Incidence of traumatic spinal cord injury worldwide: A systematic review, data integration, and update.

Objectives: This review was designed to update our earlier systematic review which evaluated both published and unpublished evidence on the incidence of traumatic spinal cord injury (TSCI) worldwide. Methods: We used various search methods including strategic searching, reference checking, searching for grey literature, contacting registries, authors, and organizations requesting unpublished data, browsing related websites, and hand searching key journals. The quality of included studies was evaluated by Joanna Briggs Institute Critical Appraisal Tools. Records published between April 2013 and May 2020 were added to the original systematic review. Results: Overall, 58 resources including 45 papers, 10 SCI registry reports, 1 book, and 2 theses were retrieved. We found TSCI incidence data for eight new countries, which overall shapes our knowledge of TSCI incidence for 49 countries. The incidence of TSCI ranges from 3.3 to 195.4 cases per million (cpm) based on subnational studies and from 5.1 to 150.48 cpm based on national studies. Most of the studies were low quality, lacked consistent case selection due to unclear definition of TSCI and unclear ascertainment methods. Conclusions: There is an increasing number of publications in the literature focusing on the epidemiologic data of TSCI. The absence of a standard form of reporting TSCI hinders the comparability of data across different data sources. Use of various definitions for TSCI may lead to heterogeneity in reports. Use of sensitivity analyses based on reasonable classification criteria can aid in offering a uniform set of case identification and ascertainment criteria for TSCI.

Autonomic cardiovascular dysfunction during simple arithmetic test in a patient with cervical spinal cord injury-a case report.

INTRODUCTION: Spinal cord injury (SCI) disrupts autonomic control of the cardiovascular system, which may lead to autonomic dysfunction. Growing amounts of evidence support the possibility that systemic and cerebral hemodynamic dysfunctions may contribute to cognitive deficits in patients with SCI. CASE PRESENTATION: We present a case of autonomic cardiovascular dysfunction in a 55-year old female patient following non-traumatic cervical SCI. This case illustrates how a simple arithmetic test may elicit fluctuations in blood pressure causing cognitive disturbances. DISCUSSION: Clinical awareness of autonomic dysfunction and cognitive deficits is relevant in neurorehabilitation of patients with SCI. Assessment of autonomic function should be evaluated according to recommendation from International Standards to document remaining Autonomic Function after Spinal Cord Injury (ISAFSCI) [1].

Autonomic dysreflexia and concurrent Horner's Syndrome: a rare presentation in a patient with spinal cord injury.

INTRODUCTION: Autonomic dysreflexia is an uninhibited sympathetic response evoked by a strong sensory input below the level of the injury in patients with spinal cord injury. As presented in this case, autonomic dysreflexia can be associated with unusual symptoms such as Horner's syndrome. CASE PRESENTATION: An 18-year-old man with a traumatic spinal cord injury (C7 AIS A) experienced symptoms of unilateral Horner's syndrome: miosis, ptosis and anhidrosis which occurred simultaneously with symptoms of autonomic dysreflexia: severe headache accompanied by increasing right-sided diaphoresis, flushing, blurred vision, and increased blood pressure. These symptoms were triggered by bladder distention and were resolved after catheterisation. DISCUSSION: The patient experienced a transient Horner's syndrome due to autonomic dysreflexia. Both Horner's syndrome and symptoms of autonomic dysreflexia resolved when eliminating the eliciting stimulus, indicating that Horner's syndrome occurred due to a transient pressure on the sympathetic fibres supplying the superior cervical ganglion. Autonomic dysreflexia may have caused increased pressure disrupting the sympathetic input, thus inducing unilateral miosis, ptosis, and facial anhidrosis.

Postprandial Hypotension and Spinal Cord Injury.

Postprandial hypotension (PPH) is defined as a fall of ≥20 mmHg in systolic blood pressure (SBP) or a SBP of <90 mmHg after having been >100 mmHg before the meal within two hours after a meal. The prevalence of PPH among persons with spinal cord injury (SCI) is unknown. Ambulatory blood pressure measurement was performed in 158 persons with SCI, 109 men, median age was 59.1 years (min.:13.2; max.: 86.2). In total, 78 persons (49.4%) had PPH after 114 out of 449 meals (25.4%). The median change in SBP during PPH was -28 mmHg (min.: -87; max.: -15 mmHg) and 96% of the PPH episodes were asymptomatic. The occurrence of PPH was correlated to older age (p = 0.001), level of injury (p = 0.023), and complete SCI (p = 0.000), but not, gender or time since injury. Further studies are needed to elucidate if PPH contributes to the increased cardiovascular mortality in the SCI population.

Multimodal Biomarkers Quantify Recovery in Autoimmune Autonomic Ganglionopathy.

OBJECTIVE: The objective of this study was to evaluate patients with ganglionic acetylcholine receptor antibody (gAChR-Ab) positive autoimmune autonomic ganglionopathy using a multimodal testing protocol to characterize their full clinical phenotype and explore biomarkers to quantify immunotherapy response. METHODS: We conducted a cohort study of 13 individuals (7 women, 21-69 years of age) with autonomic failure and gAChR-Ab >100 pM identified between 2005 and 2019. From 2018, all patients were longitudinally assessed with cardiovascular, pupillary, urinary, sudomotor, lacrimal and salivary testing, and Composite Autonomic Symptom Score (COMPASS-31) autonomic symptom questionnaires. The orthostatic intolerance ratio was calculated by dividing change in systolic blood pressure over time tolerated on head-up tilt. Eleven patients received immunotherapy. RESULTS: At first assessment, all 13 patients had cardiovascular and pupillary impairments, 7 of 8 had postganglionic sudomotor dysfunction, 9 of 11 had urinary retention and xeropthalmia, and 6 of 8 had xerostomia. After immunotherapy, there were significant improvements in orthostatic intolerance ratio (33.3 [17.8-61.3] to 5.2 [1.4-8.2], p = 0.007), heart rate response to deep breathing (1.5 [0.0-3.3] to 4.5 [3.0-6.3], p = 0.02), pupillary constriction to light (12.0 [5.5-18.0] to 19.0 [10.6-23.8]%, p = 0.02), saliva production (0.01 [0.01-0.05] to 0.08 [0.02-0.20] g/min, p = 0.03), and COMPASS-31 scores (52 to 17, p = 0.03). Orthostatic intolerance ratio correlated with autonomic symptoms at baseline (r = 0.841, p = 0.01) and following immunotherapy (r = 0.889, p = 0.02). Immunofluorescence analyses of skin samples from a patient 32 years after disease onset showed loss of nerve fibers supplying the dermal autonomic adnexa and epidermis, with clear improvements following immunotherapy. INTERPRETATION: Patients with autoimmune autonomic ganglionopathy demonstrated objective evidence of widespread sympathetic and parasympathetic autonomic failure, with significant improvements after immunotherapy. Quantitative autonomic biomarkers should be used to define initial deficits, guide therapeutic decisions, and document treatment response. ANN NEUROL 2021;89:753-768.

Fatal autonomic failure due to premanifesting Parkinson's disease only diagnosed at autopsy.

A 46-year-old male had 11-year history of cryptic autonomic dysfunction. He developed a fatal autonomic failure with diffuse hypoxic brain injury. Histology examination of medulla oblongata and the celiac ganglion revealed many α-synuclein immunoreactive Lewy bodies confirming the diagnosis of premanifesting Parkinson's disease (PD). PNS involvement in PD is underappreciated.

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.

Cannabis use in persons with traumatic spinal cord injury in Denmark.

OBJECTIVE: To evaluate recreational and medical cannabis use in individuals with traumatic spinal cord injury, including reasons and predictors for use, perceived benefits and negative consequences. DESIGN: Cross-sectional survey in Denmark. METHODS: A 35-item questionnaire was sent to 1,101 patients with spinal cord injury who had been in contact with a rehabilitation centre between 1990 and 2012. RESULTS: A total of 537 participants completed the questionnaire. Of these, 36% had tried cannabis at least once and 9% were current users. Of current users, 79% had started to use cannabis before their spinal cord injury. The main reason for use was pleasure, but 65% used cannabis partly for spinal cord injury-related consequences and 59% reported at least good effect on pain and spasticity. Negative consequences of use were primarily inertia and feeling quiet/subdued. Lower age, living in rural areas/larger cities, tobacco-smoking, high alcohol intake and higher muscle stiffness were significantly associated with cannabis use. Those who had never tried cannabis reported that they would mainly use cannabis to alleviate pain and spasticity if it were legalized. CONCLUSION: Cannabis use is more frequent among individuals with spinal cord injury in Denmark than among the general population. High muscle stiffness and various demographic characteristics (lower age, living in rural areas/larger cities, tobacco-smoking and high alcohol intake) were associated with cannabis use. Most participants had started using cannabis before their spinal cord injury. There was considerable overlap between recreational and disability-related use.

Thermodysregulation in persons with spinal cord injury: case series on use of the autonomic standards.

INTRODUCTION: The ability to maintain core body temperature (Tcore) within a narrow range (37 ± 0.6 °C), despite exposure to a wide range of ambient temperatures, is essential in order to provide an optimal environment for vital organs, the central nervous system (CNS), and cellular processes to function. High-level (above T6) spinal cord injury (SCI) interrupts the autonomic nervous system's ability to carry out hypothalamic regulation of thermoregulatory mechanisms for both heat dissipation and conservation. This interruption leaves persons with high-level SCI vulnerable to hyper and hypothermia even during exposure to relatively mild ambient temperatures. The goal of the Autonomic Standards is to enable the clinician to quickly identify those individuals with SCI who may be most at risk for thermoregulatory dysfunction. CASE PRESENTATION: Case 1: Heat Exhaustion, Case 2: Heat Stroke in absence of CNS symptoms, Case 3: Heat Exhaustion. DISCUSSION: The three cases demonstrate the signs and symptoms that may accompany hyperthermia in persons with SCI. The onset may be quite rapid and the condition persistent, despite ambient temperatures being much less intense than expected to be necessary to induce similar conditions in able-bodied (AB) persons. The responses of the persons in the case studies to the temperature regulation and autonomic control of sweating sections of the Autonomic Standards would identify them as being vulnerable and warrant providing appropriate exposure guidelines and precautions to them and their caregivers.

Ultramicronized palmitoylethanolamide in spinal cord injury neuropathic pain: a randomized, double-blind, placebo-controlled trial.

Neuropathic pain and spasticity after spinal cord injury (SCI) represent significant problems. Palmitoylethanolamide (PEA), a fatty acid amide that is produced in many cells in the body, is thought to potentiate the action of endocannabinoids and to reduce pain and inflammation. This randomized, double-blind, placebo-controlled, parallel multicenter study was performed to investigate the effect of ultramicronized PEA (PEA-um) as add-on therapy on neuropathic pain in individuals with SCI. A pain diary was completed and questionnaires were completed before and after the 12-week treatment with either placebo or PEA-um. The primary outcome measure was the change in mean neuropathic pain intensity from the 1-week baseline period to the last week of treatment measured on a numeric rating scale ranging from 0 to 10. The primary efficacy analysis was the intention to treat (baseline observation carried forward). Secondary outcomes included a per protocol analysis and effects on spasticity, evoked pain, sleep problems, anxiety, depression, and global impression of change. We randomized 73 individuals with neuropathic pain due to SCI, of which 5 had a major protocol violation, and thus 68 were included in the primary analysis. There was no difference in mean pain intensity between PEA-um and placebo treatment (P = 0.46, mean reductions in pain scores 0.4 (-0.1 to 0.9) vs 0.7 (0.2-1.2); difference of means 0.3 (-0.4 to 0.9)). There was also no effect of PEA-um as add-on therapy on spasticity, insomnia, or psychological functioning. PEA was not associated with more adverse effects than placebo.

Acute complications of spinal cord injuries.

The aim of this paper is to give an overview of acute complications of spinal cord injury (SCI). Along with motor and sensory deficits, instabilities of the cardiovascular, thermoregulatory and broncho-pulmonary system are common after a SCI. Disturbances of the urinary and gastrointestinal systems are typical as well as sexual dysfunction. Frequent complications of cervical and high thoracic SCI are neurogenic shock, bradyarrhythmias, hypotension, ectopic beats, abnormal temperature control and disturbance of sweating, vasodilatation and autonomic dysreflexia. Autonomic dysreflexia is an abrupt, uncontrolled sympathetic response, elicited by stimuli below the level of injury. The symptoms may be mild like skin rash or slight headache, but can cause severe hypertension, cerebral haemorrhage and death. All personnel caring for the patient should be able to recognize the symptoms and be able to intervene promptly. Disturbance of respiratory function are frequent in tetraplegia and a primary cause of both short and long-term morbidity and mortality is pulmonary complications. Due to physical inactivity and altered haemostasis, patients with SCI have a higher risk of venous thromboembolism and pressure ulcers. Spasticity and pain are frequent complications which need to be addressed. The psychological stress associated with SCI may lead to anxiety and depression. Knowledge of possible complications during the acute phase is important because they may be life threatening and/ or may lead to prolonged rehabilitation.

Incidence of traumatic spinal cord injury worldwide: a systematic review.

PURPOSE: Traumatic spinal cord injuries (TSCI) are among the most devastating conditions in developed and developing countries, which can be prevented. The situation of TSCI around the world is not well understood which complicates the preventive policy decision making in fight against TSCI. This study was aimed to gather the available information about incidence of TSCI around the world. METHODS: A systematic search strategy was designed and run in Medline and EMBASE, along with extensive grey literature search, personal communications, website searching, and reference checking of related papers. RESULTS: Overall, 133 resources including 101 papers, 17 trauma registries, 6 conference proceedings, 5 books, 2 theses and 2 personal communication data were retrieved. Data were found for 41 individual countries. The incidence of TSCI ranges from 3.6 to 195.4 patients per million around the world. Australia, Canada, US, and high-income European countries have various valuable reports of TSCI, while African and Asian countries lack the appropriate epidemiologic data on TSCI. CONCLUSION: Data of epidemiologic information in TSCI are available for 41 countries of the world, which are mostly European and high-income countries. Researches and efforts should be made to gather information in developing and low-income countries to plan appropriate cost-effective preventive strategies in fight against TSCI.

Management of bladder dysfunction and satisfaction of life after spinal cord injury in Norway.

BACKGROUND: There is limited knowledge about bladder dysfunction and bladder management in persons with spinal cord injury (SCI) after discharge from the hospital in Norway. The impact of bladder dysfunction on satisfaction of life has been rarely explored. SETTING: Community-based survey from Norway. METHODS: An anonymous cross-sectional postal survey. A questionnaire was sent to the registered members of the Norwegian Spinal Cord Injuries Association. A total of 400 participants, with traumatic or non-traumatic SCI, received the questionnaire. RESULTS: A total of 248 subjects (62%), 180 men and 68 women, answered the questionnaire. Mean age was 54 years and mean time since injury 13.4 years. A total of 164 participants (66.1%) used intermittent catheterization for bladder emptying (48.5% women versus 72.8% men); more paraplegics than tetraplegics (77.2% versus 55.7%). Recommendations given at the Spinal Cord Units were thoroughly followed by persons who had used catheters more than 5 years. Use of incontinence pads were associated with reduced satisfaction of life. CONCLUSIONS: The most common method of management of bladder dysfunction is clean intermittent catheterization in Norway. Recommendations were followed more thoroughly by persons who have used intermittent catheterization for more than 5 years. Spinal Cord Units are important source for information and guidance.

Comment: Increased risk for type 2 diabetes in spinal cord injury.

The prevalence of diabetes is increasing globally, with an estimated 371 million individuals affected globally and 4.8 million people who died due to diabetes in 2012.(1) Type 2 diabetes accounts for at least 90% of all cases of diabetes, and the number of people with type 2 diabetes is increasing in every country.(1) Abnormalities of carbohydrate and lipid metabolism are more common among people in the spinal cord injury (SCI) population than in the able-bodied population.(2) There is increased morbidity and mortality due to type 2 diabetes in SCI.

CFS in Children and Adolescent: Ten Years of Retrospective Clinical Evaluation.

Aim. To estimate number of children being diagnosed with chronic fatigue syndrome (CFS). Methods. For a period of 10 years (2002-2011) data from children being referred for fatigue symptoms were collected retrospectively. Results. Thirty-seven children were referred. Four were excluded due to incorrect coding. Six (18%) patients received other diagnoses at the end of evaluation time. Of the 27 who received the diagnosis G93.3, four had a previous chronic illness, while 23 patients were previously healthy. All patients reported onset of fatigue symptom in relation to an infection, and all tested positive for IgG to either Epstein-Barr virus, cytomegalovirus or borrelia, indicating previous infection. There were 16 (59%) boys among the 27 patients. The mean age at the debut of fatigue symptoms was 141 months (SD 30) for boys and 136 months (SD 31) for girls, respectively. Being underweight, defined as BMI < 17.5, was found in 12 (44%) patients. Conclusion. An increasing number of children and adolescents are evaluated for CFS. The clinical assessment of children and adolescents with possible CFS need systematically evaluation. Nutritional status, possible eating disorder, and psychosocial issues need to be addressed and evaluated carefully. A multidisciplinary approach is essential when assessing CFS in children and adolescents. There is a need for European guidelines.