Impact of haemoglobin A1c on wound infection in patients with diabetes with implanted synthetic graft.

OBJECTIVE: The aim of this study was to assess the relationship between haemoglobin A1c (HbA1c) values and operative wound infection. METHOD: During the period from 2013-2016, consecutive patients with type 2 diabetes were prospectively evaluated. Data were retrospectively analysed. All included patients were admitted for an elective surgical procedure, requiring the use of prosthetic graft in a groin wound. The patients were divided into two groups according to their preoperative HbA1c values. The main outcome was groin wound infection. The association between preoperative long-term glycoregulation and wound infection was evaluated, as well as the impact of postoperative glycaemic values, regardless of the level of HbA1c. RESULTS: Of the 93 participating patients, wound infection occurred in 20 (21.5%). Wound infection occurred in 28.2% of patients with uncontrolled diabetes (HbA1c >7%) and 16.7% of patients with controlled diabetes (HbA1c <7%); however, the difference did not reach statistical significance (p=0.181). In regression modelling, operative time (p=0.042) was a significant predictor of wound infection, while patients' age (p=0.056) was on the borderline of statistical significance. Females had a higher probability for wound infection (odds ratio (OR): 1.739; 95% confidence interval (CI):0.483-6.265), but there was no statistical significance (p=0.397). Patients with elevated levels of HbA1c had a higher chance of wound infection compared with patients with controlled diabetes (OR: 2.243; 95% CI: 0.749-6.716), nevertheless, this was not statistically significant (p=0.149). CONCLUSION: We found no statistically significant correlation between elevated values of preoperative HbA1c and postoperative groin wound infection.

Carotid Restenosis Rate After Stenting for Primary Lesions Versus Restenosis After Endarterectomy With Creation of Risk Index.

PURPOSE: Carotid artery stenting (CAS) is an option for carotid restenosis (CR) treatment with favorable outcomes. However, CAS has also emerged as an alternative to carotid endarterectomy (CEA) for the management of patients with primary carotid stenosis. This study aimed to report CR rates after CAS was performed in patients with primary lesions versus restenosis after CEA, to identify predictors of CR, and to report both neurological and overall outcomes. MATERIALS AND METHODS: From January 2000 to September 2018, a total of 782 patients were divided into 2 groups: The CAS (prim) group consisted of 440 patients in whom CAS was performed for primary lesions, and the CAS (res) group consisted of 342 patients with CAS due to restenosis after CEA. Indications for CAS were symptomatic stenosis/restenosis >70% and asymptomatic stenosis/restenosis >85%. A color duplex scan (CDS) of carotid arteries was performed 6 months after CAS, after 1 year, and annually afterward. Follow-up ranged from 12 to 88 months, with a mean follow-up of 34.6±18.0 months. RESULTS: There were no differences in terms of CR rate between the patients in the CAS (prim) and CAS (res) groups (8.7% vs 7.2%, χ2=0.691, p=0.406). The overall CR rate was 7.9%, whereas significant CR (>70%) rate needing re-intervention was 5.6%, but there was no difference between patients in the CAS (prim) and CAS (res) groups (6.4% vs 4.7%, p=0.351). Six independent predictors for CR were smoking, associated previous myocardial infarction and angina pectoris, plaque morphology, spasm after CAS, the use of FilterWire or Spider Fx cerebral protection devices, and time after stenting. A carotid restenosis risk index (CRRI) was created based on these predictors and ranged from -7 (minimal risk) to +10 (maximum risk); patients with a score >-4 were at increased risk for CR. There were no differences in terms of neurological and overall morbidity and mortality between the 2 groups. CONCLUSIONS: There was no difference in CR rate after CAS between the patients with primary stenosis and restenosis after CEA. A CRRI score >-4 is a criterion for identifying high-risk patients for post-CAS CR that should be tested in future randomized trials.

Scoring system to predict early carotid restenosis after eversion endarterectomy by analysis of inflammatory markers.

BACKGROUND: Inflammation is one of the mechanisms that leads to carotid restenosis (CR). The aim of this study was to examine the influence of increased values of inflammation markers (high-sensitivity C-reactive protein [hs-CRP], C3 complement, and fibrinogen) on CR development after eversion carotid endarterectomy (CEA). METHODS: A consecutive 300 patients were included in the study, in which eversion CEA was performed between March 1 and August 1, 2010. Demographic data, atherosclerosis risk factors, comorbidities, and ultrasound plaque characteristics were listed in relation to potential risk factors for CR. Serum concentrations of hs-CRP, fibrinogen, and C3 complement were taken just before surgery (6 hours); 48 hours after CEA; and during regular checkups at 1 month, 6 months, 1 year, and 2 years. An "inflammatory score" was also created, which consisted of six predictive values of inflammatory markers (hs-CRP just before and just after CEA, fibrinogen just before and just after CEA, and C3 complement just before and just after CEA) with a maximum score of 6 and a minimum score of 0. At every follow-up visit to the outpatient clinic, ultrasound assessment of the carotid artery for restenosis was done. RESULTS: Our results showed an increased risk of early CR within 1 year in patients with increased hs-CRP before CEA (6 hours) and increased fibrinogen 48 hours after surgery and in patients not taking aspirin after CEA. Sex was determined to be an independent predictor of CR, with female patients having a higher risk (P = .002). Male patients taking aspirin with an inflammatory score >2 had an increased risk for restenosis compared with male patients with inflammatory score <2. Not taking aspirin after CEA and fibrinogen (48 hours) were the strongest predictors, and the Fisher equation incorporating these predictors was used to predict CR. A computer program was created to calculate whether the patient was at high or low risk for CR by selecting whether the patient was taking aspirin (yes or no) and whether fibrinogen was increased 48 hours after CEA (yes or no) and to display the recommended therapeutic algorithm consisting of aspirin, clopidogrel, cilostazol, and statins. CONCLUSIONS: Increased hs-CRP before CEA, increased fibrinogen 48 hours after CEA, and not taking aspirin were the main predictors of early CR. With the clinical implementation of the Fisher equation, it is possible to identify patients at high risk for early CR and to apply an aggressive therapeutic algorithm, finally leading to a decreased CR rate.

VBM-DTI correlates of verbal intelligence: a potential link to Broca's area.

Human brain lesion studies first investigated the biological roots of cognitive functions including language in the late 1800s. Neuroimaging studies have reported correlation findings with general intelligence predominantly in fronto-parietal cortical areas. However, there is still little evidence about the relationship between verbal intelligence and structural properties of the brain. We predicted that verbal performance is related to language regions of Broca's and Wernicke's areas. Verbal intelligence quotient (vIQ) was assessed in 30 healthy young subjects. T1-weighted MRI and diffusion tensor imaging data sets were acquired. Voxel-wise regression analyses were used to correlate fractional anisotropy (FA) and mean diffusivity values with vIQ. Moreover, regression analyses of regional brain volume with vIQ were performed adopting voxel-based morphometry (VBM) and ROI methodology. Our analyses revealed a significant negative correlation between vIQ and FA and a significant positive correlation between vIQ and mean diffusivity in the left-hemispheric Broca's area. VBM regression analyses did not show significant results, whereas a subsequent ROI analysis of Broca's area FA peak cluster demonstrated a positive correlation of gray matter volume and vIQ. These findings suggest that cortical thickness in Broca's area contributes to verbal intelligence. Diffusion parameters predicted gray matter ratio in Broca's area more sensitive than VBM methodology.

White matter abnormalities and their impact on attentional performance in adult attention-deficit/hyperactivity disorder.

Inattention is the most important behavioral feature of adult patients with attention-deficit/hyperactivity disorder (ADHD). Neuroimaging studies in ADHD have demonstrated abnormalities primarily in the frontostriatal circuitry and were mostly conducted in children. We investigated white matter (WM) integrity in adult ADHD patients and the correlation of WM microstructure and neuropsychological parameters in 37 (21 men) never-medicated adult ADHD patients and 34 age- and gender-matched healthy controls. All subjects underwent clinical interviews, rating scales, and neuropsychological tests of attentional performance. Diffusion tensor imaging (DTI) was acquired, and 12 WM regions-of-interest (ROIs) within the attentional network were chosen. Group differences of mean fractional anisotropy (FA) and mean diffusivity (MD) values were calculated for each ROI, and patients' DTI measures were then correlated with measures of attentional performance. FA values in ADHD patients were significantly reduced in the left inferior longitudinal fasciculus (ILF), while MD values were significantly increased in ADHD patients in the frontal portion of the left frontooccipital fasciculus (IFO). In ADHD patients, MD values were negatively correlated with attentional performance in the left ILF. Our findings provide further support for disturbed frontostriatal structural connectivity and also point to an involvement of the left temporal white matter with an impact on attentional performance.

Broad disruption of brain white matter microstructure and relationship with neuropsychological performance in male patients with severe alcohol dependence.

AIMS: In the last years, refined magnetic resonance diffusion tensor imaging (DTI) methods have become available to study microstructural alterations in the human brain. We investigated to what extent white matter tissue abnormalities are present in male patients after chronic, excessive alcohol consumption and if these alterations are correlated with measures of alcohol consumption and neuropsychological performance. METHODS: Twenty-four detoxified adult male patients with severe alcohol dependence and 23 healthy male control subjects were included in the study. Neuropsychological tests were assessed for executive function, attention, memory and visuospatial function. DTI was acquired and preprocessing of the data was performed using tract-based spatial statistics. Group differences of fractional anisotropy (FA) as well as correlation analyses with neuropsychological measures and drinking history were calculated. RESULTS: Performance in alcoholic patients was significantly poorer in tests of non-verbal reasoning and attention. In detoxified alcoholic patients, lower FA was primarily found in the body of the corpus callosum, but these findings did not correlate directly with behavioral measures. However, executive and psychomotor performance (Trail-Making Test) correlated significantly with FA in right anterior cingulate and left motor areas. CONCLUSION: These findings provide further evidence for reduced integrity of interhemispheric connections in male patients with severe alcohol dependence, and neurocognitive performance was in part correlated with FA.

Occurence and clinical predictors of spasticity after ischemic stroke.

BACKGROUND AND PURPOSE: There is currently no consensus on (1) the percentage of patients who develop spasticity after ischemic stroke, (2) the relation between spasticity and initial clinical findings after acute stroke, and (3) the impact of spasticity on activities of daily living and health-related quality of life. METHODS: In a prospective cohort study, 301 consecutive patients with clinical signs of central paresis due to a first-ever ischemic stroke were examined in the acute stage and 6 months later. At both times, the degree and pattern of paresis and muscle tone, the Barthel Index, and the EQ-5D score, a standardized instrument of health-related quality of life, were evaluated. Spasticity was assessed on the Modified Ashworth Scale and defined as Modified Ashworth Scale >1 in any of the examined joints. RESULTS: Two hundred eleven patients (70.1%) were reassessed after 6 months. Of these, 42.6% (n=90) had developed spasticity. A more severe degree of spasticity (Modified Ashworth Scale >or=3) was observed in 15.6% of all patients. The prevalence of spasticity did not differ between upper and lower limbs, but in the upper limb muscles, higher degrees of spasticity (Modified Ashworth Scale >or=3) were more frequently (18.9%) observed than in the lower limbs (5.5%). Regression analysis used to test the differences between upper and lower limbs showed that patients with more severe paresis in the proximal and distal limb muscles had a higher risk for developing spasticity (P

Disturbed structural connectivity is related to inattention and impulsivity in adult attention deficit hyperactivity disorder.

Inattention and impulsivity are the most prominent clinical features of attention deficit hyperactivity disorder (ADHD) in adulthood. Structural and functional neuroimaging studies of subjects with ADHD have demonstrated abnormalities in several brain areas, including fronto-striatal and fronto-cerebellar networks. Mostly, these studies were based on volumetric measurements and have been conducted in children. We investigated white matter (WM) integrity and correlation with measures of attention and impulsivity in adult patients with ADHD adopting diffusion tensor imaging (DTI). N = 37 (21 males) never-medicated adult patients with ADHD combined subtype and N = 34 (16 males) healthy controls were investigated. ADHD diagnosis (DSM-IV) was assessed with clinical interviews and rating scales, subjects also underwent a large neuropsychological test battery including tests of attention and impulsivity. DTI was acquired, and group differences of fractional anisotropy (FA) and mean diffusivity (MD) as well as correlation analyses with measures of attentional performance and impulsivity were calculated using voxel-based analyses. In adult patients with ADHD, we found reduced FA as well as higher MD bilaterally in orbitomedial prefrontal WM and in the right anterior cingulate bundle, while elevated FA was present bilaterally in temporal WM structures. Measures of attention were correlated with DTI parameters in the right superior longitudinal fasciculus, whereas measures of impulsivity were correlated with FA in right orbitofrontal fibre tracts. This is the first DTI study demonstrating disturbed structural connectivity of the frontal-striatal circuitry in adult patients with ADHD. Moreover, a direct correlation between WM integrity and measures of attention and impulsivity is shown.

Angiofollicular lymphoid hyperplasia--an unusual cause of axillary artery pseudoaneurysm.

BACKGROUND: Angiofollicular lymphoid hyperplasia (Castleman's disease) is a rare inflammatory condition. Its cause is unknown and it can be both localized and general. Eighty-six percent of lesions are found in the mediastinum or hilum, and 91% are of the hyaline vascular type. Although Castleman's disease is primarily involving the chest and retroperitoneum, it may also involve neck and axilla in 2-4% of cases. In this article, we present a very rare case of Castleman's disease causing axillary artery pseudoaneurysm. METHODS AND RESULTS: A 30-year-old woman patient presented with pulsating tumefaction of the left arm which was 3.5 x 10 cm in size, and became evident 15 days before admission. History revealed that she suffered trauma of the left upper arm in childhood; therefore, it was suspected that tumefaction may be due to a post-traumatic aneurysm. Duplex scan and multislice computed tomography examinations were performed and an axillary artery pseudoaneurysm was diagnosed. Since intraoperatively the tumefaction resembled the tumorous formation but not the aneurysmal wall, specimens were sent for pathohistological analysis. Arterial reconstruction was performed using autologous vein graft. Pathohistological findings showed vascular type of angiofollicular lymphoid hyperplasia. CONCLUSION: Although angiofollicular lymphoid hyperplasia is rarely localized in the axillary area, this disease should also be considered when axillary artery pseudoaneurysm is diagnosed.

Correlation of brain white matter diffusion anisotropy and mean diffusivity with reaction time in an oddball task.

BACKGROUND: Reaction time (RT) is a frequently used measure of information processing speed, but the underlying physiological and anatomical conditions are not yet fully understood. A correlation between measures of white matter (WM) ultrastructural properties and RT is expected--particularly for those WM tracts that are involved in the attentional system of the brain. METHODS: Diffusion tensor imaging data were acquired in 43 unrelated healthy subjects (age: 22.7 +/- 1.8 years), and RT was measured during an attention-requiring visual oddball task in the same scanning session. Voxel-by-voxel and region of interest analyses were performed for the large association tracts. A linear regression model was used to correlate fractional anisotropy (FA) and mean diffusivity (MD) values with mean RT. RESULTS: Our analyses revealed significant positive correlations between RT and MD in several WM association tracts, predominantly in the right hemisphere. To a lesser extent, significant negative correlations were found between RT and FA in right temporal WM. CONCLUSION: These findings suggest that subcortical ultrastructural properties of the dorsal and ventral visual stream are relevant with regard to information processing speed. Furthermore, MD appears to be more sensitive than FA in detecting functionally relevant ultrastructural variations in WM tracts.

Surgical Treatment of Proximal Segmental Occlusion of the Internal Carotid Artery.

PURPOSE: To present the feasibility, safety, and efficacy of carotid endarterectomy in patients with type II internal carotid artery occlusions, including the long-term outcomes. METHODS: From March 2008 to August 2015, 74 consecutive patients (48 men with a mean age of 65.1 ± 8.06 years) underwent carotid endarterectomy because of internal carotid artery (ICA) segmental occlusions. These were verified with preoperative carotid duplex scans (CDS) and CT angiography (CTA). Also, brain CT scanning was performed in all these patients. The indication for treatment was made jointly by a vascular surgeon, neurologist, and an interventional radiologist in a multidisciplinary team (MDT) context. After successful treatment, all the patients were followed-up at 1, 3, 6, and 12 months, then every 6 months thereafter. RESULTS: The most common symptom at presentation was transient ischaemic attack (TIA) in 49 patients (66.2%), followed by stroke in the past six months in the 17 remaining patients (23%). Revascularisation of the ICA with endarterectomy techniques was performed successfully in all the patients with an average clamp time of 11.9 min. All the procedures were performed under general anaesthesia in combination with a superficial cervical block. The early complication rate was 8.1% and included two cardiac events (2.7%) (one rhythm disorder and one acute coronary syndrome), three TIAs (4.1%), and one intracerebral hemorrhage (1.3%). Only one patient with the intracerebral hemorrhage died 5 days after surgery giving a postoperative mortality of 1.3% for this series. During the follow-up period (mean 50.4 ± 31.3 months), the primary patency rates at 1, 3, 5, and 7 years were 98.4%, 94.9%, 92.9%, and 82.9%, respectively. Likewise, the survival rates were 98.7%, 96.8%, 89%, and 77.6%, respectively. Ultrasound Doppler controls during follow-up detected 8 ICA restenoses; however, only 3 of these patients required further endovascular treatment. CONCLUSIONS: Carotid endarterectomy of internal carotid artery (ICA) segmental occlusion is a safe and effective procedure associated with acceptable risk and good long-term results. Therefore, the current guidelines which do not recommend carotid endarterectomy in this patient group should be reassessed, with the requirement for ongoing large-scale randomized controlled trials to compare CEA with best medical therapy in this patient cohort.

Smoking impacts on prefrontal attentional network function in young adult brains.

RATIONALE: There is abundant evidence from clinical and preclinical studies that acute administration of nicotine has beneficial effects on attentional network function in the brain. In contrast, little is known about potentially neurotoxic effects on the attentional network during neurodevelopmentally critical periods, such as during adolescence and early adulthood. METHODS: Using event-related functional MRI (fMRI), we investigated prefrontal attentional network function in young adults (n=15 regular smokers and n=12 never-smokers; age: 22.6+/-1.5 years). Duration of smoking was 6.9+/-2.3 years (range of 2-10). Smokers were allowed to smoke ad libitum before the fMRI scanning was conducted. RESULTS: As expected from literature, prefrontal attentional network activity was significantly reduced in smokers compared to nonsmokers (Z=2.1; P=0.036). In smokers, we found that the history of smoking duration (years) is directly related to the extent of diminished attentional network activity (R=-0.67; P=0.012). CONCLUSIONS: To our best knowledge, the relationship between the duration of smoking history and prefrontal attentional network function has not yet been reported. This finding might suggest that several years of chronic nicotine abuse may be sufficient to exert long-lasting effects on the brain function of adolescents and young adults.

Negative-pressure wound therapy for deep groin vascular infections.

Introduction: Infection of synthetic graft in the groin is a rare but devastating complication. When it occurs, several possibilities of treatment are available. Extra-anatomic reconstruction and in-situ implantation of new, infection resistant grafts are associated with high mortality and morbidity. Therefore, more conservative approach is needed in some cases. Negative-pressure wound therapy is one of the options in treating such patients. Objective: The aim of this study was to assess the outcome for deep groin vascular graft infection treated with negative-pressure wound therapy. Methods: Seventeen patients (19 wounds), treated for Szilagyi grade III groin infections between October 2011 and June 2014, were enrolled into this observational study. Results: Majority of the wounds (11/19) were healed by secondary intention, and the rest of the wounds (8/19) were healed by primary intention after initial negative-pressure wound therapy and graft substitution with silver-coated prostheses or autologous artery/vein implantation. No early mortality was observed. Minor bleeding was observed in one patient. Reinfection was noted in three wounds. Only one graft occlusion was noted. Late mortality was observed in three patients. Conclusion: Negative-pressure wound therapy seems to be safe for groin vascular graft infections and comfortable for both patient and surgeon. However, the rate of persistent infection is high. This technique, in our opinion, can be used as a "bridge" from initial wound debridement to definitive wound management, when good local conditions are achieved for graft substitution, either with new synthetic graft with antimicrobial properties or autologous artery/vein. In selected cases of deep groin infections it can be used as the only therapeutic approach in wound treatment.

Intracardiac extension of the inferior vena cava leiomyosarcoma with Budd-Chiari syndrome presentation: a case report.

INTRODUCTION: Leiomyosarcomas of the inferior vena cava are rare malignant tumors. A limited number of these cases have been described so far. Only few of them have intracardiac propagation and surgery is rarely undertaken for their treatment. CASE OUTLINE: We present a 52-year-old female patient in whom leiomyosarcoma of the inferior vena cava with intracardiac propagation was diagnosed. The patient underwent successful surgical treatment with complete removal of the tumor and direct suture of the inferior vena cava. No additional modalities of therapy were undertaken. CONCLUSION: Surgery, without radiation therapy can be a successful option for the treatment of inferior vena cava leiomyosarcoma with a good short-term result.

Cerebellar speech representation: lesion topography in dysarthria as derived from cerebellar ischemia and functional magnetic resonance imaging.

BACKGROUND: Lesion topography and the pathophysiological background of dysarthria due to focal cerebellar lesions have not yet been fully clarified. OBJECTIVES: To investigate the lesion topography of dysarthria due to cerebellar ischemia and evaluate brainstem functions. DESIGN: Case studies. PATIENTS: Eighteen right-handed patients with sudden-onset dysarthria and cerebellar ischemia with and without brainstem involvement and 19 healthy, right-handed, monolingual, German-speaking volunteers. METHODS: In patients, we used multimodal electrophysiologic techniques to investigate brainstem functions. Functional magnetic resonance imaging (MRI) was performed in the 19 healthy volunteers. Activation tasks consisted of repetitive vertical silent movements of the tongue and lips at a self-paced rhythm. RESULTS: Cerebellar lesions and additional signs of brainstem involvement were observed in 11 patients with posterior inferior cerebellar artery, anterior inferior cerebellar artery, and superior cerebellar artery infarctions, respectively. In all other patients with isolated cerebellar infarction (n = 7), only the superior cerebellar artery territory (6 right-sided, 1 left-sided) was affected, and the common lesion site was the rostral paravermal region of the anterior lobe. Functional MRI in healthy volunteers indicated that the cerebellar representation of the tongue and orofacial muscles corresponds to that of the area involved in patients with cerebellar dysarthria. CONCLUSIONS: The results of this study demonstrate that articulatory movements of the tongue and orofacial muscles are involved in the activation of the rostral paravermal area of the anterior lobe. This location corresponds to the area involved in cerebellar ischemia in patients with dysarthria. Lesions in the upper paravermal area of the right cerebellar hemisphere, the site of coordination of articulatory movements of the tongue and orofacial muscles, may lead to the development of dysarthria that is unrelated to (often concomitant) brainstem infarctions.

Altered effective connectivity in drug free schizophrenic patients.

The present fMRI study aimed to investigate effective connectivity within a cortical-subcortical-cerebellar information processing network in drug free schizophrenic patients while performing a 2-back working memory task. The finding of enhanced thalamo-cortical and cortico-cortical intrahemispheric connectivity could be interpreted as a compensatory increase of neuronal connection strength consistent with a model of cortical inefficiency in schizophrenic patients. Additionally, the result could be integrated into a model of deficient thalamo-cortical filter functions. Conversely, lower interhemispheric connectivity of the frontal and parietal association cortex appears to be the functional correlate of reduced cognitive performance in schizophrenic patients. The study demonstrated the feasibility to model information processing within cognitive networks and provided additional evidence for the concept of cognitive dysmetria in schizophrenia.

Electrophysiological brainstem testing in the diagnosis of reversible brainstem ischemia.

The aim of this study was to evaluate the sensitivity of multimodal electrophysiological brainstem testing in the diagnosis of clinically suspected reversible ischemic deficits of the brainstem compared with diffusion weighted MR imaging. We investigated 158 consecutive patients presenting with signs of acute brainstem dysfunction. Serial electrophysiological brainstem tests including masseter reflex, blink reflex, masseter inhibitory reflex, AEP, MEP, EOG and the oculoauricular phenomenon were applied. In 14 of the 158 patients neurological deficits resolved in less than 24 hours, which was suggestive of a transitory ischemic attack (TIA), 19 patients had brainstem signs for more than 24 hours but less than 1 week, suggestive of a reversible ischemic neurological deficit (RIND). Electrophysiological data indicated acute functional brainstem lesions in 54,5 % of patients with transient clinical brainstem impairment. Lesion detection rate was significantly higher when combining electrophysiological data and MRI (60,4 %) than using acute brainstem abnormalities in diffusion weighted MRI alone (39,4 %). We conclude that diffusion weighted MRI and electrophysiological brainstem testing are complimentary sensitive indicators of acute brainstem lesions in patients with reversible neurological deficits. Correct identification of brainstem ischemia influences the therapeutic regimen and may improve patient outcome.

Emery-Dreiffus muscular dystrophy: MR imaging and spectroscopy in the brain and skeletal muscle.

Emery-Dreifuss muscular dystrophy is a rare disorder characterized by childhood onset of contractures, humeroperoneal muscle atrophy, and cardiac conduction abnormalities. This report presents the cases of two brothers with this dystrophy in whom bilateral hypomyelination of the deep periatrial white matter was noted. In the hypomyelinated regions, a prominent peak centered at 1.5 parts per million was present on short-TE MR spectra likely representing prominence of proteolipids in the macromolecular region. Major peaks (N-acetyl-aspartate, creatine, choline, and myoinositol) were normal. With respect to muscle changes, atrophy of the medial head of the gastrocnemius muscle was noted at MR imaging, and phosphorus spectroscopy of this muscle revealed decreased phosphocreatine and inorganic phosphate peaks.

Characterization of BOLD-fMRI signal during a verbal fluency paradigm in patients with intracerebral tumors affecting the frontal lobe.

Previous studies have indicated that the BOLD-fMRI signal can be modified by tumor processes in close vicinity to functional brain areas. This effect has been investigated primarily for the perirolandic area but there is only a limited number of studies concerning frontal cortical regions. Therefore, the aim of the current study was to characterize BOLD-fMRI signal and activation patterns in patients with frontal brain tumors while performing a verbal fluency task. Six patients (ages 31-56 years) suffering from frontal (5 left sided and 1 right sided) intracerebral tumors were examined with fMRI while performing a verbal fluency task in a blocked paradigm design. Eight healthy volunteers served as the control group. The patients (5 right and 1 left handed) demonstrated left frontal activation which could be clearly located outside the tumor area and adjacent edema with varying degrees of additional right frontal activation. In the predominant left frontal activation cluster, the mean voxel based z-score and cluster size were not statistically different between patients and controls. The present fMRI study is indicating that language related BOLD signal changes in the frontal cortex of patients with tumors close to functional areas were comparable to the signal in normal controls. Additionally, the temporal hemodynamic response characteristic was comparable in both groups. This is an important finding consistent with PET results and corroborates the feasibility of functional mapping approaches in patients with tumors affecting the frontal lobe. Additional studies investigating alterations of the hemodynamic response depending on tumor location and histology are required in order to further elucidate the association between pathophysiology and BOLD fMRI signal.

Anosognosia for hemiparesis after left-sided stroke.

In patients with left-sided lesions, anosognosia for hemiparesis (AHP) seems to be a rare phenomenon. It has been discussed whether this rareness might be due to an inevitable bias due to language dysfunction and whether the left hemisphere's role for our self-awareness of motor actions thus is underestimated. By applying functional magnetic resonance imaging (fMRI) we examined whether patients with AHP following a left hemisphere stroke show a regular, left-sided or a reversed, right-sided lateralization of language functions. Only the former observation would argue for an original role of the left hemisphere in self-awareness about limb function. In a consecutive series of 44 acute left-sided stroke patients, only one patient (=2%) was identified showing AHP. In this case, we could verify by using fMRI that lateralization of AHP and spatial neglect on the one hand and of language functions on the other hand were reversed. The present single case observation thus argues against an original role of the left hemisphere in self-awareness about limb function. We discuss the data in the context of previous observations in the literature.