A superficial siderosis-related transient focal neurological episode in a patient with Marfan syndrome.

Sagittal computed tomography of lumbal spine showing dural ectasia and Tarlow Cyst (red arrows) (A). Magnetic resonance imaging (MRI) axial slice, susceptibility-weighted imaging (SWI) sequences showing siderosis in cerebellar sulci (red arrows) and in the inset uncharacteristic iron deposition in dental nuclei, more pronounced on the left side (red arrows) (B, inset). MRI, SWI, axial slice, showing numerous foci of superficial siderosis (C).

Incidence of traumatic spinal cord injury worldwide: A systematic review, data integration, and update.

Objectives: This review was designed to update our earlier systematic review which evaluated both published and unpublished evidence on the incidence of traumatic spinal cord injury (TSCI) worldwide. Methods: We used various search methods including strategic searching, reference checking, searching for grey literature, contacting registries, authors, and organizations requesting unpublished data, browsing related websites, and hand searching key journals. The quality of included studies was evaluated by Joanna Briggs Institute Critical Appraisal Tools. Records published between April 2013 and May 2020 were added to the original systematic review. Results: Overall, 58 resources including 45 papers, 10 SCI registry reports, 1 book, and 2 theses were retrieved. We found TSCI incidence data for eight new countries, which overall shapes our knowledge of TSCI incidence for 49 countries. The incidence of TSCI ranges from 3.3 to 195.4 cases per million (cpm) based on subnational studies and from 5.1 to 150.48 cpm based on national studies. Most of the studies were low quality, lacked consistent case selection due to unclear definition of TSCI and unclear ascertainment methods. Conclusions: There is an increasing number of publications in the literature focusing on the epidemiologic data of TSCI. The absence of a standard form of reporting TSCI hinders the comparability of data across different data sources. Use of various definitions for TSCI may lead to heterogeneity in reports. Use of sensitivity analyses based on reasonable classification criteria can aid in offering a uniform set of case identification and ascertainment criteria for TSCI.

The impact of data quality assurance and control solutions on the completeness, accuracy, and consistency of data in a national spinal cord injury registry of Iran (NSCIR-IR).

STUDY DESIGN: Descriptive study. OBJECTIVE: This study aimed to develop and evaluate a systematic arrangement for improvement and monitoring of data quality of the National Spinal Cord (and Column) Injury Registry of Iran (NSCIR-IR)-a multicenter hospital-based registry. SETTING: SCI community in Iran. METHODS: Quality assurance and quality control were the primary objectives in improving overall quality of data that were considered in designing a paper-based and computerized case report. To prevent incorrect data entry, we implemented several validation algorithms, including 70 semantic rules, 18 syntactic rules, seven temporal rules, and 13 rules for acceptable value range. Qualified and trained staff members were also employed to review and identify any defect, inaccuracy, or inconsistency in the data to improve data quality. A set of functions were implemented in the software to cross-validate, and feedback on data was provided by reviewers and registrars. RESULTS: Socio-demographic data items were 100% complete, except for national ID and education level, which were 97% and 92.3% complete, respectively. Completeness of admission data and emergency medical services data were 100% except for arrival and transfer time (99.4%) and oxygen saturation (48.9%). Evaluation of data received from two centers located in Tehran proved to be 100% accurate following validation by quality reviewers. All data was also found to be 100% consistent. CONCLUSIONS: This approach to quality assurance and consistency validation proved to be effective. Our solutions resulted in a significant decrease in the number of missing data.

Long term follow-up results of deep brain stimulation of the Globus pallidus interna in pediatric patients with DYT1-positive dystonia.

OBJECTIVES: Primary generalized dystonia (PGD) due to heterozygous torsin 1A (TOR1A) gene mutation (DYT1) is a childhood onset dystonia with rapid deterioration of symptoms, leading to severe disability in adolescence. Globus pallidus interna deep brain stimulation (GPi-DBS) has been shown to provide significant improvement in these cases. METHODS: This was a retrospective study of TOR1A mutation positive dystonia patients, conducted at a university hospital from 2006 to 2018. Burke-Fahn-Marsden Dystonia Rating Scale (BFM-DRS) was used to evaluate dystonia severity before and after surgery. Emergence of postsurgical parkinsonian symptoms was evaluated using the Unified Parkinson Disease Rating Scale (UPDRS) part III. Montreal Cognitive Assessment (MOCA) was applied to assess cognitive dysfunction. SPSS version 18 was used for data analysis. RESULTS: Eleven patients entered for analysis with an average age of 22.36 (±3.35) years (range: 18-28). Seven patients (63.6 %) were female. Mean follow-up period was 8.72 (±0.87). Difference between baseline and most recent BFM scores was significant (disability: 10.5 ±4.52 versus 2.09 (±3.20), P: 0.001; severity: 48.45 (±17.88) versus 9.36 (±10.47), P<0.001). The mean MOCA and UPDRS III scores after 7-9 years of DBS were 27.18 (±2.99), and 6.09 (±4.15), respectively. CONCLUSION: Our experience confirms that GPi-DBS in pediatric patients with DYT1 dystonia is overall successful, with significant and long-lasting positive effects on motor and cognitive functions. There was no prominent side effect in long-term follow up.

The data set development for the National Spinal Cord Injury Registry of Iran (NSCIR-IR): progress toward improving the quality of care.

STUDY DESIGN: Descriptive study. OBJECTIVES: The aim of this manuscript is to describe the development process of the data set for the National Spinal Cord Injury Registry of Iran (NSCIR-IR). SETTING: SCI community in Iran. METHODS: The NSCIR-IR data set was developed in 8 months, from March 2015 to October 2015. An expert panel of 14 members was formed. After a review of data sets of similar registries in developed countries, the selection and modification of the basic framework were performed over 16 meetings, based on the objectives and feasibility of the registry. RESULTS: The final version of the data set was composed of 376 data elements including sociodemographic, hospital admission, injury incidence, prehospital procedures, emergency department visit, medical history, vertebral injury, spinal cord injury details, interventions, complications, and discharge data. It also includes 163 components of the International Standards for the Neurologic Classification of Spinal Cord Injury (ISNCSCI) and 65 data elements related to quality of life, pressure ulcers, pain, and spasticity. CONCLUSION: The NSCIR-IR data set was developed in order to meet the quality improvement objectives of the registry. The process was centered around choosing the data elements assessing care provided to individuals in the acute and chronic phases of SCI in hospital settings. The International Spinal Cord Injury Data Set was selected as a basic framework, helped by comparison with data from other countries. Expert panel modifications facilitated the implementation of the registry process with the current clinical workflow in hospitals.

Characteristics of neuropathic pain in individuals with chronic spinal cord injury.

OBJECTIVE: To present the characteristics of neuropathic pain in individuals with chronic spinal cord injury (SCI). METHODS: We recruited all individuals with chronic SCI referred to the Brain and Spine Injury Research center with a diagnosis of neuropathic pain from April 2013 to September 2015 into this historical cohort study. RESULTS: Forty individuals with chronic SCI-induced neuropathic pain entered this study with a mean age of 43.67+/-13.12 years and a majority of who were male (n=30, 75%). Motor vehicle collision (n=25, 62.5%) and fall (n=7, 17.5%) were the most common causes of SCI in our participants. There were 13 (32.5%) cervical, twenty (50%) thoracic, and 7 (17.5%) lumbosacral SCI. The mean `maximal pain intensity`, `overall pain intensity during the past week`, and `the pain intensity at the initial consultation in pain clinic` measured by numerical rating scale (NRS) in this cohort were 8.71+/-1.73, 6.32+/-1.60, and 6.11+/-2.48, respectively. Burning pain was the most frequently used description of pain reported by our participants. Pain intensity significantly decreased after six months of treatment for all three above categories. CONCLUSION: This study provides characteristics of neuropathic pain in a group of individuals with chronic SCI. Further large prospective studies are needed to determine the association between lesion level, completeness of injury, and region of pain.

Is there any association between adenoid biofilm and upper airway infections in pediatric patients?

AIM: To evaluate the association of the presence and extent of adenoid biofilms and the frequency of upper airway infections in children with upper airway obstruction. MATERIAL AND METHODS: This cross-sectional study was conducted from October 2014 to December 2015 on pediatric patients who were candidates for adenoidectomy due to obstructive sleep apnea. After removal of the adenoid tissue and fixation in 2.5% glutaraldehyde, the samples were sent to the electron microscopy unit. The extent of biofilm formation was examined using environmental scanning electron microscopy. These results were then confirmed using image analysis software. RESULTS: Fifty-seven children with a mean age of 7.31 (±2.65) years were included in the study. Forty-three (75.4%) were male and 14 (24.6%) were female. The average number of upper airway infections during the last 12 months before adenoidectomy was 10.01 (±5.38). Biofilm structures were detected in all (100%) samples. As the main outcome, the extent of biofilm grading exhibited a statistically significant correlation with the frequency of upper airway infections (p<0.001). There was no significant correlation between sex and adenoid size with the biofilm extent. CONCLUSION: The present study showed that the extent of adenoid biofilm had a significant relationship with the frequency of upper airway infection rate. It seems that the presence of a biofilm on the adenoid surface as a reservoir for microorganisms could cause chronic inflammation.

Anteromedial GPi deep brain stimulation in Tourette syndrome: The first case series from Iran.

OBJECTIVES: Tourette syndrome (TS) is a neuropsychiatric disorder characterized by childhood onset motor and phonic tics. In refractory cases, deep brain stimulation (DBS) with different targets including anteromedial Globus pallidus (AM-GPi) looks promising. PATIENTS AND METHODS: Patients with TS diagnosed according to DSM-IV TR criteria with severe medication-recalcitrant disease referred to our DBS clinic, were recruited for this study. They underwent bilateral AM-GPi DBS with Model 3389, Medtronic electrodes. Patients were assessed using Yale Global Tic Severity Scale (YGTSS) and Gilles de la Touretts syndrome-quality of life (GTS-QOL) questionnaire before and one year after DBS. RESULTS: Six patients (four men and two women) with severe medication-recalcitrant TS, mean age of 26.33 ±â€¯7.25 years fulfilled the follow up visits. All patients revealed significant improvement in tics severity one year after surgery. Based on YGTSS, total tic severity score decreased from 75.66 ±â€¯16.54 to 28.33 ±â€¯13.95, P-value:0.005. Quality of life improved significantly after DBS (26.66 ±â€¯20.65 before and 70.00 ±â€¯17.88 one year after surgery, P-value:0.02). CONCLUSIONS: Results of our study in accordance to previous ones suggest AM-GPi DBS as an effective and well-tolerated therapeutic modality for patients with medication refractory TS.

Association between detrusor muscle function and level of the spinal cord injury.

INTRODUCTION: Traumatic spinal cord injury (TSCI) is among the most severe disabilities with an estimation of 2.5 million people affected worldwide. The purpose of this study was to investigate the association between detrusor muscle function and the level of the spinal cord injury. MATERIAL AND METHODS: All patients with TSCI who underwent urodynamic evaluation at the Brain and Spinal Injury Research Center (BASIR) of Imam Khomeini hospital complex from March 2014 to March 2016 were retrospectively entered in this cross-sectional study. The patients were divided into three groups of suprasacral (C1-T12), sacral (L1-S5) and combined (both suprasacral and sacral) lesions. RESULTS: Medical records of 117 patients with spinal cord injury were reviewed. The mean age of the patients was 35.64 (±12.01) years. 86 patients (73.5%) were male and 31 female (26.5%). While 66 (56.4%), 28 (23.9%) and 19 (16.2%) patients had suprasacral, sacral, and combined suprasacral and sacral lesions, respectively. The relationship between the level of injury and emptying disorder (P = 0.50), storage disease (P = 0.20), first desire to void (P = 0.82), hypocompliance (P = 0.95), voided urine volume (P = 0.38) and residual urine volume (P = 0.76) were not significant. We found a significant association between the level of injury and the type of detrusor function (P = 0.019). CONCLUSIONS: Our study showed an association between detrusor muscle function and level of the spinal cord injury. However, there was no exact relationship between the level and the completeness of the spinal cord injury with the urodynamic characteristics.

Contralateral Radiculopathy: A Kernohan-Woltman Notch-like Phenomenon.

Lumbar disc herniation is the most common cause of radiculopathy. In most cases, the chief complaint is associated with radicular pain due to nerve compression on the herniated side. However, a radicular pain contralateral to the herniation side is an unusual finding rarely reported in the literature. Here, a case of right lower limb radicular pain in the presence of left extruded L4-L5 disc herniation is reported. Management of the patient is discussed in addition to a review of the literature regarding hypotheses on the mechanism of this unusual situation.

Mechanisms of spinal cord injury regeneration in zebrafish: a systematic review.

OBJECTIVES: To determine the molecular and cellular mechanisms of spinal cord regeneration in zebrafish. MATERIALS AND METHODS: Medical databases of PubMed and Scopus were searched with following key words: Zebrafish; spinal cord injuries; regeneration; recovery of function. The map of mechanisms was performed using Xmind software. RESULTS: Wnt/ß-catenin signaling, L1.1, L1.2, Major vault protein (MVP), contactin-2 and High mobility group box1 (HMGB1) had positive promoting effects on axonal re-growth while Ptena had an inhibitory effect. Neurogenesis is stimulated by Wnt/ß-catenin signaling as well as HMGB1, but inhibited by Notch signaling. Glial cells proliferate in response to fibroblast growth factor (FGF) signaling and Lysophosphatidic acid (LPA). Furthermore, fgf signaling pathway causes glia bridge formation in favor of axonal regeneration. LPA and HMGB1 in acute phase stimulate inflammatory responses around injury and suppress regeneration. LPA also induces microglia activation and neuronal death in addition to glia cell proliferation, but prevents neurite sprouting. CONCLUSION: This study provides a comprehensive review of the known molecules and mechanisms in the current literature involved in the spinal cord injury (SCI) regeneration in zebrafish, in a time course manner. A better understanding of the whole determining mechanisms for the SCI regeneration should be considered as a main goal for future studies.

A systematic review about the position-related complications of acoustic neuroma surgery via suboccipital retrosigmoid approach: Sitting versus lateral.

This systematic review wasdesigned to compare the complications of acoustic neuroma surgery via the suboccipital retrosigmoid approach in the sitting versus lateral positions. Searches for randomized trials and observational studies about the complications of acoustic neuroma surgery were performed in five medical databases (though October 2015) including PubMed, MEDLINE (In-Process and Other Non-Indexed Citations), EMBASE, Cochrane Central Register of Controlled Trials (CENTRAL) and PsycINFO. Primary outcomes in this study were venous air emboli, neuropsychological defects, CSF leak, facial and abducens nerves palsy, postoperative deafness, hydrocephalus and mortality. Secondary outcomes were total tumor removal, facial and cochlear nerves preservation and ataxia. 843 abstracts and titles were reviewed and 10 studies (two non-randomized comparative studies and 8 non-comparative case series) were included for data extraction. Because of the heterogeneity of the studies, small number of participants and methodological shortcomings, findings were evaluated qualitatively. No impressive advantage was found in surgical or neurological outcomes for use of the sitting or lateral positions in patients with acoustic neuroma surgery. According to the available evidence, it seems that both sitting and lateral positions can be used with an equivalent safety for acoustic neuroma surgery via the retrosigmoid suboccipital approach. There seems a clear need for comparative studies to compare harms and other outcomes for these two positions.

Feasibility and Data Quality of the National Spinal Cord Injury Registry of Iran (NSCIR-IR): A Pilot Study.

BACKGROUND: Spinal cord injury (SCI) is one of the most disabling consequences of trauma with unparalleled economic, social, and personal burden. Any attempt aimed at improving quality of care should be based on comprehensive and reliable data. This pilot investigation studied the feasibility of implementing the National Spinal Cord and Column Injury Registry of Iran (NSCIR-IR) and scrutinized the quality of the registered data. METHODS: From October 2015 to May 2016, over an 8-month period, 65 eligible trauma patients who were admitted to hospitals in three academic centers in mainland Iran were included in this pilot study. Certified registered nurses and neurosurgeons were in charge of data collection, quality verification, and registration. RESULTS: Sixty-five patients with vertebral column fracture dislocations were registered in the study, of whom 14 (21.5%) patients had evidence of SCI. Mechanisms of injury included mechanical falls in 30 patients (46.2%) and motor vehicle accidents in 29 (44.6%). The case identification rate i.e. clinical and radiographic confirmation of spine and SCI, ranged from 10.0% to 88.9% in different registry centers. The completion rate of all data items was 100%, except for five data elements in patients who could not provide clinical information because of their medical status. Consistency i.e. identification of the same elements by all the registrars, was 100% and accuracy of identification of the same pathology ranged from 66.6% to 100%. CONCLUSIONS: Our pilot study showed both the feasibility and acceptable data quality of the NSCIR-IR. However, effective and successful implementation of NSCIR-IR data use requires some modifications such as presence of a dedicated registrar in each center, verification of data by a neurosurgeon, and continuous assessment of patients' neurological status and complications.

Bilateral same-day endoscopic transcanal cartilage tympanoplasty: initial results / Timpanoplastia bilateral transcanal por via endoscópica com cartilagem realizada simultaneamente: resultados iniciais

Abstract Introduction: Same-day closure of bilateral tympanic membrane perforations is a quick and more comfortable procedure for the patients. However, conventional bilateral same-day tympanoplasty or myringoplasty has been rarely performed because of the theoretical risk of postoperative complications. Objective: To evaluate the advantages and outcomes of bilateral simultaneous endoscopic cartilage tympanoplasty in patients with bilateral tympanic membrane perforations. Methods: From February 2012 to March 2013, patients with bilateral dry tympanic membrane perforations who had some degree of hearing loss corresponding to the size and location of the perforation entered the study. There was no suspicion to disrupted ossicular chain, mastoid involvement or other middle or inner ear pathology. Endoscopic transcanal cartilage tympanoplasty was done using the underlay (medial) technique. The graft was harvested from cymba cartilage in just one ear with preservation of perichondrium in one side. A 1.5 cm × 1.5 cm cartilage seemed to be enough for tympanoplasty in both sides. Results: Nine patients (4 males and 5 females) with the mean age of 37.9 years underwent bilateral transcanal cartilage tympanoplasty in a same-day surgery. The mean duration of follow up was 15.8 months. There were detected no complications including hearing loss, otorrhea and wound complication with no retraction pocket or displaced graft during follow-up period. The grafts take rate was 94.44% (only one case of unilateral incomplete closure). The mean of air-bone gap overall improved from 13.88 dB preoperatively to 9.16 dB postoperatively (p < 0.05). Conclusion: Bilateral endoscopic transcanal cartilage tympanoplasty can be considered as a safe minimally invasive procedure that can be performed in a same-day surgery. It reduces the costs and operation time and is practical with a low rate of postoperative complications.

Resumo Introdução: O fechamento no mesmo dia de perfuração bilateral da membrana timpânica é um procedimento rápido e mais confortável para os pacientes. Entretanto, a timpanoplastia ou miringoplastia convencional bilateral executada no mesmo procedimento tem sido raramente feita devido ao risco teórico de complicações pós-operatórias. Objetivo: Avaliar as vantagens e os resultados da timpanoplastia bilateral simultânea com cartilagem por via endoscópica em pacientes com perfuração bilateral da membrana timpânica. Método: De fevereiro de 2012 a março de 2013, pacientes com perfuração seca bilateral da membrana timpânica que tinham algum grau de perda de audição correspondente ao tamanho e à localização da perfuração foram incluídos no estudo. Não houve suspeita de cadeia ossicular interrompida, envolvimento do mastoide ou outra doença da orelha média ou interna. Timpanoplastia com cartilagem transcanal foi executada por via endoscópica com a técnica de underlay (medial). O enxerto foi colhido de cartilagem da concha superior (cymba) em apenas uma orelha com preservação do pericôndrio em um lado. Um enxerto de 1,5 × 1,5 centímetro de cartilagem pareceu ser o suficiente para a timpanoplastia em ambos os lados. Resultados: Nove pacientes (quatro homens e cinco mulheres) com média de 37,9 anos foram submetidos à timpanoplastia bilateral com cartilagem transcanal em uma cirurgia feita em etapa única. O tempo médio de acompanhamento foi de 15,8 meses. Não foram detectadas complicações, inclusive perda de audição, otorreia e complicações como bolsa de retração ou deslocamento de enxerto durante o período de seguimento. A taxa de sucesso do enxerto foi de 94,44% (apenas um caso de fechamento unilateral incompleto). A média do gap aéreo-ósseo em geral melhorou de 13,88 dB no pré-operatório para 9,16 dB no pós-operatório (p < 0,05). Conclusão: Timpanoplastia bilateral com cartilagem transcanal por via endoscópica pode ser considerada como um procedimento minimamente invasivo, seguro e que pode ser executado em uma única cirurgia. Isso reduz os custos e tempo de operação e é prático, com um baixo índice de complicações pós-operatórias.