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We introduced the preparation of hybrid composites using technical cellulose as a matrix material from rice husks with a natural SiO2 content from 0% to 19.4%. In this work, the physicochemical characteristics of the resulting hybrid composites have been investigated. The presence of SiOC chemical bonds in the resulting hybrid composites was determined by infrared spectroscopy. Microscopic analysis showed the complete removal of the mineral component and lignin from the cell wall of the rice husk fibers. This allows obtaining of an expanded surface of the fibers (101.7 m2/g) with uniform distribution of the TiO2 aggregates. It could be shown that with content increasing of natural silicon dioxide in the hybrid composite the decomposition rate of H2O2 also increases. © 2017 Wiley Periodicals, Inc. J. Appl. Polym. Sci. 2018, 135, 45796. © 2017 Wiley Periodicals, Inc.

BACKGROUND: Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a rare early onset neurodegenerative disease that typically results in ataxia, upper motor neuron dysfunction and sensorimotor peripheral neuropathy. Dysarthria and dysphagia are anecdotally described as key features of ARSACS but the nature, severity and impact of these deficits in ARSACS are not known. A comprehensive quantitative and qualitative characterization of speech and swallowing function will support diagnostics, provide insights into the underlying pathology, and guide day-to-day clinical management. METHODS: 11 consecutive non-Quebec ARSACS patients were recruited, and compared to healthy participants from several published and unpublished cohorts. A comprehensive behavioural assessment including objective acoustic analysis and expert perceptual ratings of motor speech, the Clinical Assessment of Dysphagia in Neurodegeneration (CADN), videofluoroscopy and standardized tests of dysarthria and swallowing related quality of life was conducted. RESULTS: Speech in this ARSACS cohort is characterized by pitch breaks, prosodic deficits including reduced rate and prolonged intervals, and articulatory deficits. The swallowing profile was characterized by delayed initiation of the swallowing reflex and late epiglottic closure. Four out of ten patients were observed aspirating thin liquids on videofluoroscopy. Patients report that they regularly cough or choke on thin liquids and solids during mealtimes. Swallowing and speech-related quality of life was worse than healthy controls on all domains except sleep. CONCLUSIONS: The dysphagia and dysarthria profile of this ARSACS cohort reflects impaired coordination and timing. Dysphagia contributes to a significant impairment in functional quality of life in ARSACS, and appears to manifest distinctly from other ARSACS dysfunctions such as ataxia or spasticity.

Zusammenfassung: Eine erst kürzlich etablierte, aber häufige Ätiologie der Hüftschmerzen beim jungen Erwachsenen ist das femoroazetabuläre Impingement (FAI), das eine leichte Form der frühzeitigen degenerativen Erkrankung des Hüftgelenks darstellt. Die klinischen Aspekte und diagnostischen Möglichkeiten sollen im Folgenden diskutiert und eine komplett arthroskopische Korrektur dieser Pathologie präsentiert werden. Mit dieser Technik können die azetabuläre Komponente ebenso wie die pathologischen Veränderungen am Femurkopf behandelt werden. Das schließt — falls notwendig — auch eine Labrumnaht mit ein. Neurologische Komplikationen durch lange Distraktionszeiten können durch die Anwendung einer invasiven Distraktionsmethode vermieden werden. Die Ergebnisse der arthroskopischen Behandlung sind mit denen der offenen chirurgischen Technik vergleichbar

BACKGROUND: Mutations in the nuclear-encoded mitochondrial DNA polymerase gamma (POLG) can result in a wide spectrum of neurological deficits. A common presentation is progressive ataxia (POLG-A) which includes impaired speech and swallowing. The nature, severity and impact of these deficits in POLG-A is not known. A comprehensive quantitative and qualitative characterization of dysarthria and dysphagia in this recurrent ataxia disorder will assist in diagnostics, provide insights into the underlying pathology, and establish the foundation for future therapy trials. METHODS: 14 consecutive patients with POLG (9 females, mean age=50.1y, SD=11.2) and 34 healthy controls were enrolled. Comprehensive assessments of motor speech and swallowing function, acoustic analysis of speech, videofluoroscopy and measures of quality of life were conducted. RESULTS: The speech profile of individuals with POLG-A was characterized by poor control of pitch and strain-strangled voice quality, reduced rate of speech and longer variable silences between words, and articulatory breakdown including imprecise consonants and vowel distortions. Swallowing deficits included slower initiation of the swallow reflex, poor control of bolus and late epiglottic closure. Speech and swallowing related quality of life was worse than healthy controls. CONCLUSIONS: The dysarthria and dysphagia profiles in POLG-A are largely symptomatic of impaired timing, indicating a mainly spinocerebellar deficit. Dysarthria and dysphagia contribute to a significant impairment in functional quality of life, and progress distinctly from other POLG-A dysfunctions like ataxia or cognitive impairment. Our assessments establish meaningful patient focused outcome measures that will be suitable for use in natural history studies and clinical trials.