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Detection of Lewy Bodies in Trisomy 21 (Down's Syndrome)

Published online by Cambridge University Press:  18 September 2015

Ravi Raghavan ,
Clare Khin-Nu ,
Andrew Brown ,
Dorothy Irving ,
Paul G. Ince ,
Kenneth Day ,
Stephen P. Tyrer  and
Robert H. Perry
Ravi Raghavan*
Affiliation:
Department of Neuropathology & MRC Neurochemical Pathology Unit, Newcastle General Hospital, Newcastle-upon-Tyne (R.R., A.B., D.I., P.G.I., R.H.P.); Northgate Hospital, Morpeth, Northumberland (C.K.-N., K.D.); Prudhoe Hospital, Prudhoe, Tyne and Wear (S.P.T.); Department of Psychiatry, University of Newcastle-upon-Tyne (K.D., S.P.T.), United Kingdom Received June 9, 1992.
Clare Khin-Nu
Affiliation:
Department of Neuropathology & MRC Neurochemical Pathology Unit, Newcastle General Hospital, Newcastle-upon-Tyne (R.R., A.B., D.I., P.G.I., R.H.P.); Northgate Hospital, Morpeth, Northumberland (C.K.-N., K.D.); Prudhoe Hospital, Prudhoe, Tyne and Wear (S.P.T.); Department of Psychiatry, University of Newcastle-upon-Tyne (K.D., S.P.T.), United Kingdom Received June 9, 1992.
Andrew Brown
Affiliation:
Department of Neuropathology & MRC Neurochemical Pathology Unit, Newcastle General Hospital, Newcastle-upon-Tyne (R.R., A.B., D.I., P.G.I., R.H.P.); Northgate Hospital, Morpeth, Northumberland (C.K.-N., K.D.); Prudhoe Hospital, Prudhoe, Tyne and Wear (S.P.T.); Department of Psychiatry, University of Newcastle-upon-Tyne (K.D., S.P.T.), United Kingdom Received June 9, 1992.
Dorothy Irving
Affiliation:
Department of Neuropathology & MRC Neurochemical Pathology Unit, Newcastle General Hospital, Newcastle-upon-Tyne (R.R., A.B., D.I., P.G.I., R.H.P.); Northgate Hospital, Morpeth, Northumberland (C.K.-N., K.D.); Prudhoe Hospital, Prudhoe, Tyne and Wear (S.P.T.); Department of Psychiatry, University of Newcastle-upon-Tyne (K.D., S.P.T.), United Kingdom Received June 9, 1992.
Paul G. Ince
Affiliation:
Department of Neuropathology & MRC Neurochemical Pathology Unit, Newcastle General Hospital, Newcastle-upon-Tyne (R.R., A.B., D.I., P.G.I., R.H.P.); Northgate Hospital, Morpeth, Northumberland (C.K.-N., K.D.); Prudhoe Hospital, Prudhoe, Tyne and Wear (S.P.T.); Department of Psychiatry, University of Newcastle-upon-Tyne (K.D., S.P.T.), United Kingdom Received June 9, 1992.
Kenneth Day
Affiliation:
Department of Neuropathology & MRC Neurochemical Pathology Unit, Newcastle General Hospital, Newcastle-upon-Tyne (R.R., A.B., D.I., P.G.I., R.H.P.); Northgate Hospital, Morpeth, Northumberland (C.K.-N., K.D.); Prudhoe Hospital, Prudhoe, Tyne and Wear (S.P.T.); Department of Psychiatry, University of Newcastle-upon-Tyne (K.D., S.P.T.), United Kingdom Received June 9, 1992.
Stephen P. Tyrer
Affiliation:
Department of Neuropathology & MRC Neurochemical Pathology Unit, Newcastle General Hospital, Newcastle-upon-Tyne (R.R., A.B., D.I., P.G.I., R.H.P.); Northgate Hospital, Morpeth, Northumberland (C.K.-N., K.D.); Prudhoe Hospital, Prudhoe, Tyne and Wear (S.P.T.); Department of Psychiatry, University of Newcastle-upon-Tyne (K.D., S.P.T.), United Kingdom Received June 9, 1992.
Robert H. Perry
Affiliation:
Department of Neuropathology & MRC Neurochemical Pathology Unit, Newcastle General Hospital, Newcastle-upon-Tyne (R.R., A.B., D.I., P.G.I., R.H.P.); Northgate Hospital, Morpeth, Northumberland (C.K.-N., K.D.); Prudhoe Hospital, Prudhoe, Tyne and Wear (S.P.T.); Department of Psychiatry, University of Newcastle-upon-Tyne (K.D., S.P.T.), United Kingdom Received June 9, 1992.
*
Department of Neuropathology, Newcastle-upon-Tyne NE4 6BE, United Kingdom
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Abstract:

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The presence of cortical senile plaques and neurofibrillary tangles sufficient to warrant a neuropatho-logical diagnosis of Alzheimer's disease is well established in middle-aged individuals with Trisomy 21 (Down's syndrome). In contrast a relationship between Down's syndrome and Lewy bodies, one of the major neuropathological features of Parkinson's disease, has not been previously reported. In a cliniconeuropathological survey of 23 cases of Down's Syndrome, two patients, aged 50 and 56 years respectively, were found to have Lewy body formation in the substantia nigra in addition to cortical Alzheimer-type pathology. Neither case showed significant substantia nigra neuron loss although locus coeruleus loss was present in both. Since substantia nigra Lewy bodies are a characteristic neu-rohistological feature of idiopathic Parkinson's disease, their occurrence in cases of Down's syndrome with evidence of Alzheimer-type pathology supports an aetiopathological connection between Parkinson's disease, Alzheimer's disease, and Down's syndrome; and suggests that common pathogenic mechanisms may underlie aspects of neuronal degeneration in these three disorders, some of which may relate to aberrant chromosome 21 expression.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 20 , Issue 1 , February 1993 , pp. 48 - 51
Copyright
Copyright © Canadian Neurological Sciences Federation 1993

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