Clinical characterization of dystonia in adult patients with Huntington's disease.

BACKGROUND AND PURPOSE: Huntington's disease (HD) is an autosomal dominant, neurodegenerative movement disorder, typically characterized by chorea. Dystonia is also recognized as part of the HD motor phenotype, although little work detailing its prevalence, distribution, severity and impact on functional capacity has been published to date. METHODS: Patients (>18 years of age) were recruited from the Cardiff (UK) HD clinic, each undergoing a standardized videotaped clinical examination and series of functional assessment questionnaires (Unified Huntington's Disease Rating Scale, Burke-Fahn-Marsden Dystonia Rating Scale and modified version of the Toronto Western Spasmodic Torticollis Rating Scale). The presence and severity of dystonia were scored by four independent neurologists using the Burke-Fahn-Marsden Dystonia Rating Scale and Unified Huntington's Disease Rating Scale. Statistical analysis included Fisher's exact test, Wilcoxon test, anova and calculation of correlation coefficients where appropriate. RESULTS: Forty-eight patients [91% (48/53)] demonstrated evidence of dystonia, with the highest prevalence in the left upper limb (n = 44, 83%), right upper limb most severely affected and eyes least affected. Statistically significant positive correlations (P < 0.05) were observed between dystonia severity and increasing HD disease stage and motor disease duration. Deterioration in functional capacity also correlated with increasing dystonia severity. No significant relationship was observed with age at motor symptom onset or CAG repeat length. CONCLUSIONS: We report a high prevalence of dystonia in adult patients with HD, with worsening dystonia severity with increasing HD disease stage and motor disease duration. The recognition and management of dystonic symptoms in routine clinical practice will aid overall symptomatic treatment and functional improvement.

Evidence that hospital hygiene is important in the control of methicillin-resistant Staphylococcus aureus.

Observational and microbiological data were collected from the patients and environment of a male general surgical ward over a period of 27 months from January 1998. Isolates of methicillin-resistant Staphylococcus aureus (MRSA) from patients and environment were typed by antibiogram, bacteriophage and pulsed field gel electrophoresis of chromosomal DNA. In September 1999, an intervention was put in place which included increasing the domestic cleaning time by 57 hours per week, with emphasis on removal of dust by vacuum cleaning, and allocation of responsibility for the routine cleaning of shared medical equipment. From January 1998 to September 1999, despite standard infection control measures (emphasis on hand hygiene, isolation of affected patients and staggered closure and cleaning of ward bays), 69 patients acquired a strain of E-MRSA16. This strain was also widespread in the ward environment. Typing confirmed that isolates from patients and environment were indistinguishable from one another and that the outbreak was due to a single strain. This strain was responsible for postoperative infection in approximately one third of the patients who acquired it. In the six months following the intervention, only three patients were colonized with the outbreak MRSA and monthly surveys failed to detect this strain in the environment. Thorough and continuous attention to ward hygiene and removal of dust was needed, to terminate a prolonged outbreak of MRSA infection on a general surgical ward, in addition to standard infection control measures. Control of hospital-acquired infection with MRSA requires a combination of measures, none of which are completely effective in isolation.