Are Body Composition, Strength, and Functional Independence Similarities Between Spinal Cord Injury Classifications? A Discriminant Analysis.

CONTEXT: There seems to be no consensus on which aspects better distinguish the different levels of spinal cord injury regarding body composition, strength, and functional independence. OBJECTIVE: The study aimed to determine which variables better differentiate tetraplegia (TP) from paraplegia and high paraplegia (HP) from low paraplegia (LP). DESIGN: Cross-sectional study. SETTING: Rehabilitation hospital network. PATIENTS: Forty-five men with spinal cord injury, n = 15 for each level (TP, HP, and LP) causing complete motor impairment (American Spinal Injury Association Impairment Scale: A or B) were enrolled in the study. MAIN OUTCOME MEASURES: The 1-maximum repetition test, functional independence measure, spinal cord independence measure, and body composition (skinfold sum, body fat percentage, and body mass index) were assessed. Discriminant analysis was carried out using the Wilks lambda method to identify which strength and functional variables can significantly discriminate subjects for injury classification (TP, HP, and LP). RESULTS: The discriminant variable for TP versus HP was body mass index and for TP versus LP was 1-maximum repetition (P ≤ .05). There were no variables that discriminated HP versus LP. CONCLUSIONS: The discriminant variables for TP versus HP and TP versus LP were body mass index and 1-maximum repetition, respectively. The results showed that HP and LP are similar for strength and functional variables.

Identification and molecular characterization of non-polio enteroviruses from children with acute flaccid paralysis in West Africa, 2013-2014.

Besides polioviruses, non-polio enteroviruses (NPEVs) may also be associated with acute flaccid paralysis (AFP). Because poliomyelitis is on the verge of eradication, more attention should be paid to study NPEVs from non-polio AFP cases and their epidemic patterns. In West African countries the epidemiology of NPEVs remains largely unexplored. We investigated the genetic diversity, frequency, circulation patterns, and molecular epidemiology of NPEVs in seven West African countries by analyzing retrospectively a panel of 3195 stool samples from children with AFP collected through routine poliomyelitis surveillance activities between 2013 and 2014. VP1 sequencing and typing on 201 isolates revealed 39 NPEV types corresponding to EV-A (6.9%), EV-B (90.5%), EV-C (2%) and EV-D (0.5%) species. Echoviruses were isolated most frequently with 138 cases (68.6%), followed by coxsackievirus group B with 35 cases (17.4%). No single NPEV type was remarkably dominant. Interestingly, several rarely described types with limited detection worldwide were identified (EVA76, EVA119, EVB75, EVB77, EVB97, EVC99, CVA20, CVA21 and EVD94). This study demonstrates the extensive diversity and diverse circulation patterns of NPEVs from AFP surveillance and highlights the need to formulate effective long-term strategies to monitor NPEV circulations in West Africa.

Motor exam of patients with spinal cord injury: a terminological imbroglio.

The description of the motor deficit of patients with spinal cord injury (SCI) varies significantly, leading to confusion within the neurological terminology. This paper proposes a concise and easy to use terminology to describe the motor deficit of patients with SCI. A broad review of the origin of the nomenclature used to describe the motor deficit of patients with SCI was performed and discussed. The prefix: "hemi" should be used to describe paralysis of one half of the body; "mono" for one limb; "para" for lower limbs, di" for two symmetrical segments and/or parts in both sides of the body; "tri" for three limbs, or two limbs and one side of the face; and "tetra" for four limbs. The suffix: "plegia" should be used for total paralysis of a limb or part of the body, and "paresis" for partial paralysis. The term "brachial" refers to the upper limbs; and "podal" to the lower limbs. According to the spinal cord origin of the main key muscles for the limbs, patients with complete injury affecting spinal cord segments C1-5 usually presents with "tetraplegia"; C6-T1 presents with "paraplegia and brachial diparesis"; T2-L2 with "paraplegia"; and L3-S1 with "paraparesis".

Reliability and Validity of the Sensory Component of the International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI): A Systematic Review.

BACKGROUND: Since 1982, the International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI) has been used to classify sensation of spinal cord injury (SCI) through pinprick and light touch scores. The absence of proprioception, pain, and temperature within this scale creates questions about its validity and accuracy. OBJECTIVE: To assess whether the sensory component of the ISNCSCI represents a reliable and valid measure of classification of SCI. METHODS: A systematic review of studies examining the reliability and validity of the sensory component of the ISNCSCI published between 1982 and February 2013 was conducted. The electronic databases MEDLINE via Ovid, CINAHL, PEDro, and Scopus were searched for relevant articles. A secondary search of reference lists was also completed. Chosen articles were assessed according to the Oxford Centre for Evidence-Based Medicine hierarchy of evidence and critically appraised using the McMasters Critical Review Form. A statistical analysis was conducted to investigate the variability of the results given by reliability studies. RESULTS: Twelve studies were identified: 9 reviewed reliability and 3 reviewed validity. All studies demonstrated low levels of evidence and moderate critical appraisal scores. The majority of the articles (~67%; 6/9) assessing the reliability suggested that training was positively associated with better posttest results. The results of the 3 studies that assessed the validity of the ISNCSCI scale were confounding. CONCLUSIONS: Due to the low to moderate quality of the current literature, the sensory component of the ISNCSCI requires further revision and investigation if it is to be a useful tool in clinical trials.

[Hereditary spastic paraplegia: up to date].

Hereditary spastic paraplegia (HSP) is a clinically and genetically heterogeneous group of neurodegenerative disorders that are clinically characterized by progressive spasticity and weakness of the lower limbs. HSP genetic loci are designated SPG1-72 in order of their discovery. In 206 Japanese families with autosomal dominant HSP, SPG4 was the most common form, accounting for 38%, followed by SPG3A (5%), SPG31 (5%), SPG10 (2%), and SPG8 (1%). We have identified novel mutations in the C12orf65 gene and the LYST gene in several Japanese families with autosomal recessive HSP. JASPAC will facilitate gene discovery and mechanistic understanding of HSP. The future challenge will be the establishment of treatment of HSP.

Impact of pressure ulcers on individuals living with a spinal cord injury.

OBJECTIVE: To describe the impact of pressure ulcers on the ability to participate in daily and community activities, health care utilization, and overall quality of life in individuals living with spinal cord injury (SCI). DESIGN: Cross-sectional study. SETTING: Nationwide survey. PARTICIPANTS: Participants (N=1137) with traumatic SCI who were >1 year postinjury and living in the community were recruited. Of these, 381 (33.5%, 95% confidence interval, 30.8%-36.3%) had a pressure ulcer over the last 12 months. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Measures developed for the Rick Hansen Spinal Cord Injury Registry Community Follow-up Survey Version 2.0. RESULTS: Of the 381 individuals with pressure ulcers, 65.3% reported that their pressure ulcer reduced their activity to some extent or more. Pressure ulcers reduced the ability of individuals with SCI to participate in 19 of 26 community and daily activities. Individuals with 1 or 2 pressure ulcers were more dissatisfied with their ability to participate in their main activity than those without pressure ulcers (P=.0077). Pressure ulcers were also associated with a significantly higher number of consultations with family doctors, nurses, occupational therapists, and wound care nurses/specialists (P<.05). CONCLUSIONS: Pressure ulcers have a significant impact on the daily life of individuals with SCI. Our findings highlight the importance of implementing pressure ulcer prevention and management programs for this high-risk population and require the attention of all SCI-related health care professionals.

[Molecular genetics and gene analysis of hereditary spastic paraplegia].

Hereditary spastic paraplegia (HSP) is a neurodegenerative disorder which is characterized by spasticity of the leg. HSP is a clinically and genetically heterogeneous disorder. Mutations were detected in about 60% of autosomal dominant HSP patients. SPG4 is the most common form of autosomal dominant HSP worldwide. In autosomal recessive HSP patients, we detected mutations in about 40% using exome sequencing. Causes of autosomal recessive HSP are more heterogeneous than those of autosomal dominant HSP. We have to consider leukodystrophies/leukoencephalopathies, motor neuron diseases, spinocerebellar degenerations, or various metabolic diseases as differential diagnosis of complicated HSP. X-linked HSP or HSP with mitochondorial inheritance are rare. Further work on familial patients would lead to identify novel causative genes, which helps to understand pathophysiology of HSP and the nature of corticospinal tract and establish disease modifying therapy. Mutation detection rate for sporadic HSP is low at the moment, and molecular delineation of sporadic HSP is expected in the future.

Tetraplegia or paraplegia with brachial diparesis? What is the most appropriate designation for the motor deficit in patients with lower cervical spinal cord injury?

The authors seek to clarify the nomenclature used to describe cervical spinal cord injuries, particularly the use of the terms "tetraplegia", "quadriplegia", "quadriparesis", "tetraparesis", "incomplete quadriplegia" or "incomplete tetraplegia" when applied to patients with lower cervical cord injuries. A review of the origin of the terms and nomenclature used currently to describe the neurological status of patients with SCI in the literature was performed. The terms "tetraplegia", "quadriplegia", "quadriparesis", "tetraparesis", "incomplete quadriplegia" or "incomplete tetraplegia" have been used very often to describe patients with complete lower cervical SCI despite the fact that the clinical scenario is all the same for most of these patients. Most of these patients have total loss of the motor voluntary movements of their lower trunk and inferior limbs, and partial impairment of movement of their superior limbs, preserving many motor functions of the proximal muscles of their arms (superior limbs). A potentially better descriptive term may be "paraplegia with brachial diparesis". In using the most appropriate terminology, the patients with lower cervical SCI currently referred as presenting with "tetraplegia", "quadriplegia", "quadriparesis", "tetraparesis", "incomplete quadriplegia" or "incomplete tetraplegia", might be better described as having "paraplegia with brachial diparesis".

Functional outcome of patients 12 and 48 weeks after acute traumatic tetraplegia and paraplegia: data analysis from 2004-2009.

OBJECTIVES: During the first rehabilitation of patients with traumatic spinal cord injuries (SCIs), professional skills are learned, which can be objectified in an independent measurement score. The aims of this study were to record the skills of patients 12 and 48 weeks after acute trauma and perform an analysis of the data to identify provisions of importance. METHODS: All patients from 2004 to 2009 who experienced traumatic SCI were included in this investigation. Data recording were accomplished by the European Multi-Centre Study about Spinal Cord Injury (EMSCI) databank. Patients were divided into tetraplegia and paraplegia groups. Parameters were age at injury, the American Spinal Injury Association-Score, level of lesion and spinal cord independence measure (SCIM-Score) 12 and 48 weeks after traumatic spinal cord lesion. A questionnaire was also added to help clarify where deficiencies were prevalent. RESULTS: Data analysis of 103 tetraplegic and 110 paraplegic patients showed no correlation between the ASIA score, level of lesion, age and SCIM score. On average, tetraplegic patients had a SCIM score of 43 points 12 weeks after treatment, with 81% showing an increase to 58 points after 48 weeks. Paraplegic patients showed an average SCIM score of 60 points after 12 weeks, with 71% experiencing an increase to 71 points after 48 weeks. In all, 9% of tetraplegic patients and 19% of paraplegic patients experienced a decrease of SCIM points after 48 weeks, which occurred mainly in the bladder and intestinal control subgroups. Results of the questionnaire were not helpful for clarifying the location of the deficiencies. CONCLUSION: Most of the patients experienced an increase of SCIM points 48 weeks after traumatic SCI. However, data also showed that, especially in paraplegic patients, special attention must be given to bladder and intestinal management to avoid negative late-term consequences.

Paraparesis or incomplete paraplegia? How should we call it?

INTRODUCTION: The neurological examination terminologies and definitions of the status of spinal cord injured (SCI) patients are of great importance to establish scales and provide standard nomenclatures. There is a disagreement between the classical neurological terminology and the definitions of complete and incomplete paraplegia that have been proposed in traumatic spinal cord injured patients. OBJECTIVE: To discuss the adequacy and the impact of the terms incomplete paraplegia and paraparesis in current literature. MATERIALS AND METHODS: A review of the origin of the terms, definitions and nomenclatures applied by the most widespread assessment scales in traumatic SCI published in peer review papers was performed, searching the scales cited on the references of the latest American Spinal Injury Association classification (2002; available in http://www.asia-spinalinjury.org/ ) up to the first classification, described by Frankel et al. [14]. RESULTS: The term "incomplete paraplegia" has been used to define clinical situations classically described as "paraparesis". CONCLUSION: The terms "complete" and "incomplete" are adequately used to characterize the completeness of spinal cord lesion but inadequately used when associated to the term "plegia" as a qualifier. Therefore, patients with any preservation of motor strength below the injury level should be described as paraparetic and not as incomplete paraplegic.

Intra-rater agreement of the anorectal exam and classification of injury severity in children with spinal cord injury.

STUDY DESIGN: Intra-rater reliability study, cross-sectional design. OBJECTIVES: To report on the intra-rater agreement of the anorectal examinations and classification of injury severity in children with spinal cord injury (SCI). SETTING: Two, non-profit children's hospitals specializing in pediatric SCI. METHODS: 180 subjects had at least two trials of the anorectal examinations as defined by the International Standards for Neurological Classification of Spinal Cord Injury. Intraclass correlation coefficients (ICC) and 95% confidence intervals (CI) were used to evaluate the agreement. ICC>0.90=high agreement; ICC between 0.75-0.89=moderate agreement; ICC<0.75=poor agreement. RESULTS: When evaluated for the entire sample, agreement was moderate-high for anal sensation and contraction and injury classification. When evaluated as a function of age at examination and type of injury, agreement for anal sensation was poor for subjects with tetraplegia in the 12-15-year age group (ICC=0.56) and 16-21-year age group (ICC=0.70) and for subjects with paraplegia in the 6-11-year age group (ICC=0.69). Agreement for anal contraction was moderate for subjects with tetraplegia in the 16-21-year age group (ICC=0.81) and subjects with paraplegia in the 12-15-year age group (ICC=0.78) and poor for subjects with paraplegia in the 6-11-year age group (ICC=0.67). Agreement for injury classification was poor for subjects with tetraplegia in the 12-15-year group (ICC=0.56) and 16-21-year group (ICC=0.74) and paraplegia in the 6-11-year group (ICC=0.11) and 12-15-year group (ICC=0.63). Anorectal responses had high agreement in subjects with tetraplegia in the 6-11-year group and moderate to high agreement in subjects with paraplegia in the 16-21-year group. CONCLUSION: The data do not fully support the use of anorectal examination in children. Further work is warranted to establish the validity of anorectal examination.

Five additional mobility and locomotor items to improve responsiveness of the FIM in wheelchair-dependent individuals with spinal cord injury.

STUDY DESIGN: Repeated-measures design. OBJECTIVES: To assess the validity and responsiveness of five additional mobility and locomotor (5-AML) items when used in conjunction with the Functional Independence Measure (FIM) for assessing the mobility and locomotor function of individuals with spinal cord injury (SCI). SETTING: Specialised acute spinal and rehabilitation units in Sydney, Australia. METHODS: A previously published scale comprising five key mobility and locomotor skills was further refined. The five items included a bed mobility and vertical (floor-to-chair) transfer item and three wheelchair propulsion items (pushing 200 m over flat ground, pushing up a ramp and negotiating a kerb). A total of 43 eligible patients with SCI (ASIA A-C impairment) admitted consecutively to two acute SCI units in Sydney between 1999 and 2002 were recruited, with four being lost to follow-up. Locomotor and mobility outcomes were measured at regular intervals for up to 6 months with the FIM and the 5-AML. Construct validity of the 5-AML was assessed by testing ability of items to discriminate between different impairment groups (tetraplegia and paraplegia). Item responsiveness was assessed by analysing ability to detect changes in mobility and locomotor function over time. Factor analysis techniques were used to test the hypothesis that the 5-AML, when used in conjunction with the FIM, provides a more sensitive measure of mobility and locomotor function than the FIM alone. RESULTS: The 5-AML items were shown to be valid and responsive, measuring aspects of 'real world' mobility and locomotor function not reflected by the FIM. The bed mobility item was highly responsive to change over time for the tetraplegic group, but quickly reached a ceiling in the paraplegic group. The vertical (floor-to-chair) transfer item showed greater responsiveness over time and less ceiling effect for the paraplegic group than any of the FIM locomotor or mobility items. The three wheelchair propulsion items better discriminated between people with tetraplegia and paraplegia, and were more sensitive to changes in locomotor ability over the 6-month period than FIM locomotor items. Results of a preliminary factor analysis indicated that the 5-AML items measure different aspects of mobility and locomotor function than the FIM. CONCLUSION: The 5-AML items, when used in conjunction with the FIM, provides better delineation of function between people with tetraplegia and paraplegia and provides a more responsive measure of change in function over time than the FIM alone.

Semiquantitative assessment of hindlimb movement recovery without intervention in adult paraplegic mice.

STUDY DESIGN: Experimental laboratory investigation of hindlimb movement recovery in chronic paraplegic mice. OBJECTIVES: Development of an assessment method to discriminatively quantify motor and locomotor-like movements of paraplegic mice. SETTING: Laval University Medical Center, Quebec, Canada. METHODS: Signs of 'functional recovery' were examined in open-field condition during 1 month in adult mice with a complete spinal cord transection at the low-thoracic level. RESULTS: None of the mice exhibited hindlimb movements after spinalization. At 7 days, 33% of them displayed weak nonbilaterally alternating movements (NBA). At 14 days, increased NBA were observed and the first bilaterally alternating movements (BA) in 10% of the mice. A progressive increase of movement frequency and amplitude was found after 2-3 weeks. By the end of the month, 86% displayed mixed NBA and BA. However, none of them recovered the ability to stand or bear their own weight with the hindlimbs. CONCLUSION: This study reports signs of partial hindlimb movement recovery in chronic paraplegic mice and provides evidence of plasticity in sublesional circuits of neurons occurring in the absence of inputs from the brain, locomotor training or pharmacological treatment. This assessment method can be used to characterize hindlimb movements in complete spinal cord transected mice tested in open-field condition.

Survival and death-promoting events after transient spinal cord ischemia in rabbits: induction of Akt and caspase3 in motor neurons.

OBJECTIVE: The mechanism of spinal cord injury has been thought to be related to the vulnerability of spinal motor neuron cells to ischemia. However, the mechanisms of such vulnerability are not fully understood. We previously reported that spinal motor neurons might be lost as a result of programmed cell death and investigated a possible mechanism of neuronal death by means of immunohistochemical analysis for CPP32 (caspase3) and serine-threonine kinase (Akt). METHODS: We used a rabbit spinal cord ischemia model with use of a balloon catheter. The spinal cord was removed at 8 hours or 1, 2, or 7 days after 15 minutes of transient ischemia, and histologic changes were studied with hematoxylin and eosin staining. Western blot analysis for Akt and caspase3, temporal profiles of Akt and caspase3 immunoreactivity, and double-label fluorescence immunocytochemical studies were performed. RESULTS: The majority of motor neurons were preserved until 2 days but were selectively lost at 7 days of reperfusion. Western blot analysis revealed no immunoreactivity for Akt and caspase3 in the sham-operated spinal cords. However, such immunoreactivity became apparent at 8 hours after transient ischemia, decreased at 1 day, and returned to the baseline level at 2 days. A double-label fluorescence immunocytochemical study revealed that both Akt and caspase3 were positive at 8 hours of reperfusion in the same motor neurons, which eventually die. CONCLUSION: These results suggests that transient spinal cord ischemia activates both cell death and survival pathways after ischemia. The activation of Akt protein at the early stage of reperfusion might be one of the factors responsible for the delay in neuronal death after spinal cord ischemia.

Comparison of the revised 2000 American Spinal Injury Association classification standards with the 1996 guidelines.

OBJECTIVE: This study was undertaken to determine the level of agreement between the most recent change in the American Spinal Injury Association International Standards (2000) and the previous (1996) classification. DESIGN: In a spinal cord injury rehabilitation hospital, data were collected on 94 subjects who had an initial neurologic examination according to the International Standards within 1 wk of injury and again at 1 yr. Comparisons were examined of the level of agreement between the 1996 and 2000 revisions in classification of the motor incomplete levels and ability to prognosticate outcome at 1 yr on the basis of the initial examination. RESULTS: Near perfect agreement between the 1996 and 2000 revised guidelines in the classification of motor incomplete injuries was found, with no statistically significant difference for prognosticating neurologic recovery at 1 yr on the basis of the initial examination. CONCLUSION: The 2000 revisions do not offer a significant difference in American Spinal Injury Association impairment classification or in predicting neurologic recovery at 1 yr.

[Hereditary ataxias and paraplegias: a clinicogenetic review]. / Ataxias y paraplejías hereditarias: revisión clinicogenética.

Hereditary ataxias encompass a series of syndromes basically characterised by progressive cerebellar ataxia of slow clinical course (occasionally, periodic ataxia or spastic paraparesis) and primary spinocerebellar degeneration. The prevalence ratio of these syndromes in Spain is 20 cases per 100,000 inhabitants. Initially the ataxias were classified on the basis of clinicopathological criteria. Starting from the seminal papers by Harding published 20 years ago, a clinicogenetic classification was introduced that has given way to the present molecular classification. There have been localised about forty loci. In dominant ataxias the most frequent molecular defect is a dynamic CAG expansion responsible for abnormal polyglutamine tract transcription. The identification of such molecular defect has made it possible detection of gene carriers in clinical practice, this involving both presymptomatic and prenatal diagnosis; moreover, such molecular discoveries have contributed to develop a new pathogenetic era. A homozygous and intronic GAA expansion is the molecular basis of Friedreich's ataxia. This finding has also made it possible a molecular diagnosis in clinical practice. Molecular studies have demonstrated that hereditary spastic paraplegia is another heterogeneous genetic disorder.