Pulmonary arterial hypertension associated with systemic sclerosis in the Czech Republic

Background  

Systemic sclerosis (SSc) is an important cause of pulmonary arterial hypertension (PAH), with an estimated prevalence of 7.85–26.7%.     

Comparison of the short-term and long-term results of the Ponseti method in the treatment of idiopathic pes equinovarus

Purpose

Congenital club foot is one of the most common birth defects involving the musculoskeletal system. At present two methods are used for the treatment of this deformity: French and Ponseti method. The purpose of this study was to compare the short-term (up to three years) and long-term (three to seven years) results of treatment with the Ponseti method.

Methods

A total of 195 consecutive infants (143 boys and 52 girls) with idiopathic club foot treated with the Ponseti method in the period of 2005–2012 were included in this study; the total number of feet was 303. The severity of the foot deformity was classified according to Diméglio. When relapse occurred up until three years of age, we started with the casting again. If conservative treatment was unsuccessful we proceeded to surgical treatment.

Results

Primary correction was attained in all cases. Surgical correction of relapses was performed in 30 % of patients according to the Ponseti method (re-tenotomy of the Achilles tendon and transposition of the tibialis anterior) and in 70 % by alternative techniques. The number of relapses indicated for surgery increased with increasing period of follow-up: whereas in patients where the treatment started already in 2005 relapses occurred in 72 %, in patients included in 2011 the number of recurrences only reached 3 %.

Conclusions

It follows from our results that it is impossible to cure all club feet with casting, tenotomy of the Achilles tendon and transposition of the tibialis anterior only.     

Longitudinal study of single‐word comprehension in semantic dementia: A comparison with primary progressive aphasia and Alzheimer's disease

Background: Although semantic dementia (SD) is characterised by a multimodal loss of semantic knowledge, it has been demonstrated that lexical‐semantic representations are not equally disrupted in SD and that some categories may be recognised better than others. Little is known, however, about the pattern of the category‐specific comprehension deficits in SD and whether it differs from that of other forms of progressive aphasias.

Aims: This exploratory study aimed to investigate the evolution of category‐specific deficits of single‐word comprehension in progressive aphasias.

Methods & Procedures: A total of 19 patients with a clinical diagnosis of SD, 25 patients with primary progressive aphasia with agrammatic and relatively nonfluent speech (PPA), and 25 patients with Alzheimer's disease (AD) with aphasia were studied longitudinally with the Western Aphasia Battery (WAB). The Auditory Word Recognition subtest of the WAB was utilised to assess comprehension of words derived from different semantic categories.

Outcomes & Results: The analysis revealed that, over time, category‐specific deficits of single‐word comprehension were seen in all three groups of patients. Participants with SD as well as those with PPA and AD were impaired on both pointing to fingers and the right–left orientation task. However, patients with SD were the only group that showed defective recognition of their own body parts. Interestingly, individuals with SD had no difficulties identifying colours, letters, and numbers, even during the follow‐up testing. In addition, in all three groups the extent of category‐specific deficits was associated with the severity of aphasia.

Conclusions: These results indicate that category‐specific deficits of single‐word comprehension are frequently seen not only in patients with SD but also in individuals with PPA or AD, and that the extent of these deficits is associated with the severity of aphasia. However, the pattern of these deficits is often different in these three forms of neurodegenerative conditions and more dissociations between semantic categories are observed as each of these diseases progresses.