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Study Objectives: The objective of this study was to discern distinguishing characteristics of sleep-related breathing disorders in individuals with chronic spinal cord injury (CSCI) compared with participants without CSCI. Additionally, the study investigated factors associated with sleep-related breathing disorder severity., Methods: This is a cross-sectional analysis of 123 individuals without CSCI, 40 tetraplegics, and 48 paraplegics who underwent attended or partially supervised full polysomnography for suspected sleep-related breathing disorders in a rehabilitation center. Polysomnographic, transcutaneous capnography, and clinical data were collected and compared between the groups., Results: Among tetraplegics, apnea-hypopnea index ≥ 30 events/h (67.5%, P = .003), central apnea (17.5%, P = .007), and higher oxygen desaturation index (80.0%, P = .01) prevailed. Sleep-related hypoventilation was present in 15.4% of tetraplegics and 15.8% of paraplegics, compared with 3.2% in participants without CSCI ( P = .05). In the group without CSCI and the paraplegic group, snoring and neck circumference were positively correlated with obstructive sleep apnea (OSA) severity. A positive correlation between waist circumference and OSA severity was identified in all groups, and multivariate logistic regression analysis showed that loud snoring and waist circumference had the greatest impact on OSA severity., Conclusions: Severe OSA and central sleep apnea prevailed in tetraplegic participants. Sleep-related hypoventilation was more common in tetraplegics and paraplegics than in participants without CSCI. Loud snoring and waist circumference had an impact on OSA severity in all groups. We recommend the routine implementation of transcutaneous capnography in individuals with CSCI. We underscore the significance of conducting a comprehensive sleep assessment in the rehabilitation process for individuals with CSCI., Citation: Souza Bastos P, Amaral TLD, Yehia HC, Tavares A. Prevalences of sleep-related breathing disorders and severity factors in chronic spinal cord injury and abled-bodied individuals undergoing rehabilitation: a comparative study. J Clin Sleep Med . 2024;20(7):1119-1129., (© 2024 American Academy of Sleep Medicine.)

Study Design: Retrospective longitudinal cohort study of veterans with SCI., Objectives: Spinal cord injury (SCI) is associated with an increased risk of developing diabetes mellitus (DM), likely due to body composition alterations and autonomic nervous system dysfunction. These factors are more pronounced in persons with tetraplegia (TP) versus paraplegia (PP). However, the effect of level of injury (LOI) on DM incidence is largely unknown. Therefore, the objective is to examine the effect of LOI on DM incidence in persons with SCI., Setting: South Texas Veterans Health Care System., Methods: We obtained electronic record data on age, sex, race/ethnicity, LOI and HbA1c concentration from January 1st 2001 through December 31st 2021. Cox proportional hazard regression analyses were used to assess the association between LOI, DM and all-cause mortality., Results: Among 728 non-diabetic veterans with SCI (350 TP/ 378 PP, 52 ± 15 years, 690 male/38 female) 243 developed DM, of which 116 with TP and 127 with PP. Despite chronological variations between TP and PP, DM risk over the entire follow-up did not differ between the groups (hazard ratio (HR): 1.06, 95% CI: 0.82-1.38). Mortality was higher in TP versus PP (HR: 1.40, 95% CI: 1.09-1.78). However, developing DM did not increase the risk of death, regardless of LOI (HR: 1.07, 95% CI: 0.83-1.37)., Conclusion: Despite chronological variations between both groups, the level of injury had minimal effect on long-term DM development in this cohort of veterans with SCI. Sponsorship NIH (DK105379; MS), RR&D SPiRE (I21RX003724-01A1; MT and SH)., (© 2024. The Author(s), under exclusive licence to International Spinal Cord Society.)

Study Design: A single-institution, retrospective cohort study., Objective: The objective was to present demographic characteristics, mechanism of injuries, lengths of stay, intensive care unit (ICU) days, discharge locations, and causes of 90-day readmission for patients with subaxial spinal cord injuries resulting in paraplegia or tetraplegia., Summary of Background Data: Spinal cord injuries resulting in paraplegia or tetraplegia are rare injuries with debilitating outcomes. Numerous advances have occurred in caring for these patients, but patients still experience multiple complications. The severity of these injuries and numerous complications result in prolonged hospital stays and the need for extensive rehabilitation., Methods: Twelve patients with subaxial spinal cord injury resulting in paraplegia or tetraplegia from a level 1 adult trauma center were reviewed. The primary outcomes included hospital length of stay, ICU days, intrahospital complications, 90-day readmission rates, and discharge location. We reviewed the literature for these outcomes in spinal cord injuries., Results: For patients with subaxial spinal cord injuries resulting in paraplegia and tetraplegia, the average age was 36.0 years, and most were male [91.7% (11/12)]. The most common mechanism of injury was gunshot wounds[41.7% (5/12)]. Patients spent an average of 46.3 days in the hospital and 30.7 days in the ICU. Respiratory complications were the most common (9 patients). Fifty percent of patients (6/12) were discharged to the inpatient spinal cord rehab center, and 16.7% (2/12) expired while in the hospital. Two patients (20.0%) were readmitted within 90 days of discharge., Conclusions: Most patients with subaxial spinal cord injuries resulting in paraplegia or tetraplegia were young males with high-energy traumas. Many patients had intrahospital complications, and most were discharged to the hospital spinal rehab center. These findings likely stem from the severity of paraplegia and tetraplegia injuries and the need for rehabilitation., Competing Interests: The authors declare no conflict of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

We diagnosed a patient with dengue fever who developed acute onset of sensorimotor quadriparesis with bladder involvement, and facial nerve involvement. Despite initial negative results in routine investigations and cerebrospinal fluid analysis, spinal MRI confirmed longitudinally extensive transverse myelitis. The aetiological workup was negative, prompting an investigation into the presence of dengue in the cerebrospinal fluid, which returned positive. This case underscores the importance of considering rare neurological complications in dengue, the value of advanced diagnostic techniques and the potential effectiveness of tailored interventions in challenging cases., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Study Design: Retrospective study OBJECTIVES: To describe the presenting symptoms/signs, clinical course and outcomes in hospitalised people with spinal cord injury (SCI) and symptomatic COVID-19 infections., Setting: One university hospital and two SCI centres in Switzerland., Methods: Descriptive analysis of symptoms/signs, clinical course and outcomes of people with SCI with symptomatic COVID-19 infections and need for hospitalisation., Results: Twenty-two people with SCI were included, 15 (68%) were male, median age 64.5 years (interquartile range, IQR, 52-73 years). Nine (41%) had tetraplegia, and eight (36%) were classified with motor-complete lesions. Frequent clinical symptoms were fever (59%), coughing (54%), fatigue (50%), and dyspnoea (27%). Most frequent complications were bacterial pulmonary superinfection (18%), and acute respiratory distress syndrome (18%). Fifteen persons (68%) needed oxygen therapy during the course of hospitalisation, and 7 (32%) people were ventilated. Median length of stay (LOS) was 23 days (IQR 15-35), varying by age for people under 60 years with a median LOS of 9 days (IQR 8-27), and for those older than 60 years with a median of 34 days (IQR 17-39), respectively. In total, 3 persons (14%) died during hospitalisation, all older with paraplegia., Conclusions: Typical symptoms like fever and coughing were not present in all people. People with tetraplegia did not demonstrate worse outcomes, on the contrary, they had shorter LOS, no difference in ventilation needs, and no higher mortality compared to people with paraplegia. Older people showed longer LOS. This study recommends close supervision of the SCI population to detect early signs and symptoms of COVID-19 infection., (© 2024. The Author(s), under exclusive licence to International Spinal Cord Society.)

Traumatic spinal cord injury (SCI) is a major cause of severe and permanent disability in young adults. Overweight and obesity are commonly observed among patients affected with SCI, with reports of a prevalence of over 60 and 30% respectively. Case report: A 34 year-old woman suffering from tetraplegia after sustaining a traumatic injury to C5-C6 at age 23 as a result of a motor vehicle accident was presented to our hospital's multidisciplinary bariatric team due to class II obesity. At the time of presentation to the team, eleven years after the accident, her BMI was calculated to be 39 Kg/m 2 (weight 97 kg, height 1.57 meters). She was diagnosed with infertility while seeking pregnancy, and referred to our bariatric unit for weight loss. In addition, she had overcome the physical limitations of her injury, had a regular job and was engaged in regular physical activities such as swimming. In May 2017, she underwent laparoscopic sleeve gastrectomy (LSG) without complications and was discharged on postoperative day 2. 17 months following LSG, with a normal BMI, she became naturally pregnant. She had emergency cesarean at 35 weeks due to pneumonia but both patient and child recovered without sequelae. Currently, 4 years after surgery she maintains 37.11% total weight loss (weight 61 kg). She reports having a better quality of life with fewer medical intercurrencies. Conclusions: Patients with SCI and obesity, particularly women seeking to conceive, may be benefited by being referred to bariatric teams for assessment and treatment to improve results associated with sustained weight reduction., (© 2024. The Author(s), under exclusive licence to International Spinal Cord Society.)

Thyrotoxic periodic paralysis (TPP) is a rare complication of hyperthyroidism that manifests as painless flaccid paralysis. An East Asian man in his late 20s presented to the emergency department with an acute onset of quadriparesis associated with hypertonia and hyperreflexia. His initial symptoms and signs suggested involvement of the brain and spinal cord; however, MRI of the neuroaxis was normal. His serum potassium concentration was low, and thyroid test results were consistent with hyperthyroidism. The patient was diagnosed with TPP associated with Graves' disease and was treated with potassium supplementation, propranolol and methimazole. Motor strength improved to his baseline level of power; bulk was normal, and tone was increased. Although flaccid paralysis is a typical presentation of TPP, brisk reflexes and muscle spasticity cannot rule out this condition. This case highlights the importance of considering TPP as a possible diagnosis in patients presenting with acute quadriparesis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Study Objectives: Over 80% of people with tetraplegia have sleep-disordered breathing (SDB), but whether this is predominantly obstructive or central is unclear. This study aimed to estimate the prevalence of central sleep apnea (CSA) in tetraplegia and the contributions of central, obstructive, and hypopnea respiratory events to SDB summary indices in tetraplegia., Methods: Research and clinical data from 606 individuals with tetraplegia and full overnight polysomnography were collated. The proportions of different respiratory event types were calculated; overall and for mild, moderate, and severe disease. The prevalence of Predominant CSA (Central Apnea Index [CAI] ≥ 5 and more central than obstructive apneas) and Any CSA (CAI ≥ 5) was estimated. Prevalence of sleep-related hypoventilation (SRH) was estimated in a clinical sub-cohort., Results: Respiratory events were primarily hypopneas (71%), followed by obstructive (23%), central (4%), and mixed apneas (2%). As severity increased, the relative contribution of hypopneas and central apneas decreased, while that of obstructive apneas increased. The prevalence of Predominant CSA and Any CSA were 4.3% (26/606) and 8.4% (51/606) respectively. Being male, on opiates and having a high tetraplegic spinal cord injury were associated with CSA. SRH was identified in 26% (26/113) of the clinical sub-cohort., Conclusions: This is the largest study to characterize SDB in tetraplegia. It provides strong evidence that obstructive sleep apnea is the predominant SDB type; 9-18 times more prevalent than CSA. The prevalence of CSA was estimated to be 4%-8%, significantly lower than previously reported., (© The Author(s) 2023. Published by Oxford University Press on behalf of Sleep Research Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Solute carrier family 1 member 4 (SLC1A4), also referred to as Alanine/Serine/Cysteine/Threonine-preferring Transporter 1 (ASCT1), is a sodium-dependent neutral amino acid transporter. It is expressed in many tissues, including the brain, where it is expressed primarily on astrocytes and plays key roles in neuronal differentiation and development, maintaining neurotransmitter homeostasis, and N-methyl-D-aspartate neurotransmission, through regulation of L- and D-serine. Mutations in SLC1A4 are associated with the rare autosomal recessive neurodevelopmental disorder spastic tetraplegia, thin corpus callosum, and progressive microcephaly (SPATCCM, OMIM 616657). Psychomotor development and speech are significantly impaired in these patients, and many develop seizures. We generated and characterized a knock-in mouse model for the most common mutant allele, which results in a single amino acid change (p.Glu256Lys, or E256K). Homozygous mutants had increased D-serine uptake in the brain, microcephaly, and thin corpus callosum and cortex layer 1. While p.E256K homozygotes showed some significant differences in exploratory behavior relative to wildtype mice, their performance in assays for motor coordination, endurance, learning, and memory was normal, and they showed no significant differences in long-term potentiation. Taken together, these results indicate that the impact of the p.E256K mutation on cognition and motor function is minimal in mice, but other aspects of SLC1A4 function in the brain are conserved. Mice homozygous for p.E256K may be a good model for understanding the developmental basis of the corpus callosum and microcephaly phenotypes observed in SPATCCM patients and assessing whether they are rescued by serine supplementation., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Backgruound: In individuals with spinal cord injury (SCI), bone loss progresses rapidly to the area below the level of injury, leading to an increased risk of fracture. However, there are limited data regarding SCI-relevant characteristics for bone loss and the degree of bone loss in individuals with SCI compared with that in non-SCI community-dwelling adults., Methods: Data from men with SCI who underwent dual-energy X-ray absorptiometry at the National Rehabilitation Center (2008 to 2020) between 12 and 36 months after injury were collected and analyzed. Community-dwelling men were matched 1:1 for age, height, and weight as the control group, using data from the Korea National Health and Nutrition Examination Survey (KNHANES, 2008 to 2011)., Results: A comparison of the SCI and the matched control group revealed significantly lower hip region T-scores in the SCI group, whereas the lumbar spine T-score did not differ between groups. Among the 113 men with SCI, the paraplegia group exhibited significantly higher Z-scores of the hip region than the tetraplegia group. Participants with motor-incomplete SCI showed relatively preserved Z-scores of the hip region compared to those of the lumbar region. Moreover, in participants with SCI, the percentage of skeletal muscle displayed a moderate positive correlation with femoral neck Z-scores., Conclusion: Men with SCI exhibited significantly lower bone mineral density of the hip region than community-dwelling men. Paraplegia rather than tetraplegia, and motor incompleteness rather than motor completeness were protective factors in the hip region. Caution for loss of skeletal muscle mass or increased adiposity is also required.

Objective: To determine the positive predictive value (PPV) of a sepsis-screening protocol in patients with cervical spinal cord injury (SCI)., Design/method: Retrospective review of all patients with cervical SCI who screened positive for two or more systemic inflammatory response syndrome (SIRS) criteria while hospitalized in acute care or inpatient rehabilitation units over a 3.5-year period. Sepsis was defined by the occurrence of (1) any culture order followed by an intravenous (IV) antibiotic within 72 hours or (2) an IV antimicrobial followed by a culture order within 24 hours., Results: A total of 134 patients screened positive for two or more SIRS criteria. Of these, 36 patients (26.9%) were diagnosed with sepsis. Factors associated with a true-positive SIRS screen on multivariable analysis included American Spinal Injury Association Impairment Scale (AIS) grade A-C (vs. D; p < .001). The PPV of the screen was 38% in patients with AIS A-C and 9% in patients with AIS D. Altered mental status (AMS) was strongly associated with a diagnosis of sepsis; 16 of 18 (88.9%) of those with AMS had sepsis (p < .001). Age, sex, and neurologic level of injury were not associated with true-positive screening. For patients with new SCI, the first true-positive screen occurred a median of 31 days post-injury. The most common SIRS criteria combinations in patients with true-positive screens were elevated heart rate and either abnormal white blood cell count (43% of true positives) or abnormal temperature (26% of true positives). Abnormally low body temperature (<36°C) contributed to false-positive screening for 10 of 38 (26%) AIS D patients who screened positive., Conclusion: Sepsis screening using SIRS criteria in hospitalized patients with tetraplegia has a PPV of 26.9%; it is significantly higher in patients with AIS A-C versus D injuries. AMS, when combined with a positive SIRS screening, is strongly associated with sepsis., (© 2022 American Academy of Physical Medicine and Rehabilitation.)

Objective: High cervical spinal cord injury (SCI) results in complete loss of upper-limb function, resulting in debilitating tetraplegia and permanent disability. Spontaneous motor recovery occurs to varying degrees in some patients, particularly in the 1st year postinjury. However, the impact of this upper-limb motor recovery on long-term functional outcomes remains unknown. The objective of this study was to characterize the impact of upper-limb motor recovery on the degree of long-term functional outcomes in order to inform priorities for research interventions that restore upper-limb function in patients with high cervical SCI., Methods: A prospective cohort of high cervical SCI (C1-4) patients with American Spinal Injury Association Impairment Scale (AIS) grade A-D injury and enrolled in the Spinal Cord Injury Model Systems Database was included. Baseline neurological examinations and functional independence measures (FIMs) in feeding, bladder management, and transfers (bed/wheelchair/chair) were evaluated. Independence was defined as score ≥ 4 in each of the FIM domains at 1-year follow-up. At 1-year follow-up, functional independence was compared among patients who gained recovery (motor grade ≥ 3) in elbow flexors (C5), wrist extensors (C6), elbow extensors (C7), and finger flexors (C8). Multivariable logistic regression evaluated the impact of motor recovery on functional independence in feeding, bladder management, and transfers., Results: Between 1992 and 2016, 405 high cervical SCI patients were included. At baseline, 97% of patients had impaired upper-limb function with total dependence in eating, bladder management, and transfers. At 1 year of follow-up, the largest proportion of patients who gained independence in eating, bladder management, and transfers had recovery in finger flexion (C8) and wrist extension (C6). Elbow flexion (C5) recovery had the lowest translation to functional independence. Patients who achieved elbow extension (C7) were able to transfer independently. On multivariable analysis, patients who gained elbow extension (C7) and finger flexion (C8) were 11 times more likely to gain functional independence (OR 11, 95% CI 2.8-47, p < 0.001) and patients who gained wrist extension (C6) were 7 times more likely to gain functional independence (OR 7.1, 95% CI 1.2-56, p = 0.04). Older age (≥ 60 years) and motor complete SCI (AIS grade A-B) reduced the likelihood of gaining independence., Conclusions: After high cervical SCI, patients who gained elbow extension (C7) and finger flexion (C8) had significantly greater independence in feeding, bladder management, and transfers than those with recovery in elbow flexion (C5) and wrist extension (C6). Recovery of elbow extension (C7) also increased the capability for independent transfers. This information can be used to set patient expectations and prioritize interventions that restore these upper-limb functions in patients with high cervical SCI.

To explore the use of 5-minute heart rate variability (HRV) during inpatient rehabilitation in the acute phase of traumatic spinal cord injury (SCI)., Longitudinal observational study., Acute inpatient rehabilitation (AIR)., 10 patients with acute traumatic SCI., 5-minute HRV supine recordings twice daily on three different days per patient., HRV values were evaluated (1) within a single day (Early versus Late); (2) across the inpatient admission (initial, mid, and discharge); (3) by SCI phenotypes and by clinical outcomes (ex. pressure injuries (PI))., Patients had an average age of 38 years, 80% male, and 40% with tetraplegia. There were no HRV differences between Early and Late recordings, across the inpatient admission, demographics, or SCI phenotype. However, improvement in neurologic exam was accompanied by increased parasympathetic tone (mean RR increased by 172 ms SD 61, P = .005). Patients with PI demonstrated lower sympathetic (SNS) activity (decreased LF by 472 ms 2 SD 240, P = .049) and lower PNS activity (decreased RMSSD by 1.2 ms SD 0.5, P = .02), compared to no PI. Comparisons to uninjured reference values and chronic SCI suggest a changing autonomic nervous system (ANS) from uninjured to acute to chronic as measured by HRV., Conclusions: This preliminary evidence suggests HRV in acute SCI is stable across time and day during inpatient rehabilitation and may be correlated to clinical sequalae of ANS dysfunction and neurologic recovery. Comparisons to published work suggest that HRV may measure the progression in the ANS from acute to chronic phase after SCI.

Coronavirus disease 2019 (COVID-19) is a significant cause for intensive care unit (ICU) admission worldwide. Most COVID-19 infections are associated with lower respiratory abnormalities but it has been increasingly associated with extra-pulmonary manifestations. Guillain-Barre syndrome (GBS) is a rarely diagnosed but severe disease associated with COVID-19 infection. We describe the diagnostic process behind diagnosing GBS in an elderly male who developed acute-onset quadriparesis and respiratory muscle failure associated with severe COVID-19 pneumonia in a military ICU. A 69-year-old male was admitted to the ICU for acute hypoxemic respiratory failure due to COVID-19 pneumonia. He was subsequently intubated and treated with dexamethasone and remdesivir with improvement. On hospital day 32, the patient was extubated. Three days later, he developed acute, symmetric limb quadriparesis and respiratory muscle failure requiring reintubation. Analysis of his cerebrospinal fluid showed a cytoalbuminologic dissociation, and electromyography/nerve conduction study showed slowed nerve conduction velocity. These findings are consistent with GBS. Blood cultures, serum polymerase chain reaction testing, and clinical symptoms were not suggestive of other common pathogens causing his GBS. The patient's acute GBS in the setting of recent severe COVID-19 infection strongly suggests association between the two entities, as supported by a growing body of case literature. The patient was subjected to intravenous immunoglobulin treatment and was discharged with greatly improved strength in the upper and lower extremities. Our goal in describing this case is to highlight the need for providers to consider, accurately diagnose, and treat GBS as a consequence of severe COVID-19 infection., (Published by Oxford University Press on behalf of the Association of Military Surgeons of the United States 2021. This work is written by (a) US Government employee(s) and is in the public domain in the US.)

Study Design: Retrospective chart review., Objectives: Sepsis is a leading preventable cause of death in patients with chronic spinal cord injury (SCI). Individuals with tetraplegia may exhibit different signs and symptoms of infection compared to those with paraplegia. In this study, we examine differences in vital signs (VS) and mental status between septic patients with tetraplegia and paraplegia with the goal of improving early identification of sepsis in this population., Setting: Veterans hospital in Washington, USA., Methods: Participants consisted of 19 patients with tetraplegia and 16 with paraplegia who were transferred from an SCI Service to a higher level of care with sepsis between June 1, 2010 and June 1, 2018 (n = 35). We compared VS between patients with tetraplegia and paraplegia at baseline and during sepsis including temperature, heart rate (HR), and blood pressure as well as presence/absence of altered mental status (AMS)., Results: While there were no significant VS differences between groups at baseline, septic patients with tetraplegia had lower maximum temperature (38.2 °C versus 39.2 °C, p = 0.003), lower maximum HR (106 versus 124 beats/minute, p = 0.004), and more frequent AMS compared to septic patients with paraplegia (79% versus 31%, p = 0.007)., Conclusion: Patients with tetraplegia may not be able to mount fever and tachycardia to the same degree as patients with paraplegia and may be more prone to developing AMS during sepsis. These findings suggest that changes to VS parameter cut-offs may improve sensitivity and be useful in identifying sepsis earlier in the tetraplegic population., (© 2022. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.)

Context: Autonomic dysreflexia (AD), characterized by a transient increase in systolic blood pressure (BP), is experienced by individuals with spinal cord injury (SCI) and can be purposefully induced ('boosting') to counteract autonomic dysfunction that impairs cardiovascular responses to exercise. Herein, we demonstrate the impact of unintentional boosting observed during cardiopulmonary exercise testing (CPET) in an inactive male with SCI (C5, motor-complete)., Findings: On two separate occasions the individual performed a standard arm-crank CPET (1-min stages, 7W increase in resistance) following by a longer CPET (4-min stages, 12W increase in resistance), both to volitional exhaustion. The second CPET was performed to confirm the accuracy of exercise intensity prescription and verify peak exercise parameters. Immediately following the second CPET on the initial visit, the individual reported symptoms of AD, verified as a 58mmHg increase in systolic BP from baseline. Relative to the first CPET, performed only 35 min earlier, there were pronounced differences in peak exercise responses. In comparison to the longer CPET performed on the second visit without a concomitant episode of AD (thereby controlling for the type of CPET protocol administered), peak exercise outcomes were considerably elevated: power output (Δ19W), oxygen uptake (Δ3.61 ml·   kg· -1 min -1 ), ventilation (Δ11.4 L ·min -1 ) and heart rate (Δ9 b·min -1 )., Conclusion/clinical Relevance: This case raises important considerations around the nuances of CPET in this population. In individuals susceptible to BP instability, the physiologically boosted state may explain a significant proportion of the variance in peak aerobic capacity and should be closely monitored before and after clinical CPET.

Introduction: Neurofibromatosis type 1 (NF-1) is an autosomal-dominant disorder affecting 1 in 3000 individuals worldwide. NF-1 is characterized by café-au-lait macules and peripheral nerve sheath tumors. Patients with NF-1 frequently exhibit bony dysplasia, including spinal deformities such as scoliosis or kyphosis, pseudarthrosis of the tibia, and soft tissue tumors. Some patients with NF-1 exhibit spinal changes, including acutely angled cervical kyphosis. Prior studies have also described arteriovenous (AV) fistulas in individuals with NF-1, as well as a predisposition to cervical fistulas which display symptoms secondary to mass effect, rather than hemorrhage. Sometimes, fistulas are incidentally detected during evaluations for cervical kyphotic deformities., Case Presentation: The case herein describes a patient with NF-1 who exhibited a severe cervical spinal kyphotic deformity and a vertebral AV fistula. The patient initially presented with neck pain that radiated to all four limbs and resulted in limb weakness., Discussion: Spinal kyphosis is common in patients with NF-1, and if dystrophic changes are noted on plain radiographs, MRI should be considered for the further survey of potential spinal vascular lesions., (© 2022. The Author(s), under exclusive licence to International Spinal Cord Society.)

Context: Osteoporosis is a known complication in spinal cord injury patients and can result in an increased risk of fractures and associated morbidity. Bone demineralization is most common in long bones below the level of injury. The pathogenesis is complex and not fully understood. Findings: We present the case of a 65-year-old male with chronic spinal cord injury who was found to have multiple vertebral compression fractures causing autonomic dysreflexia and new onset spasticity. Conclusion/Clinical Relevance: This case illustrates the need for improved awareness, diagnosis, and prevention for this disease process.

Background: Puerperal mastitis, as well as breast abscess, are common complications that can arise during the breastfeeding period. Acute-onset quadriplegia has been described as a complication of bacterial or viral infections. Here we report a case of puerperal breast abscess with transient acute-onset staphylococcal mediated quadriplegia., Main Issue: A 28-year-old lactating Caucasian woman presented at our tertiary perinatal center 31 days postpartum with weakness of all four limbs, the signs and symptoms of mastitis in her left breast and a possible breast abscess with redness, a painful breast lump, and fever. A few hours after admission, the participant developed a proximal quadriplegia., Management: The participant was admitted to the intensive care unit for monitoring. The neurologists treated her as a possible case of Guillain-Barré syndrome and administered intravenous immunoglobulin therapy for 5 days. The breast abscess was diagnosed via ultrasound and treated by regular aspiration of pus and intravenous antibiotic therapy with ampicillin and sulbactam as Staphylococcus aureus was isolated from breast abscess fluid. Fifteen days after the first symptoms the participant recovered completely and could breastfeed her son exclusively, even though she developed a galactocele on the affected side., Conclusion: We report a possible association between mastitis and abscess formation, common breastfeeding issues, and transient acute onset staphylococcal mediated quadriplegia. To the best of our knowledge this is the only case in the medical literature. Independent of the systemic complications, antibiotic treatment and regular abscess aspirations have proven to be a key strategy to the resolution of puerperal mastitis and breast abscess.

Objectives: Chronic ventilator dependency in cervical tetraplegia is associated with substantial morbidity. When non-invasive weaning methods have failed the primary surgical treatment is diaphragm pacing. Phrenic nerve integrity and diaphragm motor units are requirements for effective pacing but may need to be restored for successful weaning. A surgical algorithm that includes: 1. Diaphragm pacing, 2. Phrenic nerve reconstruction, and 3. Diaphragm muscle replacement, may provide the capability of reducing or reversing ventilator dependency in virtually all cervical tetraplegics. Design: Prospective case series. Setting: A university-based hospital from 2015 to 2019. Participants : Ten patients with ventilator-dependent cervical tetraplegia. Interventions : I. Pacemaker alone, II. Pacemaker + phrenic nerve reconstruction, or III. Pacemaker + diaphragm muscle replacement. Outcome measures : Time from surgery to observed reduction in ventilator requirements (↓VR), ventilatory needs as of most recent follow-up [no change (NC), partial weaning (PW, 1-12 h/day), or complete weaning (CW, >12 h/day)], and complications. Results: Both patients in Group I achieved CW at 6-month follow-up. Two patients in Group II achieved CW, and in another two patients PW was achieved, at 1.5-2-year follow-up. The remaining two patients are NC at 6 and 8-month follow-up, respectively. In group III, both patients achieved PW at 2-year follow-up. Complications included mucous plugging ( n  = 1) and pacemaker malfunction requiring revision ( n  = 3). Conclusion: Although more investigation is necessary, phrenic nerve reconstruction or diaphragm muscle replacement performed (when indicated) with pacemaker implantation may allow virtually all ventilator-dependent cervical tetraplegics to partially or completely wean.

Study Design: Retrospective descriptive study., Objectives: To identify the characteristics of and epidemiological trends in traumatic spinal cord injuries (TSCIs) in Korea from 1990 to 2019., Setting: National Rehabilitation Center affiliated with the Ministry of Health and Welfare in Korea., Methods: The medical records of 3395 individuals with TSCIs were retrospectively reviewed. Three groups were formed based on onset period (1990-1999, 2000-2009, and 2010-2019) and six groups based on age (≤15, 16-30, 31-45, 46-60, 61-75, and ≥76 years). Pearson's chi-square and analysis of variance tests were used for statistical analysis., Results: From 1990 to 2019, the mean age (standard deviation, [SD]) at the time of injury increased from 32.4 (SD = 12.4) years in the 1990s to 47.1 (SD = 16.2) years in the 2010s (F = 222.317 p = <0.001). Land transport and falls were the most common causes of TSCIs. The number of injuries from land transport gradually decreased, while that from falls increased (24.9% in 1990s to 46.3% in 2010s [χ 2  = 134.415 p < 0.001]). In the >60 years group, falls were the most common cause of injury, which resulted in 42.9% TSCIs in the 1990s to 59.1% in the 2010s (χ 2  = 10.398, p > 0.05). Tetraplegia (n = 769, 58.6%) was more common than paraplegia; incomplete tetraplegia (entire population: =564, 43%; >60 years group: n = 186, 43%) was the highest in the 2010s., Conclusions: Falls have been the most common cause of TSCIs after 2010s. Implementing national education and campaigns for preventing falls is important to reduce/prevent TSCIs caused by falls in the aged population., (© 2021. The Author(s), under exclusive licence to International Spinal Cord Society.)

Acute-onset quadriparesis is not only debilitating and a grave concern for the patient but also perturbs the clinician as it demands early diagnosis and prompt management to prevent catastrophic outcome due to respiratory failure. Guillain-Barré syndrome (GBS) and hypokalaemia are notorious causes of acute-onset lower motor neuron (LMN) quadriparesis and warrant a rapid evaluation to necessitate early management. However, coexistence of these two entities is extremely rare and may pose a diagnostic and therapeutic challenge and mandates exclusion of either condition to avoid a poor outcome. We hereby report a case of a young woman who presented with an acute-onset LMN quadriparesis, initially found to have significant hypokalaemia with poor response to supplementation and was further evaluated to have an axonal variant of GBS., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Case: A 44-year-old man developed urinary retention due to a spontaneous spinal epidural hematoma (SSEH) at the cervicothoracic junction, without paraplegia. Symptoms improved with surgical treatment., Conclusion: SSEH is rare and causes acute neck or back pain and progressive paralysis. Patients with advanced myelopathy due to spinal cord compression lesion including SSEH often present with bladder and bowel disorders after exacerbation of quadriplegia. However, SSEH can cause predominant bladder and bowel disorders without paraplegia or quadriplegia. Physicians should consider that there can be a manifestation of myelopathy with bladder and bowel dysfunction without quadriplegia., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSCC/B871)., (Copyright © 2022 by The Journal of Bone and Joint Surgery, Incorporated.)

Study Design: Qualitative Survey study., Objective: One might assume that those with higher and more complete spinal cord injuries are at higher risk of depression and anxiety. The objective of this study was to assess the association between level and severity of injury with the degree of anxiety and depression in chronic spinal cord injury., Setting: Spinal Cord Injury Rehabilitation outpatient clinic., Methods: 49 subjects were recruited from our research database. Time from injury ranged from 6 months to 20+ years. Sample included 23 motor complete and 26 motor incomplete participants; of these 24 were people with paraplegia, 25 were people with tetraplegia. They answered HADS, BDI-FS and PHQ-9 questionnaires. Participants were grouped based on time post injury and then were further divided by motor complete (AIS A/B) and incomplete (AIS C/D) and level (tetraplegia cf. paraplegia). Two-sample t-tests were conducted to evaluate the differences in depression and anxiety scores, between individuals with different completeness and injury type., Results: There were no significant differences amongst the groups tested regarding severity of depression and anxiety, however, those with paraplegia or incomplete injuries had higher but not significantly higher average scores for depression and anxiety., Conclusion: We found there were no statistically significant differences in depression and anxiety scores among the groups. These results suggest that all people with SCI may be at similar risk of depression and anxiety, regardless of SCI severity and level., (© 2022. The Author(s), under exclusive licence to International Spinal Cord Society.)

Sensorimotor function of patients with spinal cord injury (SCI) is commonly assessed according to the International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI). From the ISNCSCI segmental motor and sensory assessments, upper and lower extremity motor scores (UEMS and LEMS), sum scores of pinprick (PP) and light touch (LT) sensation, the neurological level of injury (NLI) and the classification of lesion severity according to the American Spinal Injury Association Impairment Scale (AIS) grade are derived. Changes of these parameters over time are used widely to evaluate neurological recovery. Evaluating recovery based on a single ISNCSCI scoring or classification variable, however, may misestimate overall recovery. Here, we propose an Integrated Neurological Change Score (INCS) based on the combination of normalized changes between two time points of UEMS, LEMS, and total PP and LT scores. To assess the agreement of INCS with clinical judgment of meaningfulness of neurological changes, changes of ISNCSCI variables between two time points of 88 patients from an independent cohort were rated by 20 clinical experts according to a five-categories Likert Scale. As for individual ISNCSCI variables, neurological change measured by INCS is associated with severity (AIS grade), age, and time since injury, but INCS better reflects clinical judgment about meaningfulness of neurological changes than individual ISNCSCI variables. In addition, INCS is related to changes in functional independence measured by the Spinal Cord Independence Measure (SCIM) in patients with tetraplegia. The INCS may be a useful measure of overall neurological change in clinical studies.

Return to work is a challenging aspect of community integration for individuals with disabilities. The reintegration of individuals with spinal cord injury (SCI) is multifactorial; hence, regional challenges need to be investigated in the context of their clinical attributes and perceptions. A total of 121 male participants above 18 years of age with diagnosis of SCI and living at home were included in this cross-sectional survey. The study was conducted at a tertiary care rehabilitation facility in Saudi Arabia. The most common reported clinical barriers to employment were mobility, bladder incontinence, spasticity, musculoskeletal pain, and neuropathic pain. Bladder incontinence and musculoskeletal pain were the most common perceived clinical barriers for individuals with paraplegia and tetraplegia, respectively. A significant difference was observed for bowel incontinence as a reported barrier ( p = 0.024) among adults less than thirty years of age in comparison with those older than thirty years. Spasticity as a barrier was reported more among patients who were older than thirty years (54.0%) compared to those younger than thirty years of age (37.9%) ( p = 0.077). Twenty-two (23.7%) participants with paraplegia reported transfers as a perceived barrier to employment, which was significant ( p = 0.014), and it was also reported as a significant barrier ( p = 0.001) in individuals with tetraplegia (56%). This study shows that clinical conditions associated with SCI are considered potential barriers to employment by individuals with SCI. In terms of priority, the perceived barriers between individuals with tetraplegia and paraplegia were mostly different. This shows the need to consider relevant secondary health care conditions in goal setting while planning for employment in individuals with SCI.

Sjogren syndrome is an autoimmune disease characterised by lymphocytic infiltration and inflammation of the exocrine glands resulting in decreased secretion of involved glands which manifests mostly as dry eye and dry mouth. The prevalence of the disease is reported to be about 10.3 per 10,000 population. It is more common in females with a male: female ratio of 16:1. Extra glandular manifestations are seen in up to 1/3rd of the cases. Renal involvement is seen in 4.9% of patients with Sjogren syndrome., Material: Here we present three cases of Sjogren Syndrome who presented to our hospital with hypokalaemic quadriparesis., Observation: On evaluation all three of the patients were found to have renal tubular acidosis type 1. None of these patients had any symptom of Sjogren syndrome before the onset of quadriparesis. All of these patients had acute onset progressive areflexic quadriparesis with involvement of facial muscles and drooping of eyelids without sensory or bladder bowel involvement. One of these patients had respiratory muscle paralysis severe enough to mandate mechanical ventilation. Arterial Blood Gas analysis and urine electrolyte analysis were suggestive of type 1 renal tubular acidosis. ANA positive in 2 of the 3 patients. Anti-SSA & anti-SSB antibodies were positive in all three patients. Supportive measures and IV fluid and electrolyte correction was done. There was complete recovery of power in all three patients and were discharged on oral medications.Renal Tubular Acidosis is characterised by inability of the nephrons to maintain physiologic acid base balance. This usually results from a defect in the tubular transport mechanisms. Distal Renal tubular acidosis (as in these patients) is further defined by an alkalotic urinary pH(&gt;5.5) and profound hypokalemia due to impairment in H+ secretion in ditstal tubular alpha-intercalated cells. Owing to this imbalance of ionic transport in distal tubules there can be nephrocalcinosis, nephrolithiasis, rickets and severe muscle weakness. Sjogren syndrome is one of the etiologies leading to development of T1RTA.T1RTA can be the presenting feature of Sjogren Syndrome., Conclusion: Though a rare manifestation of the disease if can be the presenting symptom. Work up for RTA (ABG, urine electrolytes, Urine PH and osmolarity etc) in patients with hypokalaemic paresis can help establish the etiological diagnosis(ANA, anti-SSA,anti-SSB) and help prevent future relapses of the disease., (© Journal of the Association of Physicians of India 2011.)

Objective: To determine the differences in neurologic recovery in persons with initial cervical American Spinal Cord Injury Association Impairment Scale (AIS) grades A and B over time., Design: Retrospective analysis of data from people with traumatic cervical spinal cord injury (SCI) enrolled in the National Spinal Cord Injury Model Systems (SCIMS) database from 2011-2019., Setting: SCIMS centers., Participants: Individuals (N=187) with traumatic cervical (C1-C7 motor level) SCI admitted with initial AIS grade A and B injuries within 30 days of injury, age 16 years or older, upper extremity motor score (UEMS) ≤20 on both sides, and complete neurologic data at admission and follow-up between 6 months and 2 years., Interventions: Not applicable., Main Outcome Measures: Conversion in AIS grades, UEMS and lower extremity motor scores (LEMS), and sensory scores., Results: Mean time to initial and follow-up examinations were 16.1±7.3 days and 377.5±93.4 days, respectively. Conversion from an initial cervical AIS grades A and B to motor incomplete status was 13.4% and 50.0%, respectively. The mean UEMS change for people with initial AIS grades A and B did not differ (7.8±6.5 and 8.8±6.1; P=.307), but people with AIS grade B experienced significantly higher means of LEMS change (2.3±7.4 and 8.8±13.9 (P≤.001). The increased rate of conversion to motor incomplete status from initial AIS grade B appears to be the primary driving factor of increased overall motor recovery. Individuals with initial AIS grade B had greater improvement in sensory scores., Conclusions: While UEMS recovery is similar in persons with initial AIS grades A and B, the rate of conversion to motor incomplete status, LEMS, and sensory recovery are significantly different. This information is important for clinical as well as research considerations., (Copyright © 2021. Published by Elsevier Inc.)

Study Design: Retrospective descriptive study., Objectives: To summarize epidemiologic data on nontraumatic spinal cord injury (NTSCI) for the last 30 years and compare these findings to data from individuals with traumatic spinal cord injury (TSCI)., Setting: National Rehabilitation Center affiliated with the Ministry of Health and Welfare in Korea., Methods: The medical records of 948 individuals with NTSCIs were retrospectively reviewed. Three groups were created based on onset period (1990-1999, 2000-2009, 2010-2019) and six groups based on age (≤15, 16-30, 31-45, 46-60, 61-75, and ≥76 years). Pearson's chi-square and analysis of variance tests were used for statistical analysis., Results: The male-to-female ratio was 1.30:1 for NTSCI individuals and 3.47:1 for TSCI individuals. The mean age (standard deviation [SD]) at the time of injury increased from 38.7 (SD = 18.1) years in the 1990s to 55.5 (SD = 16.6) years in the 2010s (F = 44.597, p ≤ 0.001). Vertebral column degenerative disorder was the primary cause of injury in 28.9% of the cases, and the most common neurologic level was T12-L2. Paraplegia occurred in 74.6% of cases, and tetraplegia in 25.4% of cases. The proportion of NTSCI individuals increased from 11.2% to 29.3% during the observation period., Conclusions: The proportion of NTSCI among total spinal cord injuries has increased in Korea over the last 30 years (1990-2019). Compared to individuals with TSCI, those with NTSCI had a higher age at onset, with different male-to-female ratios. Our study is the most comprehensive investigation of NTSCI in Korea, and our findings can inform research directions and medical guidelines., (© 2021. The Author(s), under exclusive licence to International Spinal Cord Society.)

Study Design: Pre-post design; before and after vibration intervention., Objectives: To explore effect of a focal, self-applied upper extremity (UE) vibration intervention on UE spasticity for individuals with tetraplegia. The secondary objectives were to explore the acceptability and ease of use of this intervention., Setting: Specialty rehabilitation center in Georgia, USA., Methods: Eleven participants each completed one session of focal, self-applied vibration to the UEs. UE spasticity was measured using the Modified Ashworth Scale (MAS). UE function was measured using the Box & Block (B&B) test which measures the effectiveness of grasp, transport, and release. These measurements were taken pre-intervention, immediately post-intervention, and 20 min post-intervention. Participants also self-reported the acceptability and usability of the intervention, their perception of change in their spasticity and completed the Qualities of Spasticity Questionnaire., Results: In the full group analysis of the spasticity measures, no significant effects were found. Subgroup analysis, however, indicated participants with higher spasticity demonstrated significantly more change on the MAS than the lower spasticity group. Analysis did not reveal any impact of the intervention on UE function as measured by the B&B. Ten out of eleven participants indicated that they agreed or strongly agreed that the intervention would be valuable to have at home., Conclusions: Participants with higher spasticity demonstrated decreased spasticity after focal UE vibration, although there was no clear effect on grasp, transport and release function. Participants were satisfied with the intervention; most were able to use it independently and indicated it would be a valuable home intervention., (© 2022. The Author(s), under exclusive licence to International Spinal Cord Society.)

Background: Research has evaluated the effect of surgical timing on patient functional recovery in individuals with spinal cord injury (SCI); however, there is a critical need to assess how demographics, clinical characteristics, and process of care affect functional outcomes., Objective: We examined the association between demographic, clinical, and process of care factors with post-acute functional status (locomotion and transfer mobility scores) and discharge disposition (home vs. institution) in individuals with SCI., Methods: This study was a retrospective cohort analysis of the Pennsylvania Trauma Systems Outcomes Study (PTOS) database for individuals with traumatic SCI ( N  = 2223). We conducted multinomial and binomial logistic regression analyses to examine post-acute functional status and discharge disposition, respectively., Results: The results indicated that older age, longer length of stay, lower Glasgow Coma Scale (GCS), higher Injury Severity Score (ISS), and individuals with tetraplegia had significantly lower motor functional score at discharge from an acute hospital. In addition, older age, individuals with public-sponsored insurance, longer length of stay, lower GCS, and higher ISS had significantly higher odds of being discharged to an institution, as compared to home. Individuals of Hispanic ethnicity, as compared to White, had lower odds of being discharged to an institution., Conclusions: The regression models developed in this study were able to better classify discharge destinations compared to the functional outcomes at discharge from the acute hospital. Further research is necessary to determine how these factors and their associations vary nationally across the US, which have the potential to inform trauma and acute care post-SCI.

Pathogenic variants in GEMIN4 contribute to a hereditary disorder characterized by neurodevelopmental features, microcephaly, cataracts, and renal abnormalities (known as NEDMCR). To date, only two homoallelic variations have been linked to the disease. Moreover, clinical features associated with the variants have not been fully elucidated yet. Here, we identified a novel variant in GEMIN4 (NM&#95;015721:exon2:c.440A>G:p.His147Arg) in two siblings from a consanguineous Saudi family by using whole exome sequencing followed by Sanger sequence verification. We comprehensively investigated the patients' clinical features, including brain imaging and electroencephalogram findings, and compared their phenotypic characteristics with those of previously reported cases. In silico prediction and structural modeling support that the p.His147Arg variant is pathogenic.

Context: Takotsubo cardiomyopathy (TC) is a transient stress-induced cardiomyopathy with left ventricular dysfunction of unknown etiology. A well accepted theory for the pathophysiology of TC is attributed to a massive catecholamine release [1]. This case report will review a chronic tetraplegia patient who was diagnosed with TC after a severe episode of autonomic dysreflexia (AD). He experiences mild episodes of AD several times a day; however, he had never experienced the severity of symptoms that was associated with this episode which led to his hospitalization. Autonomic dysreflexia is a syndrome of imbalanced sympathetic input secondary to loss of descending central sympathetic control in spinal cord injury due to noxious stimuli below the level of the injury, which occurs when the injury level is at thoracic level 6 (T6) or above [2]. Findings: In this specific case, it is presumed that the massive catecholamine release associated with this severe AD episode resulted in TC. Although TC has been diagnosed after other instances of acute stress, it is unknown for it to be diagnosed after AD in a chronic setting. Clinical Relevance: The long-term effects of AD have not been well studied, and this case illustrates the importance of education to recognize and manage AD in the spinal cord patient who frequently has episodes of AD.

Previous studies showed reorganised and/or altered activity in the primary sensorimotor cortex after a spinal cord injury (SCI), suggested to reflect abnormal processing. However, little is known about whether somatotopically specific representations can be activated despite reduced or absent afferent hand inputs. In this observational study, we used functional MRI and a (attempted) finger movement task in tetraplegic patients to characterise the somatotopic hand layout in primary somatosensory cortex. We further used structural MRI to assess spared spinal tissue bridges. We found that somatotopic hand representations can be activated through attempted finger movements in the absence of sensory and motor hand functioning, and no spared spinal tissue bridges. Such preserved hand somatotopy could be exploited by rehabilitation approaches that aim to establish new hand-brain functional connections after SCI (e.g. neuroprosthetics). However, over years since SCI the hand representation somatotopy deteriorated, suggesting that somatotopic hand representations are more easily targeted within the first years after SCI., Competing Interests: SK, DP, MV, PF, NW No competing interests declared, (© 2021, Kikkert et al.)

Aim: Spinal cord injury (SCI) patients may have intestinal dysmotility and digestive symptoms that are associated with small intestinal bacterial overgrowth (SIBO). The aim of this study is to describe the prevalence of SIBO in SCI patients and the risk factors of its development., Methods: Twenty-nine consecutive SCI patients were studied (10 women/19 men; mean age 47 years), 16 with subacute injuries (<9 months) and 13 with chronic injuries (>1 year). Nine patients were affected by tetraplegia and 15 by paraplegia. Each patient underwent a glucose breath test according to the North American Consensus and the presence of abdominal symptoms was evaluated during the test. The results were compared with 15 non-neurological patients with SIBO., Results: Six patients tested positive for SIBO (21%), all of them affected by SCI in the subacute phase, 6/16 vs. 0/13 in the chronic phase (P<.05) and the majority with tetraplegia, 5/9 vs. 1/19 with paraplegia (P<.05). No statistically significant relationship was found with other clinical characteristics. All the tests were positive for methane or mixed (methane and hydrogen), while only 67% of the controls had methane-predominant production (P>.05)., Conclusion: SCI patients can develop SIBO, more frequently in the subacute phase and in tetraplegic patients, highlighting a high production of methane. This complication should be considered in neurogenic bowel management., (Copyright © 2021 Elsevier España, S.L.U. All rights reserved.)

A 44-year-old male person with tetraplegia (C-5 AIS-A (American Spinal Cord Injury Association Standard Neurological Classification of Spinal Cord Injury Impairment Scale)) developed urinary tract infection and received appropriate antibiotic. Subsequently, he started sweating and shivering when he was sitting up; these symptoms resolved while lying on his back. Autonomic dysreflexia triggered by truncal movements continued to occur for 3 months. CT of the spine showed L5-S1 discitis. MRI of the spine showed diffuse marrow oedema in L5 and S1 vertebrae and a large abscess at L5/S1 level. Blood culture yielded Serratia marcescens sensitive to meropenem. Meropenem followed by ertapenem was given for 12 weeks. After 11 months, MRI showed resolution of discitis and epidural collection. The patient was able to sit up for 9 hours without developing autonomic dysreflexia. If a person with cervical spinal cord injury develops posture-related autonomic dysreflexia (eg, in sitting position, lying on sides or while hoisted), disco-vertebral pathology should be suspected., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Respiratory complications (RC) are a leading cause of death after spinal cord injury (SCI) due to compromised immune function and respiratory muscle weakness. Thus, individuals with SCI are at high risk of developing COVID-19 related RC. Results of a SCI clinical trial showed a supervised respiratory muscle training (RMT) program decreased risk of developing RC. The feasibility of conducting unsupervised RMT is not well documented. Four publications (n = 117) were identified in which unsupervised RMT was performed. Significant improvements in respiratory outcomes were reported in two studies: Maximal Inspiratory and Expiratory Pressure (MIP40% and MEP25%, respectively), Peak Expiratory Flow (PEF9%), seated and supine Forced Vital Capacity (FVC23% and 26%, respectively), and Peak Cough Flow (28%). This review and case report will attempt to show that an inspiratory muscle training (IMT) home exercise program (HEP) is feasible and may prepare the respiratory system for RC associated with COVID-19 in patients with SCI., Case Presentation: A 23-year-old with tetraplegia (P1), history of mechanical ventilation, and hospitalization for RC, completed 27 IMT HEP sessions in one month. MIP and sustained MIP (SMIP) increased from baseline by 28% and 26.5%, respectively. Expiratory volumes and rates also improved (FVC, FEV1, and PEF: 11.7%, 8.3%, and 14.2%, respectively)., Discussion: The effects of COVID-19 on patients with SCI remains inconclusive, but recent literature and the results of this case suggest that unsupervised IMT is feasible and may limit the severity of RC in patients with SCI who contract COVID-19.

Introduction: Blood flow-restricted exercise (BFRE) appears to hold considerable potential in spinal cord injury (SCI) rehabilitation, due to its ability to induce beneficial functional changes and morphological alterations from low-intensity, low-load exercise. However, it remains unclear if this training approach is feasible and safe in individuals with autonomic dysreflexia (AD)., Case Presentation: A 23-year-old male with traumatic, cervical (C6), motor-complete (AIS: B) SCI and diagnosed AD completed eight sessions of BFRE for the upper extremities over 4 weeks. Blood pressure and heart rate recordings and perceptual pain responses were collected repeatedly during exercise. Blood samples were drawn pre- and post-training. Training was carried out in a neurorehabilitation hospital setting with appertaining medical staff readiness, and was supervised by a physiotherapist with expertise in AD in general as well as prior knowledge of the present patient's triggers and symptoms. Four incidences of AD (defined as systolic blood pressure increase >20 mmHg) were recorded across all training sessions, of which one was symptomatic. The patient's blood profile did not change considerably from pre- to post-training sessions. Self-reported average pain during training corresponded from "mild" to "moderate"., Discussion: The patient was able to perform 4 weeks of BFRE, but encountered episodes of AD. Similarly, two AD episodes were registered during a single conventional, free-flow resistance training session. Evidence from clinically controlled safety studies is needed in order to establish if and how BFRE can be applied in a rehabilitation strategy in SCI individuals with neurological level of injury at or above T6 level.

Respiratory dysfunction is one of the most debilitating effects of spinal cord injury (SCI) impacting the quality of life of patients and caregivers. In addition, breathing difficulties impact the rehabilitation routine a patient may potentially undergo. Transcutaneous electrical spinal cord neuromodulation (TESCoN) is a novel approach to reactivate and retrain spinal circuits after paralysis. We demonstrate that acute and chronic TESCoN therapy over the cervical spinal cord positively impacts the breathing and coughing ability in a patient with chronic tetraplegia. ln addition, we show that the improved breathing and coughing ability are not only observed in the presence of TESCoN but persisted for a few days after TESCoN was stopped. NEW & NOTEWORTHY Noninvasive spinal neuromodulation improves breathing and coughing in a patient with severe and complete tetraplegia.

Purpose: Our aim was to test whether the newly modified rhizotomy protocol which could be effectively used to guide single-level approach selective dorsal rhizotomy (SL-SDR) to treat spastic hemiplegic cases by mainly releasing those spastic muscles (target muscles) marked pre-operatively in their lower limbs was still applicable in spastic quadriplegic or diplegic cerebral palsy (CP) cases in pediatric population., Methods: In the current study, we retrospectively conducted a cohort review of cases younger than 14 years of age diagnosed with spastic quadriplegic or diplegic CP who undergone our modified protocol-guided SL-SDR in the Department of Neurosurgery, Children's Hospital of Shanghai since July 2016 to November 2017 with at least 12 months post-op intensive rehabilitation program (pre-op GMFCS level-based). Clinical data including demographics, intra-operative EMG responses interpretation, and relevant assessment of included cases were taken from the database. Inclusion and exclusion criteria were set for the selection of patients in the current study. Muscle tone (modified Ashworth scale) and strength of those spastic muscles (muscle strength grading scale), range of motion (ROM) of those joints involved, the level of Gross Motor Function Classification System (GMFCS), and Gross Motor Function Measure 66 items (GMFM-66) score of those cases were our focus., Results: A total of 86 eligible cases were included in our study (62 boys). Among these patients, 61.6% were quadriplegic. Pre-operatively, almost 2/3 of our cases were with GMFCS levels II and III. Mean age at the time of surgery in these cases was 6.2 (3.5-12) years. Pre-op assessment marked 582 target muscles in these patients. Numbers of nerve rootlets tested during SDR procedure were between 52 and 84 across our cases, with a mean of 66.5 ± 6.7/case. Among those tested (5721 in 86 cases), 47.9% (2740) were identified as lower limb-related sensory rootlets. Our protocol successfully differentiated sensory rootlets which were considered to be associated with spasticity of target muscles across all our 86 cases (ranged from 3 to 21). Based on our protocol, 871 dorsal nerve rootlets were sectioned 50%, and 78 were cut 75%. Muscle tone of those target muscles reduced significantly right after SL-SDR procedure (3 weeks post- vs. pre-op, 1.7 ± 0.5 vs. 2.6 ± 0.7). After an intensive rehabilitation program for 19.9 ± 6.0 months, muscle tone continued to decrease to 1.4 ± 0.5. With the reduction of muscle tone, strength of those target muscles in our cases improved dramatically with statistical significance achieved (3.9 ± 1.0 at the time of last follow-up vs. 3.3 ± 0.8 pre-op), and as well as ROM. Increase in GMFCS level and GMFM-66 score was observed at the time of last follow-up with a mean of 0.4 ± 0.6 and 6.1 ± 3.2, respectively, when compared with that at pre-op. In 81 cases with their pre-op GMFCS levels II to V, 27 (33.3%) presented improvement with regard to GMFCS level upgrade, among which 4 (4.9%) even upgraded over 2 levels. Better results with regard to upgrading in level of GMFCS were observed in cases with pre-op levels II and III when compared with those with levels IV and V (24/57 vs. 3/24). Upgrading percentage in cases younger than 6 years at surgery was significantly greater than in those older (23/56 vs. 4/25). Cases with their pre-op GMFM-66 score ≥ 50 had greater score increase of GMFM-66 when compared with those less (7.1 ± 3.4 vs. 5.1 ± 2.8). In the meanwhile, better score improvement was revealed in cases when SDR performed at younger age (6.9 ± 3.3 in case ≤ 6 years vs. 4.7 ± 2.7 in case > 6 years). No permanent surgery-related complications were recorded in the current study., Conclusion: SL-SDR when guided by our newly modified rhizotomy protocol was still feasible to treat pediatric CP cases with spastic quadriplegia and diplegia. Cases in this condition could benefit from such a procedure when followed by our intensive rehabilitation program with regard to their motor function.

Introduction: While autonomic dysreflexia caused by severe spinal cord lesions can be life-threatening, relevant reports on non-traumatic spinal lesions are rare. Furthermore, modes of innervation of the supraspinal inhibitory pathways at each spinal sympathetic segment remain unknown. Herein, I report the case of a patient with autonomic dysreflexia and radiation myelopathy. The laterality of autonomic dysreflexia was investigated with special reference to the sudomotor function., Case Presentation: A 51-year-old man with a history of epipharynx carcinoma, radiotherapy, and cisplatin chemotherapy was referred for the evaluation of autonomic function. He was ambulant but displayed spastic tetraparesis, areflexia of the extremities, sensory disturbance below C4 dermatome, dysuria, and impotence. Spinal magnetic resonance imaging demonstrated a cervical lesion involving the lateral portion of C2-C5, bilaterally. The thermal sweating test showed that sweating was lower on the left side of the face and neck, left shoulder, and arm than the corresponding parts on the right side. The rest of the body was anhidrotic. Sweating due to autonomic dysreflexia was symmetric, but more abundant on the left side of the face. Acetylcholine-induced sweating was markedly reduced on the left leg., Discussion: This might be the first documentation of autonomic dysreflexia observed in a patient with radiation myelopathy. The present observations suggested that the supraspinal inhibitory pathway to spinal preganglionic neurons may descend on the same side as thermal sudomotor facilitatory pathways at the cervical level. Furthermore, autonomic dysreflexia was more prominent in the standing position suggesting that the pressure stimulus might enhance autonomic dysreflexia.

During the pandemic of coronavirus disease 2019, it is possible for rehabilitation physicians and personnel to take care of patients with concurrent spinal cord injury and coronavirus disease 2019. Here, we describe a case of acute cervical spinal cord injury resulting in complete tetraplegia C5 American Spinal Injury Association Impairment Scale A with unrecognized, severe acute respiratory syndrome coronavirus 2 infection. This resulted in large-scale quarantines of related surgical and rehabilitation staff, and the unexpected death of the patient despite receiving the treatments according to the standard guideline. Rehabilitation personnel who take care of acute spinal cord injury patients with coronavirus disease 2019 should consider the effect of spinal cord injury on the course of coronavirus disease 2019, the effect of coronavirus disease 2019 and its treatments on the course of spinal cord injury, and risks of severe acute respiratory syndrome coronavirus 2 transmission between patients and rehabilitation staff, to continue providing safe and effective rehabilitation programs.

Spinal cord injuries (SCIs) present distinct physiological and social considerations for the emergency physician. During the COVID-19 pandemic, these considerations may generate unique challenges for emergency physicians managing patients with SCIs. Physiological disruptions may alter the way SCI patients present with COVID-19. The same disruptions can affect management of this vulnerable patient group, perhaps warranting early aggressive treatment. The medical picture will often be complicated by unique social characteristics. The reliance on caregivers for activities of daily living can, as an example, increase the human resource requirement of an ED. Considering the vulnerabilities and complexities of patients with SCI, the community should prioritise prevention of COVID-19 infections in this group. In the event that they do present to an ED, planning for and understanding their complexities will facilitate optimal management., (© 2020 Australasian College for Emergency Medicine.)

Introduction: Bladder rupture in patients with indwelling urethral catheters is rare. Herein, we describe two spinal cord injured (SCI) patients with neurogenic bladder dysfunction managed with chronic indwelling catheters who presented with extraperitoneal bladder rupture related to bladder instillation. One case was during continuous bladder irrigation for hematuria, the other during routine cystography., Case Presentation: One patient is a tetraplegic male with a C5 ASIA impairment scale (AIS) SCI and a chronic catheter who presented with gross hematuria and autonomic dysreflexia (AD). Continuous irrigation was complicated by ongoing AD and poor catheter drainage. A CT scan revealed an extraperitoneal bladder rupture which was managed with surgical repair and suprapubic catheter. The second patient is a tetraplegic female who underwent gravity cystography to evaluate for vesicoureteric reflux. She experienced AD, followed by a witnessed extraperitoneal rupture. The rupture resolved with continued catheter drainage. No long term complications were noted., Discussion: We present two cases of extraperitoneal rupture in chronically catheterized SCI patients following bladder instillation. Both patients were undergoing instillation of fluid through balloon catheters which likely occluded the outlet. We believe that rupture in both cases was iatrogenic, from elevated intravesical pressures during gravity instillation of fluid. Both patients experienced AD during these events. A procedure involving bladder instillation in chronically catheterized SCI patients should be performed by providers familiar with management of AD. Risk factors for iatrogenic bladder rupture during instillation procedures likely include chronic catheterization, small bladder capacity, instillation under significant pressure, and occlusion of the bladder outlet by a balloon catheter.

Introduction: We present the report of the first, to our best knowledge, case of COVID-19 in a tetraplegic person., Case Presentation: A 56-year-old male with AIS A C4 tetraplegia developed fever during the night, without any prodrome. His general practitioner suspected a urinary tract infection and prescribed him antibiotic therapy. After 2 days of antibiotic therapy the fever still persisted, so the individual was admitted to the local hospital and treated with broad-spectrum antibiotics. After 2 days he was transferred to our spinal unit. Considering the worsening of the chest X-ray and fever despite 48 h of broad-spectrum antibiotic therapy, we strongly suspected viral pneumonia. SARS-CoV-2 was detected and antiviral therapy with Lopinavir/Ritonavir, associated with hydroxychloroquine, was promptly started. Fever ceased after 2 days of therapy., Discussion: Blood test and chest X-ray findings in this patient were similar to previously published findings regarding COVID-19. One difference between this case and the known clinical course of COVID-19 is that did not develop cough. Another interesting feature of our case is that, despite tetraplegia, the clinical course was not severe. Persons with COVID-19 remain asymptomatic, these results underscore the need for rehabilitation and SCI professionals to have a high index of suspicion for COVID-19 in their inpatient and outpatient clients. Only inpatient with fever hase being tested for COVID-19. All new patients are submitted to SARS-COV-2 Test. Moreover, routine testing of patients who have to participate in therapy in common gym areas may be warranted.

Study Design: Validation cross-sectional study., Objective: To adapt the Spinal Cord Injury Secondary Conditions Scale (SCI-SCS) to Italian and to assess the validity and reliability of this instrument., Setting: Multicentre study in outpatient clinics of three urban spinal units across Italy., Methods: After a five-step translation/validation process, the Italian SCI-SCS was administered in a toolset composed of a sociodemographic questionnaire, the Modified Barthel Index, the Short-Form 8, the Patient Health Questionnaire 9, and the General Anxiety Disorder 7. The Italian SCI-SCS construct validity was assessed through exploratory factor analysis (EFA). The internal consistency and test-retest reliability of the instrument were evaluated using Cronbach's α and the intraclass correlation coefficient (ICC) for the total scale and its subscales. Pearson's correlation coefficient with all administered instruments was calculated to evaluate the concurrent validity., Results: One-hundred fifty-six participants were recruited from February to October 2018. EFA suggested a three-factor structure explaining 45% of the total variance. After experts' consideration about the clinical relevance of its components, a final version of the Italian SCI-SCS with four different subscales and 15 items was proposed. The total scale Cronbach's α was 0.73. The ICC agreement for test-retest reliability was 0.91. Correlations of the Italian SCI-SCS with the administered instruments were statistically significant (p < 0.05), highlighting congruent hypothesized relations., Conclusion: Findings of this study provided a first psychometric evaluation of the SCI-SCS. The modified Italian version of this tool may represent a valuable instrument for the longitudinal assessment of the impact of secondary conditions in people with SCI.

Introduction: The AP4B1 gene encodes a subunit of adaptor protein complex-4 (AP4), a component of intracellular transportation of proteins which plays important roles in neurons. Bi-allelic mutations in AP4B1 cause autosomal recessive spastic paraplegia-47(SPG47)., Case Presentation: Here we present a Chinese patient with spastic tetraplegia, moderate psychomotor development delay and febrile seizures plus. Brain MRIs showed dilated supratentorial ventricle, thin posterior and splenium part of corpus callosum. The patient had little progress through medical treatments and rehabilitating regimens. Whole exome sequencing identified novel compound heterozygous truncating variants c.1207C > T (p.Gln403*) and c.52&#95;53delAC (p.Cys18Glnfs*7) in AP4B1 gene. Causal mutations in AP4B1 have been reported in 29 individuals from 22 families so far, most of which are homozygous mutations., Conclusions: Our study enriched the genetic and phenotypic spectrum of SPG47. Early discovery, diagnosis and proper treatment on the conditions generally increase chances of improvement on the quality of life for patients.

Background: Respiratory complications remain a leading cause of morbidity and mortality in people with acute and chronic tetraplegia. Respiratory muscle weakness following spinal cord injury-induced tetraplegia impairs lung function and the ability to cough. In particular, inspiratory muscle strength has been identified as the best predictor of the likelihood of developing pneumonia in individuals with tetraplegia. We hypothesised that 6 weeks of progressive respiratory muscle training (RMT) increases respiratory muscle strength with improvements in lung function, quality of life and respiratory health., Methods: Sixty-two adults with tetraplegia participated in a double-blind randomised controlled trial. Active or sham RMT was performed twice daily for 6 weeks. Inspiratory muscle strength, measured as maximal inspiratory pressure (PImax) was the primary outcome. Secondary outcomes included lung function, quality of life and respiratory health. Between-group comparisons were obtained with linear models adjusting for baseline values of the outcomes., Results: After 6 weeks, there was a greater improvement in PImax in the active group than in the sham group (mean difference 11.5 cmH 2 O (95% CI 5.6 to 17.4), p<0.001) and respiratory symptoms were reduced (St George Respiratory Questionnaire mean difference 10.3 points (0.01-20.65), p=0.046). Significant improvements were observed in quality of life (EuroQol-Five Dimensional Visual Analogue Scale 14.9 points (1.9-27.9), p=0.023) and perceived breathlessness (Borg score 0.64 (0.11-1.17), p=0.021). There were no significant improvements in other measures of respiratory function (p=0.126-0.979)., Conclusions: Progressive RMT increases inspiratory muscle strength in people with tetraplegia, by a magnitude which is likely to be clinically significant. Measurement of baseline PImax and provision of RMT to at-risk individuals may reduce respiratory complications after tetraplegia., Trial Registration Number: Australian New Zealand Clinical Trials Registry (ACTRN 12612000929808)., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To assess pressure ulcer (PU) risk in persons with mobility impairments using a large data set to identify demographic, laboratory, hemodynamic, and pharmacologic risk factors., Methods: The cohort of interest was persons with disabilities who have mobility impairments and are diagnostically at risk of PUs. To define this cohort, diagnoses that qualify patients for skin protection wheelchair cushions were used. Data were obtained from the Cerner Health Facts data warehouse. Two cohorts were defined: persons with and without a history of PUs. Analysis included descriptive statistics and multivariate logistic regression modeling. Variables retained in the model were identified using LASSO, gradient boosting, and Bayesian model averaging., Main Results: The resulting cohorts included more than 87,000 persons with a history of PUs and more than 1.1 million persons who did not have a PU. The data revealed seven disability groups with the greatest prevalence of PUs: those with Alzheimer disease, cerebral palsy, hemiplegia, multiple sclerosis, paraplegia/quadriplegia, Parkinson disease, and spina bifida. Ulcers in the pelvic region accounted for 82% of PUs. Persons with disabilities who were male or black had a greater prevalence of PUs. Physiologic risk factors included the presence of kidney or renal disease, decreased serum albumin, and increased serum C-reactive protein., Conclusions: The results indicate that, although persons with disabilities can exhibit a wide functional range, they remain at risk of PUs and should be evaluated for proper preventive measures, including support surfaces and wheelchair cushions.

Castleman disease is a rare lymphoproliferative disease the exact cause of which is not known. The diagnosis is based on the adequate histological examination. While in the unifocal form, the disease most commonly affects the chest, and symptoms may resolve as a result of intact excision of the tumour; other treatment methods may be performed in addition to or instead of surgical incision in the case of the multifocal form. We present the case of a patient with Castleman disease who received multidisciplinary treatment. Speech difficulty, dysphagia, and progressive paresis occurred in the upper and lower extremities of the 56-year-old male patient 18 weeks before his check-up examinations. Although the complaints temporarily resolved with plasmapheresis, surgical sampling could not confirm the origin of the mediastinal lymphadenomegaly detected with thoracic CT. The patient was admitted to our department to remove the 5 cm large subcarinal lymph node or to gain a tissue sample from it. On admission, significant atrophy, hypotonia and tetraplegia were seen in the four extremities, and areflexia was detected all over the body. The 5.5 × 3.5 cm large subcarinal lymph node conglomerate was removed from posterolateral thoracotomy. Histology was performed, Castleman disease was confirmed. 3 days after the surgery, the patient was able to move the extremities, and then on the 9th postoperative day, the patient could walk using a walking frame, and he was transferred back to the Department of Neurorehabilitation. At transfer, the muscle strength of the upper extremities was almost intact, and 4/5 muscle strength was detected in the lower extremities. After this, methylprednisolone, vitamin B 1 , calcium citrate, famotidine therapy was administered, and 2 weeks after his transfer, he was discharged home; at that time, the patient was able to walk safely without a walking frame. The symptoms resolved almost completely 3 months after the surgery. Diagnosis and treatment of Castleman disease are multidisciplinary tasks. If the patient is suitable for surgery, surgical removal has to play a key role in the treatment of unifocal Castleman disease. Orv Hetil. 2020; 161(1): 33-38.