No abstract

The aim of this study was to investigate cardiac involvement in patients with idiopathic inflammatory myopathies excluding inclusion body myositis with cardiac magnetic resonance tomography (CMR). A case series of 53 patients with polymyositis, dermatomyositis, or non-specific myositis underwent CMR including functional imaging, T1-weighted, and late gadolinium enhancement (LGE) imaging. T1-weighted and LGE images were analyzed for myocardial enhancement. Reduced left ventricular function (LVF) was found in 9 (7%) patients. Patients with reduced LVF more often presented with early and late myocardial enhancement (p = 0.014 and p = 0.001). In 33 (62.3%) patients, LGE was observed by CMR. These patients had significantly lower left ventricular ejection fractions (p < 0.001) compared to patients without LGE. LGE was mainly present in the lateral (p < 0.01) and inferior (p < 0.02) segments. No correlations of LGE presence or reduced LVF to cardiovascular risk factors were found. Myocardial inflammation is very frequent in polymyositis, dermatomyositis, and non-specific myositis. In our patient, cohort CMR demonstrated signs of myocardial inflammation in 62.3%. CMR seems to offer a measurable and quantifiable diagnostic tool for cardiac involvement of idiopathic inflammatory myopathies and can thus be used to monitor disease progress and therapeutic success in these patients.

Ischemic stroke in patients with cancer is thought to be associated with a worse prognosis and might be the initial symptom of an unknown malignancy. However, diagnostic algorithms to reliably identify cancer-associated stroke have not been developed. In this retrospective single-centre analysis, 68 patients with ischemic stroke and an active solid malignancy were identified. Neurological assessment and outcome, cardiovascular risk factors, neuroimaging studies as well as laboratory findings were compared to 68 age- and sex-matched control subjects with ischemic stroke without diagnosis of cancer. Lung, pancreatic and renal cancer showed increased prevalences compared to those of the general population in Germany. Diagnosis of cancer was most often made within the 12 months preceding (32.4%) or during the diagnostic work-up for stroke (17.7%). Cancer-associated stroke was characterized by a more severe clinical deficit, frequent clinical deterioration (13.2 vs. 1.5%) or death (25 vs. 4.4%). Ischemic lesions often involved multiple territories (51.6 vs. 12.7%), more often with co-existing subacute and acute infarctions in imaging studies (54.8 vs. 11.1%). Patients with cancer had significantly higher levels of C-reactive protein, relative granulocytosis and serum lactate dehydrogenase activity. Using receiver operating characteristics-based multiple analysis, we developed a model using these parameters which detected cancer-associated stroke with a sensitivity of 75% and specificity of 95%. Our analysis suggests that a multiple algorithm combining the number of territories involved and laboratory signs of inflammation and cell turnover might identify patients with stroke suffering from previously unknown malignancy.

In amyotrophic lateral sclerosis (ALS), cognition is affected. Cortical atrophy in frontal and temporal areas has been associated with the cognitive profile of patients. Additionally, reduced metabolic turnover and regional cerebral blood flow in frontal areas indicative of reduced neural activity have been reported for ALS. We hypothesize that functional connectivity in non-task associated functional default mode network (DMN) is associated with cognitive profile and white matter integrity. This study focused on specific cognitive tasks known to be impaired in ALS such as verbal fluency and attention, and the relationship with functional connectivity in the DMN and white matter integrity. Nine patients and 11 controls were measured with an extensive neuropsychological battery. Resting-state functional magnetic resonance imaging (fMRI) and diffusion tensor imaging (DTI) data were acquired. Results showed that ALS patients performed significantly worse in attention and verbal fluency task. Patients showed increased functional connectivity in parahippocampal and parietal areas of the non-task associated DMN compared to controls. The more pronounced the cognitive deficits, the stronger the increase in functional connectivity in those areas. White matter integrity was reduced in frontal areas in the patients. In conclusion, increased connectivity in the DMN in parahippocampal and parietal areas might represent recruitment of accessory brain regions to compensate for dysfunctional frontal networks.

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.