Peripheral T-cell lymphoma emerging in a patient with aggressive polymyositis: molecular evidence for neoplastic transformation of an oligoclonal T-cell infiltrate

We report a rare case of peripheral T-cell lymphoma arising in a 52-year-old man with biopsy-proven aggressive polymyositis, who had cardiac involvement, progressive bulbar symptoms, and died 11 months post diagnosis due to multiorgan failure. Using a multimodality approach including immunohistochemistry, genome-wide single nucleotide polymorphism (SNP)-array analysis, and high-throughput sequencing of the complementary determining region 3 (CDR3) of T-cell receptor beta (TCRβ) genes, our study demonstrates a molecular link between polymyositis and T-cell lymphoma, and provides evidence of the rapid and possibly late occurrence of genomic instability during neoplastic transformation of an oligoclonal T-cell population. Immunohistochemical analysis revealed loss of CD5, CD7, and CD8 antigen expression in autopsy tissue samples, as well as the occurrence of aberrant CD56 expression, not seen in pre-mortem biopsies, supporting the emergence of a neoplastic T-cell population. Multiplex polymerase chain reaction and next-generation sequencing of the TCRβ CDR3 region displayed two unique T-cell clones in both the diagnostic biopsy confirming polymyositis and the autopsy muscle tissue exhibiting T-cell lymphoma, linking the two pathological processes. SNP-array analysis revealed complex genomic abnormalities at autopsy but not in the pre-mortem muscle biopsies displaying polymyositis, confirming malignant transformation of the oligoclonal T-cell infiltrate. Our findings raise the possibility that clinically aggressive polymyositis might represent a preneoplastic condition in some instances, similar to certain other autoimmune and inflammatory disorders.     

Development of cortical influences on superior colliculus multisensory neurons: effects of dark‐rearing

Rearing cats from birth to adulthood in darkness prevents neurons in the superior colliculus (SC) from developing the capability to integrate visual and non‐visual (e.g. visual‐auditory) inputs. Presumably, this developmental anomaly is due to a lack of experience with the combination of those cues, which is essential to form associative links between them. The visual‐auditory multisensory integration capacity of SC neurons has also been shown to depend on the functional integrity of converging visual and auditory inputs from the ipsilateral association cortex. Disrupting these cortico‐collicular projections at any stage of life results in a pattern of outcomes similar to those found after dark‐rearing; SC neurons respond to stimuli in both sensory modalities, but cannot integrate the information they provide. Thus, it is possible that dark‐rearing compromises the development of these descending tecto‐petal connections and the essential influences they convey. However, the results of the present experiments, using cortical deactivation to assess the presence of cortico‐collicular influences, demonstrate that dark‐rearing does not prevent the association cortex from developing robust influences over SC multisensory responses. In fact, dark‐rearing may increase their potency over that observed in normally‐reared animals. Nevertheless, their influences are still insufficient to support SC multisensory integration. It appears that cross‐modal experience shapes the cortical influence to selectively enhance responses to cross‐modal stimulus combinations that are likely to be derived from the same event. In the absence of this experience, the cortex develops an indiscriminate excitatory influence over its multisensory SC target neurons.     

Treatment of recurrent,multiloculated, obstructive hydrocephalus with suprahepatic peritoneal catheter placement

The suprahepatic space represents a potential reservoir for cerebrospinal fluid absorption and becomes critical when extensive abdominal adhesions prevent passage of a peritoneal shunt catheter toward the pelvis. We present a patient with refractory hydrocephalus in which a multishunt system with drainage into the suprahepatic space was ultimately successful in decompressing the patient’s trapped ventricular spaces.     

Prioritizing core components of successful transitions from child to adult mental health care: a national Delphi survey with youth,caregivers, and health professionals

Youth accessing mental health care often experience a disruption in care as they attempt to transition between child and adolescent mental health services (CAMHS) and adult mental health services (AMHS). Few studies have evaluated interventions seeking to improve the experience and outcomes of CAMHS–AMHS transitions, in part due to lack of consensus on what constitutes best practices in intervention success. As such, the aim of this study was to engage patients, caregivers, and clinicians to prioritize core components of successful CAMHS–AMHS transitions which can be used in the design or evaluation of transition interventions. As such, a Delphi study was conducted to determine core components of successful CAMHS–AMHS transitions. Guided by the principles of patient-oriented research, three balanced expert panels consisting of youth, caregivers, and clinicians ranked and provided feedback on the importance and feasibility of core components of CAMHS-AMHS transitions. Components endorsed as feasible or important with ≥ 70% agreement from any panel moved to the next round. As a result, a list of 26 core components of CAMHS–AMHS transitions has been refined which can be used in the design, implementation, or evaluation of interventions intended to improve transition experiences and outcomes for youth in mental health care. Youth and families were engaged in an expert advisory role throughout the research process, contributing their important perspectives to the design and implementation of this study, as well as interpretation of the findings.

     

The Communication Supports Inventory-Children & Youth (CSI-CY), a new instrument based on the ICF-CY

Purpose: Two studies are presented that evaluated the Communication Supports Inventory-Children & Youth (CSI-CY), an instrument designed to facilitate the development of communication-related educational goals for students with complex communication needs (CCN). The CSI-CY incorporates a code set based on the ICF-CY. The studies were designed to determine the effect of using the CSI-CY on IEP goals for students with CCN and to evaluate consumer satisfaction.

Method: In Study 1, sixty-one educators and speech–language pathologists were randomly assigned to either (a) provide a student’s current IEP (control group) or (b) complete the CSI-CY prior to preparing a student’s next IEP and to submit the new IEP (experimental group). Study 2 was a field test to generate consumer satisfaction data.

Results: Study 1 showed that IEP goals submitted by participants in the experimental group referenced CSI-CY-related content significantly more frequently than did those submitted by control participants. Study 2 revealed high satisfaction with the instrument.

Conclusions: The code set basis of the CSI-CY extends the common language of the ICF-CY to practical educational use for children with CCN across diagnostic groups. The CSI-CY is well regarded as an instrument to inform the content of communication goals related to CCN.

• Implications for Rehabilitation

• The CSI-CY will guide rehabilitation professionals to develop goals for children with complex communication impairments.

• The CSI-CY is a new instrument that is based on the ICF-CY for documentation of communication goals.

     

Guidelines on suicide amongst anaesthetists 2019

Anaesthetists are thought to be at increased risk of suicide amongst the medical profession. The aims of the following guidelines are: increase awareness of suicide and associated vulnerabilities, risk factors and precipitants; to emphasise safe ways to respond to individuals in distress, both for them and for colleagues working alongside them; and to support individuals, departments and organisations in coping with a suicide.