A case of recurrent urinary tract infections with neurogenic bladder due to spinal tumors

Neuroblastic tumors are the most common extracranial solid tumors in children. They are manifested by different clinical presentations ranging from cord compression symptoms to asymptomatic cases. A 2.5-year girl with a history of vaginal delivery at 39 gestational weeks and low Apgar score presented by repeated episodes of urinary tract infections and progressive paraplegia started at the age of 8 months.Brain MRI and EEG were normal. Voiding cystourethrography revealed grade II vesicoureteral reflux in the left kidney. Lumbar MRI with and without contrast showed a dumbbell shape mass, the hyper signal in T2 -weighted image and low signal in T1 -weighted image, extramedullary, and intramural with mass effect on the cord.Microscopic examination of tissue obtained by surgery reported ganglioneuroma.Our case was interesting because of her presentation, neurogenic bladder associated with repeated episodes of urinary tract infections, and secondary paraplegia. Neurogenic bladder dysfunction is rarely reported in cases with ganglioneuroma

A Study on the Risk Factors for Obstetrical Brachial Plexus Palsy

ObjectiveConsiderable medical and legal debates have surrounded the prognosis and outcome of obstetrical brachial plexus injuries and obstetricians are oftenconsidered responsible for the injury. In this study, we assessed the factors related to the outcome of brachial plexus palsy.Material & MethodsDuring 24 months, 21 neonates with obstetrical brachial plexus injuries were enrolled.Electrophysiology studies were done at the age of three weeks. They received physiotherapy and occupational therapy. They were examined every 3 monthsfor one year and limbs function was assessed according to Mallet scores; also, maternal and neonatal factors were collected by a questionnaire.Results There were 10 boys and 11 girls.Of all, 76.2% had Erb's palsy, 19% had total brachial palsy and 4.8% hadklumpke paralysis.Risk factors including primiparity, high birth weight, shoulder dystocia, andprolonged second stage of labor were assessed.Electrophysiology studies showed neuropraxia in 52.4% and axonal injuries in42.9% of the patients.At the end of the first year, 81% of the patients had functioned recovery aroundgrade III or IV of Mallet scores.There were only significant relationships between functional improvement andneurophysiologic findings.ConclusionOutcome of obstetrical brachial injuries has a close relationship toneurophysiologic study results than other risk factors

The Results of Whole Exome Sequencing Performed On Previously Undiagnosed Pediatric Neurology Patients

  Objective Whole exome sequencing (WES) is a new molecular diagnostic test, used in pediatric medicine, especially pediatric neurology. The diagnostic yield of WES is higher than conventional methods. Therefore, this study aimed to assess the diagnostic yield of WES in a pediatric neurology clinic and to report positive results. Materials & Methods This retrospective study was performed on patients, presenting to the pediatric neurology clinic of Ghaem Hospital in Mashhad, Iran, between March 2015 and March 2017, with various neurological disabilities and unrevealing workup before WES. The patients’ clinical features and molecular diagnoses based on the WES Results were reported in this study. The overall diagnostic yield of WES was 82.71% (67/81 patients). Two patients were excluded for the lack of data. Sixty-five patients with pathogenic or possibly pathogenic variants exhibited various abnormalities, including intellectual disability/developmental delay (n=44), seizure (n=27), developmental regression (n=11), myopathy (n=9), microcephaly (n=8), neuropathy (n=2), autism spectrumdisorder (n=2), and neuromuscular disease (n=2). Overall, 93.84% of the patients were born to consanguineous parents. Also, 62 patients had an autosomal recessive disorder, and three patients had an autosomaldominant disorder. Conclusion The present findings indicating the high diagnostic yield of WES, besides the important role of this test in determining the etiology of non-specific and atypical presentations of genetic disorders, support the use of WES in pediatric neurology practice

A study of nurses' observance rate of hygienic principles and nosocomial infections control

Background: Health-care personnel's familiarity with infection control methods and pathogen transmission channels play a determining role in reducing nosocomial infections. This study aimed at investigating the nurses' performance in observing hygienic principles and nosocomial infections control.Methods: This descriptive cross-sectional study was conducted on 34 nurses working at Afshar Hospital in Yazd, Iran in 2016. Nurses' performance on infections prevention was surveyed via a checklist using self-reporting by nurses. The gleaned data were analyzed using independent t-test and ANOVA.Results: Our findings suggested that 69.5% of nurses had moderate performance on nosocomial infections control with regard to themselves, 63.2% had it with regard to patients, and 71.5% had it with regard to hospital setting. Also, there was a significant correlation between performance quality and nurses' working experience (P=0.01), on the one hand, and participation in an orientation workshop (P=0.001), on the other hand.Conclusions: Based on our findings, it is rendered as mandatory for hospital authorities to provide the required conditions for improving nurses' awareness and performance with regard to observing the hygienic principles and nosocomial infections control.

HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) versus adult T-cell leukemia/lymphoma (ATLL)

ObjectivesHuman T cell leukemia virus-1 (HTLV-1) infection may lead to one or both diseases including HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) or adult T cell leukemia lymphoma (ATLL). The complete interactions of the virus with host cells in both diseases is yet to be determined. This study aims to construct an interaction network for distinct signaling pathways in these diseases based on finding differentially expressed genes (DEGs) between HAM/TSP and ATLL.ResultsWe identified 57 hub genes with higher criteria scores in the primary protein-protein interaction network (PPIN). The ontology-based enrichment analysis revealed following important terms: positive regulation of transcription from RNA polymerase II promoter, positive regulation of transcription from RNA polymerase II promoter involved in meiotic cell cycle and positive regulation of transcription from RNA polymerase II promoter by histone modification. The upregulated genes TNF, PIK3R1, HGF, NFKBIA, CTNNB1, ESR1, SMAD2, PPARG and downregulated genes VEGFA, TLR2, STAT3, TLR4, TP53, CHUK, SERPINE1, CREB1 and BRCA1 were commonly observed in all the three enriched terms in HAM/TSP vs. ATLL. The constructed interaction network was then visualized inside a mirrored map of signaling pathways for ATLL and HAM/TSP, so that the functions of hub genes were specified in both diseases.Peer reviewe

Acoustic Neurinomas

Acoustic neuromas (AN) are schwann cell-derived tumors that commonly arise from the vestibular portion of the eighth cranial nerve also known as vestibular schwannoma(VS) causes unilateral hearing loss, tinnitus, vertigo and unsteadiness. In many cases, the tumor size may remain unchanged for many years following diagnosis, which is typically made by MRI. In the majority of cases the tumor is small, leaving the clinician and patient with the options of either serial scanning or active treatment by gamma knife radiosurgery (GKR) or microneurosurgery. Despite the vast number of published treatment reports, comparative studies are few. The predominant clinical endpoints of AN treatment include tumor control, facial nerve function and hearing preservation. Less focus has been put on symptom relief and health-related quality of life (QOL). It is uncertain if treating a small tumor leaves the patient with a better chance of obtaining relief from future hearing loss, vertigo or tinnitus than by observing it without treatment.   In this paper we review the literature for the natural course, the treatment alternatives and the results of AN. Finally, we present our experience with a management strategy applied for more than 30 years

Surgery for Temporal Lobe Epilepsy in Children

This study aimed to assess the efficacy of magnetic resonance imaging (MRI) and single-photon emission computed tomography (SPECT) in localizing epileptic foci in children with temporal lobe epilepsy (TLE). This prospective study was conducted on 12 patients including five males and seven females aged between 2 and 16 years old with a clinical diagnosis of TLE. All the patients underwent high-resolution MRI and if no abnormality was identified, SPECT was used for further assessment. In all the patients, visual inspection identified unilateral mesial temporal sclerosis, and the patients underwent craniotomy and lesionectomy.According to the results, eight patients were categorized in class I and two cases were classifies as class II, and all the patients survived. About 25% of the patients showed adequate memory function on the non-operated temporal lobe, and general intelligence quotient increased by 10% in 50% of the patients.According to the results, in patients with clinically suspected TLE, MRI alone is not able to localize the epileptic foci correctly, and SPECT can be helpful to localize these lesions

Sleep Apnea Syndrome after Posterior Fossa Surgery: A Case of Acquired Ondine's Curse

Introduction: Ondine’s Curse is a catastrophic but rare condition in adults. It is referred to as a congenital or acquired condition, in which the patient cannot breathe automatically while asleep. Acquired causes of this disease can be any cause affecting the ventrolateral part of the medulla, which is considered to be the breathing center in humans.    Case Report:   A 51-year-old woman, with ataxia and the symptoms and signs of rising Intra-Cranial Pressure, who underwent ventriculoperitoneal shunting and removal of tumour, developed episodic apnea during sleep after surgery and hypercapnia when awake. In her post-operative CT scan, some fine spots of hypodensity in the left lateral part of the medulla were observed. She was managed pharmacologically and underwent tracheotomy. After 50 days, she was discharged from the hospital when she was able to breathe normally.   Conclusion:  Having experience with this condition after resection of a fourth ventricle tumor, it was found that Ondine’s Curse can be considered as one of the complications of  posterior fossa surgery and is curable by proper management

Huge Intracanal lumbar Disc Herniation: a Review of Four Cases

Lumbar disc herniation (LDH) is the most common cause of sciatica and only in about 10% of the affected patients, surgical intervention is necessary. The side of the patient (the side of most prominent clinical complaints) is usually consistent with the side of imaging (the side with most prominent disc herniation on imaging scans). In this case series, we presented our experience in four cases with huge intracanal LDH that a mismatch between the patient’s side and the imaging’s side was present. In these cases, for deciding to do the operation, the physicians need to rely more on clinical findings, but for deciding the side of discectomy, imaging characteristic (imaging side) may be a more important criterion

Hypoglossal Schowannoma: A Case Report and Review of Literature

Introduction: Schawannomas (neuromas, neurilemmomas) are benign tumors originating from showann cells or nerve fiber sheet cells. They are solitary, encapsulated tumors usually attached to, or surrounded by a nerve. Case Report: We present a case of left hypoglossal nerve schwannoma in a 19 year old man who was admitted with progressive left tongue atrophy. Conclusion: Schwannoma of the hypoglossal nerve usually develops in the intracranial and extracranial portion or both in the intracranial and extracranial components forming a dumbbell shape tumor .The peripheral hypoglossal schwannomas are extremely rare