Dutch-Flemish translation and cross-cultural adaption of the Spinal Cord Injury-Quality of Life (SCI-QOL) short forms.

PURPOSE: A spinal cord injury (SCI) has a large impact on a person's physical, psychological, and social health. Previous studies focused on physical recovery in patients with SCI and assessed their health-related quality of life (HRQOL) with general questionnaires, which often contain irrelevant questions. The SCI-QOL questionnaire was developed to specifically assess the HRQOL of the SCI population. A comprehensive translation and cross-cultural adaptation are required to use this questionnaire in the Netherlands and Flanders, Belgium. METHODS: All 23 short forms of the SCI-QOL for adults were translated from English into Dutch-Flemish using the Functional Assessment of Chronic Illness Therapy translation methodology, with a cognitive debriefing being performed with 10 patients with SCI and 10 persons from the general population. RESULTS: The Dutch-Flemish PROMIS group previously translated 46 of the 207 items in the 23 short forms. Here, we achieved an acceptable translation of the other 161 items. A single Dutch-Flemish translation was obtained for 20 short forms, while separate Dutch and Flemish translations were necessary for the short forms Ambulation, Manual Wheelchair, and Stigma. CONCLUSION: The Dutch-Flemish translation of the SCI-QOL is now available for clinical and research purposes. Future studies should focus on the psychometric properties of this cross-culturally adapted version.

Oral itraconazole for epistaxis in hereditary hemorrhagic telangiectasia: a proof of concept study.

The inhibiting effects of itraconazole, an antifungal drug on vascular endothelial growth factor (VEGF) have recently been discovered. By inhibiting VEGF, itraconazole has shown potential in clinical trials as anti-cancer treatment. In hereditary hemorrhagic telangiectasia (HHT) patients, VEGF levels are elevated and inhibition of VEGF can decrease bleeding. Itraconazole could potentially serve as anti-angiogenic therapy for HHT-related bleeding. We report a proof of concept study with HHT patients and severe epistaxis. Patients were treated with daily 200 mg orally administered itraconazole for sixteen weeks. Twenty-one HHT patients, 8 females (38%), 13 males (62%), median age of 59 years (interquartile range (IQR) 55-69) were enrolled. Of these patients, 13 (62%) were diagnosed with HHT type 1, seven (33%) with HHT type 2 and in one patient (5%), no pathognomonic HHT mutation was found. Four patients (19%) prematurely terminated the study (3 due to mild or moderate side-effects) resulting in 17 patients included in the analyses. The median epistaxis severity score significantly decreased during treatment from 6.0 (IQR 5.1-7.2) to 3.8 (IQR 3.1-5.2) (p = 0.006). The monthly epistaxis frequency decreased from 56 to 38 epistaxis episodes (p = 0.004) and the monthly duration from 407 to 278 minutes (p = 0.005). Hemoglobin levels did not significantly change. The quality of life showed a small but significant improvement. In conclusion, oral itraconazole significantly improved epistaxis in HHT patients. The potential benefit of itraconazole in HHT should be further investigated.

Reproducibility of right-to-left shunt quantification using transthoracic contrast echocardiography in hereditary haemorrhagic telangiectasia.

AIM: Transthoracic contrast echocardiography (TTCE) is recommended for screening of pulmonary arteriovenous malformations (PAVMs) in hereditary haemorrhagic telangiectasia. Shunt quantification is used to find treatable PAVMs. So far, there has been no study investigating the reproducibility of this diagnostic test. Therefore, this study aimed to describe inter-observer and inter-injection variability of TTCE. METHODS: We conducted a prospective single centre study. We included all consecutive persons screened for presence of PAVMs in association with hereditary haemorrhagic telangiectasia in 2015. The videos of two contrast injections per patient were divided and reviewed by two cardiologists blinded for patient data. Pulmonary right-to-left shunts were graded using a three-grade scale. Inter-observer and inter-injection agreement was calculated with κ statistics for the presence and grade of pulmonary right-to-left shunts. RESULTS: We included 107 persons (accounting for 214 injections) (49.5% male, mean age 45.0 ± 16.6 years). A pulmonary right-to-left shunt was present in 136 (63.6%) and 131 (61.2%) injections for observer 1 and 2, respectively. Inter-injection agreement for the presence of pulmonary right-to-left shunts was 0.96 (95% confidence interval (CI) 0.9-1.0) and 0.98 (95% CI 0.94-1.00) for observer 1 and 2, respectively. Inter-injection agreement for pulmonary right-to-left shunt grade was 0.96 (95% CI 0.93-0.99) and 0.95 (95% CI 0.92-0.98) respectively. There was disagreement in right-to-left shunt grade between the contrast injections in 11 patients (10.3%). Inter-observer variability for presence and grade of the pulmonary right-to-left shunt was 0.95 (95% CI 0.91-0.99) and 0.97 (95% CI 0.95-0.99) respectively. CONCLUSION: TTCE has an excellent inter-injection and inter-observer agreement for both the presence and grade of pulmonary right-to-left shunts.

Definitions of traumatic conus medullaris and cauda equina syndrome: a systematic literature review.

STUDY DESIGN: A systematic review. OBJECTIVES: Conus medullaris syndrome (CMS) and cauda equina syndrome (CES) are well-known neurological entities. It is assumed that these syndromes are different regarding neurological and functional prognosis. However, literature concerning spinal trauma is ambiguous about the exact definition of the syndromes. METHODS: A MEDLINE, EMBASE and Cochrane literature search was performed. We included original articles in which clinical descriptions of CMS and/or CES were mentioned in patients following trauma to the thoracolumbar spine. RESULTS: Out of the 1046 articles, we identified 14 original articles concerning patients with a traumatic CMS and/or CES. Based on this review and anatomical data from cadaveric and radiological studies, CMS and CES could be more precisely defined. CONCLUSION: CMS may result from injury of vertebrae Th12-L2, and it involves damage to neural structures from spinal cord segment Th12 to nerve root S5. CES may result from an injury of vertebrae L3-L5, and it involves damage to nerve roots L3-S5. This differentiation between CMS and CES is necessary to examine the hypothesis that CES patients tend to have a better functional outcome.

Cancer and hereditary haemorrhagic telangiectasia.

OBJECTIVE: To examine associations between cancer incidence and hereditary haemorrhagic telangiectasia (HHT). METHODS: Two studies with contrasting conclusions were compared. The first had used a registry-based, matched-pairs approach, while the second utilised HHT family-based, survey methodology. RESULTS: The first manuscript captured data on cancer incidence in a total of 316,581 matched cancer patients-non-cancer controls, which included 431 HHT cases. No association was found between HHT and pooled cases of lung, breast, prostate, and colorectal cancer (adjusted OR 0.978, 95% CI [0.795, 1.204]). The second, which was powered to examine these four cancers individually, captured data from 2161 HHT cases and 2817 related controls. Fewer HHT-affected individuals had cancer (398/2161, [18.4%]) compared to 668/2817 (23.7%) related controls (p = 0.0012). Of the four most common cancers, prostate and colorectal cancer rates were equivalent, but lung cancers were significantly less frequent in HHT (adjusted OR 0.48 [0.30, 0.70], p = 0.0012), and breast cancer was more frequent (adjusted OR 1.52 [1.07, 2.14] p = 0.018). CONCLUSIONS: The respective studies had different methodological strengths and weaknesses. Potential reasons for the discrepant conclusions include study power, particularly important to dissect specific cancers where differential contributions from HHT genotypes and environmental confounders might be predicted.

Life expextancy of parents with Hereditary Haemorrhagic Telangiectasia.

BACKGROUND: Hereditary Haemorrhagic Telangiectasia (HHT) is an autosomal dominant disease associated with epistaxis, arteriovenous malformations and telangiectasias. Disease complications may result in premature death. METHOD: We investigated life-expectancies of parents of HHT patients compared with their non-HHT partners using self- or telephone-administered questionnaires sent to their children. Patients were extracted from the databases of 2 participating HHT Centres: the Toronto HHT Database (Toronto, Canada) and the St. Antonius Hospital HHT Database (Nieuwegein, The Netherlands). RESULTS: Two hundred twenty five/407 (55%) of respondents were included creating HHT- (n = 225) and control groups (n = 225) of equal size. Two hundred thirteen/225 (95%) of the HHT group had not been screened for organ involvement of the disease prior to death. The life expectancy in parents with HHT was slightly lower compared to parents without (median age at death 73.3 years in patients versus 76.6 years in controls, p0.018). Parents with ACVRL 1 mutations had normal life expectancies, whereas parents with Endoglin mutations died 7.1 years earlier than controls (p = 0.024). Women with Endoglin mutations lived a median of 9.3 years shorter than those without (p = 0.04). Seven/123 (5%) of deaths were HHT related with a median age at death of 61.5 years (IQ range 54.4-67.7 years). CONCLUSION: Our study showed that the life expectancy of largely unscreened HHT patients was lower than people without HHT. Female patients with Endoglin mutations were most strikingly at risk of premature death from complications. These results emphasize the importance of referring patients with HHT for screening of organ involvement and timely intervention to prevent complications.

Pre-hospital and acute management of traumatic spinal cord injury in the Netherlands: survey results urge the need for standardisation.

STUDY DESIGN: Questionnaire survey. OBJECTIVES: Although a range of novel therapeutic approaches for traumatic spinal cord injury (tSCI) are being trialled in highly standardised, pre-clinical research models, little has been published about the extent of standardisation in health service delivery for newly injured tSCI patients. SETTING: All Emergency Medical Services (EMSs) and 11 level-1 trauma centres (L1TCs) in the Netherlands. METHODS: A survey assessing the organisation of pre-hospital and acute tSCI management was developed and distributed across all 23 pre-hospital EMSs and 11 L1TCs based in the Netherlands. RESULTS: Response rates for EMSs and L1TCs were 82 and 100%, respectively. Thirteen EMSs (68%) transported all patients who are suspected of having tSCI to L1TCs. The decision to transfer tSCI patients to L1TCs was primarily made by paramedics at the scene of accident (79%). Nonetheless, no EMS reported the use of validated neurological assessments for determining the likelihood of tSCI. The International Standards for Neurological Classification of SCI were used to determine the level and severity of tSCI in four centres, and three centres performed magnetic resonance imaging in all tSCI patients. Three L1TCs had spinal cord perfusion support protocols in place, and two centres administered methylprednisolon to acute tSCI patients. CONCLUSION: We found a large variance in the delivery of pre-hospital and acute tSCI management in a well-defined geographical catchment area. This survey urges the need for implementing standardised assessments and developing best-practice guidelines, which should be endorsed by all pre-hospital and acute tSCI health-care providers.

Follow-up of Thalidomide treatment in patients with Hereditary Haemorrhagic Telangiectasia.

BACKGROUND: Patients with a hereditary vascular disorder called Rendu-Osler-Weber syndrome (Hereditary Haemorrhagic Telangiectasia, HHT) haemorrhage easily due to weak-walled vessels. Haemorrhage in lungs or brain can be fatal but patients suffer most from chronic and prolonged nosebleeds (epistaxis), the frequency and intensity of which increases with age. Several years ago, it was discovered serendipitously that the drug Thalidomide had beneficial effects on the disease symptoms in several of a small group of HHT patients: epistaxis and the incidence of anaemia were reduced and patients required fewer blood transfusions. In addition, they reported a better quality of life. However, Thalidomide has significant negative side effects, including neuropathy and fatigue. METHODS: We followed up all HHT patients in the Netherlands who had been taking Thalidomide at the time the original study was completed to find out (i) how many had continued taking Thalidomide and for how long (ii) the nature and severity of any side-effects and (iii) whether side-effects had influenced their decision to continue taking Thalidomide. RESULTS: Only a minority of patients had continued taking the drug despite its beneficial effects on their symptoms and that the side effects were the primary reason to stop. CONCLUSION: Despite symptom reduction, alternative treatments are still necessary for epistaxis in HHT patients and a large-scale clinical trial is not justified although incidental use in the most severely affected patients can be considered.

Structural biomarkers in the cerebrospinal fluid within 24 h after a traumatic spinal cord injury: a descriptive analysis of 16 subjects.

STUDY DESIGN: Prospective cohort study. OBJECTIVES: To characterize the cerebrospinal fluid (CSF) concentrations of glial fibrillary acidic protein, neuron specific enolase (NSE), S-100ß, tau and neurofilament heavy chain (NFH) within 24 h of an acute traumatic spinal cord injury (SCI), and to correlate these concentrations with the baseline severity of neurologic impairment as graded by the American Spinal Injury Association impairment scale (AIS). METHODS: A lumbar puncture was performed to obtain CSF from 16 acute traumatic SCI patients within 24 h post injury. Neurological examinations were performed within 24 h of injury and again at 6 or 12 months post injury. The correlations between the CSF concentrations and initial AIS were calculated by using Pearson correlation coefficients. In addition, an independent Student's t-test was used to test for differences in CSF concentrations between patients of different AIS grades. RESULTS: The CSF NSE concentrations were significantly correlated with the baseline neurologic impairment being either 'motor complete' (AIS A, B) or 'motor incomplete' (AIS C, D) (r=0.520, P<0.05). The mean S-100ß concentration in motor complete patients was significantly higher compared with motor incomplete patients; 377.2 µg l(-1) (s.d.±523 µg l(-1)) vs 57.1 µg l(-1) (s.d.±56 µg l(-1)) (P<0.05), respectively. Lastly, the mean NFH concentration in motor complete patients was significantly higher compared with motor incomplete patient, 11 813 ng l(-1) (s.d.±16 195 ng l(-1)) vs 1446.8 ng l(-1) (s.d.±1533 ng l(-1)), (P<0.05), respectively. CONCLUSION: In this study we identified differences in the structural CSF biomarkers NSE, S-100ß and NFH between motor complete and motor incomplete SCI patients. Our data showed no clear differences in any of the protein concentrations between the different AIS grades.

Acute sinusitis and blindness as the first presentation of chronic lymphocytic leukaemia.

Chronic lymphocytic leukaemia (CLL) is the most frequent form of leukaemia among adults in the Western world, presenting at a median age of 65 years. The diagnosis is usually made incidentally during routine blood examination while the disease is still in its early phase. We report a case of blindness of 24 hours due to acute sinusitis based on CLL localisation in a patient with undiagnosed CLL. Emergency endoscopic sinus surgery and intra- and extra-ocular orbital decompression were performed. The sinusitis resolved after surgery and intravenous antibiotics. Her vision improved within 24 hours and eventually recovered completely after six months. Her CLL remained in an indolent state, needing no active treatment. This case illustrates that blindness from a lymphoproliferative disorder may be treated with emergency endoscopic sinus surgery instead of conventional chemotherapy in order to salvage the vision first, even if the vision is lost for more than 24 hours.

Design and rationale of a Prospective, Observational European Multicenter study on the efficacy of acute surgical decompression after traumatic Spinal Cord Injury: the SCI-POEM study.

OBJECTIVES: Despite many years of research, there is currently no treatment available that results in major neurological or functional recovery after traumatic spinal cord injury (tSCI). In particular, no conclusive data related to the role of the timing of decompressive surgery, and the impact of injury severity on its benefit, have been published to date. This paper presents a protocol that was designed to examine the hypothesized association between the timing of surgical decompression and the extent of neurological recovery in tSCI patients. STUDY DESIGN: The SCI-POEM study is a Prospective, Observational European Multicenter comparative cohort study. This study compares acute (<12 h) versus non-acute (>12 h, <2 weeks) decompressive surgery in patients with a traumatic spinal column injury and concomitant spinal cord injury. The sample size calculation was based on a representative European patient cohort of 492 tSCI patients. During a 4-year period, 300 patients will need to be enrolled from 10 trauma centers across Europe. The primary endpoint is lower-extremity motor score as assessed according to the 'International standards for neurological classification of SCI' at 12 months after injury. Secondary endpoints include motor, sensory, imaging and functional outcomes at 3, 6 and 12 months after injury. CONCLUSION: In order to minimize bias and reduce the impact of confounders, special attention is paid to key methodological principles in this study protocol. A significant difference in safety and/or efficacy endpoints will provide meaningful information to clinicians, as this would confirm the hypothesis that rapid referral to and treatment in specialized centers result in important improvements in tSCI patients.

Diffusion-weighted MR imaging within 24 h post-injury after traumatic spinal cord injury: a qualitative meta-analysis between T2-weighted imaging and diffusion-weighted MR imaging in 18 patients.

STUDY DESIGN: Only few studies have been published about diffusion-weighted imaging (DWI) within 24 h of traumatic spinal cord injury (tSCI). OBJECTIVES: The purpose of this study was to compare the imaging findings from conventional magnetic resonance imaging (MRI) and DWI in seven tSCI patients with findings in the existing literature. METHODS: Seven patients with tSCI at neurologic levels C2-T10 were examined with conventional MRI and DWI within 24 h post-injury. DWI was obtained with a b-factor of 1000 s mm(-2). American Spinal Injury Association (ASIA) scores and Spinal Cord Independence Measurement (SCIM) II item 12 after 12 months were collected. In addition, MEDLINE was searched from 1995 to 2010 to identify clinical tSCI studies reporting on MRI, DWI and apparent diffusion coefficient maps within 24 h post-injury to perform a meta-analysis. Images obtained with a b-factor of 1000 s mm(-2) were compared with lower b-factors. Differences were calculated using χ (2) tests. RESULTS: No associations were identified between the images of the seven tSCI patients and ASIA or SCIM II scores. Eighteen SCI patients (11 from the retrieved publications) were included in the meta-analysis. The detection rates of hyperintense signals on T2-weighted and DW imaging did not show significant differences at 94 and 72%, respectively. In addition, there were no significant differences in detection rates or diffusion abnormalities between subjects in whom DW images were obtained with a maximum b-factor of 1000 or <1000 s mm(-2). CONCLUSION: Our analysis suggests that T2-weighted and DW imaging have comparable detection rates for spinal cord damage in tSCI patients within 24 h post-injury.

How does knowledge about spinal cord injury-related complications develop in subjects with spinal cord injury? A descriptive analysis in 214 patients.

STUDY DESIGN: Monocentric cohort study. OBJECTIVE: To investigate the acquisition of knowledge about spinal cord injury (SCI)-related complications in SCI patients. SETTING: Level 1 trauma center. METHODS: All patients with a traumatic or non-traumatic SCI were included in the study. Data were collected at admission, post-admission at 1 and 3 months and post-discharge at 6, 18 and 30 months. The discharge of all patients was between 3 and 6 months post-admission. Knowledge about pressure ulcers and bladder management was tested using the 'Knowledge' score. This score has a minimum and maximum of 0 and 20 points. To detect differences across the multiple time intervals, the Friedman test was used. Differences in the number of patients with poor (0-8), average (9-12) and good knowledge (13-20) between the different age classifications (age at injury) were calculated using a χ (2)-test. RESULTS: A total of 214 patients were included. At discharge subjects had increased their knowledge score to 11.2 compared with 5.4 on admission (P < 0.001). After 30 months, however, the mean score decreased to 10.8 points. At the time of discharge, the number of patients who achieved poor, average or good knowledge were 48 (22.4%), 65 (30.4%) and 101 (47.2%), respectively. Subjects of ∼50 years old and tetraplegics had better (P < 0.001) knowledge compared with subjects of ∼50 years old and paraplegics, respectively. CONCLUSION: In this study, less than 50% of SCI patients had good knowledge about bladder management and pressure ulcers after being discharged.

Is the outcome in acute spinal cord ischaemia different from that in traumatic spinal cord injury? A cross-sectional analysis of the neurological and functional outcome in a cohort of 93 paraplegics.

STUDY DESIGN: Retrospective cohort study. OBJECTIVES: To compare the neurological outcome between paraplegic patients with acute spinal cord ischaemia syndrome (ASCIS) or traumatic spinal cord injury (tSCI) and to investigate the influence of SCI aetiology on the total Spinal Cord Independence Measure (SCIM)-II score. SETTING: Level 1 trauma centre. METHODS: Initial (0-40 days) and chronic-phase (6-12 months) American Spinal Injury Association (ASIA) sensory scores, lower extremity motor score (LEMS) and chronic-phase total SCIM-II scores were analysed. Differences between ASCIS and tSCI patients were calculated using Student's t-tests and Wilcoxon signed-rank tests. To assess which variables give rise to the prediction of total SCIM-II score, a multiple linear regression analysis was used. These predictor variables included complete (ASIA impairment scale A) or incomplete SCI (AIS B, C, and D), aetiology, age and gender. RESULTS: Out of 93 included patients, 20 ASCIS and 73 tSCI patients were identified. In the complete SCI group, the initial pinprick scores were higher (P<0.05) in ASCIS patients compared with tSCI patients, 37.9 (95% Confidence Interval (CI), 23.3-52.5) and 27.3 (95% CI, 24.1-30.4), respectively. No other relevant differences in neurological outcome were identified between ASCIS and tSCI patients; however, the total SCIM-II scores were higher (P<0.05) in tSCI patients after 12 months. Using the linear regression analysis, we were able to predict 31.4% of the variability. The aetiology was not significant in this model. CONCLUSION: The neurological outcome was independent of the diagnosis ASCIS or tSCI. Furthermore, the diagnosis ASCIS or tSCI was not a significant predictor for total SCIM II scores after 12 months. SPONSORSHIP: This study was granted by the 'Internationale Stiftung für Forschung in Paraplegie' (IFP), Zürich, Switzerland.

Diagnostic criteria of traumatic central cord syndrome. Part 3: descriptive analyses of neurological and functional outcomes in a prospective cohort of traumatic motor incomplete tetraplegics.

STUDY DESIGN: Prospective multicenter cohort study. OBJECTIVES: To compare the neurological recovery and functional outcomes between traumatic central cord syndrome (TCCS) patients and motor incomplete tetraplegic patients. SETTING: European Multicenter Study of human spinal cord injury. METHODS: In 248 traumatic motor incomplete tetraplegics, initial phase (0-15 days) American Spinal Injury Association (ASIA) impairment grading, upper and lower extremity motor scores (UEMS and LEMS), upper and lower sensory scores and chronic phase (6 or 12 months) neurological outcomes were analyzed. In addition, chronic phase self-care and indoor mobility Spinal Cord Independence Measure (SCIM) items were studied. Tetraplegics were subdivided into three groups: (1) non-TCCS group (UEMSLEMS), (2) intermediate-TCCS group (UEMS=(1-9 points)

Diagnostic criteria of traumatic central cord syndrome. Part 2: a questionnaire survey among spine specialists.

STUDY DESIGN: A questionnaire survey. OBJECTIVES: To evaluate the need for the introduction of quantitative diagnostic criteria for the traumatic central cord syndrome (TCCS). SETTING: An online questionnaire survey with participants from all over the world. METHODS: An invitation to participate in an eight-item online survey questionnaire was sent to surgeon members of AOSpine International. RESULTS: Out of 3340 invited professionals, 157 surgeons (5%) from 41 countries completed the survey. Whereas most of the respondents (75%) described greater impairment of the upper extremities than of the lower extremities in their own TCCS definitions, symptoms such as sensory deficit (39%) and bladder dysfunctions (24%) were reported less frequently. Initially, any difference in motor strength between the upper and lower extremities was considered most frequently (23%) as a 'disproportionate' difference in power. However, after presenting literature review findings, the majority of surgeons (61%) considered a proposed difference of at least 10 points of power (based on the Medical Research Council scale) in favor of the lower extremities as an acceptable cutoff criterion for a diagnosis of TCCS. Most of the participants (40%) felt that applying a single criterion to the diagnosis of TCCS is insufficient for research purposes. CONCLUSION: Various definitions of TCCS were used by physicians involved in the spinal trauma care. The authors consider a difference of at least 10 motor score points between upper and lower extremity power a clear diagnostic criterion. For clinical research purposes, this diagnostic criterion can be considered as a face valid addendum to the commonly applied TCCS definition as introduced by Schneider et al.

Diagnostic criteria of traumatic central cord syndrome. Part 1: a systematic review of clinical descriptors and scores.

STUDY DESIGN: Systematic review. BACKGROUND: The applied definition of traumatic central cord syndrome (TCCS) lacks specific quantified diagnostic criteria. OBJECTIVE: To review currently applied TCCS diagnostic criteria and quantitative data regarding the 'disproportionate weakness' between the upper and lower extremities described in original studies reporting on TCCS subjects. METHODS: A MEDLINE (1966 to 2008) literature search was conducted. The descriptors applied to define TCCS were extracted from all included articles. We included original studies that reported on the differences in motor score (based on the Medical Research Council scale) between the total upper extremity motor score (UEMS) and the total lower extremity motor score (LEMS), in a minimum of five TCCS patients at the time of hospital admission. The mean difference between the total UEMS and the total LEMS of the patients included in each study was calculated. Case reports were excluded. RESULTS: None of the identified studies on TCCS patients reported inclusion and/or exclusion criteria using a quantified difference between the UEMS and LEMS. Out of 30 retrieved studies, we identified seven different clinical descriptors that have been applied as TCCS diagnostic criteria. Nine studies reporting on a total of 312 TCCS patients were eligible for analysis. The mean total UEMS was 10.5 motor points lower than the mean total LEMS. CONCLUSIONS: There is no consensus on the diagnostic criteria for TCCS. Nevertheless, this review revealed an average of 10 motor points between the UEMS and LEMS as a possible TCCS diagnostic criterion. However, further discussion by an expert panel will be required to establish definitive diagnostic criteria.

Relevance of the diagnosis traumatic cervical Brown-Séquard-plus syndrome: an analysis based on the neurological and functional recovery in a prospective cohort of 148 patients.

STUDY DESIGN: Prospective multi-center cohort study. OBJECTIVES: To compare the neurological and functional recovery between tetraplegic Brown-Séquard-plus syndrome (BSPS) and incomplete tetraplegia (non-BSPS). SETTING: European Multicenter Study of Human Spinal Cord Injury (EM-SCI). METHODS: BSPS was defined as a traumatic incomplete spinal cord injury (SCI) with ipsilateral weakness and contralateral loss of pinprick sensation at neurologic levels C2-T1. Acute (0-15 days) and chronic phase (6 or 12 months) were assessed for the American Spinal Injury Association (ASIA) sensory scores, upper extremity motor scores and lower extremity motor scores. Furthermore, chronic phase scores of all Spinal Cord Independence Measure (SCIM) II items were analyzed. Differences in neurological and functional outcome between BSPS patients and non-BSPS patients were calculated using Student's t-tests and Wilcoxon signed rank tests. RESULTS: Out of 148 tetraplegic patients, 30 were diagnosed with BSPS. Patients with an ASIA impairment scale (AIS) B were significantly (P<0.001) more identified in non-BSPS patients (25%) compared with BSPS patients (3%), respectively. After 12 months, the median scores for sphincter management of the bladder for both BSPS and non-BSPS patients were 15. Both 25 and 75% quartile median scores were 15 for BSPS patients and 12 and 15 for non-BSPS patients (P<0.02). Except for the difference in bladder function, no significant differences were identified in other SCIM II subitems and ASIA motor or sensory scores between BSPS and non-BSPS patients when stratified for injury severity by excluding AIS B patients. CONCLUSION: Compared with incomplete tetraplegic patients, patients with cervical BSPS have a similar neurological and functional recovery when matched for the AIS.

Radicular dysfunction due to spinal deformities in Marfan syndrome at older age: three case reports.

Marfan syndrome is a inherited connective tissue disorder due to mutations in fibrillin-1. It presents with cardiovascular, ocular, skeletal, pulmonary and dural signs and symptoms. Some of the symptoms of later onset are those associated with scoliosis and dural ectasia. This is the enlargement of the neural canal especially in the lower lumbar and sacral region and occurs in over 90% of Marfan patients. We here report three patients with lumbar and/or sacral radiculopathy due to (kypho)scoliosis and dural ectasia with spinal meningeal cysts. The pain, muscle weakness, muscle atrophy, and sensory disturbances illustrate the severe neurological complications which may occur in Marfan syndrome, especially at later age. Awareness of these complications and development of management protocols is essential since life expectancy of Marfan patients has increased. Marfan syndrome might gradually become recognized as an inherited connective tissue disorder with potentially severe neurological complications during ageing.

Is determination between complete and incomplete traumatic spinal cord injury clinically relevant? Validation of the ASIA sacral sparing criteria in a prospective cohort of 432 patients.

STUDY DESIGN: Prospective multicenter longitudinal cohort study. OBJECTIVE: To validate the prognostic value of the acute phase sacral sparing measurements with regard to chronic phase-independent ambulation in patients with traumatic spinal cord injury (SCI). SETTING: European Multicenter Study of Human Spinal Cord Injury (EM-SCI). METHODS: In 432 patients, acute phase (0-15 days) American Spinal Injury Association (ASIA)/International Spinal Cord Society neurological standard scale (AIS) grades, ASIA sacral sparing measurements, which are S4-5 light touch (LT), S4-5 pin prick (PP), anal sensation and voluntary anal contraction; and chronic phase (6 or 12 months) indoor mobility Spinal Cord Independence Measure (SCIM) measurements were analyzed. Calculations of positive and negative predictive values (PPV/NPV) as well as univariate and multivariate logistic regressions were performed in all four sacral sparing criteria. The area under the receiver-operating characteristic curve (AUC) ratios of all regression equations was calculated. RESULTS: To achieve independent ambulation 1-year post injury, a normal S4-5 PP score showed the best PPV (96.5%, P<0.001, 95% confidence interval (95% CI): 87.9-99.6). Best NPV was reported in the S4-5 LT score (91.7%, P<0.001, 95% CI: 81.6-97.2). The use of the combination of only voluntary anal contraction and the S4-5 LT and PP sensory scores (AUC: 0.906, P<0.001, 95% CI: 0.871-0.941) showed significantly better (P<0.001, 95% CI: 0.038-0.128) discriminating results in prognosticating 1-year independent ambulation than with the use of currently used distinction between complete and incomplete SCI (AUC: 0.823, P<0.001, 95% CI: 0.781-0.864). CONCLUSIONS: Out of the four sacral sparing criteria, the acute phase anal sensory score measurements do not contribute significantly to the prognosis of independent ambulation. The combination of the acute phase voluntary anal contraction and the S4-5 LT and PP scores, predicts significantly better chronic phase-independent ambulation outcomes than the currently used distinction between complete and incomplete SCI. SPONSORSHIP: This study was granted by 'Acute Zorgregio Oost' and the 'Internationale Stiftung für Forschung in Paraplegie (IFP)'.