TY - JOUR
T1 - Essential tremor
T2 - evolving clinicopathological concepts in an era of intensive post-mortem enquiry
AU - Louis, Elan D.
N1 - Funding Information:
EDL is funded through R01 NS042859 and R01 NS 039422 from the National Institutes of Health and the Parkinson's Disease Foundation .
Copyright:
Copyright 2010 Elsevier B.V., All rights reserved.
PY - 2010/6
Y1 - 2010/6
N2 - Essential tremor (ET) is one of the most common neurological disorders. In recent years, as a result of systematic post-mortem examinations, our knowledge of the pathophysiology of this disease has grown substantially. Clearly identifiable structural changes (ie, Purkinje cell loss, Lewy bodies) have been observed in the brains of individuals with ET. These changes are not uniform and seem to follow several patterns, localising to the cerebellum itself or to a collection of brainstem neurons that synapse directly with Purkinje cells. Furthermore, these changes are similar to those seen in degenerative diseases. A wealth of clinical, epidemiological, and now post-mortem data indicate that this disease, or perhaps this family of diseases, is likely to be neurodegenerative. The molecular mechanisms that underlie these structural changes in ET are unknown. However, with more controlled, tissue-based studies being done, it is hoped that these mechanisms will be elucidated, thereby laying the foundation for the development of more targeted, effective pharmacotherapeutic interventions.
AB - Essential tremor (ET) is one of the most common neurological disorders. In recent years, as a result of systematic post-mortem examinations, our knowledge of the pathophysiology of this disease has grown substantially. Clearly identifiable structural changes (ie, Purkinje cell loss, Lewy bodies) have been observed in the brains of individuals with ET. These changes are not uniform and seem to follow several patterns, localising to the cerebellum itself or to a collection of brainstem neurons that synapse directly with Purkinje cells. Furthermore, these changes are similar to those seen in degenerative diseases. A wealth of clinical, epidemiological, and now post-mortem data indicate that this disease, or perhaps this family of diseases, is likely to be neurodegenerative. The molecular mechanisms that underlie these structural changes in ET are unknown. However, with more controlled, tissue-based studies being done, it is hoped that these mechanisms will be elucidated, thereby laying the foundation for the development of more targeted, effective pharmacotherapeutic interventions.
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U2 - 10.1016/S1474-4422(10)70090-9
DO - 10.1016/S1474-4422(10)70090-9
M3 - Review article
C2 - 20451458
AN - SCOPUS:77952551416
SN - 1474-4422
VL - 9
SP - 613
EP - 622
JO - The Lancet Neurology
JF - The Lancet Neurology
IS - 6
ER -