Tuberous sclerosis complex with unilateral perisylvian polymicrogyria and contralateral hippocampal sclerosis - a case report.

Malformations of cortical development (MCD) encompass a wide spectrum of brain disorders. Although rare, the combination of certain MCD may occur. We report a rare case of combination of three distinct cerebral pathologies: tuberous sclerosis, polymicrogyria and hippocampal sclerosis in a patient with intractable epilepsy. Ictal EEG and ictal SPECT suggested right perisylvian area as a seizure onset zone. However, on MRI multiple potentially epileptogenic lesions were identified. The neurobiological background of the coexistence of different types of malformations of cortical development remains enigmatic.

Unilateral polymicrogyria with ipsilateral cerebral hemiatrophy: a distinct syndrome?

INTRODUCTION: There are sporadic reports of unilateral polymicrogyria with ipsilateral hemiatrophic cerebri associated with epilepsy, focal neurological deficit and mental retardation. The mechanisms which cause this condition are not well understood. The aim of our study was to delineate further, clinical and neuroimaging features of this malformation of cortical development and to explore its possible etiological background. PATIENTS AND METHODS: Four patients (two males and two females), aged from 23 to 31 years (mean age range 27.5 years) were evaluated. Subjects underwent clinical, electrophysiological, neuropsychological and high resolution magnetic resonance imaging assessment. RESULTS: No significant perinatal event or exposure to intrauterine infection was noted. None suffered from birth asphyxia or ischemic injury. The parents of two patients were first cousins. Every subject had delayed developmental milestones, mental disability and congenital, non-progressive, spastic hemiparesis. They had epilepsy with seizure-onset ranging from three months to 17 years (mean 6.8 years); two had intractable seizures. In all patients, unilateral, right-sided polymicrogyria was associated with ipsilateral cerebral hemiatrophy. Polymicrogyria involved mainly anterior perisylvian areas; occipital regions were relatively spared. CONCLUSION: The evaluated patients showed homogenous clinical and neuroimaging characteristics. We support the idea that the disorder could constitute a clinical entity with an underlying genetic cause.

Cerebral vasospasm following temporal lobe epilepsy surgery.

OBJECTIVE: Selective amygdalohippocampectomy (AHE) has been associated with postoperative cerebral vasospasm (CVS) in patients with medically intractable temporal lobe epilepsy. The incidence in temporal lobe resection (TLR) is unknown. This retrospective cohort study evaluates the incidence of and risk factors for the development of CVS in patients with TLR and AHE. METHODS: A total of 119 patients were included between 1998 and 2009. All patients were evaluated by standardized preoperative and postoperative transcranial Doppler sonography (TCD) evaluations and neurologic examinations. Postoperative CT scans were evaluated by an independent radiologist and the volume of bleeding within the resection cavity was quantified. RESULTS: Of 107 patients with longitudinal TCD data, 35 (32.7%) developed postoperative CVS. The incidence of CVS did not differ between patients with TLR and AHE. CVS was associated with female gender and a higher bleeding volume in the postoperative CT scan (p = 0.035 and 0.046). Patients with CVS showed a significantly higher incidence of postoperative neurologic signs and symptoms (48.6%) compared to patients without CVS (25%, p = 0.015). The mean length of stay was significantly prolonged in patients with diffuse CVS compared to patients with localized CVS or no CVS (28.8 ± 10.9, 24.2 ± 6.6, and 18.2 ± 6.1 days, p < 0.001). CONCLUSION: CVS is a frequent complication of surgery for temporal lobe epilepsy irrespective of the resection method. Important risk factors for the development of postoperative CVS are female gender and a higher amount of bleeding in the postoperative CT. Patients with CVS more frequently have neurologic signs and symptoms resulting in prolonged hospital stay.

Hippocampal abnormalities in malformations of cortical development: MRI study.

OBJECTIVES: Hippocampal abnormalities may coexist with malformations of cortical development (MCD). This cross-sectional MRI study aimed at categorizing hippocampal abnormalities in a large group of MCD and comparing MCD patients with (group W) and without (group W/O) hippocampal abnormalities. METHODS: Hippocampal anatomy, rotation, size, internal structure, and MRI signal alterations were assessed visually by 3 independent raters in patients with MCD and epilepsy. Four types of hippocampal abnormalities were examined in 220 patients (116 women, mean age 31 +/- 16.6, range 2-76 years): partially infolded/hypoplastic hippocampus (HH), hippocampal sclerosis (HS), malrotated hippocampus (MH), and enlarged hippocampus (EH). The commonest MCD in the cohort were focal cortical dysplasia (27%), polymicrogyria (PMG) (21%), developmental tumors (15%), and periventricular nodular heterotopia (PNH) (14%). RESULTS: Hippocampal abnormalities were seen in 69/220 (31%) patients: HH in 34/69 (49%); HS in 18/69 (26%); MH in 15/69 (22%); and EH in 2/69 (3%). PNH (21/30 [70%]) and PMG (22/47 [47%]) were most commonly associated with hippocampal abnormalities. Compared to the W/O group, patients in the W group had a higher rate of learning disability (W 41/69 [59%] vs W/O 56/151 [37%]; p = 0.003) and delayed developmental milestones (W 36/69 [52%] vs W/O 53/151 [35%]; p = 0.025); groups did not differ otherwise with regard to clinical presentation. HH was associated with symptomatic generalized epilepsies (11/34 [32%]) and high rate of learning disability (27/34 [79%]), neurologic deficits (25/34 [73%]), and delayed developmental milestones (23/34 [68%]). CONCLUSIONS: About a third of patients with malformations of cortical development had hippocampal abnormalities. Patients with hypoplastic hippocampus had the most severe clinical phenotype.

Variability of BOLD response evoked by foot vibrotactile stimulation: influence of vibration amplitude and stimulus waveform.

The aim of the present was study to evaluate cortical and subcortical neural responses on vibrotactile stimulation of the food and to assess somatosensory evoked BOLD responses in dependence of vibration amplitude and stimulus waveform. Sixteen healthy male subjects received vibrotactile stimulation at the sole of the right foot. The vibration stimulus was delivered through a moving magnet actuator system (MMAS). In an event-related design, a series of vibration stimuli with a duration of 1 s and a variable interstimulus interval was presented. Four stimulation conditions were realized using a 2 (amplitudes 0.4 mm or 1.6 mm) x 2 (waveform sinusoidal or amplitude modulated) factorial design. Stimulating with 0.4 mm amplitude compared to 1.6 mm stimulus amplitude more strongly activated the pre- and postcentral gyrus bilaterally and the right inferior, medial and middle frontal gyrus. In the reverse comparison significant differences were observed within the left inferior parietal lobule, the left superior temporal gyrus, and the left temporal transverse gyrus. In the comparison of sinusoidal versus modulated waveform and vice versa no significant activation differences were obtained. The inter-subject variability was high but when all four stimulation conditions were jointly analyzed, a significant activation of S1 was obtained for every single subject. This study demonstrated that the BOLD response is modulated by the amplitude but not by the waveform of vibrotactile stimulation. Despite high inter-individual variability, the stimulation yielded reliable results for S1 on the single-subject level. Therefore, our results suggest that vibrotactile testing could evolve into a clinical tool in functional neuroimaging.

Does caffeine modulate verbal working memory processes? An fMRI study.

To assess the effect of caffeine on the functional MRI signal during a 2-back verbal working memory task, we examined blood oxygenation level-dependent regional brain activity in 15 healthy right-handed males. The subjects, all moderate caffeine consumers, underwent two scanning sessions on a 1.5-T MR-Scanner separated by a 24- to 48-h interval. Each participant received either placebo or 100 mg caffeine 20 min prior to the performance of the working memory task in blinded crossover fashion. The study was implemented as a blocked-design. Analysis was performed using SPM2. In both conditions, the characteristic working memory network of frontoparietal cortical activation including the precuneus and the anterior cingulate could be shown. In comparison to placebo, caffeine caused an increased response in the bilateral medial frontopolar cortex (BA 10), extending to the right anterior cingulate cortex (BA 32). These results suggest that caffeine modulates neuronal activity as evidenced by fMRI signal changes in a network of brain areas associated with executive and attentional functions during working memory processes.

[Functional magnetic resonance imaging before motor cortex stimulation for phantom limb pain]. / fMRT vor Motorkortexstimulation beim Phantomschmerz.

This study deals with the diagnostic value of functional magnetic resonance imaging (fMRI) in a patient with phantom limb pain following traumatic amputation of the right arm. After failure with medication, resection of stump neurinoma, and spinal cord stimulation, fMRI with evidence of cortical reorganization was performed. Tactile stimulation of the perioral region and motor imagery with cranial, tactile stimulation of the stump led to a caudal shift in fMRI activity. Subsequent motor cortex stimulation brought relief from the pain. By detecting cortical reorganization, fMRI contributes to the indication for motor cortex stimulation for phantom pain and aids in electrode positioning.

Learning by strategies and learning by drill--evidence from an fMRI study.

The present fMRI study investigates, first, whether learning new arithmetic operations is reflected by changing cerebral activation patterns, and second, whether different learning methods lead to differential modifications of brain activation. In a controlled design, subjects were trained over a week on two new complex arithmetic operations, one operation trained by the application of back-up strategies, i.e., a sequence of arithmetic operations, the other by drill, i.e., by learning the association between the operands and the result. In the following fMRI session, new untrained items, items trained by strategy and items trained by drill, were assessed using an event-related design. Untrained items as compared to trained showed large bilateral parietal activations, with the focus of activation along the right intraparietal sulcus. Further foci of activation were found in both inferior frontal gyri. The reverse contrast, trained vs. untrained, showed a more focused activation pattern with activation in both angular gyri. As suggested by the specific activation patterns, newly acquired expertise was implemented in previously existing networks of arithmetic processing and memory. Comparisons between drill and strategy conditions suggest that successful retrieval was associated with different brain activation patterns reflecting the underlying learning methods. While the drill condition more strongly activated medial parietal regions extending to the left angular gyrus, the strategy condition was associated to the activation of the precuneus which may be accounted for by visual imagery in memory retrieval.

[Ultrasound contrast agents and prostate cancer]. / Ultraschallkontrastmittel und Prostatakarzinom.

Prostatic carcinoma is the most frequent malignant disease in men and associated with very high mortality. The diagnostic work-up of prostatic carcinoma is based on tests to determine the level of prostate-specific antigen (PSA), digital rectal examination, and transrectal sonography. Due to diagnostic limitations, ultrasound-guided prostate biopsy is the method of choice for diagnosis of prostatic carcinoma. New imaging technologies allow detection of prostatic carcinoma, thus facilitating removal of specific biopsy specimens from these regions. Introduction of ultrasound contrast agents ("echo signal enhancers") significantly increased the diagnostic potential of this method, making it possible to visualize tumor vascularization.

Modulatory effects on human sensorimotor cortex by whole-hand afferent electrical stimulation.

OBJECTIVE: To investigate the effect of electrical stimulation of the nerve afferents of the hand on cortical activity elicited by whole-hand subthreshold stimulation for sensation in healthy human subjects. METHODS: Ten healthy volunteers were studied using BOLD-fMRI with 1) a test motor-task with finger-to-thumb tapping of the left hand, 2) a whole-hand afferent electrical stimulation of the left hand below the sensory level for sensation for 30 minutes, 3) a second fMRI run with the same paradigm as in the test motor-task immediately after electrical stimulation, and 4) a final identical fMRI run 2 hours post-stimulation to test the cortical changes induced by electrical stimulation. Experiments were carried out on a 1.5 T MR scanner and for fMRI echoplanar sequences were used. Data analysis was performed with SPM99. RESULTS: An increase of movement-related responses was seen within the primary motor and primary somatosensory areas of both hemispheres when comparing the test motor-task with the motor-task after electrical stimulation relative to the baseline or sham stimulation. Two hours post-stimulation the modulatory effects of mesh-glove stimulation diminished to baseline level except within the contralateral primary motor region. CONCLUSIONS: The increased BOLD response spatially localized within the sensorimotor cortex reflects an increase in neuronal activity that may provide augmented neuronal excitability.

[The value of ultrasound (US) in the diagnosis of prostate cancer]. / Sonographie des Prostatakarzinoms. Derzeitiger Stand und Zukunftsperspektiven.

The value of ultrasound (US) in the diagnosis of prostate cancer has increased in importance in the past decade, which is mainly related due to the increasing incidence of prostate cancer, the most common malignancy in men. The value of conventional gray-scale US for prostate cancer detection has been extensively investigated. The introduction of US contrast agents has dramatically changed the role of US for prostate cancer detection. Advances in US techniques were introduced to further increase the role of US contrast agents. Although most of these advances in US techniques, which use the interaction of the contrast agent with the transmitted US waves are very sensitive for the detection of microbubbles, are mostly unexplored, in particular for prostate applications. First reports of contrast-enhanced US investigations of blood flow of the prostate have shown that contrast-enhanced US adds important information to the conventional US technique. We present a critical evaluation of the current status of transrectal US imaging for prostate cancer detection. Furthermore, we give background information on US contrast agents and imaging modalities. Early results of contrast-enhanced US suggest the feasibility of the use of US contrast agents to enhance US imaging of the prostate. The application of US contrast agents for the detection and clinical staging of prostate cancer is promising. However, future clinical trials will be needed to determine the promise of contrast-enhanced US of the prostate evolves into clinical application.