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Synucleinopathies from bench to bedside.

Puschmann, Andreas LU ; Bhidayasiri, Roongroj and Weiner, William J (2012) In Parkinsonism & Related Disorders 18 Suppl 1. p.24-27
Abstract
Accumulation of alpha-synuclein is a pathological feature in several neurological diseases. Its characterization has allowed for a re-grouping of diseases according to the expected pathology. The clinical syndrome of PD can now be classified into forms with and without alpha-synuclein pathology. DLB and PDD are synucleinopathies, and MSA shows alpha-synuclein pathology with glial inclusions. ADHD symptoms commonly occur in persons that will subsequently develop DLB. A similar phenomenon may be the early personality changes and frontotemporal atrophy in patients with SNCA multiplication. RLS is not known to have alpha-synuclein pathology, but as PD and ADHD, involves a hypodopaminergic state. Furthermore, PD and RLS co-occur in families in... (More)
Accumulation of alpha-synuclein is a pathological feature in several neurological diseases. Its characterization has allowed for a re-grouping of diseases according to the expected pathology. The clinical syndrome of PD can now be classified into forms with and without alpha-synuclein pathology. DLB and PDD are synucleinopathies, and MSA shows alpha-synuclein pathology with glial inclusions. ADHD symptoms commonly occur in persons that will subsequently develop DLB. A similar phenomenon may be the early personality changes and frontotemporal atrophy in patients with SNCA multiplication. RLS is not known to have alpha-synuclein pathology, but as PD and ADHD, involves a hypodopaminergic state. Furthermore, PD and RLS co-occur in families in a way that suggests common inheritance. A proportion of patients with ET have brainstem Lewy body pathology. Gaucher disease and other lysosomal storage disorders also have alpha-synuclein pathology. Alpha-synuclein is a naturally unfolded protein. Non-fibrillar oligomeres may be the toxic species, and Lewy body formation may in fact be protective. Inhibiting alpha-synuclein toxicity seems to be an attractive novel treatment strategy and several approaches are being developed. When such treatments become available, clinicians will need to be familiar with the clinical features that distinguish the synucleinopathies from their look-alikes. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Parkinsonism & Related Disorders
volume
18 Suppl 1
pages
24 - 27
publisher
Elsevier
external identifiers
  • pmid:22166445
  • scopus:84858662511
ISSN
1873-5126
DOI
10.1016/S1353-8020(11)70010-4
language
English
LU publication?
yes
id
573c6984-7054-4729-a405-58cde445cf01 (old id 2273746)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/22166445?dopt=Abstract
date added to LUP
2012-01-03 20:59:07
date last changed
2017-08-06 04:47:46
@article{573c6984-7054-4729-a405-58cde445cf01,
  abstract     = {Accumulation of alpha-synuclein is a pathological feature in several neurological diseases. Its characterization has allowed for a re-grouping of diseases according to the expected pathology. The clinical syndrome of PD can now be classified into forms with and without alpha-synuclein pathology. DLB and PDD are synucleinopathies, and MSA shows alpha-synuclein pathology with glial inclusions. ADHD symptoms commonly occur in persons that will subsequently develop DLB. A similar phenomenon may be the early personality changes and frontotemporal atrophy in patients with SNCA multiplication. RLS is not known to have alpha-synuclein pathology, but as PD and ADHD, involves a hypodopaminergic state. Furthermore, PD and RLS co-occur in families in a way that suggests common inheritance. A proportion of patients with ET have brainstem Lewy body pathology. Gaucher disease and other lysosomal storage disorders also have alpha-synuclein pathology. Alpha-synuclein is a naturally unfolded protein. Non-fibrillar oligomeres may be the toxic species, and Lewy body formation may in fact be protective. Inhibiting alpha-synuclein toxicity seems to be an attractive novel treatment strategy and several approaches are being developed. When such treatments become available, clinicians will need to be familiar with the clinical features that distinguish the synucleinopathies from their look-alikes.},
  author       = {Puschmann, Andreas and Bhidayasiri, Roongroj and Weiner, William J},
  issn         = {1873-5126},
  language     = {eng},
  pages        = {24--27},
  publisher    = {Elsevier},
  series       = {Parkinsonism & Related Disorders},
  title        = {Synucleinopathies from bench to bedside.},
  url          = {http://dx.doi.org/10.1016/S1353-8020(11)70010-4},
  volume       = {18 Suppl 1},
  year         = {2012},
}