Neuropathology of two members of a German-American kindred (Family C) with late onset parkinsonism

Acta Neuropathologica
Z K WszolekD W Dickson

Abstract

We present genealogical and longitudinal clinical observations and autopsy findings of a previously reported kindred, Family C (German-American), with late-onset autosomal dominant parkinsonism with evidence for linkage on chromosome 2p13. The clinical phenotype includes the cardinal features of idiopathic Parkinson's disease. In addition, postural tremor and dementia are detected in some individuals. Two members of the kindred, one affected and one unaffected have recently come to autopsy. The unaffected family member was an 82-year-old woman whose brain showed only mild age-related pathology and no evidence of subclinical Lewy body disease. In contrast, the affected family member was an 83-year-old man whose brain had neuronal loss, gliosis and Lewy bodies in the substantia nigra and other monoaminergic brain stem nuclei, as well as the basal forebrain and amygdala. Lewy bodies and Lewy neurites had a distribution typical of cases of idiopathic Parkinson's disease. Thus, the clinical and pathological findings in this family with autosomal dominant parkinsonism are similar to those of sporadic Parkinson's disease.

Citations

Sep 5, 2002·Movement Disorders : Official Journal of the Movement Disorder Society·Katrina Gwinn-Hardy
Mar 17, 2004·Current Opinion in Pharmacology·Weidong Le, Stanley H Appel
Mar 6, 2003·Parkinsonism & Related Disorders·Anna Krygowska-WajsZbigniew K Wszolek
Mar 18, 2008·Parkinsonism & Related Disorders·C WiderF J G Vingerhoets
Jun 15, 2004·Brain Pathology·Masaki TakaoAtsuo Koto
Sep 23, 2006·The Neurologist·Jacobo Lester, Enrique Otero-Siliceo
Mar 24, 2005·Journal of Neural Transmission·A Krygowska-WajsZ K Wszolek

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