ObjectivesCentral nervous system involvement is one important clinical aspect of myotonic dystrophy type 1 and 2 (DM1 and DM2). We assessed CNS involvement DM1 and DM2 by 3T MRI and correlated clinical and neuocognitive symptoms with brain volumetry and voxel-based morphometry (VBM).Methods12 patients with juvenile or classical DM1 and 16 adult DM2 patients underwent 3T MRI, a thorough neurological and neuropsychological examination and scoring of depression and daytime sleepiness. Volumes of brain, ventricles, cerebellum, brainstem, cervical cord, lesion load and VBM results of the patient groups were compared to 33 matched healthy subjects.ResultsClinical symptoms were depression (more pronounced in DM2), excessive daytime sleepiness (more pronounced in DM1), reduced attention and flexibility of thinking, and deficits of short-term memory and visuo-spatial abilities in both patient groups. Both groups showed ventricular enlargement and supratentorial GM and WM atrophy, with prevalence for more GM atrophy and involvement of the motor system in DM1 and more WM reduction and affection of limbic structures in DM2. White matter was reduced in DM1 in the splenium of the corpus callosum and in left-hemispheric WM adjacent to the pre- and post-central gyrus. In DM2, the bilateral cingulate gyrus and subgyral medio-frontal and primary somato-sensory WM was affected.Significant structural-functional correlations of morphological MRI findings (global volumetry and VBM) with clinical findings were found for reduced flexibility of thinking and atrophy of the left secondary visual cortex in DM1 and of distinct subcortical brain structures in DM2. In DM2, depression was associated with brainstem atrophy, Daytime sleepiness correlated with volume decrease in the middle cerebellar peduncles, pons/midbrain and the right medio-frontal cortex.ConclusionGM and WM atrophy was significant in DM1 and DM2. Specific functional-structural associations related morphological changes to cognitive impairment, depression and daytime sleepiness, partly indicating involvement of complex neuronal networks.

The authors' first results show that tumor resections with 5-ALA in combination with intraoperative cortical stimulation have the advantages of both methods and, thus, provide additional safety for the neurosurgeon during resections of primary malignant brain tumors in eloquent areas. Nonetheless, more cases and additional studies are necessary to further prove the advantages of this multimodal strategy.

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We combined diffusion tensor imaging (DTI) measures of the corpus callosum (CC) and the superior longitudinal fascicle (SLF) with calculation of brain atrophy in 53 patients with relapsing-remitting multiple sclerosis (MS) and 15 healthy controls, to analyze their interrelation and their correlation with disease duration and clinical impairment. The lateral ventricle volume in MS patients was increased; the fractional anisotropy in the CC was decreased as was the fiber volume. Perpendicular (in the literature also referred to as radial) diffusivity (ped), which reflects the diffusion perpendicular to the long axis of the axons within the fiber bundle, was increased in the SLF and the posterior CC, but contrary to our predictions, parallel (also called axial) diffusivity (pad) that refers to the amount of diffusion in the direction of the axon was increased, too. Brain atrophy and DTI-derived parameters were highly intercorrelated and both correlated with disease duration. Discriminant analysis showed that DTI-derived atrophy measures are superior to brain atrophy measures in classifying patients and controls. In light of our results, animal studies focusing on demyelination and axonal loss are reinterpreted.

The California Verbal Learning Test (CVLT) is recognized as a standard clinical tool for assessing episodic memory difficulties in multiple sclerosis (MS), but its neural correlates have not yet been examined in detail in this patient population. We combined neuropsychological examination and diffusion tensor imaging (DTI) analysis in a group of MS patients (N = 50) and demographically matched healthy participants (N = 20). We investigated the degree of impairment of the uncinate fascicle (UF), the superior longitudinal fascicle (SLF), the fornix (FX) and the cingulum (CG). The patients were impaired on all CVLT parameters and the DTI parameters correlated moderately with disease-related variables. Regression analyses in the complete study sample showed that CVLT learning scores correlated with impairment of the right UF. This association reached marginal significance in the patient sample. In contrast to other studies claiming retrieval deficits, our results suggest that encoding and consolidation deficits may play a major role in verbal memory impairments in MS. The findings also provide evidence for an association between degree of myelination of prefrontal fibre pathways and encoding efficiency. Finally, DTI-derived measurements appear to reflect disease progression in MS. The results are discussed in light of functional MRI studies investigating compensatory brain activity during cognitive processing in MS.

BackgroundFatigue is a disabling syndrome in multiple sclerosis (MS), which may be associated with inflammation and faster disease progression.ObjectiveTo analyze the significance of cognitive fatigue for subsequent disease progression.MethodWe followed 46 MS patients and 14 healthy controls in a study over 17 months. At the beginning (t1) and at the end (t2) of the study participants scored their fatigue, performed the Multiple Sclerosis Functional Composite and received MRI scanning, encompassing MPR T1, FLAIR, and DTI sequences. At t1, MS patients were divided into those with and those without cognitive fatigue (cut-off score for moderate cognitive fatigue of the Fatigue Scale for Motor and Cognition). We calculated ANCOVAs for repeated measurement to analyze the relevance of cognitive fatigue status for the number of relapses and for MRI parameters.ResultsAt t1, but not at t2, patients with cognitive fatigue showed increased axial and radial diffusivity of corpus callosum fibers. At t2, these patients showed significantly more loss of brain parenchyma and greater enlargement of lateral ventricles. Moreover, they developed more relapses, but there was no difference in lesion load or in performance deterioration. Additional analyses showed that only cognitive fatigue but not a more general score for fatigue (Fatigue Severity Scale) had an impact on the worsening of the disease status.ConclusionPatients with cognitive fatigue may develop more brain atrophy and relapses during the next 17 months than patients without cognitive fatigue. Hence, experiencing cognitive fatigue might indicate more aggressive inflammatory processes and subsequent neurodegeneration.

We evaluated a rehabilitation programme for executive deficits in multiple sclerosis patients by comparing outcome scores of a cognitive intervention group (CIG; n = 11) with those of a placebo group (n = 14) and an untreated group (n = 15). Executive functioning and verbal learning improved significantly more in the CIG. The treatment effect on verbal learning was still present at 1-year follow-up. Baseline brain atrophy, quantified by the brain parenchymal fraction, was associated with treatment effects for one aspect of executive functioning. Consequently, cognitive intervention may be beneficial and baseline brain atrophy has some predictive value in determining treatment outcome for executive functioning.

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