Utility of motor-evoked potentials in contemporary open thoracoabdominal aortic repair.

OBJECTIVE: Paraplegia remains one of the major complications of contemporary open thoracoabdominal aortic aneurysm (TAAA) repair. Intraoperative motor-evoked potentials (MEPs) act as a surrogate measure for spinal cord homeostasis. The purpose of this study was to evaluate the results of intraoperative neuromonitoring in contemporary TAAA repair and its association with postoperative spinal cord ischemia (SCI). METHODS: Patients who underwent open type 2 or 3 TAAA or completion aortic repair using intraoperative neuromonitoring were identified between May 2006 and November 2023. Patient demographics, comorbidities, indication for the procedure, procedural details, and outcomes were recorded. The groups were divided based on type of repair, and univariate statistics were then used to evaluate the association of these metrics vs the type of repair. RESULTS: Seventy-nine patients underwent open type 2 (N = 41) and 3 (N = 23) TAAA and completion aortic (N = 15; open in 14 and endovascular in 1) repairs by a single surgeon. The cohort was predominantly male (N = 48, 60.8%) with a mean age of 52.5 ± 16.2 years. There was a high incidence of hypertension (N = 53, 67.1%), smoking history (N = 42, 53.1%), and connective tissue disorders (N = 37, 46.8%). Operative indications included dissection-related (N = 50, 63.3%) and degenerative (N = 26, 32.9%) TAAA and dissection-related malperfusion (N = 3, 3.8%). Left heart bypass was often (N = 73, 92.4%) used for distal aortic perfusion, and cerebrospinal fluid drainage (N = 77, 97.5%) was a common adjunct. MEPs were classified as no change (N = 43, 54.4%), reversible change (N = 26, 32.9%), irreversible change (N = 4, 5.1%), and unreliable (N = 6, 7.6%). MEP changes were predominantly bilateral (N = 70, 88.6%) and occurred most often during repair of the abdominal aortic segment (N = 13, 16.5%). The median number of replaced vertebral levels was associated with MEP changes (P = .013). SCI was only observed in repairs greater than 6 replaced vertebral levels with an overall frequency of 17.7%. It was most prevalent in completion aortic repairs (26.7%). Immediate and delayed SCI occurred in 10.1% and 7.6% of patients, respectively; it was most commonly (71.8%) reversible. Permanent paraplegia occurred in four patients (5.1%), with equal immediate and delayed onsets. MEPs demonstrated poor sensitivity (53.9%) and specificity (62.3%) for SCI; however, there was a high negative predictive value (86.4%) in this population. In-hospital mortality occurred in five (6.3%) patients. CONCLUSIONS: No changes in intraoperative MEPs are highly predictive of spinal cord homeostasis. The number of replaced vertebral levels and previous aortic repair should guide intraoperative neuroprotective measures including intercostal reimplantation and should take precedence over intraoperative monitoring, especially when MEP changes occur.

Effect of high-intensity interval training on cardiometabolic component risks in persons with paraplegia: Results of a randomized controlled trial.

The aim of this work is to determine the effect of upper-body high intensity interval training (HIIT) on cardiometabolic risks in individuals with chronic paraplegia. Twenty-seven individuals (14 females, 13 males, mean ± SD age: 46 ± 9 years) with chronic paraplegia (spinal cord injury between T2 and L5 >1-year post-injury) took part in a randomized controlled trial and were included in the final analysis. Participants in the HIIT group (n = 18) performed ∼30 min of arm crank exercise (60 s intervals at 80%-90% peak heart rate) four times per week, for 6 weeks. Participants in the control (CON) group (n = 9) were asked to maintain their habitual diet and physical activity patterns over the study period. Outcome measures were taken at baseline and follow-up. The primary outcome measures were fasting insulin, peak power output (PPO) and peak aerobic capacity ( V ̇ O 2 peak ${{\dot{V}}_{{{{\mathrm{O}}}_{\mathrm{2}}}{\mathrm{peak}}}}$ ). Secondary outcome measures included body composition, postprandial glycaemic control, fasting blood lipids, inflammatory biomarkers and resting blood pressure. Differences between groups were assessed by ANCOVA, using baseline values as a covariate. PPO was higher in the HIIT (101 W, 97-106) compared to the CON (90 W, 83-96) group at follow-up (P = 0.006). There were no differences in fasting insulin (P = 0.415) or relative V ̇ O 2 peak ${{\dot{V}}_{{{{\mathrm{O}}}_{\mathrm{2}}}{\mathrm{peak}}}}$ (P = 0.417). Postprandial Matsuda insulin sensitivity index (ISIMatsuda) was higher in the HIIT (5.42, 4.69-6.15) compared to the CON (3.75, 2.46-5.04) group at follow-up (P = 0.036). Six weeks of upper-body HIIT increased PPO and ISIMatsuda, with no other beneficial effect on cardiometabolic component risks in persons with chronic paraplegia. HIGHLIGHTS: What is the central question of this study? What is the effect of upper-body high intensity interval training (HIIT) on cardiometabolic component risks in individuals with chronic paraplegia? What is the main finding and its importance? Six weeks of upper-body HIIT increased PPO and improved insulin sensitivity, but had no beneficial effect on other cardiometabolic component risks in persons with chronic paraplegia. The large effect size observed for insulin sensitivity may be important in terms of reducing the risk of type-2 diabetes in this population.

Topographic mapping of the sensorimotor qualities of empathic reactivity: A psychophysiological study in people with spinal cord injuries.

The experience of empathy for pain is underpinned by sensorimotor and affective dimensions which, although interconnected, are at least in part behaviorally and neurally distinct. Spinal cord injuries (SCI) induce a massive, below-lesion level, sensorimotor body-brain disconnection. This condition may make it possible to test whether sensorimotor deprivation alters specific dimensions of empathic reactivity to observed pain. To explore this issue, we asked SCI people with paraplegia and healthy controls to observe videos of painful or neutral stimuli administered to a hand (intact) or a foot (deafferented). The stimuli were displayed by means of a virtual reality set-up and seen from a first person (1PP) or third person (3PP) visual perspective. A number of measures were recorded ranging from explicit behaviors like explicit verbal reports on the videos, to implicit measures of muscular activity (like EMG from the corrugator and zygomatic muscles that may represent a proxy of sensorimotor empathy) and of autonomic reactivity (like the electrodermal response and Respiratory Sinus Arrhythmia that may represent a general proxy of affective empathy). While no across group differences in explicit verbal reports about the pain stimuli were found, SCI people exhibited reduced facial muscle reactivity to the stimuli applied to the foot (but not the hand) seen from the 1PP. Tellingly, the corrugator activity correlated with SCI participants' neuropathic pain. There were no across group differences in autonomic reactivity suggesting that SCI lesions may affect sensorimotor dimensions connected to empathy for pain.

High intensity functional training for people with spinal cord injury & their care partners.

STUDY DESIGN: Non-randomized clinical trial. OBJECTIVES: Examine the feasibility, physical and psychosocial effects of a high intensity functional training (HIFT) exercise program for people with spinal cord injury (pSCI) and their care partners (CPs). SETTING: Community fitness center in a Medically Underserved Area (Fort Smith, USA.) METHODS: A single-group design with three assessment points (before the program, at midpoint (13 weeks), and post-program (25 weeks) was used to examine the effects of up to 49 HIFT sessions over 25-weeks. Sessions were 60 to 75 min in duration and adapted to the abilities of participants. Feasibility measures included recruitment, retention, attendance, safety and fidelity (exercise intensity rated via session-Rating of Perceived Exertion (RPE). Physical measures included cardiovascular endurance, anaerobic power, and muscular strength. Psychosocial measures included perceived social support for exercise, exercise self-efficacy and health-related quality of life. RESULTS: Fourteen pSCI (7 with paraplegia and 7 with tetraplegia, 2 females) and 6 CPs (4 females) were included (median age = 60) (IQR = 15.8). Recruitment rates were 40% for pSCI and 32% for CPs. On average, participants attended 73% (22%) of exercise sessions with a median session-RPE of 5 (IQR = 1). Retention rates were 83% and 67% for pSCI and CPs, respectively. For pSCI and their CPs, large effect sizes were observed for cardiovascular endurance, anaerobic power, muscular strength, and social support for exercise. CONCLUSIONS: For pSCI and their CPs, HIFT appears feasible and potentially leads to improvements in physical and psychosocial health for both groups.

Chasing Gold: Heat Acclimation in Elite Handcyclists with Spinal Cord Injury.

Thermoregulation is impaired in individuals with a spinal cord lesion (SCI), affecting sweat capacity, heat loss, and core temperature. This can be particularly problematic for athletes with SCI who exercise in hot and humid conditions, like those during the Tokyo 2020 Paralympic Games. Heat acclimation can support optimal preparation for exercise in such challenging environments, but evidence is limited in endurance athletes with SCI. We evaluated whether seven consecutive days of exercise in the heat would result in heat acclimation. Five elite para-cycling athletes with SCI participated (two females, three males, median (Q1-Q3) 35 (31-51) years, four with paraplegia and one with tetraplegia). All tests and training sessions were performed in a heat chamber (30°C and 75% relative humidity). A time-to-exhaustion test was performed on day 1 (pretest) and day 7 (posttest). On days 2-6, athletes trained daily for one hour at 50-60% of individual peak power (PPeak). Comparing pretest and posttest, all athletes increased their body mass loss (p=0.04), sweat rate (p=0.04), and time to exhaustion (p=0.04). Effects varied between athletes for core temperature and heart rate. All athletes appeared to benefit from our heat acclimation protocol, helping to optimize their preparation for the Tokyo 2020 Paralympic Games.

Formation of a novel supraspinal-spinal connectome that relearns the same motor task after complete paralysis.

Having observed that electrical spinal cord stimulation and training enabled four patients with paraplegia with motor complete paralysis to regain voluntary leg movement, the underlying mechanisms involved in forming the newly established supraspinal-spinal functional connectivity have become of great interest. van den Brand et al. (Science 336: 1182-1185, 2012) subsequently, demonstrated the recovery, in response to spinal electro-neuromodulation and locomotor training, of voluntary stepping of the lower limbs in rats that received a lesion that is assumed to eliminate all long-descending cortical axons that project to lumbosacral segments. Here, we used a similar spinal lesion in rats to eliminate long-descending axons to determine whether a novel, trained motor behavior triggered by a unique auditory cue learned before a spinal lesion, could recover after the lesion. Hindlimb stepping recovered 1 mo after the spinal injury, but only after 2 mo, the novel and unique audio-triggered behavior was recovered, meaning that not only was a novel connectivity formed but also further evidence suggested that this highly unique behavioral response was independent of the recovery of the circuitry that generated stepping. The unique features of the newly formed supraspinal-spinal connections that mediated the recovery of the trained behavior is consistent with a guidance mechanism(s) that are highly use dependent.NEW & NOTEWORTHY Electrical spinal cord stimulation has enabled patients with paraplegia to regain voluntary leg movement, and so the underlying mechanisms involved in this recovery are of great interest. Here, we demonstrate in rodents the recovery of trained motor behavior after a spinal lesion. Rodents were trained to kick their right hindlimb in response to an auditory cue. This behavior recovered 2 mo after the paralyzing spinal cord injury but only with the assistance of electrical spinal cord stimulation.

Supraspinal nociceptive networks in neuropathic pain after spinal cord injury.

Neuropathic pain following spinal cord injury involves plastic changes along the whole neuroaxis. Current neuroimaging studies have identified grey matter volume (GMV) and resting-state functional connectivity changes of pain processing regions related to neuropathic pain intensity in spinal cord injury subjects. However, the relationship between the underlying neural processes and pain extent, a complementary characteristic of neuropathic pain, is unknown. We therefore aimed to reveal the neural markers of widespread neuropathic pain in spinal cord injury subjects and hypothesized that those with greater pain extent will show higher GMV and stronger connectivity within pain related regions. Thus, 29 chronic paraplegic subjects and 25 healthy controls underwent clinical and electrophysiological examinations combined with neuroimaging. Paraplegics were demarcated based on neuropathic pain and were thoroughly matched demographically. Our findings indicate that (a) spinal cord injury subjects with neuropathic pain display stronger connectivity between prefrontal cortices and regions involved with sensory integration and multimodal processing, (b) greater neuropathic pain extent, is associated with stronger connectivity between the posterior insular cortex and thalamic sub-regions which partake in the lateral pain system and (c) greater intensity of neuropathic pain is related to stronger connectivity of regions involved with multimodal integration and the affective-motivational component of pain. Overall, this study provides neuroimaging evidence that the pain phenotype of spinal cord injury subjects is related to the underlying function of their resting brain.

Cross training effects of non-paralytic dorsiflexion muscle strengthening exercise on paralytic dorsiflexor muscle activity, gait ability, and balancing ability in patients with chronic stroke: A randomized, controlled, pilot trial.

OBJECTIVE: To investigate the effects of non-paralytic dorsiflexion muscle strengthening exercise on functional abilities in chronic hemiplegic patients after stroke. METHODS: A total of 21 patients with chronic stroke underwent dorsiflexion muscle strengthening exercise (MST) 5 times a week for 6 weeks (the experimental group, MST to non-paralytic dorsiflexion muscles, n=11; the control group, MST to paralytic dorsiflexion muscles; n=10). Paralytic dorsiflexor muscle activities (DFA) and 10 m walking tests (10MWT) and timed up and go tests (TUG) were measured before and after intervention. RESULTS: A significant increase in DFA was observed after intervention in the experimental and control groups (p<0.05) (experimental 886.6% for reference voluntary contraction (RVC), control 931.6% for RVC). TUG and 10MWT results showed significant reductions post-intervention in the experimental and control groups (experimental group -5.6 sec, control -4.8 sec; experimental group -3.1 sec, control, -3.9 sec; respectively). No significant intergroup difference was observed between changes in DFA or between changes in TUG and 10MWT results after intervention (p>.05). CONCLUSION: Strengthening exercise performed on non-paralytic dorsiflexion muscles had positive cross-training effects on paralytic dorsiflexor muscle activities, balance abilities, and walking abilities in patients with chronic stroke.

Oxygen Uptake During Exoskeletal-Assisted Walking in Persons With Paraplegia.

OBJECTIVE: To determine the cardiometabolic demands associated with exoskeletal-assisted walking (EAW) in persons with paraplegia. This study will further examine if training in the device for 60 sessions modifies cost of transport (CT). DESIGN: Prospective cohort study. Measurements over the course of a 60-session training program, approximately 20 sessions apart. SETTING: James J. Peters Bronx Veterans Affairs Medical Center, Center for the Medical Consequences of Spinal Cord Injury Research Center. PARTICIPANTS: The participants' demographics (N=5) were 37-61 years old, body mass index (calculated as weight in kilograms divided by height in meters squared) of 22.7-28.6, level of injury from T1-T11, and 2-14 years since injury. INTERVENTIONS: Powered EAW. MAIN OUTCOME MEASURES: Oxygen consumption per unit time (V˙O2, mL/min/kg), velocity (m/min), cost of transport (V˙O2/velocity), and rating of perceived exertion (RPE). RESULTS: With training: EAW velocity significantly improved (Pre: 51±51m; 0.14±0.14m/s vs Post: 99±42m; 0.28±0.12m/s, P=.023), RPE significantly decreased (Pre: 13±6 vs Post: 7±4, P=.001), V˙O2 significantly improved (Pre: 9.76±1.23 mL/kg/m vs Post: 12.73±2.30 mL/kg/m, P=.04), and CT was reduced from the early to the later stages of training (3.66±5.2 vs 0.87±0.85 mL/kg/m). CONCLUSIONS: The current study suggests that EAW training improves oxygen uptake efficiency and walking velocities, with a lower perception of exertion.

Shoulder Pain Is Associated With Rate of Rise and Jerk of the Applied Forces During Wheelchair Propulsion in Individuals With Paraplegic Spinal Cord Injury.

OBJECTIVE: To investigate the association between propulsion biomechanics, including variables that describe smoothness of the applied forces, and shoulder pain in individuals with spinal cord injury (SCI). DESIGN: Cross-sectional, observational study. SETTING: Non-university research institution. PARTICIPANTS: Community dwelling, wheelchair dependent participants (N=30) with chronic paraplegia between T2 and L1, with and without shoulder pain (age, 48.6±9.3y; 83% men). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Rate of rise and jerk of applied forces during wheelchair propulsion. Participants were stratified in groups with low, moderate, and high pain based on their Wheelchair User Shoulder Pain Index score on the day of measurement. RESULTS: A mixed-effect multilevel analysis showed that wheelchair users in the high pain group propelled with a significantly greater rate of rise and jerk, measures that describe smoothness of the applied forces, compared with individuals with less or no pain, when controlling for all covariables. CONCLUSIONS: Individuals with severe shoulder pain propelled with less smooth strokes compared to individuals with less or no pain. This supports a possible association between shoulder pain and rate of rise and jerk of the applied forces during wheelchair propulsion.

Paraplegia Following Type B Acute Aortic Dissection Can Spare the Spinal Cord.

Ischemic lumbosacral plexopathy secondary to an acute aortic dissection is a rare condition that is usually unilateral and frequently accompanied by a simultaneous spinal cord infarction. The functional prognosis relies on the severity of the nervous system involvement being usually worse when the spinal cord is involved. We present a case of a 46-year-old man who suffered an acute type B aortic dissection presenting as acute paraplegia due to bilateral ischemic lumbosacral plexopathy treated with thoracic endovascular aortic repair. An up-to-date review of the literature on ischemic lumbosacral plexus injury is provided.

Caffeine-induced hypokalemia: a case report.

BACKGROUND: With an increase in the global popularity of coffee, caffeine is one of the most consumed ingredients of modern times. However, the consumption of massive amounts of caffeine can lead to severe hypokalemia. CASE PRESENTATION: A 29-year-old man without a specific past medical history was admitted to our hospital with recurrent episodes of sudden and severe lower-extremity weakness. Laboratory tests revealed low serum potassium concentration (2.6-2.9 mmol/L) and low urine osmolality (100-130 mOsm/kgH2O) in three such prior episodes. Urinary potassium/urinary creatinine ratio was 12 and 16 mmol/gCr, respectively. The patient was not under medication with laxatives, diuretics, or herbal remedies. Through an in-depth interview, we found that the patient consumed large amounts of caffeine-containing beverages daily, which included > 15 cups of coffee, soda, and various kinds of tea. After the cessation of coffee intake and concomitant intravenous potassium replacement, the symptoms rapidly resolved, and the serum potassium level normalized. CONCLUSIONS: An increased intracellular shift of potassium and increased loss of potassium in urine due to the diuretic action have been suggested to be the causes of caffeine-induced hypokalemia. In cases of recurring hypokalemia of unknown cause, high caffeine intake should be considered.

An investigation into the validity and reliability of the Chinese version of Spinal Cord Independence Measure III (SCIM III).

OBJECTIVE: To investigate the validity and reliability of a Chinese version of Spinal Cord Independence Measure III (SCIM III) in individuals with spinal cord injury. DESIGN: Study on psychometric properties. SETTING: An inpatient rehabilitation facility in China. SUBJECTS: 102 participants with spinal cord injury. Mean (SD) age was 48.8 (15.6) years; tetraplegia/paraplegia ratio was 50/52; median time post injury was 2 months. INTERVENTION: SCIM III was translated into Chinese. Chinese versions of Barthel Index and SCIM III were filled out for each participant by Rater 1. SCIM III was then administered by Rater 2 after 24 hours (n = 67) and 7 days (n = 65). MAIN MEASURES: Validity, inter-rater/test-retest reliability, and internal consistency of the Chinese version of SCIM III. RESULTS: The total scores between the two raters were similar (mean ± SD: 33.8 ± 25.8 vs 33.8 ± 25.5, P = 0.95). Total agreement between the raters in each item was >80%, with both Pearson and intraclass correlation coefficients >0.97 (P < 0.01) for each subscale and total score. The Pearson correlation coefficients of the two independent assessments performed by Rater 2 were also >0.97 (P < 0.01) for each subscale and the total score. Cronbach α was >0.7 for each subscale and the total score for both raters. High consistency was found between Barthel Index and SCIM III total scores (Pearson correlation coefficient = 0.88, P < 0.01). CONCLUSION: The Chinese version of SCIM III is valid and reliable for the functional assessment of patients with SCI.

Defective kinesin binding of TUBB2A causes progressive spastic ataxia syndrome resembling sacsinopathy.

Microtubules participate in fundamental cellular processes, including chromosomal segregation and cell division, migration and intracellular trafficking. Their proper function is required for correct central nervous system development and operative preservation, and mutations in genes coding tubulins, the constituting units of microtubules, underlie a family of neurodevelopmental and neurodegenerative diseases, collectively known as 'tubulinopathies', characterized by a wide range of neuronal defects resulting from defective proliferation, migration and function. Here, we causally link a previously unreported missense mutation in TUBB2A (c.1249G>A, p.D417N), encoding one of the neuron-specific ß-tubulin isotype II, to a disorder characterized by progressive spastic paraplegia, peripheral sensory-motor polyneuropathy and ataxia. Asp417 is a highly conserved solvent-exposed residue at the site mediating binding of kinesin superfamily motors. Impaired binding to KIF1A, a neuron-specific kinesin required for transport of synaptic vesicle precursors of the disease-associated TUBB2A mutant, was predicted by structural analyses and confirmed experimentally in vitro. We show that overexpression of TUBB2AD417N disrupts the mitotic spindle bipolarity and morphology and affects the M phase entry and length. Differently from the TUBB2AN247K and TUBB2AA248V, two mutants previously identified to affect neurodevelopment, TUBB2AD417N retains the ability to assemble into microtubules. Consistent with the differential clinical and structural impact, TUBB2AA248V does not drastically affect TUBB2A binding to KIF1A, nor mitotic spindle bipolarity. Overall, our data demonstrate a pathogenic role of the p.D417N substitution that is different from previously reported TUBB2A mutations and expand the phenotypic spectrum associated with mutations in this gene.

Spinal cord injury alters purinergic neurotransmission to mesenteric arteries in rats.

Complications associated with spinal cord injury (SCI) result from unregulated reflexes below the lesion level. Understanding neurotransmission distal to the SCI could improve quality of life by mitigating complications. The long-term impact of SCI on neurovascular transmission is poorly understood, but reduced sympathetic activity below the site of SCI enhances arterial neurotransmission (1). We studied sympathetic neurovascular transmission using a rat model of long-term paraplegia (T2-3) and tetraplegia (C6-7). Sixteen weeks after SCI, T2-3 and C6-7 rats had lower blood pressure (BP) than sham rats (103 ± 2 and 97 ± 4 vs. 117 ± 6 mmHg, P < 0.05). T2-3 rats had tachycardia (410 ± 6 beats/min), and C6-7 rats had bradycardia (299 ± 10 beats/min) compared with intact rats (321 ± 4 beats/min, P < 0.05). Purinergic excitatory junction potentials (EJPs) were measured in mesenteric arteries (MA) using microlectrodes, and norepinephrine (NE) release was measured using amperometry. NE release was similar in all groups, while EJP frequency-response curves from T2-3 and C6-7 rats were left-shifted vs. sham rats. EJPs in T2-3 and C6-7 rats showed facilitation followed by run-down during stimulation trains (10 Hz, 50 stimuli). MA reactivity to exogenous NE and ATP was similar in all rats. In T2-3 and C6-7 rats, NE content was increased in left cardiac ventricles compared with intact rats, but was not changed in MA, kidney, or spleen. Our data indicate that peripheral purinergic, but not adrenergic, neurotransmission increases following SCI via enhanced ATP release from periarterial nerves. Sympathetic BP support is reduced after SCI, but improving neurotransmitter release might maintain cardiovascular stability in individuals living with SCI.NEW & NOTEWORTHY This study revealed increased purinergic, but not noradrenergic, neurotransmission to mesenteric arteries in rats with spinal cord injury (SCI). An increased releasable pool of ATP in periarterial sympathetic nerves may contribute to autonomic dysreflexia following SCI, suggesting that purinergic neurotransmission may be a therapeutic target for maintaining stable blood pressure in individuals living with SCI. The selective increase in ATP release suggests that ATP and norepinephrine may be stored in separate synaptic vesicles in periarterial sympathetic varicosities.

SPG8 mutations in Italian families: clinical data and literature review.

BACKGROUND: Spastic paraplegia type 8 (SPG8) is an autosomal-dominant form of hereditary spastic paraplegia (AD-HSP) caused by a mutation in the KIAA0196 gene. SPG8 accounts for 1% of less of all AD-HSP and the genotype-phenotype correlation remains poorly understood. METHODS: We report the first clinical and genetic description of SPG8 disease in Italian patients. We identified four new mutations in KIAA0196 gene. These variants were identified using a multigene targeted resequencing HSP panel. We took this opportunity to review the pertinent literature. RESULTS: Age at disease onset was in the third or fourth decade of life. Stiffness of the lower limb with spastic gait, walking impairment, and decreased vibration sense were common early symptoms. Subjects of two families had bladder control abnormalities. Unlike previous reported cases, Italian SPG8 subjects have pure form of spastic paraparesis without cranial nerve involvement, and onset is in adult life. DISCUSSION: By a clinical point of view, it is hard to differentiate SPG8 from the SPG4, in which bladder and vibration sense dysfunctions are frequent signs. The differential diagnosis with other forms of AD-HSPs seems relatively easier if one considers the early-onset manifestations in SPG3A and the peripheral nervous system and cerebellar involvement seen in SPG31.

Muscle Strength Cutoff Points for Functional Independence and Wheelchair Ability in Men With Spinal Cord Injury.

OBJECTIVE: Determine trunk and shoulder muscle strength cutoff points for functional independence and wheelchair skills, and verify the predictive capacity of relative and absolute peak torque in men with spinal cord injury (SCI). DESIGN: Cross-sectional study. SETTING: Rehabilitation hospital setting. PARTICIPANTS: Men (N=54) with SCI were recruited and stratified into high and low paraplegia groups. INTERVENTIONS: All participants performed maximum strength tests for shoulder abduction or adduction (isokinetic) and trunk flexion or extension (isometric) to determine relative and absolute peak torque cutoff points for the Spinal Cord Independence Measure version III (SCIM-III) and Adapted Manual Wheelchair Circuit (AMWC). MAIN OUTCOME MEASURES: The primary outcome measures were SCIM-III, AMWC-Brazil test, and strength variables (peak torques). Demographic characteristics obtained from participants' electronic medical records were the secondary outcomes used as predictor variables of functional independence. RESULTS: The best predictive model for SCIM-III (R=0.78, P≤.05) used the sum of trunk flexion and extension relative peak torque values to determine the cutoff points (1.42 N·m/kg for a score of 70). Relative shoulder abduction peak torque was used in the predictive models for AMWC outcomes: performance score (R=0.77, P≤.05, cutoff points of 0.97 N·m/kg for 300.0m) and 3-minute overground wheeling (R=0.72, P≤.05, cutoff points of 0.96 N·m/kg for 18.5s). CONCLUSIONS: Relative peak torque showed better predictive capacity compared to absolute peak torque. Cutoff points were established for relative muscle strength and could help health professionals set appropriate goals for individuals with SCI to achieve high functional independence and wheelchair ability.

Exoskeletal-Assisted Walking During Acute Inpatient Rehabilitation Leads to Motor and Functional Improvement in Persons With Spinal Cord Injury: A Pilot Study.

OBJECTIVE: To explore the potential effects of incorporating exoskeletal-assisted walking (EAW) into spinal cord injury (SCI) acute inpatient rehabilitation (AIR) on facilitating functional and motor recovery when compared with standard of care AIR. DESIGN: A quasi-experimental design with a prospective intervention group (AIR with EAW) and a retrospective control group (AIR only). SETTING: SCI AIR facility. PARTICIPANTS: Ten acute inpatient participants with SCI who were eligible for locomotor training were recruited in the intervention group. Twenty inpatients with SCI were identified as matched controls by reviewing an AIR database, Uniform Data System for Medical Rehabilitation, by an individual blinded to the study. Both groups (N=30) were matched based on etiology, paraplegia/tetraplegia, completeness of injury, age, and sex. INTERVENTION: EAW incorporated into SCI AIR. MAIN OUTCOME MEASURES: FIM score, International Standards for Neurological Classification of Spinal Cord Injury Upper Extremity Motor Score and Lower Extremity Motor Scores (LEMS), and EAW session results, including adverse events, walking time, and steps. RESULTS: Changes from admission to discharge LEMS and FIM scores were significantly greater in the intervention group (LEMS change: 14.3±10.1; FIM change: 37.8±10.8) compared with the control group (LEMS change: 4.6±6.1; FIM change: 26.5±14.3; Mann-Whitney U tests: LEMS, P<.01 and FIM, P<.05). One adverse event (minor skin abrasion) occurred during 42 walking sessions. Participants on average achieved 31.5 minutes of up time and 18.2 minutes of walk time with 456 steps in one EAW session. CONCLUSIONS: Incorporation of EAW into standard of care AIR is possible. AIR with incorporated EAW has the potential to facilitate functional and motor recovery compared with AIR without EAW.

Spinal Cord Ischemia Rescue after Hybrid Total Arch Repair with Frozen Elephant Trunk: A Case Report.

Frozen elephant trunk repair is a technique described to simplify total arch repair for Stanford type A aortic dissection. Spinal cord ischemia is a devastating complication after frozen elephant trunk repair. In this report, we describe a case of spinal cord ischemia resulting in paralysis after frozen elephant trunk repair. Our spinal cord ischemia protocol was implemented and rescued patients from paraplegia. We report a dedicated spinal cord ischemia protocol that can rescue patients from paraplegia after hybrid arch repair with frozen elephant trunk.

A Case Report of Thoracic Endovascular Aneurysm Repair under Local Anesthesia with Resolution of Acute Onset Lower Extremity Paraplegia from an Acute Complicated Type B Aortic Dissection.

Spinal cord ischemia (SCI) is a rare presenting symptom of acute complicated type B aortic dissection, occurring in approximately 3% of patients . We present a case report of a patient with this presentation who had observed resolution of his paraplegia symptoms immediately after placement of a thoracic stent graft under local anesthesia. The temporal association between true lumen flow restoration and paraplegia resolution intraoperatively is a novel finding. We feel that this case report may provide support for recognized cord perfusion theory , as well as contribute to the understanding of the time frame associated with SCI and reversibility of paraplegia.