Proceedings of the Association of British Neurologists and the British Neuropsychiatry Association, Royal College of Physicians, 2-4 October 2002
2003
Journal of Neurology, Neurosurgery and Psychiatry
The present syndromic variants of frontotemporal lobar degeneration (FTLD)-frontotemporal dementia, progressive non-fluent aphasia, semantic dementia-reflect focal pathological damage to the frontal lobes or to the left temporal lobe. This study aims to identify the clinical correlates arising from focal degeneration of the right temporal lobe. Methods: Ten patients with focal right temporal lobe atrophy (RTLA) were identified on the basis of the MRI appearances. The associated clinical,
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... ychological, and behavioural features are documented. Volumetric MRI analysis of the whole temporal lobe, hippocampus, and amygdala are compared with data obtained on patients with focal left temporal lobe atrophy (LTLA) who present with semantic dementia (SD) and with controls. Results: The most frequently identified symptoms associated with RTLA patients were: impairment of episodic memory (90%), impairment of face recognition (60%), getting lost (70%), disinhibition (70%), and change in dietary preference (50%). By contrast, patients with LTLA typically exhibited preservation of episodic memory until later in the disease and did not get lost. Other symptoms observed in RTLA patients were: depression (30%), aggressive behaviour (30%), hyperreligiosity (20%), somatisation disorders (20%), and visual hallucinations (20%). In RTLA patients the mean whole right temporal lobe volume was 62% of the control value. A similar reduction was observed in the volume of the right hippocampus (mean volume 61% of normal) but there was more severe atrophy of the right amygdala (55%). Lesser atrophy of the left hippocampus (90% of normal) and left amygdala (72%) were also observed. This pattern in RTLA patients of asymmetric, predominantly right sided, temporal lobe atrophy with disproportionately severe amygdala involvement represented the mirror image of the pattern of temporal lobe atrophy observed in SD patients. Discussion: Patients with right temporal lobe atrophy exhibit clinical features that differ from those that characterise the current syndromic variants of FTLD. The disproportionate severity of right amygdala atrophy may underpin some of the behavioural features associated with RTLA atrophy. These findings will help to define a "right temporal" variant of FTLD. Background: Previous work used CSF volume as a marker for brain atrophy in dementing diseases, and showed that simple measures of CSF volume in 12 regions within the skull could provide diagnostic information. Here we hypothesise that separating cortical from ventricular CSF will increase the diagnostic capability of these measurements, since we assume that increases in cortical and ventricular CSF arise from different disease processes. Methods: A combination of ventricle location using an active appearance model, and intensity based segmentation is used to segment the lateral ventricles automatically, from MR scans of patients with Alzheimer's disease (AD; n=26), Frontotemporal dementia (FTD; n=18), and vascular dementia (VaD; n=8), and age matched normal volunteers (n=34). Regional CSF volumes are combined to form five measures of the relative degrees of atrophy in different regions of the ABN MEDAL AWARD 2002 Lord Walton of Detchant Lord Walton of Detchant has made outstanding contributions in neurology, medical education, and scientific research. He is arguably the most renowned British neurologist of his generation. He was born in the north east of England and trained at King's College in the University of Durham, now the University of Newcastle on Tyne, where he graduated in 1945 with First Class Honours and Distinctions in Medicine, Surgery, and Midwifery. During National Service he served in the Western Approaches and the Middle East, later joining the TA and gaining the TD. He demonstrated his potential for research and writing during his MD thesis on subarachnoid haemorrhage, on which he was examined by Professor Natrass and Sir Charles Symonds, and which he later turned into an outstanding book in 1956. He was persuaded by Natrass and Henry Miller to forego an initial interest in paediatrics and, after a research fellowship, part of which was at the National Hospital, he spent time in Boston with Raymond Adams before writing his second book, a comprehensive text on polymyositis, the start of his lifelong interest in muscle disease. He founded the Muscular Dystrophy Laboratories at NGH and his phenotypic classification of muscle disease laid the foundation for subsequent studies in molecular genetics. In 1961 he wrote the book that became a standard text for medical students, Essentials of Neurology. In 1964 he edited the first edition of Disorders of Voluntary Muscle and in 1969, shortly after his appointment as Professor, he was invited to follow Lord Brain as author of Diseases of the Nervous System. In 1971 he succeeded Henry Miller as Dean of Medicine, a post which he held for a decade and during which he was knighted in 1979. Towards the end of his time in Newcastle he became successively President of the BMA, President of the GMC, then President of the RSM, he moved to Oxford as Warden of Green College in 1983. His writing continued and there followed Skeletal Muscle Pathology, Introduction to Clinical Neurosciences, and The Oxford Companion to Medicine. In 1989 he left Green College, became President of the WFN, and was appointed to a Life Peerage. During his stewardship the WFN increased in strength and importance and his tenure culminated in the successful British bid for the World Congress in 2001. His work in the House of Lords continues, his is a respected opinion whenever matters medical, scientific, or educational are discussed, and he has served as member and chairman of several important committees and reports. What Lord Walton has given to neurology, medicine, and research is evident to all, but to those who have worked for him and with him his interest in the individual, willingness to spend time with juniors, kindness, and generosity are equally apparent and more important. Colleagues around the world will applaud this Association for its recognition of his work and this award of the ABN Medal for 2002. Lord Walton, we look forward to your talk on "Fifty Years in Neurology".
doi:10.1136/jnnp.74.3.397
fatcat:fx5k362d6jatpodtafkws2ko2u