37 research outputs found

    FDG-PET underscores the key role of the thalamus in frontotemporal lobar degeneration caused by C9ORF72 mutations

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    C9ORF72 mutations are the most common cause of familial frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). MRI studies have investigated structural changes in C9ORF72-associated FTLD (C9FTLD) and provided first insights about a prominent involvement of the thalamus and the cerebellum. Our multicenter, F-18-fluorodeoxyglucose positron-emission tomography study of 22 mutation carriers with FTLD, 22 matched non-carriers with FTLD, and 23 cognitively healthy controls provided valuable insights into functional changes in C9FTLD: compared to non-carriers, mutation carriers showed a significant reduction of glucose metabolism in both thalami, underscoring the key role of the thalamus in C9FTLD. Thalamic metabolism did not correlate with disease severity, duration of disease, or the presence of psychotic symptoms. Against our expectations we could not demonstrate a cerebellar hypometabolism in carriers or non-carriers. Future imaging and neuropathological studies in large patient cohorts are required to further elucidate the central role of the thalamus in C9FTLD

    Postablation asymptomatic cerebral lesions: Long-term follow-up using magnetic resonance imaging

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    BACKGROUND Catheter ablation of atrial fibrillation (AF) is complicated by cerebral emboli resulting in acute ischemia. Recently, cerebral ischemic microlesions have been identified with diffusion-weighted magnet resonance imaging (MRI). OBJECTIVE The clinical course and longer-term characteristics of these lesions are not known and were investigated in this study. METHODS Of 86 patients, 33 (38%) had new asymptomatic cerebral lesions documented on MRI after catheter ablation for AF; 14 of these 33 (42%) underwent repeat MRI at different time intervals (2 weeks to 1 year) during follow-up, and clinical symptoms as well as size and number of residual lesions were documented. RESULTS In postablation cerebral MRI, 50 new lesions were identified (3.6 lesions/patient) in 14 patients. No patient presented any neurological symptoms. Distribution of the lesions was predominantly in the left hemisphere (60%) and the cerebellum (26%); 52% of the lesions were small (10 mm. Follow-up MRI after a median of 3 months revealed 3 residual lesions in 3 of 14 patients corresponding to the large acute postablation lesions (>10 mm). The re-maining 47 of 50 (94%) of the small or medium-sized lesions were not detectable at follow-up evaluation. CONCLUSIONS Most asymptomatic cerebral lesions observed acutely after AF ablation procedures were 2 weeks after ablation. The larger acute lesions produced chronic glial scars. Neither chronic nor acute lesions were associated with neurological symptoms
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