sopranuclear progressive palsy

Background: Aggregates of TAR DNA-binding protein of 43 kDa (TDP-43) represent the pathological hallmark of most amyotrophic lateral sclerosis (ALS) and of nearly 50% of frontotemporal dementia (FTD) cases but were also observed to occur as secondary neuropathology in the nervous tissue of patients with different neurodegenerative diseases, including Parkinson's disease (PD) and atypical parkinsonism. Mutations of TARDBP gene, mainly in exon 6 hotspot, have been reported to be causative of some forms of ALS and FTD, with clinical signs of parkinsonism observed in few mutation carriers...

Rotigotine (RTG) is a non-ergot dopamine agonist developed as a new transdermal formulation, indicated for use in early and advanced Parkinson's disease (PD). The potential advantages of the RTG patch include immediacy of effect onset, constant drug delivery, better tolerability avoiding drug peaks and easy of use, helping patient's compliance. So, RTG patch appears to be a suitable candidate in the treatment of patients with atypical parkinsonism. The present is an observational study to evaluate the efficacy and tolerability of RTG in patients affected by atypical parkinsonian disorders...

Neuroimaging studies of the dopaminergic system with positron emission tomography and single photon emission computed tomography radioligands are useful in the assessment of neuronal degeneration in Parkinson's disease. Abnormalities can be identified early in the disease process and used to monitor progression as well as the effect of treatment. Studies of dopamine receptors and brain metabolism can help identify those patients who have other Parkinsonian syndromes, such as multiple system atrophy, progressive sopranuclear palsy or diffuse Lewy body dementia...