Impacts of postoperative changes of segmental mobility on neurological improvement after laminoplasty for cervical ossification of the posterior longitudinal ligament.

ABSTRACT: Several studies have demonstrated that the dynamic factor at the mobile segment affects the severity of myelopathy in patients with cervical ossification of the posterior longitudinal ligament (C-OPLL), and posterior decompression supplemented with posterior instrumented fusion at the mobile segment provides good neurological improvement. However, there have been few reports of changes in range of motion at the mobile segment (segmental ROM) after laminoplasty (LP). The aim of this study was thus to retrospectively investigate changes in segmental ROM after LP and the impacts of these changes on neurological improvement in patients with C-OPLL.A total of 51 consecutive patients who underwent LP for C-OPLL since May 2010 and were followed for at least 2 years after surgery were included in this study. Neurological status was assessed using the Japanese Orthopaedic Association (JOA) score before surgery and at 2-year follow-up. Segmental ROM at the responsible level for myelopathy was measured preoperatively and at 2-year follow-up using lateral flexion-extension radiographs of the cervical spine.The mean JOA score improved significantly from 10.7 points preoperatively to 13.5 points at 2 years after surgery (mean recovery rate, 45.0%). The mean segmental ROM decreased significantly from 6.5 degrees before surgery to 3.2 degrees at 2 years after surgery. In the good clinical outcome group (recovery rate of the JOA score ≥50%; n = 22), the mean segmental ROM decreased significantly from 5.8 degrees preoperatively to 3.0 degrees postoperatively. It also decreased significantly from 7.1 degrees to 3.4 degrees in the poor clinical outcome group (recovery rate of the JOA score <50%; n = 29).This study showed that segmental ROM was stabilized after LP in most patients with C-OPLL. Neither preoperative nor postoperative segmental ROM showed significant differences between the good and poor clinical outcome groups and neither a postoperative increase nor decrease of segmental ROM significantly affected the recovery rate of the JOA score.

Assessment of the cutaneous trunci muscle reflex in neurologically abnormal cats.

OBJECTIVES: The aim of this study was to evaluate the presence of the cutaneous trunci reflex (CTR) in a population of neurologically abnormal cats in regard to age, body condition score (BCS), sex, breed, evidence of traumatic injury, pain, known metabolic disease, mentation, neurolocalization and diagnostic classification. METHODS: A retrospective medical record review was performed to identify cats with a history of neurologic disease undergoing a complete neurologic assessment between 24 September 2012 and 20 March 2019. CTR outcome (present, absent), signalment, evidence of traumatic injury, pain, known metabolic disease, mentation, neurolocalization and diagnostic classification were recorded. RESULTS: A total of 182 cats were identified. The CTR was present in 118 cats (64.8%) and absent in 64 cats (35.2%). Statistical analysis revealed no association between CTR outcome and age, BCS, sex, breed, evidence of traumatic injury, non-spinal pain, known metabolic disease, mentation, neurolocalization or diagnostic classification. A significant association was found between spinal pain and CTR outcome (P = 0.037). CONCLUSIONS AND RELEVANCE: These findings suggest that elicitation of the CTR in the cat can be unreliable. Further prospective controlled studies are warranted to determine whether continued inclusion of the CTR in feline neurologic examinations is justified. Consideration of the reliability of the CTR is indicated, particularly in the context of fractious or anxious patients for which only a limited window for examination may be present.

A Phase 2 Study of Post-Operative Stereotactic Body Radiation Therapy (SBRT) for Solid Tumor Spine Metastases.

PURPOSE: In patients with spinal instability, cord compression, or neurologic deficits, the standard of care is surgery followed by radiation therapy (RT). Recurrence rates after conventional RT remain high. The purpose of this study is to prospectively examine the efficacy of postoperative stereotactic body RT (SBRT) in patients who have undergone surgical intervention for spine metastases. We hypothesize that postoperative SBRT to the spine would be associated with higher local control than historical rates after conventional RT. METHODS AND MATERIALS: Thirty-five adult patients with a Karnofsky Performance Status score ≥40 and spine metastases from solid tumors with no prior overlapping RT and target volumes ≤3 consecutive vertebral levels were enrolled. Thirty-three patients were treated. Two patients underwent treatment to 2 target volumes for a total of 35 target volumes. All patients received SBRT 30 Gy in 5 fractions. Patients were followed with neurological examinations and computed tomography and/or magnetic resonance imaging every 3 months. Neurologic function was assessed at the same time points using the American Spinal Injury Association (ASIA) impairment score. Pain was rated according to the 10-point visual analogue scale and MD Anderson Cancer Center brief pain index. Toxicity was recorded according to National Cancer Institute Common Toxicity Criteria for Adverse Events Version 4. The primary objective was the rate of radiographic local recurrence at 12 months after completion of SBRT. RESULTS: Patient characteristics were as follows: 34.3% had radioresistant primaries; 71.4% were ASIA E and the remainder ASIA D; and the median baseline Karnofsky Performance Status score was 70 (range, 50-100). Radiographic and symptomatic local control at 1 year were 90% (95% confidence interval, 76%-98%). The median time to recurrence in these 3 patients was 3.5 months (range, 3.4-5.8 months), all had radiosensitive tumors, and all recurrences were epidural. No patients experienced wound dehiscence, hardware failure, or spinal cord myelopathy. The median time to return to systemic therapy was 0.5 months (range, 0-9.4 months). CONCLUSIONS: This prospective study of postoperative spine SBRT demonstrates excellent local control with low toxicity. These data suggest superior rates of local control compared with conventional RT; however, a formal comparative study is warranted.

Twenty Years of a Pre-Symptomatic Testing Protocol for Late-Onset Neurological Diseases in Portugal.

INTRODUCTION: The national protocol of genetic counselling and pre-symptomatic testing for late-onset neurological diseases began in Portugal in 1995. Initially, it was accessible only to adults at-risk for Machado-Joseph disease, but was later extended to other hereditary ataxias, to Huntington's disease and to familial amyloid polyneuropathy caused by Val30Met mutation at the transthyretin gene. The aim of this study was to describe the profile of the population seeking pre-symptomatic testing, while also reflecting on the experience of conducting the protocol of multidisciplinary sessions since 1996. MATERIAL AND METHODS: We conducted a retrospective study and collected data from clinical records of consultands who requested pre-symptomatic testing at our centre in Porto (Portugal) during the first twenty years of practice (1996 - 2015). RESULTS: A total of 1446 records were reviewed. The most common reason for testing was to reduce uncertainty (41.7%). The rate of withdrawals before results disclosure was lower (16%) than reported in other international experiences with pre-symptomatic testing, while 45% of the consultands dropped out the protocol after learning the test results (73.5% of them were non-carriers). As far as the mutation carriers were concerned, 29.6% adhered to the protocol a year after test disclosure. Consultands that had learned about presymptomatic testing through healthcare professionals tended to adhere more to pre-symptomatic testing consultations. DISCUSSION: The profile of Portuguese consultands at risk for late-onset neurological diseases is similar to those reported in other international programs. The largest group in this data set was the one comprising the subjects at risk for familial amyloid polyneuropathy caused by Val30Met mutation at the transthyretin gene, and it is likely that therapeutic options for this condition may have influenced this result. Adherence to pre-symptomatic testing may change in the future since effective therapies are available (or given the fact that people think effective treatments are imminent). CONCLUSION: This study reflects the first comprehensive description of a Portuguese experience with pre-symptomatic testing for late onset neurological diseases. The development of innovative approaches to improve the consultands' experience with pre-symptomatic testing and their engagement in genetic departments is still a challenge in Portuguese genetics healthcare departments. A better coordination among primary care and genetics healthcare services is needed.

Introdução: Em 1995 foi iniciado em Portugal um protocolo nacional para o aconselhamento genético e teste pré-sintomático de doenças neurológicas de início tardio. Inicialmente, foi disponibilizado para indivíduos adultos em risco para a doença de Machado-Joseph e posteriormente estendido a outras ataxias hereditárias, doença de Huntington e polineuropatia amiloidótica familiar ATTR Val30Met. O objetivo deste estudo é descrever o perfil dos consultandos envolvidos no teste pré-sintomático desde 1996, e refletir no protocolo de sessões multidisciplinares. Material e Métodos: Realizámos um estudo retrospetivo com recolha de dados dos processos clínicos dos utentes que solicitaram teste pré-sintomático ao longo dos primeiros 20 anos do Centro de Genética Preditiva e Preventiva (1996 - 2015), localizado no Porto, Portugal. Resultados: Analisámos um total de 1446 processos clínicos; a principal motivação para a realização do teste pré-sintomático foi o alívio da incerteza (41,7%). A taxa de abandono do protocolo antes da comunicação dos resultados do pré-sintomático (16% dos casos) foi mais baixa do que em outras experiências internacionais; 45% dos consultandos abandonaram o protocolo depois de saberem o resultado do teste pré-sintomático (73,5% dos quais eram não-portadores). 29,6% de consultandos portadores continuaram envolvidos no protocolo um ano após saberem o resultado do teste pré-sintomático. Os consultandos encaminhados para o protocolo através de outros profissionais de saúde revelaram maior adesão ao protocolo. Discussão: O perfil sociodemográfico dos consultandos no Centro de Genética Preditiva e Preventiva é similar ao reportado noutras experiências internacionais. Os consultandos em risco para polineuropatia amiloidótica familiar ATTR Val30Met representaram o maior grupo nos nossos dados, sendo provável que as opções terapêuticas disponíveis para esta doença tenham influenciado este resultado. A adesão ao teste pré-sintomático poderá alterar-se no futuro quando terapias eficazes estiverem disponíveis (ou as pessoas as percepcionem como estando iminentes). Conclusão: Este trabalho constitui a descrição mais completa até ao momento publicada acerca da realização de teste pré-sintomático em Portugal. O desenvolvimento de abordagens com vista à melhoria da experiência dos consultandos com os testes pré-sintomáticos e ao seu envolvimento nos serviços de genética é um desafio atual, assim como a melhor articulação dos mesmos com os cuidados de saúde primários.

Median Nerve Symptoms, Signs, and Electrodiagnostic Abnormalities Among Working Adults.

INTRODUCTION: Diagnostic screening tests for carpal tunnel syndrome (CTS) have not been rigorously assessed in large populations. METHODS: This study is a cross-sectional analysis from a prospective cohort study. Participants' (n = 1,194) symptoms and disease prevalence were measured. Sensitivity, specificity, and positive and negative predictive values (NPVs) were calculated. RESULTS: When defining CTS as tingling/numbness in at least two median nerve-served digits and an abnormal median nerve conduction study, the prevalence was 8.9%. The sensitivity of paresthesias with nocturnal awakening was 77.4%. The sensitivity of the Phalen sign was 52.8% and that of the Hoffman-Tinel sign was only 37.7%. DISCUSSION: The highest sensitivity (77.4%) for a case definition of CTS in this population of workers was for nocturnal tingling/numbness in a median nerve distribution, and the highest specificity (97.5%) was for continuous tingling/numbness. The Phalen sign has a sensitivity of 52.8% and NPV of 95%, suggesting that the NPV is of particular diagnostic value. Hoffman-Tinel signs seem primarily helpful for the NPV (93.7%). LEVEL OF EVIDENCE: Level II diagnostic study.

Development and Validation of an Empiric Tool to Predict Favorable Neurologic Outcomes Among PICU Patients.

OBJECTIVES: To create a novel tool to predict favorable neurologic outcomes during ICU stay among children with critical illness. DESIGN: Logistic regression models using adaptive lasso methodology were used to identify independent factors associated with favorable neurologic outcomes. A mixed effects logistic regression model was used to create the final prediction model including all predictors selected from the lasso model. Model validation was performed using a 10-fold internal cross-validation approach. SETTING: Virtual Pediatric Systems (VPS, LLC, Los Angeles, CA) database. PATIENTS: Patients less than 18 years old admitted to one of the participating ICUs in the Virtual Pediatric Systems database were included (2009-2015). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 160,570 patients from 90 hospitals qualified for inclusion. Of these, 1,675 patients (1.04%) were associated with a decline in Pediatric Cerebral Performance Category scale by at least 2 between ICU admission and ICU discharge (unfavorable neurologic outcome). The independent factors associated with unfavorable neurologic outcome included higher weight at ICU admission, higher Pediatric Index of Morality-2 score at ICU admission, cardiac arrest, stroke, seizures, head/nonhead trauma, use of conventional mechanical ventilation and high-frequency oscillatory ventilation, prolonged hospital length of ICU stay, and prolonged use of mechanical ventilation. The presence of chromosomal anomaly, cardiac surgery, and utilization of nitric oxide were associated with favorable neurologic outcome. The final online prediction tool can be accessed at https://soipredictiontool.shinyapps.io/GNOScore/. Our model predicted 139,688 patients with favorable neurologic outcomes in an internal validation sample when the observed number of patients with favorable neurologic outcomes was among 139,591 patients. The area under the receiver operating curve for the validation model was 0.90. CONCLUSIONS: This proposed prediction tool encompasses 20 risk factors into one probability to predict favorable neurologic outcome during ICU stay among children with critical illness. Future studies should seek external validation and improved discrimination of this prediction tool.

Bony ankylosis of the facet joint of the cervical spine in rheumatoid arthritis: Its characteristics and relationship to the clinical findings.

OBJECTIVES: The purpose of this study was to clarify the characteristics of bony ankylosis of the facet joint of the cervical spine in rheumatoid arthritis (RA) patients who required cervical spine surgery, and its relationship to the clinical findings. METHODS: Eighty consecutive RA patients with cervical spine disorder who received initial surgery were reviewed. The occurrence of bony ankylosis of the facet joint of the cervical spine was investigated using computed tomography (CT) before surgery. We also evaluated the severity of neurological symptoms and the plain wrist radiographs taken before surgery; furthermore, we evaluated each patient's medical history for total knee arthroplasty (TKA) or hip arthroplasty (THA). RESULTS: The preoperative CT imaging demonstrated bony ankylosis of the facet joint of the cervical spine in 45 facet levels of 19 cases (BA + group). In all patients, responsible instability or stenosis was demonstrated just caudal or on the cranial side of those bony ankylosis. Before surgery, the BA + group included significantly more patients showing severe cervical myelopathy (p < 0.05), and significantly more cases showing progressed ankylosis in the wrist joint bilaterally (p < 0.01). There were also significantly more patients who received two or more TKA or THA before the cervical spine surgery in the BA + group (p < 0.01). CONCLUSIONS: Bony ankylosis of the facet joint of the cervical spine may be a risk factor of instability or stenosis at the adjacent disc level and severe cervical myelopathy. Furthermore, its ankylosis was demonstrated in RA patients with severe destroyed joints.

Developmental neuropsychological assessment of 4- to 5-year-old children born following Preimplantation Genetic Diagnosis (PGD): A pilot study.

The purpose of this pilot study was to evaluate developmental neuropsychological profiles of 4- to 5-year-old children born after Preimplantation Genetic Diagnosis (PGD). Twenty-seven participants received a neurological examination and a battery of neuropsychological assessments including Wechsler Preschool & Primary Scale of Intelligence - Third Edition (WPPSI-III; cognitive development), Preschool Language Scale, Fourth Edition (PLS-4; language development), Wide Range Assessment of Visual Motor Abilities (visual motor abilities), Childhood Autism Rating Scales II (a screening test for autistic spectrum disorders), and the Miles ABC Test (ocular dominance). Parental questionnaires included the Behavior Rating Inventory of Executive Function Preschool Version (BRIEF-P; executive function), Child Behavior Checklist (CBCL) and the Carey Temperament Scales Behavioral Style Questionnaire (socioemotional development and temperament), and the Vineland Adaptive Behavior Scales, Interview Edition, Second Edition (general adaptive behavior). Subjects' tests results were compared to each test's norms. Children born after PGD demonstrated scores within the normal or above-normal ranges for all developmental outcomes (mean ± SD): WPPSI-III-VIQ 107.4 ± 14.4 (p = .013), PLS-4-Total 113.2 ± 12.4, p < .001), CBCL-Total 41.1 ± 8.6 (p < .001), BRIEF-P-Global Executive Composite 44.8 ± 9.5 (p = .009). Twelve (44%) of the PGD children had a significant difference between their VIQ and PIQ scores (compared to 27% in the general population). One subject was found to show possible signs of autistic spectrum disorder, although a family history of autism was noted. In conclusion, in this pilot study, children assessed at age 4-5 years and conceived after PGD displayed developmental neuropsychological outcomes within normal limits as compared to their chronologic peers. A larger study is needed to evaluate and follow the neuropsychological development of children born after PGD.

The Diagnostic Value of Neuropathy Symptom and Change Score, Neuropathy Impairment Score and Michigan Neuropathy Screening Instrument for Diabetic Peripheral Neuropathy.

AIMS: This study aims to evaluate the diagnostic capabilities of neuropathy symptom and change (NSC) score, neuropathy impairment score (NIS) and Michigan neuropathy screening instrument (MNSI) in diagnosing diabetic peripheral neuropathy (DPN). METHODS: A total of 131 patients with type II diabetes received NSC, NIS and MNSI scoring systems. Electromyography/nerve conduction velocity (EMG/NCV) test was taken as gold standard. Correlations between EMG/NCV test and the 3 scorings, and their sensitivity, specificity, positive and negative predictive values, accuracy and kappa (x03BA;) value were analyzed. RESULTS: The prevalence of DPN was 43.5% according to EMG/NCV findings. EMG/NCV test was significantly positive correlated with all the 3 scorings, highest with NIS scoring (r = 0.653, p < 0.001). Compared with EMG/NCV test, NSC score was most sensitive (85.96%) but least specific (77.03%); NIS score had lower sensitivity (59.65%) but best specificity (98.65%) and accuracy (81.68%). Both had high concordance with EMG/NCV test (x03BA; = 0.61). Sensitivity, specificity and accuracy of MNSI were highest (70.18, 98.65 and 80.15%) at the cutoff values of >1.0, >2.5 and >1.5, respectively (x03BA; = 0.58). CONCLUSIONS: Both NSC and NIS were accurate and reliable diagnostic methods for DPN. The combined application of NSC and NIS was recommended in DPN diagnosis.

Ataxia, ophthalmoplegia and laryngeal stridor related to glutamic acid decarboxylase antibodies / Ataxia, oftalmoplegia e estridor laríngeo relacionados a anticorpos antidescarboxilase do ácido glutâmico

Glutamic acid decarboxylase (GAD) is the enzyme responsible for the conversion of glutamate to gamma-aminobutyric acid (GABA) in the central nervous system. The presence of anti-GAD antibody in cerebrospinal fluid and high levels in blood have been described in some neurological disorders, such as stiff person syndrome and cerebellar ataxia. It is postulated that African descent with anti-GAD may exhibit more severe neurological impairment. We report a case of a young adult African descent with cerebellar syndrome associ-ated with ophthalmoplegia and laryngeal stridor. We found in the literature relationship of ophthalmoplegia plus ataxia with anti-GAD, but no reports of these symptoms with laryngeal stridor, apparently being the first reported case.

Descarboxilase do ácido glutâmico (GAD) é a enzima responsável pela conversão do glutamato em ácido gama-aminobutírico (GABA) no sistema nervoso central. A presença do anticorpo anti-GAD no líquido cefalorraquidiano e em altos níveis no sangue tem sido descrita em alguns distúrbios neurológicos, tais como a síndrome da pessoa rígida e ataxia cerebelar. Postula-se que pacientes afrodescendentes podem apresentar comprometimento neurológico mais severo. Relatamos o caso de um adulto jovem afrodescendente com síndrome cerebelar associada a oftalmoplegia e estridor laríngeo. Encontramos na literatura relação entre a oftalmoplegia com ataxia e anti-GAD, mas nenhum relato desses sintomas com estridor laríngeo, sendo aparentemente o primeiro caso reportado.

Preliminary reliability and validity of a battery for assessing functional skills in children with Sturge-Weber syndrome.

PURPOSE: The purpose of this study was to evaluate inter-rater reliability and validity of a proposed functional outcome battery for clinical trials in children with Sturge-Weber syndrome (SWS). METHODS: Ten children were evaluated twice on the same day using a series of functional outcome measures selected for sensitivity to the range of age and function of children with SWS: Modified Rankin Scale, Pediatric Evaluation of Disability Index, Modified House Functional Classification, and a modified version of the Erhardt Developmental Prehension Assessment. Inter-rater reliability was calculated, and criterion validity was explored through correlations with the Sturge-Weber Syndrome-Neurological Rating Score (SWS-NRS). RESULTS: Inter-rater reliability was high across all measures. Correlations were identified between the SWS-NRS and the study measures. CONCLUSIONS: The proposed battery of functional outcome measures captures child's functioning at the levels of impairment, activity and participation and is robust to evaluation by different raters and across sessions on the same day. This battery is expected to be sensitive to treatment-related changes in qualitative patterns of hand use, functional skills, and/or change in independence in daily living.

The Democritos Movement Screening Tool for Preschool Children (DEMOST-PRE©): development and factorial validity.

The aim of this study was (a) to develop an assessment tool (the Democritos Movement Screening Tool for Preschool Children - DEMOST-PRE), designed to provide preschool educators, clinicians and researchers with information about assessment and screening of the motor proficiency of children aged 4-6 years, as well as the development and control of movement programmes and (b) to assess its factorial validity. First, tool's content and face validity were established and its final structure was determined. Then, the DEMOST-PRE was administered to 435 children (197 girls) aged 48-71 months (M=60.48 months, SD=6.98). The factor analysis conducted revealed two distinct components. Present evidence combined with the DEMOST-PRE administrative traits make it promising for preschool aged children's assessment.

[Quantitative Sensory Testing in the facial area: a review]. / Quantitative Sensorische Testung im Gesichtsbereich - eine Übersicht.

Quantitative Sensory Testing is an established method to evaluate somatosensory function. In the facial area, the procedures depend on the localisation of disorders and the modalities of interest. The test stimuli are of thermal or mechanical nature (touch, pain, vibration, or pressure stimuli). According to the protocol of the German Neuropathic Pain Network, comprehensive information on the function of afferent nerves can be generated in the facial area as well. Standard values have been obtained for the cheek and intraoral mucosa. For various orofacial pain conditions, studies concerning the somatosensory function are available. Changed functional patterns are not limited to neuropathic pain, but also occur in other orofacial pain conditions, indicating, for example, central sensitisation. The standardised collection of QST parameters may improve the understanding of the pathophysiology of orofacial pain and effect therapeutic approaches. Comprehensive studies may lead to the development of specific screenings that are feasible in a clinical setting.

CT scan utilization patterns in pediatric patients with recurrent headache.

UNLABELLED: Although unnecessary for children with headache and normal history, computed tomography (CT) scans are widely used. This study sought to determine current practice patterns of neuroimaging to diagnose pediatric headache in a variety of treatment settings and to identify factors associated with increased use of neuroimaging. METHODS: This retrospective claims analysis included children (aged 3­17 years) with ≥2 medical claims for headache. The primary outcome was CT scan utilization on or after first presentation with headache in a physician's office or emergency department (ED). RESULTS: Of 15 836 patients, 26% (4034 patients; mean age: 11.8 years) had ≥1 CT scan, 74% within 1 month of index diagnosis. Patients with ED visits were 4 times more likely to undergo a CT scan versus those without ED visits (P < .001 [95% confidence interval: 3.9­4.8]). However, even outside the ED, use of CT scans remained widespread. Two-thirds of patients with CT scans had no ED use.Among patients with no ED utilization, >20% received a CT scan during the study period. Evaluation by a neurologist was strongly associated with a lower likelihood of CT scan compared with other provider specialties (odds ratio: 0.37; P < .01 [95% confidence interval: 0.30­0.46]). CONCLUSIONS: Use of CT scans to diagnose pediatric headache remains high despite existing guidelines, low diagnostic yield, and high potential risk. Implementing quality improvement initiatives to ensure that CT scans in children are performed only when truly indicated will reduce unnecessary exposure to ionizing radiation and associated cancer risks.

MOON-test - determination of motor performance in the pediatric oncology.

BACKGROUND: Pediatric cancer patients suffer from various negative consequences due to the disease, the medical therapy and the inactivity during the intensive treatment. Only few studies have systematically identified the adverse effects of cancer on motor performance in childhood. METHODS: To determine the motor performance of pediatric cancer patients, a motor performance test was developed which is applicable for this specific patient group. Eight test items with reference values for healthy children were merged to the MOON-test (test for motor performance in the oncology). RESULTS: MOON was tested for feasibility and acceptance in 33 patients aged 4-18 years. Feasibility was confirmed for children with different types of cancer (hematological malignancies and solid tumors) and with amputation, endoprosthesis, during aplasia as well as reduced general condition. Furthermore the patients showed a broad acceptance. CONCLUSION: Based on the study findings, the use of MOON-test as a standardized motor performance diagnostic tool in clinical routine of oncological acute clinics as well as rehabilitation clinics can be recommended.·

[Should a more interventionist approach be taken in A and E departments with atypical febrile seizures? Three years' experience in a tertiary hospital]. / Se debe ser más intervencionista en urgencias con las convulsiones febriles atípicas? Experiencia en un hospital terciario durante tres años.

INTRODUCTION: Atypical febrile seizures (AFS) have been related with a higher incidence of severe pathologies of the central nervous system (CNS). Recent studies show a reduction in the prevalence of some of these diseases, a fact that could affect their management. AIMS. To determine the prevalence of severe pathologies of the CNS in patients treated for AFS in A and E departments and to detect any differences between these and patients suffering from AFS that is not associated to any severe pathology. PATIENTS AND METHODS: A retrospective study was conducted by reviewing the medical records of patients diagnosed with AFS between November 2008 and November 2011. RESULTS: Altogether, the sample consisted of 231 episodes of AFS (223 patients), with an average age of 1.7 years (p25-75=1.2-2.3 years), 133 (57.6%) of whom were males. Twelve patients (5.2%; 95% CI=2.7-8.9) were diagnosed with a severe pathology of the CNS. In patients with a severe pathology of the CNS, AFS is on most occasions the first episode (91.7% versus 63%; p=0.036) and more than one diagnostic criterion is present (50% versus 15.1%; p=0.007). Moreover, focal seizures (50% versus 12.8%; p=0.003) or epileptic status (25% versus 5.9%; p=0.041) are more common, and patients present altered levels of awareness that persist after the episode (66.7% versus 31.5%; p=0.002). CONCLUSIONS: Given the fact that the prevalence of severe pathology of the CNS in patients with AFS is low, carrying out complementary tests or admission to hospital on a routine basis are not recommended. Certain characteristics of the episode increase the likelihood of AFS being the manifestation of a severe pathology of the CNS (being a first episode, presenting more than one diagnostic criterion for AFS and being a focal seizure or epileptic status), and should therefore be taken into account in the management of the patient.

Time to diagnosis of paediatric posterior fossa tumours: an 11-year West of Scotland experience 2000-2011.

INTRODUCTION: Brain tumours are the most common solid childhood malignancy accounting for 20% of all paediatric cancers. Of these, posterior fossa tumours comprise approximately 60-70% of all brain tumours in children. Several studies have estimated the median pre-diagnostic interval (PSI) of paediatric brain tumours as approximately 60 days. OBJECTIVES: The objectives of this retrospective analysis were to (a) identify the common presenting symptoms of posterior fossa tumours, (b) determine the time interval from the first attributable symptom to the radiological diagnosis of a posterior fossa tumour, (c) compare the West of Scotland with other international centres and (d) identify which factors correlate with outcome for these children. MATERIALS AND METHODS: A retrospective case note review of 69 children diagnosed with posterior fossa tumours from January 2000 to September 2011. Of the 69 children diagnosed during this period, complete data were available for 66 children (M:F = 31:35, Mean age (SD): 7.50 + 4.53 years). Results. Nausea and vomiting (75.8%), headaches (63.6%) and incoordination (51.5%) were recorded as the most common presenting symptoms followed by lethargy (28.8%), cranial nerve palsy (25.8%) and diplopia (24.2%). Fifty-three of the sixty-six children (i.e., 80.3%) demonstrated radiological evidence of hydrocephalus on their initial scan. The majority of children were assessed by less than three specialists after a median PSI of 43.5 days. The only variable significantly associated with PSI was tumour grade (r = - 0.202, p = 0.036). Neither age at diagnosis, number of specialists seen, nor outcome was significantly correlated with PSI. The only factor associated with outcome was tumour grade (r = 0.337, p = 0.006). CONCLUSION: Despite recent reports indicating poor performance of the UK with respect to time to diagnosis of paediatric brain tumours, the present data indicate that the experience of this cohort is favourably comparable to international standards.

Interleukin 6-preconditioned neural stem cells reduce ischaemic injury in stroke mice.

Transplantation of neural stem cells provides a promising therapy for stroke. Its efficacy, however, might be limited because of massive grafted-cell death after transplantation, and its insufficient capability for tissue repair. Interleukin 6 is a pro-inflammatory cytokine involved in the pathogenesis of various neurological disorders. Paradoxically, interleukin 6 promotes a pro-survival signalling pathway through activation of signal transducer and activator of transcription 3. In this study, we investigated whether cellular reprogramming of neural stem cells with interleukin 6 facilitates the effectiveness of cell transplantation therapy in ischaemic stroke. Neural stem cells harvested from the subventricular zone of foetal mice were preconditioned with interleukin 6 in vitro and transplanted into mouse brains 6 h or 7 days after transient middle cerebral artery occlusion. Interleukin 6 preconditioning protected the grafted neural stem cells from ischaemic reperfusion injury through signal transducer and activator of transcription 3-mediated upregulation of manganese superoxide dismutase, a primary mitochondrial antioxidant enzyme. In addition, interleukin 6 preconditioning induced secretion of vascular endothelial growth factor from the neural stem cells through activation of signal transducer and activator of transcription 3, resulting in promotion of angiogenesis in the ischaemic brain. Furthermore, transplantation of interleukin 6-preconditioned neural stem cells significantly attenuated infarct size and improved neurological performance compared with non-preconditioned neural stem cells. This interleukin 6-induced amelioration of ischaemic insults was abolished by transfecting the neural stem cells with signal transducer and activator of transcription 3 small interfering RNA before transplantation. These results indicate that preconditioning with interleukin 6, which reprograms neural stem cells to tolerate oxidative stress after ischaemic reperfusion injury and to induce angiogenesis through activation of signal transducer and activator of transcription 3, is a simple and beneficial approach for enhancing the effectiveness of cell transplantation therapy in ischaemic stroke.

MRI and neurological findings in patients with spinal metastases.

BACKGROUND: Magnetic resonance imaging (MRI) is the recommended primary investigation method for metastatic spinal cord compression (MSCC). Initiating treatment before the development of motor deficits is essential to preserve neurological function. However, the relationship between MRI-assessed grades of spinal metastatic disease and neurological status has not been widely investigated. PURPOSE: To analyze the association between neurological function and MRI-based assessment of the extent of spinal metastases using two different grading systems. MATERIAL AND METHODS: A total of 284 patients admitted to our institution for initial radiotherapy or surgery for symptomatic spinal metastases were included in the study. Motor and sensory deficits were categorized according to the Frankel classification system. Pre-treatment MRI evaluations of the entire spine were scored for the extent of spinal metastases, presence and severity of spinal cord compression, and nerve root compression. Two MRI-based scales were used to evaluate the degree of cord compression and spinal canal narrowing and relate these findings to neurological function. RESULTS: Of the patients included in the study, 28 were non-ambulatory, 49 were ambulatory with minor motor deficits, and 207 had normal motor function. Spinal cord compression was present in all patients with Frankel scores of B or C, 23 of 35 patients with a Frankel score of D (66%), and 48 of 152 patients with a Frankel score of E (32%). The percentage of patients with severe spinal canal narrowing increased with increasing Frankel grades. The grading according to the scales showed a significant association with the symptoms according to the Frankel scale (P < 0.001). CONCLUSION: In patients with neurological dysfunction, the presence and severity of impairment was associated with the epidural tumor burden. A significant number of patients had radiological spinal cord compression and normal motor function (occult MSCC).

Application of futility analysis to refine jitter recordings in myasthenia gravis.

INTRODUCTION: The current practice of single-fiber electromyography (SFEMG) requires that 20 fiber pairs with normal jitter be collected to exclude myasthenia gravis (MG). We applied principles of futility analysis from clinical trials in an attempt to reduce that requirement. METHODS: We utilized conditional power futility analysis to assess the probability of an abnormal 20-pair SFEMG based on ongoing analysis of jitter as each pair is collected. Rules for early test termination in the presence of 0, 1, or 2 abnormal pairs were identified. These rules were then applied to previously collected SFEMG data. RESULTS: SFEMG could be stopped at just 12 pairs if all are normal and at 17 pairs if 1 is abnormal. The rules successfully determined when SFEMG could be stopped in 104 of 106 (98%) studies originally reported to be normal. CONCLUSIONS: If the first 12 SFEMG pairs have normal jitter, the study can be terminated and interpreted as normal.