Lancet neurology
-
In patients with multiple sclerosis (MS), grey matter damage is widespread and might underlie many of the clinical symptoms, especially cognitive impairment. This relation between grey matter damage and cognitive impairment has been lent support by findings from clinical and MRI studies. However, many aspects of cognitive impairment in patients with MS still need to be characterised. ⋯ Imaging measures of both lesioned and normal-appearing white matter lend support to the hypothesis of the existence of an underlying disconnection syndrome that causes clinical symptoms to trigger. Findings on cortical reorganisation support the contribution of brain plasticity and cognitive reserve in limiting cognitive deficits. The development of clinical and imaging biomarkers that can monitor disease development and treatment response is crucial to allow early identification of patients with MS who are at risk of cognitive impairment.
-
Review Case Reports
C9orf72 expansions in frontotemporal dementia and amyotrophic lateral sclerosis.
C9orf72 hexanucleotide repeat expansions are the most common cause of familial frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) worldwide. The clinical presentation is often indistinguishable from classic FTD or ALS, although neuropsychiatric symptoms are more prevalent and, for ALS, behavioural and cognitive symptoms occur more frequently. ⋯ Like in many patients with FTD and ALS, neuronal inclusions that contain TARDBP are seen, but are not universal, and the characteristic pathological finding is of dipeptide repeat (DPR) proteins, formed by unconventional repeat-associated non-ATG translation. Possible mechanisms of neurodegeneration include loss of C9orf72 protein and function, RNA toxicity, and toxicity from the DPR proteins, but which of these is the major pathogenic mechanism is not yet certain.
-
Multicenter Study
Development and validation of nomograms to provide individualised predictions of seizure outcomes after epilepsy surgery: a retrospective analysis.
Half of patients who have resective brain surgery for drug-resistant epilepsy have recurrent postoperative seizures. Although several single predictors of seizure outcome have been identified, no validated method incorporates a patient's complex clinical characteristics into an instrument to predict an individual's post-surgery seizure outcome. ⋯ Cleveland Clinic Epilepsy Center.
-
Diabetes mellitus is associated with an increase in the risk of dementia and the proportion of patients who convert from mild cognitive impairment (MCI) to dementia. In addition to MCI and dementia, the stages of diabetes-associated cognitive dysfunction include subtle cognitive changes that are unlikely to affect activities of daily life or diabetes self-management. ⋯ Acknowledgment of diabetes-associated cognitive decrements can help to improve understanding of patients' symptoms and guide management. Future challenges are to establish the importance of screening for cognitive impairment in people with diabetes, to identify those at increased risk of accelerated cognitive decline at an early stage, and to develop effective treatments.