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 CBD:
* “The clinical presentation of CBD is variable and includes the CBS, PSP, progressive aphasia, and frontotemporal dementia. However, CBD research is hampered by the rarity of the disorder, patients being scattered over a large geographic area.”
* “A recent clinicopathological study showed that only the minority of postmortem confirmed CBD cases were diagnosed correctly in life (five out of 19 cases) and that the clinical syndrome of CBS is not specific for CBD. Intriguingly, most of the pathologically confirmed CBD cases were characterized clinically by a PSP-like presentation with delayed onset of vertical supranuclear gaze palsy.”
* “Intriguingly, an Alzheimer’s disease-like CSF pattern was associated with early memory decline in patients with CBS according to a recent study.”
Copied below is the abstract.
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.
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.
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.
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)