Frequency and Pathophysiology of Apathy in Huntington Disease: A Systematic Review and Meta-Analysis.

OBJECTIVE: Apathy is a common behavioral symptom of Huntington disease (HD). This systematic review describes current evidence on the pathophysiology, assessment, and frequency of apathy in HD. METHODS: This systematic review was conducted in accordance with PRISMA guidelines. Using a comprehensive search strategy, the investigators searched the MEDLINE, Embase, and PsycINFO databases. All studies that evaluated apathy in HD patients with a valid scale and reported apathy frequency or scores were included. Apathy scores were analyzed by mean or standardized mean differences in accordance with Cochrane guidelines. RESULTS: A total of 1,085 records were screened and 80 studies were ultimately included. The Problem Behaviors Assessment-Short was the most frequently used apathy assessment tool. Apathy frequency generally ranged from 10%-33% in premanifest HD to 24%-76% in manifest HD. A meta-analysis of 5,311 records of patients with premanifest HD showed significantly higher apathy scores, with a standardized mean difference of 0.41 (CI=0.29-0.52; p<0.001). A comparison of 1,247 patients showed significantly higher apathy scores in manifest than premanifest HD, with a mean difference of 1.87 (CI=1.48-2.26; p<0.001). There was evidence of involvement of various cortical and subcortical brain regions in HD patients with apathy. CONCLUSIONS: Apathy was more frequent among individuals with premanifest HD compared with those in a control group and among individuals with manifest HD compared with those with premanifest HD. Considering the complexity and unique pattern of development in neurodegenerative disease, further studies are required to explore the pathophysiology of apathy in HD.

The impact of data quality assurance and control solutions on the completeness, accuracy, and consistency of data in a national spinal cord injury registry of Iran (NSCIR-IR).

STUDY DESIGN: Descriptive study. OBJECTIVE: This study aimed to develop and evaluate a systematic arrangement for improvement and monitoring of data quality of the National Spinal Cord (and Column) Injury Registry of Iran (NSCIR-IR)-a multicenter hospital-based registry. SETTING: SCI community in Iran. METHODS: Quality assurance and quality control were the primary objectives in improving overall quality of data that were considered in designing a paper-based and computerized case report. To prevent incorrect data entry, we implemented several validation algorithms, including 70 semantic rules, 18 syntactic rules, seven temporal rules, and 13 rules for acceptable value range. Qualified and trained staff members were also employed to review and identify any defect, inaccuracy, or inconsistency in the data to improve data quality. A set of functions were implemented in the software to cross-validate, and feedback on data was provided by reviewers and registrars. RESULTS: Socio-demographic data items were 100% complete, except for national ID and education level, which were 97% and 92.3% complete, respectively. Completeness of admission data and emergency medical services data were 100% except for arrival and transfer time (99.4%) and oxygen saturation (48.9%). Evaluation of data received from two centers located in Tehran proved to be 100% accurate following validation by quality reviewers. All data was also found to be 100% consistent. CONCLUSIONS: This approach to quality assurance and consistency validation proved to be effective. Our solutions resulted in a significant decrease in the number of missing data.

National Guidelines for Cognitive Assessment and Rehabilitation of Iranian Traumatic Brain Injury Patients.

BACKGROUND: Individuals with moderate to severe traumatic brain injury (TBI) often have prolonged cognitive impairments, resulting in long-term problems with their real-life activities. Given the urgent need for evidence-based recommendations for neuropsychological management of Iranian TBI patients, the current work aimed to adapt eligible international guidelines for cognitive assessment and rehabilitation of the TBI patients in Iran. METHODS: The project was led by an executive committee, under the supervision of the Iranian Ministry of Health and Medical Education (MOHME). Following a systematic literature search and selection process, four guidelines were included for adaptation. Clinical recommendations of the source guidelines were tabulated as possible clinical scenarios for 90 PICO clinical questions covering all relevant phases of care. After summing up the scenarios, our initial list of recommendations was drafted according to the Iranian patients' conditions. The final decision-making, with the contribution of a national interdisciplinary panel of 37 experts from across the country, was conducted in two rounds using online and offline survey forms (Round 1), and face-to-face and telephone meetings (Round 2). RESULTS: A total of 63 recommendations in six sections were included in the final list of recommendations, among which 24 were considered as key recommendations. In addition, some of the recommendations were identified as fundamental, meaning that proper implementation of the other recommendations is largely dependent on their implementation. CONCLUSION: Iranian health policy makers and rehabilitation program managers are recommended to address some fundamental issues to provide the necessary infrastructure to set up an efficient cognitive rehabilitation service system.

Feasibility and Data Quality of the National Spinal Cord Injury Registry of Iran (NSCIR-IR): A Pilot Study.

BACKGROUND: Spinal cord injury (SCI) is one of the most disabling consequences of trauma with unparalleled economic, social, and personal burden. Any attempt aimed at improving quality of care should be based on comprehensive and reliable data. This pilot investigation studied the feasibility of implementing the National Spinal Cord and Column Injury Registry of Iran (NSCIR-IR) and scrutinized the quality of the registered data. METHODS: From October 2015 to May 2016, over an 8-month period, 65 eligible trauma patients who were admitted to hospitals in three academic centers in mainland Iran were included in this pilot study. Certified registered nurses and neurosurgeons were in charge of data collection, quality verification, and registration. RESULTS: Sixty-five patients with vertebral column fracture dislocations were registered in the study, of whom 14 (21.5%) patients had evidence of SCI. Mechanisms of injury included mechanical falls in 30 patients (46.2%) and motor vehicle accidents in 29 (44.6%). The case identification rate i.e. clinical and radiographic confirmation of spine and SCI, ranged from 10.0% to 88.9% in different registry centers. The completion rate of all data items was 100%, except for five data elements in patients who could not provide clinical information because of their medical status. Consistency i.e. identification of the same elements by all the registrars, was 100% and accuracy of identification of the same pathology ranged from 66.6% to 100%. CONCLUSIONS: Our pilot study showed both the feasibility and acceptable data quality of the NSCIR-IR. However, effective and successful implementation of NSCIR-IR data use requires some modifications such as presence of a dedicated registrar in each center, verification of data by a neurosurgeon, and continuous assessment of patients' neurological status and complications.

Subarachnoid Space Transplantation of Schwann and/or Olfactory Ensheathing Cells Following Severe Spinal Cord Injury Fails to Improve Locomotor Recovery in Rats.

Treatment of spinal cord injury by exogenous cells has brought both successful and unsuccessful results. Olfactory ensheathing cells and Schwann cells have been widely used for transplantation purposes. In this study, we investigated the effects of these cells on contused spinal cord by introducing cells into subarachnoid space. Fifty thousand Schwann cells or olfactory ensheathing cells or a mixture of both cell types were transplanted one week after a 3-second clip compression injury at T-9 spinal cord level in rats. Starting from the day one of spinal cord injury, animals were assessed for six months by BBB test and then were sacrificed for immunohistochemistry labeling of the spinal cord injury site. There was no locomotor recovery in any of the treatment groups including controls. Immunohistochemistry assessment indicated positive labeling of P75 and S100 markers in the cell-transplanted groups compared with control. Our data suggest that transplantation of Schwann cells and/or olfactory ensheathing cells into the subarachnoid space does not improve motor recovery in severely injured spinal cord, at least with the number of cells transplanted here. This, however, should not be regarded as an essentially negative outcome, and further studies which consider higher densities of cells are required.

Adaptation of Traumatic Brain Injury Guidelines in Iran.

CONTEXT: The National institute for health and care excellence (NICE) and scottish intercollegiate guidelines network (SIGN) are two well-known sources of clinical guideline development. In the past years, they have developed clinical guidelines for the management of head injury. In this report, we will highlight our modifications to these guidelines according to the domestic situation in a developing country. EVIDENCE ACQUISITION: The guidelines were appraised using the appraisal of guidelines for research and evaluation (AGREE) instrument. All key recommendations were reviewed by 14 prominent Iranian neurosurgeons; levels of evidence were evaluated and items with limited evidence were determined. Available evidence for selected items were reviewed and discussed. RESULTS: The following items were the most challenging when accounting for the domestic situation in Iran: age as a risk factor for referral, computed tomography scan, the impact of medical comorbidities, pregnancy, consultation, referral to a neurosurgical unit, and teleconsulting and observation before discharge. CONCLUSIONS: The evidence in the discussed topics was limited and controversial. This report is important because it exposes the current knowledge gap in head trauma studies in Iran.

The effect of timing of decompression on neurologic recovery and histopathologic findings after spinal cord compression in a rat model.

Prior animal models have shown that rats sustaining 3-second immediate spinal cord compression had significantly better functional recovery and smaller lesion volumes than rats subjected to compression times of 1 hour, 6 hours, 3 weeks, and 10 weeks after spinal cord injury. We compare locomotor rating scales and spinal cord histopathology after 3 seconds and 10 minute compression times. . Ten rats were assigned into two early (3-second) and late (10-minute) compressive surgery groups. Compressive injury was produced using an aneurysmal clip method. Rats were followed-up for 11 weeks, and behavioral assessment was done by inclined plane test and tail-flick reflex. At the end of the study, the rats were sacrificed, and spinal cord specimens were studied in light and EM. Basso, Beattie and Bresnahan (BBB) locomotor rating scales were significantly better in the early compression group after the 4th week of evaluation (P<0.05) and persisted throughout the remainder of the study. Histopathology demonstrated decreased normal tissue, more severe gliosis and cystic formation in the late group compared to the early group (P<0.05). In EM study, injuries in the late group including injury to the myelin and axon were more severe than the early compression group, and there was more cytoplasmic edema in the late compression group. Spinal cord injury secondary to 3-second compression improves functional motor recovery, spares more functional tissue, and is associated with less intracellular edema, less myelin and axon damage and more myelin regeneration in rats compared to those with 10 minutes of compression. Inclined plane test and tail-flick reflex had no significant difference.