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Nervous System Diseases - Top 30 Publications

Delivering at the country level: the International Coordinating Group on Vaccine Provision and its impact in 2016 and 2017.

Update: Dura Mater Graft-Associated Creutzfeldt-Jakob Disease - Japan, 1975-2017.

Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder that, according to the most well accepted hypothesis (1), is caused by replicating, transmissible, abnormal forms of a host-encoded prion protein (prions). Most CJD cases occur spontaneously (sporadic CJD) or are inherited (genetic CJD). Iatrogenic CJD can occur after exposure to prion-contaminated instruments or products in medical/surgical settings. Cadaveric dura mater graft-associated CJD (dCJD) accounts for a common form of iatrogenic CJD. This report summarizes the epidemiologic features of 154 cases of dCJD identified in Japan during 1975-2017; these cases account for >60% of dCJD cases reported worldwide (1,2). The unusually high prevalence of dCJD in Japan was first reported in 1997 (3). In 2008, a single brand of graft (Lyodura [B. Braun Melsungen AG, Melsungen, Germany]), frequently used as a patch in neurosurgical procedures, was identified as the probable vehicle of transmission (4). No international recall of the implicated Lyodura occurred, the product had a relatively long shelf life, and the grafts were used frequently in Japanese patients with non-life-threatening conditions (4,5). Since 2008, additional cases have been ascertained, reflecting the identification of previously missed cases and the occurrence of new cases with longer latency periods (interval from exposure to symptom onset) for dCJD (up to 30 years), underscoring the importance of maintaining surveillance for dCJD.

Hearing Loss in Adults.

Hearing Loss in Adults.

Hearing Loss in Adults.

Post-traumatic scleromalacia.

We here report the case of a 18-year old patient who was referred for evaluation of pigmented subconjunctival tumor of the left eye mimicking uveal melanoma. The patient reported that the lesion had evolved rapidly during the previous months. Eye exam showed prominent dark brown lesion adjacent to the limbus between 3 and 9 hours, measuring 10 mm x 6 mm (A). Ultrasonographic biomicroscopy revealed cystic structure communicating with the vitreous cavity, suggesting the diagnosis of uveal hernia rather of melanoma. During a more thorough interview, the patient reported that he had undergone transfixing keratoplasty for cloudy patch secondary to blunt trauma from stone's throw dating back 10 years. We assumed that the scleral wall had been damaged, leading to the development of scleromalacia with uveal hernia. The patient had recently had an acute episode of vomiting which could have lead to an increase in lesion size. The patient was followed up for several weeks, during which the lesion was stable. The patient underwent surgery with successful uveal tissue reintegration and sclera closure (B).

Pseudotumor cerebri revealing sarcoidosis.

Sarcoidosis is a multivisceral granulomatosis of unknown aetiology which may have various clinical and radiological manifestations. Cerebral sarcoidosis, although rare, can appears as a misleading pseudotumor. We report the case of a young Tunisian adult hospitalized for intracranial hypertension associated with pseudotumoral lesion on radiology, which was revealed to be systemic sarcoidosis.

Bullous pemphigoid and Parkinson's disease: about a case.

Bullous pemphigoid (BP) is an organ-specific autoimmune disease which can be associated with many pathologies including degenerative neurological diseases such as Parkinson's and Alzheimer's disease. Studies have suggested the possibility of cross-immune responses through "epitope spreading". Bullous dermatosis has been reported to be secondary to neurological disease, at intervals ranging from a few months to a maximum of a few years. Then is it an unusual association or a causation? It has been suggested that drug consumption, decubitus lesions, traumatic events as well as reduced immunity can be triggers for BP in patients with neurological disease. We report the case of a 93-year old patient with a 10-year history of advanced Parkinson's disease, hospitalized for the treatment of a common bullous pemphigoid confirmed by histology and immunohistochemistry. The patient was treated with oral corticosteroids. After a week of treatment, the patient died due to septic shock. Neurological disorders represent a real risk factor for BP. BP could be considered as a marker for neurological disorder. These associations are of broad interest, because they can play a role in the etiopathogenesis of BP and contribute to a complete understanding of the causes of these neurodegenerative diseases.

Hearing Loss in Adults.

Case 7-2018: A 25-Year-Old Man with New-Onset Seizures.

The Effect of Sleep Loss on Dual Time-Based Prospective Memory Tasks.

The aim of the present study was to deepen knowledge about the effect of a lowered vigilance state on time-based prospective memory (TBPM) performance. For this purpose 2 TBPM tasks (primary and interpolated), which shared a portion of the retention interval, and 3 reasoning tasks, as ongoing activities, were administered after total sleep deprivation and in a regular sleep condition. The results showed a detrimental effect of sleep deprivation on prospective memory performance and a partial dissociation between clock-checking behavior and time estimation for prospective compliance. This study clearly indicates that total sleep deprivation im- pairs the ability to complete multiple prospective task assignments in a timely fashion. Results are discussed suggesting the existence of different mechanisms involved in time monitoring and other cognitive functions underlying TBPM performance.

High incidence of rainbow glare after femtosecond laser assisted-LASIK using the upgraded FS200 femtosecond laser.

To compare the incidence of rainbow glare (RG) after femtosecond laser assisted-LASIK (FS-LASIK) using the upgraded FS200 femtosecond laser with different flap cut parameter settings.

Neuroprotective effect of ethanol in acute carbon monoxide intoxication: A retrospective study.

In acute carbon monoxide (CO) intoxication, treatment of neurologic injury and prevention of neurological sequelae are primary concerns. Ethanol is the one of the frequent substances which is co-ingested in intentional CO poisoning. Neuroprotective effect of ethanol was highlighted and demonstrated in isolated brain injury recently. We assessed the neuroprotective effect of ethanol in acute CO intoxication using magnetic resonance imaging (MRI).We retrospectively reviewed medical records for patients who visited an emergency medical center of a university-affiliated hospital during a period of 73 months, from March 2009 to April 2015. Enrolled patients were divided into 2 groups, patients with or without abnormal brain lesion in brain MRI. Multivariate logistic regression analysis was performed to assess the factors associated with brain injury in MRI.A total of 109 patients with acute CO intoxication were evaluated of which 66 (60.55%) tested positive in brain MRI. MRI lesion-positive patients were more likely to have electrocardiogram change, elevation of serum troponin I and s100 protein level and lower serum ethanol level. Serum ethanol positivity was an independent factor for prevalence of brain injury in MRI in acute CO poisoning.This study revealed that ethanol which is co-ingested in acute CO intoxication may work the neuroprotective effect and could consequence more favorable neurological outcome in acute CO intoxication.

Case report of a pituitary thyrotropin-secreting macroadenoma with Hashimoto thyroiditis and infertility.

Thyrotropin-secreting adenoma (TSHoma) is rare, diagnosis and treatment are often delayed if the condition coexists with Hashimoto thyroiditis. The enlarged pituitary adenoma may eventually induce panhypopituitarism, infertility, or the compression of optic nerves and optic chiasma.

High EMP3 expression might independently predict poor overall survival in glioblastoma and its expression is related to DNA methylation.

In this study, we analyzed the prognostic value of epithelial membrane protein 3 (EMP3) in terms of overall survival (OS) in glioblastoma multiforme (GBM) and the association between its expression and DNA methylation.Bioinformatic analysis was performed by using data from the Cancer Genome Atlas (TCGA) database.EMP3 expression was markedly higher in GBM tissues than in normal brain tissues. High EMP3 expression was associated with significantly worse OS in patients with GBM. Univariate and multivariate analysis showed that EMP3 expression was an independent prognostic factor of poor OS no matter converting its expression into categorical variables (Hazard Ratio [HR] = 1.359, 95%CI: 1.118-1.652, P = .002) or setting it as a continuous variable (HR = 1.178, 95%CI: 1.101-1.260, P < .001). Among different subtypes of GBM, proneural subtype had the lowest EMP3 expression. The lowest EMP3 expression was observed in cluster 5 DNA methylation, which all belong to G-CIMP phenotype. Regression analysis confirmed a moderate negative correlation between EMP3 expression and its DNA methylation (Pearson's r = -0.61).Based on these findings, we infer that high EMP3 expression might be an independent indicator of unfavorable OS in GBM. EMP3 expression might be repressed by DNA methylation.

Recombinant human erythropoietin for treating severe traumatic brain injury.

This study aimed to explore the efficacy and safety of recombinant human erythropoietin (RHE) for the treatment of severe traumatic brain injury (STBI).

Encephalopathy associated with propofol infusion syndrome: A case report.

Propofol infusion syndrome (PRIS) is a rare but potentially fatal complication of propofol infusion. It is clinically characterized by metabolic acidosis, refractory bradycardia, rhabdomyolysis, renal failure, hyperlipidemia, and hepatomegaly. Brain lesion was only reported once in a pediatric patient. We present the 1st adult case with colon polyp and cancer who was diagnosed with PRIS. Her brain magnetic resonance imaging (MRI) and computed tomography (CT) scans reveal prominent bilateral brain lesions, matching with the proposed pathophysiologic mechanism of the syndrome. The patient received prompt acidosis correction and cardiorespiratory support. At last, she died from refractory circulatory failure.

Cardiovascular risk factors in patients with combined central retinal vein occlusion and cilioretinal artery occlusion: Case report.

To analyze cardiovascular risk factors and comorbidity of acute unilateral visual loss due to combined central retinal vein occlusion (CRVO) and cilioretinal artery occlusion (CLRAO).

Longitudinally extensive transverse myelitis with pulmonary tuberculosis: Two case reports.

Longitudinally extensive transverse myelitis (LETM) is characterized by contiguous inflammatory lesions of spinal cord extending to ≥3 vertebral segments. The etiology of LETM is complicated, including various infection, autoimmune disease, and so on. Neuromyelitis optic spectrum disorder (NMOSD) is the most common cause of LETM. Several case reports have suggested the associations between NMOSD and pulmonary tuberculosis (PTB).

Effectiveness and neural mechanisms of home-based telerehabilitation in patients with stroke based on fMRI and DTI: A study protocol for a randomized controlled trial.

Stroke is one of leading diseases causing adult death and disability worldwide. Home-based telerehabilitation has become a novel approach for stroke patients as effective as conventional rehabilitation, and more convenient and economical than conventional rehabilitation. However, there is no study assessing the mechanism of home-based telerehabilitation in promoting motor recovery among stroke patients with hemiplegic.

A new clinical decision support tool for improving the adequacy of anticoagulant therapy and reducing the incidence of stroke in nonvalvular atrial fibrillation: A randomized clinical trial in primary care.

Atrial fibrillation (AF) is the most common cardiac arrhythmia and increases the risk of ischemic stroke 4 to 5-fold. The first choice of anticoagulant therapy (AT) is the vitamin K antagonist (VKA). Contraindication to VKA or poor control of the International Normalized Ratio leads to the administration of direct-acting oral anticoagulants. There is a trend toward inadequate AT in nonvalvular AF (NVAF) patients.

Autoimmune encephalitis positive for both anti-γ-aminobutyric acid B receptor and anticollapsin response-mediator protein 5 antibodies: A case report.

Autoimmune encephalitis (AE) is a heterogeneous group of recently identified disorders. Despite severe and even prolonged neurologic deficits, dramatic improvements may occur with proper immunotherapy in some patients with AE. Antineuronal antibodies have been discovered in patients' serum and cerebrospinal fluid (CSF). However, AE with multiple antineuronal antibodies is rare. To date, there are no published reports of AE with both anti-γ-aminobutyric acid B receptor (GABABR) and anticollapsin response-mediator protein 5 (CV2) antibodies.

Three-dimensional brain arteriovenous malformation models for clinical use and resident training.

To fabricate three-dimensional (3D) models of brain arteriovenous malformation (bAVM) and report our experience with customized 3D printed models of patients with bAVM as an educational and clinical tool for patients, doctors, and surgical residents.

Bilateral abducens nerve palsies and urinary retention caused by the rupture of a vertebral artery aneurysm: A case report and literature review.

Bilateral abducens nerve deficits caused by vertebral artery (VA) aneurysm have been reported, but there have been no reports regarding accompanying urinary retention.

Carpal Tunnel Syndrome: Making Evidence-Based Treatment Decisions.

Carpal tunnel syndrome (CTS) is one of the most common musculoskeletal disorders of the upper extremity. Comorbidities associated with the development of CTS include diabetes and obesity. Although a high rate of repetitive hand/wrist motions is a risk factor, there is insufficient evidence to implicate computer use in the development of CTS. Initial treatment generally is nonoperative, with the strongest evidence supporting bracing/splinting. Strong evidence supports operative treatment, regardless of technique, as superior to nonoperative treatment. Complications are infrequent and most are minor and transient.

Hip Surveillance in Children with Cerebral Palsy.

The hip is the second most common involved joint in cerebral palsy. Hip displacement occurs in more than 33% of children with cerebral palsy, with a higher prevalence in nonambulatory children. Hip displacement in this population is typically progressive. Hip dislocation can result in pain and difficulty with sitting and perineal care. Since early stage of hip displacement can be silent, and hip surveillance programs are recommended. Most programs use the degree of hip dysplasia and Growth Motor Function Classification System level for screening recommendations. Treatment depends on the degree of dysplasia, functional status of the patient, and patient's age.

Visual field defects and retinal nerve fiber imaging in patients with obstructive sleep apnea syndrome and in healthy controls.

To assess the retinal sensitivity in obstructive sleep apnea hypopnea syndrome (OSAHS) patients evaluated with standard automated perimetry (SAP). And to correlate the functional SAP results with structural parameters obtained with optical coherence tomography (OCT).

The fast exodrift after the first surgical treatment of exotropia and its correlation with surgical outcome of second surgery.

To compare the rate of exodrift after a second surgery for recurrent exotropia, in patients grouped to fast versus slow exodrift after their first surgery. To determine whether there is a correlation with surgical outcome, and to evaluate the factors associated with fast exodrift.

Progress towards poliomyelitis eradication: Nigeria, January–December 2017.

Improving the auditory environment for patients with intellectual disabilities and hearing loss.