How people living with motor neurone disease and their carers experience healthcare decision making: a qualitative exploration.

PURPOSE: Healthcare decision making in motor neurone disease (MND) focuses on symptom management and quality of life. Decision making may be affected by personal approach to receiving information, decision making style, and disease symptoms. This study explored decision making from the perspectives of people living with motor neurone disease (plwMND). The issues impacting engagement and involvement in healthcare decisions were investigated. METHODS: Semi-structured interviews were conducted with 19 plwMND and 15 carers. Interview data was inductively analysed to identify and describe patterns and themes. RESULTS: Data analysis identified six overarching themes: Dimensions of decision making; Window of opportunity for choice; Intrinsic influences on decision making; Extrinsic influences impacting decision making; Planning in uncertainty; and, Communication is core. Many participants did not identify a process of "decision-making" except if considering early gastrostomy placement. Information provision requires a balance between ensuring patients are informed but not overwhelmed. Communication impairment impacts involvement. Healthcare professionals' communication style influences engagement in decision making. CONCLUSION: PlwMND perceive a lack of clinical decisions to make because disease symptoms and clinical phenotypes dictate necessary interventions. PlwMND describe communication impairment as a barrier to involvement in decision making and extra support is required to ensure they maintain engagement.IMPLICATIONS FOR REHABILITATIONPeople living with MND (plwMND) perceive they have few clinical decisions to make and viewed this process as "accepting a recommendation", rather than "making a decision" although early gastrostomy placement is the exception with considerable deliberation evident.Specialist multidisciplinary clinic advice is especially helpful for plwMND without dysphagia (swallowing problems) when considering early gastrostomy placement.Communication impairment may be a barrier to involvement in healthcare decisions and extra support to remain engaged is required.Some plwMND choose not to involve others in their decisions, and patients/families with medical or scientific backgrounds are more likely to collaborate with each other outside the context of clinic appointments.

Speech metrics, general disability, brain imaging and quality of life in multiple sclerosis.

BACKGROUND AND PURPOSE: Objective measurement of speech has shown promising results to monitor disease state in multiple sclerosis. In this study, we characterize the relationship between disease severity and speech metrics through perceptual (listener based) and objective acoustic analysis. We further look at deviations of acoustic metrics in people with no perceivable dysarthria. METHODS: Correlations and regression were calculated between speech measurements and disability scores, brain volume, lesion load and quality of life. Speech measurements were further compared between three subgroups of increasing overall neurological disability: mild (as rated by the Expanded Disability Status Scale ≤2.5), moderate (≥3 and ≤5.5) and severe (≥6). RESULTS: Clinical speech impairment occurred majorly in people with severe disability. An experimental acoustic composite score differentiated mild from moderate (P < 0.001) and moderate from severe subgroups (P = 0.003), and correlated with overall neurological disability (r = 0.6, P < 0.001), quality of life (r = 0.5, P < 0.001), white matter volume (r = 0.3, P = 0.007) and lesion load (r = 0.3, P = 0.008). Acoustic metrics also correlated with disability scores in people with no perceivable dysarthria. CONCLUSIONS: Acoustic analysis offers a valuable insight into the development of speech impairment in multiple sclerosis. These results highlight the potential of automated analysis of speech to assist in monitoring disease progression and treatment response.

Consensus Paper: Neurophysiological Assessments of Ataxias in Daily Practice.

The purpose of this consensus paper is to review electrophysiological abnormalities and to provide a guideline of neurophysiological assessments in cerebellar ataxias. All authors agree that standard electrophysiological methods should be systematically applied in all cases of ataxia to reveal accompanying peripheral neuropathy, the involvement of the dorsal columns, pyramidal tracts and the brainstem. Electroencephalography should also be considered, although findings are frequently non-specific. Electrophysiology helps define the neuronal systems affected by the disease in an individual patient and to understand the phenotypes of the different types of ataxia on a more general level. As yet, there is no established electrophysiological measure which is sensitive and specific of cerebellar dysfunction in ataxias. The authors agree that cerebellar brain inhibition (CBI), which is based on a paired-pulse transcranial magnetic stimulation (TMS) paradigm assessing cerebellar-cortical connectivity, is likely a useful measure of cerebellar function. Although its role in the investigation and diagnoses of different types of ataxias is unclear, it will be of interest to study its utility in this type of conditions. The authors agree that detailed clinical examination reveals core features of ataxia (i.e., dysarthria, truncal, gait and limb ataxia, oculomotor dysfunction) and is sufficient for formulating a differential diagnosis. Clinical assessment of oculomotor function, especially saccades and the vestibulo-ocular reflex (VOR) which are most easily examined both at the bedside and with quantitative testing techniques, is of particular help for differential diagnosis in many cases. Pure clinical measures, however, are not sensitive enough to reveal minute fluctuations or early treatment response as most relevant for pre-clinical stages of disease which might be amenable to study in future intervention trials. The authors agree that quantitative measures of ataxia are desirable as biomarkers. Methods are discussed that allow quantification of ataxia in laboratory as well as in clinical and real-life settings, for instance at the patients' home. Future studies are needed to demonstrate their usefulness as biomarkers in pharmaceutical or rehabilitation trials.

The impacts of injury at the individual, community and societal levels: a systematic meta-review.

OBJECTIVES: Injury is a major public health problem. While the impacts of injury on the injured person are well documented, there is relatively little knowledge about the impacts of injury on those individuals and groups in the community connected to the injured person. This study seeks to describe this breadth of research using a meta-review methodology. STUDY DESIGN: Systematic meta-review. METHODS: To gain a better understanding of the known impacts of injury on family, community and society, a systematic meta-review of injury outcomes research literature was conducted. Seventy-eight peer-reviewed published literature reviews were included in the meta-review. RESULTS: Of these, 70 reported outcomes at the level of the injured person including mortality, body functions, activity and participation limitations. Nine reviews reported impacts at the level of the community including impacts on family members, work colleagues and carers. Six studies reported impacts at the societal level including economic, health system and injury compensation system impacts. CONCLUSIONS: In summary, the meta-review identified a substantial body of knowledge at the individual level outcomes of injury, and a relative lack of information regarding the community and societal impacts of injury. An injury outcome framework is proposed on the basis of the findings of the meta-review to guide future research activity, particularly with regard to injury outcome domains where there is currently limited evidence. A comprehensive framework that takes account of all levels of impact is necessary for effective policies, systems and strategies to support recovery following injury.

Health care utilisation following hospitalisation for transport-related injury.

BACKGROUND: Transport injuries are a substantial cause of disability and death internationally. There is little published information regarding patterns of healthcare utilisation following transport injury. AIMS: To investigate patterns of in-hospital and post-discharge healthcare use following transport injury. METHODS: Analysis of all accepted adult claims from the database of the transport accident compensation regulator in Victoria, Australia between 1995 and 2008. The analyses focused on injuries resulting in hospitalisation. Indicators of in-hospital and post-discharge healthcare utilisation (e.g. number of services per practitioner group) within the first 12-months were summarised. RESULTS: More than a third (33.6%, n = 68,639) of all accepted compensable transport injuries resulted in admission to an acute care facility within 28 days of injury. In this group, the compensation authority paid for a total of 4.5 million healthcare services in the 12 months post-discharge (median of 19 services per claim). Services provided by medical practitioners were accessed by nearly all claimants (95.7%) at a median of 11 (5-26) per claimant. Less than half of claimants (46.7%) accessed paramedical or allied health services but the median number of services accessed was higher at 29 (9-82) per claimant. CONCLUSION: Transport-related injury cases require a substantial interaction with multiple components of the healthcare system in the year following hospital discharge. Compensation system data may provide a detailed understanding of healthcare utilisation, a key element of injury burden.

Role of cerebellum in fine speech control in childhood: persistent dysarthria after surgical treatment for posterior fossa tumour.

Dysarthria following surgical resection of childhood posterior fossa tumour (PFT) is most commonly documented in a select group of participants with mutism in the acute recovery phase, thus limiting knowledge of post-operative prognosis for this population of children as a whole. Here we report on the speech characteristics of 13 cases seen long-term after surgical treatment for childhood PFT, unselected for the presence of post-operative mutism (mean time post-surgery=6y10m, range 1;4-12;6 years, two had post-operative mutism), and examine factors affecting outcome. Twenty-six age- and sex- matched healthy controls were recruited for comparison. Participants in both groups had speech assessments using detailed perceptual and acoustic methods. Over two-thirds of the group (69%) with removal of PFT had a profile of typically mild dysarthria. Prominent speech deficits included consonant imprecision, reduced rate, monopitch and monoloudness. We conclude that speech deficits may persist even up to 10 years post-surgery in participants who have not shown mutism in the acute phase. Of cases with unilateral lesions, poorer outcomes were associated with right cerebellar tumours compared to left, consistent with the notion based on adult data that speech is controlled by reciprocal right cerebellar/left frontal interactions. These results confirm the important role of the cerebellum in the control of fine speech movements in children.

A Cochrane review of treatment for dysarthria following acquired brain injury in children and adolescents.

BACKGROUND: The expression ''acquired brain injury'' (ABI) incorporates a range of etiologies including cerebrovascular accident, brain tumour and traumatic brain injury. ABI is a common cause of disability in the pediatric population, and dysarthria is a common and often persistent sequelae associated with ABI in children. OBJECTIVES: The aim of this study was to assess the efficacy of intervention delivered by Speech and Language Pathologists/Therapists targeting dysarthric speech in children resulting from acquired brain injury. METHODS: Several electronic databases were searched up to January 2007. The review considered randomised controlled trials (RCTs) and quasi-randomised studies of children aged 3 to 16 years with acquired dysarthria grouped by aetiology (e.g., brain tumour, traumatic brain injury, cerebrovascular accident). Both authors independently assessed the titles and abstracts for relevance (100% inter-rater reliability) and the full text version of all potentially relevant articles was obtained. No studies met inclusion criteria. RESULTS: Of 2091 titles and abstracts identified, full text versions of only three were obtained. The remaining 2 088 were excluded, largely on the basis of not including dysarthria, being diagnostic or descriptive papers, and for concerning adults rather than children. All obtained articles were excluded due to including populations without ABI, adults with dysarthria, or inappropriate design. Thus, no studies met inclusion criteria. CONCLUSIONS: The review demonstrates a critical lack of studies, let alone RCTs, addressing treatment efficacy for dysarthria in children with ABI. Possible reasons to explain this lack of data include 1) a lack of understanding of the characteristics or natural history of dysarthria associated with this population; 2) the lack of a diagnostic classification system for children precluding the development of well targeted intervention programs; and 3) the heterogeneity of both the etiologies and resultant possible dysarthria types of pediatric ABI. Efforts should first be directed at modest well-controlled studies to identify likely efficacious treatments that may then be trialled in multicentre collaborations using quasi-randomised or RCT methodology.

A Cochrane review of treatment for childhood apraxia of speech.

BACKGROUND: The diagnosis of Childhood Apraxia of Speech (CAS) remains heavily debated, and there is no agreement upon the etiology for the disorder. Whilst there is some consensus on the potential broad array of presenting features of children with CAS, only three key features are recognised as ubiquitous for diagnosis: 1) inconsistent error production on both consonants and vowels across repeated productions of syllables or words; 2) lengthened and impaired coarticulatory transitions between sounds and syllables; and 3) inappropriate prosody. Despite the typically severe and complex presentation of CAS, there is little evidence reported on intervention approaches for the disorder, perhaps as a result of the ongoing deliberation over etiology and diagnosis. OBJECTIVES: The aim of this paper was to assess the efficacy of intervention delivered by Speech and Language Pathologists(s)/Speech and Language Therapists targeting CAS in children and adolescents. METHOD: Several electronic databases were searched up to January 2007. The review considered randomised controlled trials (RCTs) and quasi-randomised studies of children aged 3 to 16 years with CAS, grouped by treatment types (e.g., perceptual and instrumentally-based biofeedback treatment techniques). Two authors independently assessed references identified from the searches and obtained full text versions of all potentially relevant articles. RESULTS: Of 825 titles and abstracts searched, only 31 abstracts appeared to meet inclusion criteria. The remaining 794 papers were excluded predominantly on the basis of not including participants with CAS (e.g., focused on other developmental speech disorders or adult acquired apraxia of speech), or for not being intervention studies (i.e. being diagnostic or descriptive). All 31 full text articles obtained were excluded following evaluation as they did not meet inclusion criteria on design. CONCLUSION: The review demonstrates a critical lack of well controlled treatment studies addressing treatment efficacy for CAS, making it impossible for conclusions to be drawn about which interventions are most effective for treating CAS in children or adolescents.

Aerosol infection of rhesus macaques with Junin virus.

The purpose of our work was to determine if aerosols of Junin virus can infect rhesus macaques and if the disease is the same as that produced by virus inoculated parenterally. The 6 macaques exposed to the virus by aerosol became acutely ill during the 3rd week after exposure, and all died. Three died by day 21, while the remainder died after 1 month. Junin virus was found primarily in visceral organs of those animals dying before 21 days after infection and in the central nervous system tissues from animals dying later. Histological changes were similar to those reported in rhesus monkeys after parenteral Junin viral infection. Gastrointestinal necrosis, however, was less severe in aerosol-infected animals and the associated septicemia was not seen. High levels of alpha interferon were detected by the 3rd day in all infected macaques. Experimental Argentine hemorrhagic fever induced by aerosol infection in rhesus macaques was similar to that seen after parenteral challenge and mimicked closely the clinical syndrome observed in humans.

Simian immunodeficiency virus infection of macaques: end-stage disease is characterized by widespread distribution of proviral DNA in tissues.

Four simian immunodeficiency virus (SIV)-infected macaques in the terminal stages of AIDS were studied. Easily detectable unintegrated proviral DNA was present in nearly every tissue (lymphoid and nonlymphoid) examined. SIV-specific immunohistochemistry revealed that macrophages or macrophage-like cells were the predominant reservoirs for SIV. High burdens of infectious SIV were also characteristic of end-stage disease. At the molecular level, sequence analyses of molecular clones derived directly from tissue demonstrated that SIV, like other RNA viruses, exists as a mixture of closely related genomes. Viruses derived by cocultivation seemed to be selected for their ability to grow in tissue culture and did not represent the wide spectrum of genotypes in tissues. These data indicate that SIV-induced immunodeficiency is a complex, multisystem disease and that SIV replication, in addition to impairing the immune system, may have direct deleterious effects on multiple organ systems.

Ambulatory electrocardiography (Holter monitoring) in caged monkeys.

A swivel-tethering and jacket system was used in conjunction with vinyl patch electrodes and Holter recorders to obtain continuous ECG recordings in 12 rhesus monkeys on a long-term (12 day) study. Animals were custom-fitted with nylon mesh jackets that were connected to a swivel unit by a flexible, stainless steel tether. Lead wires from the chest electrodes passed through the tether to the electrical swivel apparatus located at the top of the cage. Wires from the upper part of the swivel were attached to a reel-to-reel Holter recorder. This technique was used to obtain 24-h continuous ECG recordings, which were later processed using a computer-assisted Holter analysis system.

Coronavirus-like virions associated with a wasting syndrome in guinea pigs.

An apparent wasting syndrome was observed in newly arriving 3 to 4 week old guinea pigs characterized by anorexia, weight loss, diarrhea, perineal staining and death. Diagnostic efforts to attribute the disease to husbandry, environmental factors or to known guinea pig pathogens were unsuccessful. Clinical signs, enteric histopathological lesions and diagnostic transmission electron microscopy identification of typical coronavirus-like virions in fecal samples were consistent with enteric coronaviral diseases seen in other species.