Reliability of five-minute vs. one-hour heart rate variability metrics in individuals with spinal cord injury.

Background: A previous study showed low reliability of 1-h HRV outcomes in participants with spinal cord injury (SCI), but it was not certain whether the low reliability was due to the unrestricted activity of participants. We aimed to investigate test-retest reliability of HRV metrics in individuals with SCI using a 1-h measurement in a supine position. Methods: Individuals with SCI underwent two sessions of 1-h recording of the time between consecutive R waves (RR-intervals) in a supine position. HRV outcomes were obtained from a single 5-min data segment and for the full 1-h recording. HRV parameters of interest were: standard deviation of all normal-to-normal R-R intervals (SDNN) and square root of the mean of the squared differences between successive R-R intervals (RMSSD) (time domain); and high frequency power (HF), low frequency power (LF), very low frequency power (VLF), ultra-low frequency power (ULF) and total power (TP) (frequency domain). Relative reliability was assessed by intraclass correlation coefficient (ICC). Absolute reliability was assessed by coefficient of variation (CV) and Bland-Altman limits of agreement (LoA). Results: Data from 37 individuals (14 with tetraplegia and 23 with paraplegia) were included. Relative reliability was higher for the 1-h (ICCs ranged from 0.13-0.71) than for the 5-min duration (ICCs ranged from 0.06-0.50) in the overall SCI group for all HRV metrics. Participants with tetraplegia had lower relative reliability compared to participants with paraplegia in all HRV metrics for the 5-min duration (ICCs ranged from -0.01-0.34 vs. 0.21-0.57). For the 1-h duration, participants with paraplegia showed higher relative reliability than participants with tetraplegia in all HRV metrics (ICCs ranged from 0.18-0.79 vs. 0.07-0.54) except TP (ICC 0.69 vs. 0.82). In terms of absolute reliability, the CVs and LoAs for the 1-h duration were better than for the 5-min duration. In general, time domain metrics showed better reliability than frequency domain metrics for both durations in participants with tetraplegia and paraplegia. The lowest CV and narrowest 95% LoA were found for SDNN in 5-min and 1-h durations overall and in both lesion levels. Conclusions: The supine position did not provide better reliability compared to unrestricted activity in participants with SCI. HRV analysis using a 5-min duration is of limited value in SCI due to poor reliability. For the 1-h analysis duration, interpretation of the reliability of HRV varies according to lesion level: it is recommended to take lesion level into account when interpreting reliability measures.

Test-retest reliability of short- and long-term heart rate variability in individuals with spinal cord injury.

STUDY DESIGN: Cross-sectional. OBJECTIVES: To investigate test-retest reliability of heart rate variability (HRV) metrics in SCI without restriction of activity over long (24-h) and shorter durations (5-min, 10-min, 1-h, 3-h and 6-h). SETTINGS: University hospital in Khon Kaen, Thailand. METHODS: Forty-five participants (11 with tetraplegia and 34 with paraplegia) underwent two 24-h recordings of RR-intervals to derive time and frequency HRV metrics. Relative reliability was assessed by intraclass correlation coefficient (ICC) and absolute reliability by coefficient of variation (CV) and Bland-Altman limits of agreement (LoA). RESULTS: For 5- and 10-min durations, eight of eleven HRV metrics had moderate to excellent reliability (ICC 0.40-0.76); the remaining three were poor (ICC < 0.4). HRV values from 1-h and 3-h durations showed moderate to excellent reliability (ICC of 0.46-0.81), except for 1-h reliability of ULF and TP (ICC of 0.06 and 0.30, respectively). Relative reliability was excellent (ICC of 0.77-0.92) for 6-h and 24-h durations in all HRV metrics. Absolute reliability improved as recording duration increased (lower CVs and narrower LoAs). Participants with high AD risk (SCI level at or above T6) showed lower test-retest reliability of HF and LF values than participants with low AD risk. CONCLUSION: Relative reliability of HRV was excellent for 6-h and 24-h. The best absolute reliability values were for 24-h duration. Time-domain outcomes were more reliable than frequency domain outcomes. Participants with high risk of AD, particularly those with tetraplegia, showed lower reliability, especially for HF and LF.

Grasp and Release Test for Tetraplegic Hand Assessment: an update of the Grasp and Release Test.

The Grasp and Release Test (GRT) was originally developed to measure effectiveness of an implanted neuroprosthesis in people with tetraplegia. Its ease of use and lack of floor and ceiling effects culminated in recommendations for inclusion in a battery of tests to measure outcome following upper limb reconstructive surgery. However, the length of time taken to administer the GRT in a clinical setting, lack of instructions of accepted grasp patterns in the upper limb reconstructive surgery population and scoring procedures lead to differences in reporting outcomes using this measure. In order to ensure clinical utility for the upper limb reconstructive surgery population, revisions of the original test instructions have been made and are reported in this article. Further testing of the psychometric properties of the new measure are currently underway.

PAK1 c.1409 T > a (p. Leu470Gln) de novo variant affects the protein kinase domain, leading to epilepsy, macrocephaly, spastic quadriplegia, and hydrocephalus: Case report and review of the literature.

The p-21-activated kinase 1 (PAK1) protein, encoded by the PAK1 gene, is an evolutionarily conserved serine/threonine-protein kinase that regulates key cellular developmental processes. To date, seven de novo PAK1 variants have been reported to cause the Intellectual Developmental Disorder with Macrocephaly, Seizures, and Speech Delay (IDDMSSD). In addition to the namesake features, other common characteristics include structural brain anomalies, delayed development, hypotonia, and dysmorphic features. Here, we report a de novo PAK1 NM_002576.5: c.1409 T > A variant (p.Leu470Gln) identified by trio genome sequencing (GS) in a 13-year-old boy with postnatal macrocephaly, obstructive hydrocephalus, medically refractory epilepsy, spastic quadriplegia, white matter hyperintensities, profound developmental disabilities, and a horseshoe kidney. This is the first recurrently affected residue identified in the protein kinase domain. Combined assessment of the eight pathogenic PAK1 missense variants reveal that the variants cluster in either the protein kinase or autoregulatory domains. Although interpretation of the phenotypic spectrum is limited by the sample size, neuroanatomical alterations were found more often in individuals with PAK1 variants in the autoregulatory domain. In contrast, non-neurological comorbidities were found more often in individuals with PAK1 variants in the protein kinase domain. Together, these findings expand the clinical spectrum of PAK1-associated IDDMSSD and reveal potential correlations with the affected protein domains.

The Berlin Bimanual Test for Tetraplegia (BeBiTT): development, psychometric properties, and sensitivity to change in assistive hand exoskeleton application.

BACKGROUND: Assistive hand exoskeletons are promising tools to restore hand function after cervical spinal cord injury (SCI) but assessing their specific impact on bimanual hand and arm function is limited due to lack of reliable and valid clinical tests. Here, we introduce the Berlin Bimanual Test for Tetraplegia (BeBiTT) and demonstrate its psychometric properties and sensitivity to assistive hand exoskeleton-related improvements in bimanual task performance. METHODS: Fourteen study participants with subacute cervical SCI performed the BeBiTT unassisted (baseline). Thereafter, participants repeated the BeBiTT while wearing a brain/neural hand exoskeleton (B/NHE) (intervention). Online control of the B/NHE was established via a hybrid sensorimotor rhythm-based brain-computer interface (BCI) translating electroencephalographic (EEG) and electrooculographic (EOG) signals into open/close commands. For reliability assessment, BeBiTT scores were obtained by four independent observers. Besides internal consistency analysis, construct validity was assessed by correlating baseline BeBiTT scores with the Spinal Cord Independence Measure III (SCIM III) and Quadriplegia Index of Function (QIF). Sensitivity to differences in bimanual task performance was assessed with a bootstrapped paired t-test. RESULTS: The BeBiTT showed excellent interrater reliability (intraclass correlation coefficients > 0.9) and internal consistency (α = 0.91). Validity of the BeBiTT was evidenced by strong correlations between BeBiTT scores and SCIM III as well as QIF. Wearing a B/NHE (intervention) improved the BeBiTT score significantly (p < 0.05) with high effect size (d = 1.063), documenting high sensitivity to intervention-related differences in bimanual task performance. CONCLUSION: The BeBiTT is a reliable and valid test for evaluating bimanual task performance in persons with tetraplegia, suitable to assess the impact of assistive hand exoskeletons on bimanual function.

Positive predictive value of a sepsis-screening protocol for patients with tetraplegia.

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.

Progressive motor quadriparesis with wasting: a diagnostic conundrum.

The case of a man in his early 30s, presenting with slowly progressive, asymmetrical onset, pure motor quadriparesis over 9 months with bulbar involvement, absence of upper motor neuron features and significant weight loss, is reported. In view of presentation with diffuse lower motor neuron involvement of short duration, the diagnostic possibilities are discussed. Apart from motor neuron disease, its mimics including muscle and neuromuscular junction disorders were also considered and evaluated for. Carefully assessed clinical and electrophysiological markers which finally clinched the diagnosis of the rare disorder Lambert-Eaton myasthenic syndrome are elaborated. In the present context, recognition of the oddities on clinical and laboratory evaluation is of paramount importance to rule in causes of pure motor quadriparesis.

Accidental boosting in an individual with tetraplegia - considerations for the interpretation of cardiopulmonary exercise testing.

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·â€…kg·-1min-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.

Acute non-traumatic tetraparesis - Differential diagnosis.

INTRODUCTION: Potentially life-threatening disorders may present in the emergency department with acute tetraparesis, and their recognition is crucial for an appropriate management and timely treatment. Our review aims to systematize the differential diagnosis of acute non-traumatic tetraparesis. RESULTS: Causes of tetraparesis can be classified based on the site of defect: upper motor neuron (UMN), peripheral nerve, neuromuscular junction or muscle. History of present illness should include the distribution of weakness (symmetric/asymmetric or distal/proximal/diffuse) and associated clinical features (pain, sensory findings, dysautonomia, and cranial nerve abnormalities such as diplopia and dysphagia). Neurological examination, particularly tendon reflexes, helps further in the localization of nerve lesions and distinction between UMN and lower motor neuron. Ancillary studies include blood and cerebral spinal fluid analysis, neuroaxis imaging, electromyography, muscle magnetic resonance and muscle biopsy. CONCLUSIONS: Acute tetraparesis is still a debilitating and potentially serious neurological condition. Despite all the supplementary ancillary tests, the neurological examination is the key to achieve a correct diagnosis. The identification of life-threatening neurologic disorders is pivotal, since failing to identify patients at risk of complications, such as acute respiratory failure, may have catastrophic results.

Concomitant Guillain-Barre syndrome with COVID-19: a case report.

BACKGROUND: During the Coronavirus disease 2019 (COVID-19) pandemic, different neurological manifestations have been observed. However, only a few cases of Guillain-Barre syndrome (GBS) and COVID-19 have been reported. Therefore, the aim of this study is to investigate a case of concomitant GBS with COVID-19 in Colombia. CASE PRESENTATION: A 39-year-old woman was admitted to a teaching hospital in Barranquilla, Colombia with a history of progressive general weakness with lower limb dominance. Previous symptoms such as ageusia, anosmia and intense headache were reported. Upon admission, facial diplegia, quadriparesis with lower extremity predominance and Medical Research Council muscular strength of 2/5 in the lower limbs and 4/5 in the upper limbs were reported. During clinical evolution, due to general areflexia, hypertensive emergency and progressive diaphragmatic weakness, the patient was admitted to an intensive care unit. The cerebrospinal fluid analysis showed protein-cytological dissociation and the GBS diagnosis was confirmed via a nerve conduction and electromyography test. With regard to the symptoms before hospitalisation, SARS-CoV-2 diagnostic testing was performed with positive results in the second test. The patient was managed with supportive care and was discharged after 20 days of hospitalization with clinical improvement. CONCLUSIONS: Only a few cases of COVID-19 with GBS have been reported. Different subtypes have been previously identified, such as Miller-Fisher syndrome and dysautonomic GBS with SARS-CoV-2 infection. This study investigated the first confirmed case of COVID-19 with concomitant GBS in Colombia. In patients with GBS, several viral and bacterial pathogens have been found in case-control studies but the factors that induce the immune-mediated destruction of the nerve tissues have not been determined. Further studies are needed to determine the possible association between COVID-19 exposure and GBS.

Internal consistency and validity of the Italian version of the Jebsen-Taylor hand function test (JTHFT-IT) in people with tetraplegia.

STUDY DESIGN: Psychometric study. OBJECTIVES: This study aimed to evaluate the internal consistency and validity of the Italian version of the Jebsen-Taylor hand function test (JTHFT-IT) in people with tetraplegia. SETTING: Italian spinal units. METHODS: Psychometric properties of the JTHFT-IT were assessed following international guidelines. The internal consistency was examined using Cronbach's alpha coefficient. Pearson's correlation coefficient was calculated for the concurrent validity of JTHFT-IT with a dynamometer, while the construct validity was calculated in comparison to that of the Van Lieshout test short version in Italian (VLT-SV-IT). RESULTS: The test was administered to 48 right-hand dominant people with tetraplegia. Cronbach's alpha calculation resulted in a value of 0.96 for the right hand and 0.94 for the left hand. In terms of the validity of the scale, the Pearson's correlation, as measured in relation to the VLT-SV-IT and dynamometer, showed statistically significant results (range for the correlation coefficient of between -0.96 and -0.12, p < 0.05). CONCLUSIONS: The findings of this study support the internal consistency and validity of the JTHFT-IT and its use among a population with cervical SCI as a measure of hand functionality.

Is Guillain-Barrè syndrome triggered by SARS-CoV-2? Case report and literature review.

BACKGROUND: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is the infectious agent responsible for coronavirus disease 2019 (COVID-19). Respiratory and gastrointestinal manifestations of SARS-CoV-2 are well described, less defined is the clinical neurological spectrum of COVID-19. We reported a case of COVID-19 patient with acute monophasic Guillain-Barré syndrome (GBS), and a literature review on the SARS-CoV-2 and GBS etiological correlation. CASE DESCRIPTION: A 68 years-old man presented to the emergency department with symptoms of acute progressive symmetric ascending flaccid tetraparesis. Oropharyngeal swab for SARS-CoV-2 tested positive. Neurological examination showed bifacial nerve palsy and distal muscular weakness of lower limbs. The cerebrospinal fluid assessment showed an albuminocytologic dissociation. Electrophysiological studies showed delayed distal latencies and absent F waves in early course. A diagnosis of Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) subtype of GBS was then made. CONCLUSIONS: Neurological manifestations of COVID-19 are still under study. The case we described of GBS in COVID-19 patient adds to those already reported in the literature, in support of SARS-CoV-2 triggers GBS. COVID-19 associated neurological clinic should probably be seen not as a corollary of classic respiratory and gastrointestinal symptoms, but as SARS-CoV-2-related standalone clinical entities. To date, it is essential for all Specialists, clinicians and surgeons, to direct attention towards the study of this virus, to better clarify the spectrum of its neurological manifestations.

Predictive Value of Midsagittal Tissue Bridges on Functional Recovery After Spinal Cord Injury.

BACKGROUND: The majority of patients with spinal cord injury (SCI) have anatomically incomplete lesions and present with preserved tissue bridges, yet their outcomes vary. OBJECTIVE: To assess the predictive value of the anatomical location (ventral/dorsal) and width of preserved midsagittal tissue bridges for American Spinal Injury Association (ASIA) Impairment Scale (AIS) grade conversion and SCI patient stratification into recovery-specific subgroups. METHODS: This retrospective longitudinal study includes 70 patients (56 men, age: 52.36 ± 18.58 years) with subacute (ie, 1 month) SCI (45 tetraplegics, 25 paraplegics), 1-month neuroimaging data, and 1-month and 12-month clinical data. One-month midsagittal T2-weighted scans were used to determine the location and width of tissue bridges. Their associations with functional outcomes were assessed using partial correlation and unbiased recursive partitioning conditional inference tree (URP-CTREE). RESULTS: Fifty-seven (81.4%) of 70 patients had tissue bridges (2.53 ± 2.04 mm) at 1-month post-SCI. Larger ventral (P = .001, r = 0.511) and dorsal (P < .001, r = 0.546) tissue bridges were associated with higher AIS conversion rates 12 months post-SCI (n = 39). URP-CTREE analysis identified 1-month ventral tissue bridges as predictors of 12-month total motor scores (0.4 mm cutoff, P = .008), recovery of upper extremity motor scores at 12 months (1.82 mm cutoff, P = .002), 12-month pin-prick scores (1.4 mm cutoff, P = .018), and dorsal tissue bridges at 1 month as predictors of 12-month Spinal Cord Independence Measure scores (0.5 mm cutoff, P = .003). CONCLUSIONS: Midsagittal tissue bridges add predictive value to baseline clinical measures for post-SCI recovery. Based on tissue bridges' width, patients can be classified into subgroups of clinical recovery profiles. Midsagittal tissue bridges provide means to optimize patient stratification in clinical trials.

Caring for Patients with Spinal Cord Injuries.

The column in this issue is supplied by Juan Jose Olivero, MD, who was a nephrologist at Houston Methodist Hospital and a member of the hospital's Nephrology Training Program before his retirement in 2019. Dr. Olivero obtained his medical degree from the University of San Carlos School of Medicine in Guatemala, Central America, and completed his residency and nephrology fellowship at Baylor College of Medicine in Houston, Texas. He currently serves on the journal's editorial board and is editor of the "Points to Remember" section.

Feasibility and safety of 4 weeks of blood flow-restricted exercise in an individual with tetraplegia and known autonomic dysreflexia: a case report.

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.

Quadriplegia and quadriparesis after endovascular aortic procedures: a catastrophic and under-reported complication?

In this study are presented three cases of spinal cord ischemia (SCI) involving the cervical-dorsal level and leading to quadriplegia and quadriparesis, following thoraco-abdominal aortic aneurysm (TAAA) endovascular repair. A 79-year-old woman with an extent III TAAA was scheduled for a multi-step fenestrated/branched endovascular aortic repair. Immediately after the first step, consisting of standard proximal thoracic stent-graft implantation, she developed quadriplegia that did not resolve despite all therapeutic actions, and died therefore on postoperative day 32. A 72-year old male with an extent IV TAAA underwent endovascular repair, using a customized fenestrated aortic stent-graft. Five hours after the procedure, he developed an asymmetric quadriparesis, that progressively resolved after spinal fluid drainage and arterial pressure increase, even if signs of SCI were documented at magnetic resonance imaging (MRI). A 79-year old man, referred for a type II TAAA with rapid enlargement, underwent a one-stage endovascular repair, using a customized branched aortic stent-graft. As soon as the procedure was completed, the patient presented inferior limbs paralysis and upper limbs paresis. Although no signs of SCI were documented at MRI, the patient did not recover and died therefore three months after the procedure. Although rare, cervical-dorsal SCI may develop during TAAA endovascular aortic repair. This possibly catastrophic event should be considered in the decisional process of TAAA repair and considered to allow prompt recognition and treatment.

A Patient With Acute Cervical Cord Injury and COVID-19: A First Case Report.

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.