This recently-published paper is about “new insights” into atypical parkinsonism, including MSA, DLB, PSP, and CBD. The paper is based upon medical journal articles published in 2010. Two of the three authors are well-known Austrian researchers in the atypical parkinsonism community.
The authors describe atypical parkinsonian disorders (APDs) as “characterized by relentlessly progressive and levodopa refractory parkinsonism associated with a range of distinctive atypical features.”
Here are the key points made in the article related to PSP:
* “During the last years, infrequent variants due to PSP tau pathology have been separated from the ‘classical’ syndrome (Richardson syndrome). These variants that challenge the concept of clinicopathologic PSP include PSP-parkinsonism (PSP-P), pure akinesia with gait freezing (PAGF), corticobasal syndrome (CBS), and progressive nonfluent aphasia. … The regional differences in pathological severity almost certainly account for the clinical differences and logically correlate with the different clinical features.”
* “Familial PSP is rare and sometimes related to familial frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17).”
* “Male sex, older age at disease-onset and higher PSP rating scale scores were independent predictors of shorter survival.”
* “A natural history study confirmed previous observations of high levels of cognitive impairment associated with PSP occurring in up to 50% of patients at early disease stages. Furthermore, the cognitive profile characterized by frontal-executive dysfunction was similar to a control cohort of patients with MSA.”
* “The NINDS-SPSP (National Institute of Neurological DisordersSociety for Progressive Supranuclear Palsy) criteria were established 15 years ago focusing on the classic Richardson syndrome presentation.”
Copied below is the abstract.
Robin
Current Opinion in Neurology. 2011 May 13. [Epub ahead of print]
New insights into atypical parkinsonism.
Wenning GK, Krismer F, Poewe W.
Department of Neurology, Medical University, Innsbruck, Austria.
Abstract
PURPOSE OF REVIEW:
Atypical parkinsonian disorders (APDs) comprise a heterogenous group of disorders including multiple system atrophy (MSA), dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). Based on literature published in 2010, we here review recent advances in the APD field.
RECENT FINDINGS:
Genome-wide association studies have provided robust evidence of increased disease risk conferred by synuclein and tau gene variants in MSA and PSP. Furthermore, advanced imaging tools have been established in the differential diagnosis and as surrogate markers of disease activity in patients with APDs. Finally, although therapeutic options are still disappointing, translational research into disease-modifying strategies has accelerated with the increasing availability of transgenic animal models, particularly for MSA.
SUMMARY:
Remarkable progress has been achieved in the field of APDs, and advances in the genetics, molecular biology and neuroimaging of these disorders will continue to facilitate intensified clinical trial activity.
PubMed ID#: 21577106 (see pubmed.gov for this abstract only)