[Rofecoxib-induced psychosis].

Acute psychosis is a possible side effect of many centrally acting drugs. Its appearance is facilitated by certain conditions like advanced age, comedication, or comorbidity. We report two cases of acute psychotic syndromes with visual (Case 1 + 2) and auditory (Case 1) hallucinations under rofecoxib, a cyclooxygenase-2-inhibitor. This is one of the first reports of psychotic disorders under rofecoxib intake. We present a review of the literature on mental side effects of NSAID and on the pharmacological basis of the association of cyclooxygenase-inhibitors and mental disorders.

Aged↗
PubMed2006

Restless legs syndrome in hereditary spastic paraparesis.

This study was designed to investigate the prevalence and determinants of the association of restless legs syndrome (RLS) and hereditary spastic paraparesis (HSP). Therefore, 132 patients with HSP were evaluated concerning the symptoms of RLS by a standardized questionnaire. RLS was supposed when patients met all of the established four essential criteria of RLS defined by the International RLS Study Group. In addition, we studied the relationship between RLS and age at HSP symptom onset and evaluated the severity of RLS symptoms. Out of 59 responses, RLS was found in 27 HSP patients (15 male, 12 female) so that RLS was more frequent in the total HSP group (20.5%; 27/132) than in previous population-based studies (about 11%). In all 27 patients, the diagnosis of RLS was established based on an additional personal interview. The probability to develop RLS did not increase with higher age. Age at onset of HSP symptoms in the HSP group with RLS (27.7 +/- 12.6 years) and the HSP group without RLS (37.0 +/- 16.9 years) differed significantly (p = 0.04). Most of the patients with RLS showed a moderate and severe grade on the RLS severity score. Only 8 patients had previously been diagnosed to have RLS and were on medication. The data of this screening for RLS provided evidence that patients with HSP are particularly susceptible to develop RLS. Consequently, special emphasis should be put on the diagnosis criteria of RLS in HSP patients.

Female↗
PubMed2006

Primary progressive aphasia accompanied by environmental sound agnosia: a neuropsychological, MRI and PET study.

As part of the frontotemporal dementias, primary progressive aphasia (PPA) is typically characterized by nonfluent speech with paraphasias, but there is growing evidence that also a fluent variant of PPA exists. We describe a patient suffering from PPA who adds to the broad clinical spectrum of this disorder. Moreover, we report for the first time that PPA may be associated with severe impairment in meaningful nonverbal sound recognition (environmental sound agnosia). These neuropsychological findings were found to be associated with distinct focal alterations in functional and structural neuroimaging.

Aged↗
PubMed2006

Head of the caudate nucleus is most vulnerable in chorea-acanthocytosis: a voxel-based morphometry study.

Chorea-acanthocytosis (ChAc; OMIM 200150) is a rare autosomal recessive disease with dysfunction of the erythrocyte membrane, presenting with acanthocytes and neurological manifestations characterized by progressive hyperkinesias (chorea, dystonia) and neuropsychological impairment. Damage to the basal ganglia was described previously in neuropathological and neuroimaging investigations. We analyzed high-resolution MRI of six ChAc patients with mutations in the VPS13A gene (median age, 37 years; mean time since clinical onset, 13 years) with respect to regional atrophy by use of the observer-independent technique of voxel-based morphometry in comparison to 15 age-matched healthy controls. Additionally, global brain atrophy was determined using the standardized brain parenchymal fraction (BPF) method. A robust regional reduction of gray matter density was observed in the head of the caudate nucleus bilaterally and was nearly symmetrical (P < 0.001, corrected for small volumes). No additional gray matter changes were found. In the BPF analysis, there was no significant global brain atrophy. The predilection of atrophy in the head of the caudate nucleus, as suggested by our results, argues for a particular vulnerability of this part of the striatum in ChAc and is in agreement with pronounced neuropsychological disturbances that are thought to rely on these regions.

Adult↗
PubMed2006

Neurofilament heavy-chain NfH(SMI35) in cerebrospinal fluid supports the differential diagnosis of Parkinsonian syndromes.

We aimed to evaluate the potential of the cerebrospinal fluid (CSF) axonal damage biomarker NfH(SMI35) in the laboratory-supported differential diagnosis of parkinsonian syndromes. Patients with idiopathic Parkinson's disease (PD; n = 22), multiple-system atrophy (MSA; n = 21), progressive supranuclear palsy (PSP; n = 21), corticobasal degeneration (CBD; n = 6), and age-matched controls (n = 45) were included. CSF levels of NfH(SMI35) were measured using ELISA. Levels of CSF NfH(SMI35) were elevated in PSP compared to PD and controls (P < 0.05 each). They were also significantly higher in MSA than in PD and controls (P < 0.05 each). NfH(SMI35) differentiated PD from PSP with a sensitivity of 76.5% and a specificity of 94.4%. Axonal damage as measured by CSF NfH(SMI35) is most prominent in the more rapidly progressive syndromes PSP and MSA as compared to PD or CBD. CSF NfH(SMI35) may therefore be of some value for the laboratory-supported differential diagnosis of atypical parkinsonian syndromes.

Adult↗
PubMed2006

Grey-matter abnormalities in boys with Tourette syndrome: magnetic resonance imaging study using optimised voxel-based morphometry.

The genesis of Tourette syndrome is still unknown, but a core role for the pathways of cortico-striatal-thalamic-cortical circuitry (CSTC) is supposed. Volume-rendering magnetic resonance imaging data-sets were analysed in 14 boys with Tourette syndrome and 15 age-matched controls using optimised voxel-based morphometry. Locally increased grey-matter volumes (corrected P < 0.001) were found bilaterally in the ventral putamen. Regional decreases in grey matter were observed in the left hippocampal gyrus. This unbiased analysis confirmed an association between striatal abnormalities and Tourette syndrome, and the hippocampal volume alterations indicate an involvement of temporolimbic pathways of the CSTC in the syndrome.

Adolescent↗
PubMed2006

Laryngospasm in neurological diseases.

Laryngospasm is a clinical symptom characterized by involuntary spasms of the laryngeal muscles, which leads to paroxysms of coughing, inspiratory stridor, and sometimes to episodes of complete upper-airway occlusion. Although laryngospasm is a symptom mainly seen in otolaryngeal diseases and in the context of anesthesiological complications, it also occurs in neurological disorders. In this review of the occurrence of laryngospasms in neurological diseases, the clinical symptomatology, additional circumstances, possible underlying causes, and therapeutic options are presented.

Gastroesophageal Reflux↗
PubMed2005

Magnetic resonance investigation of the upper spinal cord in pure and complicated hereditary spastic paraparesis.

Neuropathological studies of hereditary spastic paraparesis (HSP) have described axonal loss involving corticospinal and somatosensory tracts in the spinal cord. This MRI-based study was intended to investigate in vivo diameter alterations of the spinal cord in HSP, including both pure HSP (p-HSP, n = 20) and complicated HSP (c-HSP, n = 10). Standard MRI examinations of the cervical and thoracic spinal cord in HSP patients and a control group (n = 54) were analyzed by standardized spinal cord planimetry. In HSP patients, significant atrophy of the upper spinal cord compared to controls was observed at p < 0.001 both at the cervical and at the thoracic level. Myelon diameters at both levels were also significantly reduced in the two HSP subgroups in an additional comparison with age-matched subgroups of controls each, but p-HSP and c-HSP groups themselves did not differ. Marked atrophy of the upper spinal cord seems to be associated with HSP, assumedly due to the central-distal axonopathy. However, the differences between p-HSP and c-HSP could not be visualized by structural MRI at spinal cord level.

Adult↗
PubMed2005

Safety and therapeutical benefit of hemicraniectomy combined with mild hypothermia in comparison with hemicraniectomy alone in patients with malignant ischemic stroke.

INTRODUCTION: Both for hemicraniectomy and for hypothermia, several reports describe a beneficial effect in patients with malignant supratentorial cerebral ischemia. We compared the safety and the clinical outcome in patients with a malignant supratentorial infarction who were treated with hemicraniectomy alone (HA) or received a combination therapy with hemicraniectomy and hypothermia of 35 degrees C (HH), respectively. METHODS: In a prospective and randomized study, 25 consecutive patients were treated after an ischemic infarction of more than two thirds of one hemisphere by HA (n=13 patients) or the HH combination therapy (n=12 patients). Safety parameters were compared between both treatment groups, the clinical outcome was assessed during treatment and after 6 months. RESULTS: Age, cranial CT or MRI findings, initial National institutes of Health Stroke Scale Score (NIHSSS) and level of consciousness were not significantly different between both groups. Hemicraniectomy was performed within 15+/- 6 h after the ischemic event. Hypothermia was induced immediately after surgery. Overall mortality was 12% (2/13 vs. 1/12 in the two groups), but none of these 3 patients died due to treatment-related complications. There were no severe side effects of hypothermia. Duration of need for intensive care or for mechanical ventilation and infectious status did not differ significantly between both groups, but the need for catecholamine application was increased in the HH group. The clinical outcome showed a tendency for a better outcome in the HH compared with the HA group with respect to status after 6 months, as assessed by the NIHSSS (10+/-1 vs. 11+/-3, p<0.08). DISCUSSION: The present study suggests that a combined therapy of mild hypothermia and hemicraniectomy in malignant brain infarction does not imply additional risks by side effects and improves functional outcome as compared with hemicraniectomy alone.

Adult↗
PubMed2005

Executive dysfunction in early stages of Huntington's disease is associated with striatal and insular atrophy: a neuropsychological and voxel-based morphometric study.

BACKGROUND: Huntington's disease (HD) is characterized by a progressive multisystem neuronal atrophy in the brain. Apart from motor signs, cognitive symptoms, particularly executive dysfunctions, are proposed to be recognizable in early stages of disease. The aim of the present study was to clarify if cognitive dysfunction in early stages of HD is correlated with loco-regional structural changes in 3D-MRI. METHODS: Twenty-five patients with genetically confirmed HD in early clinical stages were included in the study and underwent neuropsychological testing, i.e., the executive tasks Tower of Hanoi (ToH), Stroop Colour Word Interference Test (STROOP), and modified Wisconsin Card Sorting Test (mWCST). High-resolution volume-rendering MRI scans (MP-RAGE) were acquired on a 1.5 T scanner in all patients and were analyzed by statistical parametric mapping and voxel-based morphometry (VBM) in comparison to an age-matched control group. RESULTS: Group analysis of HD patients demonstrated robust regional decreases of gray matter volumes (p<0.05, corrected for multiple comparisons) in the caudate and the putamen bilaterally with a global maximum at Talairach coordinates 11/4/11 (Z-score=7.06). Executive dysfunction was significantly correlated with the areas of highest significant differences out of VBM results which were located bilaterally in the caudate (ToH: r=0.647, p<0.001; STROOP: r=0.503, p<0.01; mWCST: r=0.452, p<0.05). Moreover, subgroup analyses revealed marked insular atrophy (Talairach coordinates 43/-3/1; Z-score=5.64) in HD patients who performed worse in the single executive tasks. CONCLUSION: Two aspects were most remarkable in this correlational study: (i) striatal atrophy in HD patients in early stages plays an important role not only in impaired motor control but also in executive dysfunction, and (ii) extrastriatal cortical areas, i.e., the insular lobe, seem to be involved in executive dysfunction as assessed by neuropsychological tests requiring for planning and problem solving, stimulus response selectivity and concept formation.

Adult↗
PubMed2005

Enhanced visualization of blurred gray-white matter junctions in focal cortical dysplasia by voxel-based 3D MRI analysis.

PURPOSE: Focal cortical dysplasia (FCD), a frequent cause of partial epilepsy, is often associated with blurring of the gray-white matter junction in magnetic resonance images (MRI). To improve the recognition and delineation of FCD we developed a novel voxel-based image post-processing method for enhanced visualization of blurred gray-white matter junctions. METHODS: Using standard algorithms of statistical parametric mapping software (SPM99) a T1-weighted MRI volume data set is normalized and segmented. The distribution of gray and white matter is analyzed on a voxelwise basis and compared with a normal database. Based on this analysis, a three-dimensional feature map is created which highlights brain areas with blurred gray-white matter transition. This method was applied to the MRI data of 25 epilepsy patients with histologically proven FCD. RESULTS: In 18/25 patients the new feature maps clearly showed that the dysplastic lesions were accompanied by blurring of the gray-white matter junction. Combined with a formerly published method of voxel-based 3D MRI analysis, 21/25 FCD lesions were shown to be associated with either blurring or abnormal extension of gray matter beyond the normal cortical ribbon, including four cases with lesions not or incompletely recognized on conventional MRI. CONCLUSIONS: The MRI post-processing presented here improves the visualization of FCD and may increase the diagnostic yield of MRI. Thereby, it provides a valuable additional diagnostic tool in the presurgical evaluation of epilepsy patients.

Adolescent↗
PubMed2005

The cerebral control of speech tempo: opposite relationship between speaking rate and BOLD signal changes at striatal and cerebellar structures.

So far, only sparse data on the cerebral organization of speech motor control are available. In order to further delineate the neural basis of articulatory functions, fMRI measurements were performed during self-paced syllable repetitions at six different frequencies (2-6 Hz). Bilateral hemodynamic main effects, calculated across all syllable rates considered, emerged within sensorimotor cortex, putamen, thalamus and cerebellum. At the level of the caudatum and the anterior insula, activation was found restricted to the left side. The computation of rate-to-response functions of the BOLD signal revealed a negative linear relationship between syllable frequency and response magnitude within the striatum whereas cortical areas and cerebellar hemispheres exhibited an opposite activation pattern. Dysarthric patients with basal ganglia disorders show unimpaired or even accelerated speaking rate whereas, in contrast, cerebellar dysfunctions give rise to slowed speech tempo which does not fall below a rate of about 3 Hz. The observed rate-to-response profiles of the BOLD signal thus might help to elucidate the pathophysiological mechanisms of dysarthric deficits in central motor disorders.

Acoustic Stimulation↗
PubMed2005

Emotional responding in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a fatal disease, leaving the patient in a partially or completely deafferented state. In an explorative study, we investigated responses to visual socio-emotional stimuli in ALS patients. Pictures from the International Affective Picture System (IAPS) were verbally judged by 12 moderately affected ALS patients with a spinal onset and a slow progression and 18 age-matched controls, and data were compared with psychophysiological responses. Verbal emotional judgments of patients were more positive than ratings of controls. Regarding arousal, patients neutralized extreme pictures, in that they rated calm pictures as more exciting than controls and exciting pictures as more calm. These changes of emotional processing were unrelated to depression or frontal lobe dysfunction. There were no major differences between patients and controls concerning physiological responses to emotional stimuli. We conclude that emotional responses of ALS patients tend to be altered towards positive valence and towards a more balanced arousal state in early stages of the disease. These findings contradict assumptions of a generally negative impact of the disease on the emotional disposition and may indicate compensatory cognitive or neuroplastic changes.

Aged↗
PubMed2005

MR-pathologic comparison of the upper spinal cord in different motor neuron diseases.

This MRI study was performed to evaluate in vivo alterations of the spinal cord in defined subgroups of motor neuron diseases. Standard MRI examinations of the cervical and thoracic spinal cord in sporadic amyotrophic lateral sclerosis (ALS; n = 39), sporadic lower motor neuron disease (LMND; n = 19), Kennedy's disease (KD; n = 19) and a control group (n = 96) were analyzed with respect to spinal cord signal changes and the thickness of the spinal cord. No significant changes in thickness or signal alterations were observed when comparing ALS, LMND and control groups with one another. However, in KD patients significant upper spinal cord atrophy was detected at the cervical level as compared with all other groups. At the thoracic level, KD patients had significant upper cord atrophy as compared with controls and LMND. Marked atrophy of the upper spinal cord seems to be a feature of the KD-associated central-peripheral distal axonopathy.

Age of Onset↗