Neuritis Research Articles

Evidence-based guidelines for diagnosis and treatment of demyelinating optic neuritis in China(2021)

Demyelinating optic neuritis (DON) is an inflammatory demyelinating disease of the central nervous system, which can lead to visual loss in young adults as a major factor. The key points concerned by neuro-ophthalmologists are to reduce the rate of recurrence and improve the prognosis in the course of diagnosis and treatment of DON. Companied with the deep research of diagnostic markers, different classifications and therapies, and evidence-based medicine, there are more and more clinical evidences for the development and standardization of evidence-based guidelines for diagnosis and treatment. According to the routine procedures, the expert steering committee, the guideline development group, the external review expert group and the working group were established. The clinical concerns were collected directly from neuro-ophthalmologists, ophthalmologists of other subspecialties and neurologists. The guideline development group comprehensively searched and systematically evaluated the relevant research evidence at home and abroad, analyzed the preferences and values of patients, and organized the expert consensus meeting. Sixteen recommendations for the diagnosis and treatment of DON were formulated, including classification and diagnosis, therapies in acute and chronic phases, and the follow-up management, which has important guiding significance for the improvement of clinical treatment and reduction of the recurrence rate. (Chin J Ophthalmol, 2021, 57:171-186).

Optic Neuritis Incidence, Prevalence, Risks, and Etiologies

The differential for optic neuritis (ON) is broad. While multiple sclerosis (MS) is at the top of the list, various other neurologic and systemic

Aquaporin-4 and Myelin Oligodendrocyte Glycoprotein Antibody-Associated Optic Neuritis: Diagnosis and Treatment

Optic neuritis (ON) is a frequent manifestation of aquaporin-4 (AQP4) antibody-mediated neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis (MOG-EM; also termed MOG antibody-associated disorders, MOGAD). The past few years have seen major advances in the diagnosis and treatment of these two relatively new entities: international diagnostic criteria for NMOSD and MOG-EM have been proposed, improved antibody assays developed, and consensus recommendations on the indications and methodology of serological testing published. Very recently, the results of four phase III trials assessing new treatment options for NMOSD have been presented. With eculizumab, a monoclonal antibody inhibiting complement factor C5, for the first time a relapse-preventing long-term treatment for NMOSD - which has so far mostly been treated off-label with rituximab, azathioprine, and other immunosuppressants - has been approved. Data from recent retrospective studies evaluating treatment responses in MOG-ON suggest that rituximab and other immunosuppressants are effective also in this entity. By contrast, many drugs approved for the treatment of multiple sclerosis (MS) have been found to be either ineffective or to cause disease exacerbation (e.g., interferon-β). Recent studies have shown that not only NMOSD-ON but also MOG-ON usually follows a relapsing course. If left untreated, both disorders can result in severe visual deficiency or blindness, though MOG-ON seems to have a better prognosis overall. Acute attacks are treated with high-dose intravenous methylprednisolone and, in many cases, plasma exchange (PEX) or immunoadsorption (IA). Early use of PEX/IA may prevent persisting visual loss and improve the long-term outcome. Especially MOG-ON has been found to be frequently associated with flare-ups, if steroids are not tapered, and to underlie many cases of "chronic relapsing inflammatory optic neuropathy" (CRION). Both NMOSD-ON and MOG-ON are often associated with simultaneous or consecutive attacks of myelitis and brainstem encephalitis; in contrast to earlier assumptions, supratentorial MRI brain lesions are a common finding and do not preclude the diagnosis. In this article, we review the current knowledge on the clinical presentation, epidemiology, diagnosis, and treatment of these two rare yet important differential diagnoses of both MS-associated ON und idiopathic autoimmune ON.

G protein regulated inducer of neurite outgrowth 1 is a potential marker for lung cancer prognosis.

Lung cancer mortality remains high, and only approximately 15% of patients with non-small cell lung cancer survive for more than five years. The purpose of this research was to investigate the prognostic value and biological functions of G protein regulated inducer of neuritis outgrowth 1(GPRIN1) in lung cancer. We used the Kaplan-Meier method to analyze the correlation between GPRIN1 and overall survival, and performed Cox regression to determine whether GPRIN1 might be an independent predictive factor for lung adenocarcinoma prognosis. qRT-PCR and Western blot assays were conducted to detect GPRIN1 expression in lung cancer cells and normal control cells. To detect the functional effects of knockdown/overexpression of GPRIN1 on lung cancer cells, we performed CCK-8, colony formation and Transwell assays. Through the Kaplan-Meier method, we found that GPRIN1 expression correlated with overall survival and adverse prognosis, and Cox regression indicated that GPRIN1 is as an independent predictive factor for lung adenocarcinoma. Furthermore, the mRNA and protein expression levels of GPRIN1 in lung cancer cells were markedly higher than those in normal cells. Downregulation of GPRIN1significantly decreased cell viability, colony formation, the number of invasive and migrating cells, and levels of epithelial-mesenchymal transition-related proteins in A549 cells. Overexpression of GPRIN1showed the opposite effect in Calu-1 cells. Together, these results indicated that GPRIN1 facilitates lung cancer proliferation and migration, possibly by affecting the epithelial-mesenchymal transition of lung cancer cells, suggesting that GPRIN1may be used as an effective target for the treatment of lung cancer.

Comparison of the low frequency fluctuation between AQP4 antibody positive and MOG antibody positive patients with optic neuritis

Objective To compare the changes of spontaneous brain activity in myelin oligodendrocyte glycoprotein antibody (MOG-Ab) positive and Aquaporin 4 antibody (AQP4-Ab) positive neuromyelitis optica spectrum disorder (NMOSD) by using resting-state functional magnetic resonance imaging (fMRI). Methods A case control study was designed.A total of 11 NMOSD patients with positive MOG-Ab and 21 NMOSD patients with positive AQP4-Ab were enrolled from October 2006 to May 2017 in PLA General Hospital.Thirty-four healthy controls closely matched in age, sex and education were recruited and underwent resting-state fMRI scans.The amplitude of low-frequency fluctuation (ALFF) was extracted to investigate the spontaneous brain activity.This study was approved by Ethics Committee of PLA General Hospital (S2019-111-01). All subjects enrolled signed informed consent. Results Two patients in the MOG-Ab positive group had seizure history, and no seizure history was observed in AQP4-Ab positive group and healthy control group.Compared with healthy control group, all patients in MOG-Ab positive group and AQP4-Ab positive group had significantly increased ALFF values of prefrontal gyrus.The ALFF values of bilateral anterior central gyrus and bilateral posterior central gyrus in AQP4-Ab positive group were 1.89±0.56 and 2.10±0.69, respectively, which were lower than 3.32±1.15 and 3.61±1.23 in MOG-Ab positive group, the differences were statistically significant (both at P<0.001, AlphaSim correction). Conclusions Resting-state fMRI could provide new evidence of possibly multi-focal disease mechanisms.Hyperactivity in prefrontal cortex, motor cortex and somatosensory cortex might reflect differences in pathological processes between MOG-Ab positive and AQP4-Ab positive NMOSD patients. Key words: Optic neuritis; Resting-state functional magnetic resonance imaging; Amplitude of low frequency fluctuation; Neuromyelitis optica spectrum disorder; Aquaporin 4 antibody; Myelin oligodendrocyte glycoprotein antibody

Clinical analysis of relapsed demyelinating disease associated with myelin oligodendrocyte glycoprotein antibody in children

Objective To investigate the clinical characteristics, treatment and prognosis of relapsed demyeli-nating disease (RDD) associated with myelin oligodendrocyte glycoprotein antibodies (MOG abs) children in southern China. Methods Children with RDD associated with MOG abs at Department of Neurology in Guangzhou Women and Children′s Medical Center from January 2015 to December 2018 were retrospectively analyzed.The annualized relapse rates (ARRs) and expand disability status scale (EDSS) were used to assess the recurrence frequency and neurological dysfunction respectively. Results Ten children were included with the age of (6.4±3.6) years old, and male to female ratio was 4∶6.(1)Clinical phenotype: all children had 24 episodes during follow-up, with acute disseminated encephalomyelitis (ADEM)(7/10 cases) and neuromyelitis optica spectrum disorders (NMOSD)(3/10 cases) on the first episode.Among 14 recurrent episodes, ADEM (9/14 times) was the most common, followed by optic neuritis(ON)(3/14 times)and brainstem encephalitis (2/14 times). By the final follow-up, the final diagnosis was multiphasic disseminated encephalomyelitis(MDEM)(6/10 cases), NMOSD(3/10 cases), ADEM-ON(1/10 case), respectively.(2)Laboratory examination: all the children had positive serum MOG abs in the acute stage.The serum MOG abs titer high group(≥1∶640)(6 cases)on the first episode complicated ON (3 cases) and long segment myelitis (3 cases) more common than those of low group(1∶320)(4 cases). (3)Imaging changes: 25 times of bain magnetic resonance imaging (MRI) were performed in the acute stage, MRI changes mostly involved the cortex and subcortical white matter.Four cases had abnormal spinal cord MRI.(4)Treatment and prognosis: intravenous methylprednone (IVMP) combined with intravenous immunoglobulin (IVIG) were administrated in acute stage.Rituximab (2/10 cases), mycophenolate mofetil (4/10 cases), IVIG (2/10 cases) monthly and low dose prednisone orally (2/10 cases) were given respectively in maintains stage.ARRs decreased from 1.4 to 0 and EDSS score improved significantly after these treatments above.Seven cases had residual neurological dysfunction with 3 cases of NMOSD, 3 cases of MDEM and 1 case of ADEM-ON, including motor dysfunction, learning disability and inattention, symptomatic epilepsy and visual impairment. Conclusions ADEM is the most common form of RDD associated with MOG abs in children.Those with high serum MOG abs titer on the first episode are prone to have ON or long segment myelitis.Immunomodification therapy is effective in the relapsed patients, residual neurological sequelae were related to the type of repeated demyelination. Key words: Myelin oligodendrocyte glycoprotein; Relapsed demyelinating disease; Child

Study on the variation of visual evoked potential and its diagnostic value in children with optic neuritis

Objective To investigate the characteristics and clinical value of visual evoked potentials(VEP) in children with optic neuritis. Methods The VEP of 33 children with optic neuritis were tested by NICOLET evoked potential instrument.The results were compared with those of cranial and/or orbital MRI and fundus examination, and the consistency with visual performance was analyzed.The correlation between visual sensitivity and VEP results was analyzed. Results Among 33 children with optic neuritis, the abnormal rate of VEP in 52 abnormal eyes was 88.5%; the abnormal rate of cranial and/or orbital MRI was 38.5%; the abnormal rate of fundus examination was 62.2%; the abnormal rate of VEP examination was significantly higher than that of cranial and/or orbital MRI and fundus examination (P<0.05); the consistency rate between VEP examination and visual acuity was 84.8%.The consistency rate between pattern reversal visual evoked potential (P-VEP) and visual acuity was 86.8%, and that between flash visual evoked potential (F-VEP) and visual acuity was 82.1%.With the increase of visual impairment, the percentage of P100 wave loss increased gradually.There was no correlation between visual acuity and the prolongation of P100 wave latency. Conclusion There are significant differences between VEP and fundus examination and MRI, the sensitivity of VEP is superior to both.P-VEP is more consistent with visual acuity than F-VEP.VEP has certain value in evaluating the degree of visual impairment in children with optic neuritis.It can not be used to evaluate the level of optic sensitivity when the latency of P100 wave is prolonging. Key words: Optic neuritis; Visual evoked potentials; Visual; Children

A teenager with Kikuchi-Fujimoto disease and brachial plexus neuritis

Introduction: Kikuchi- Fujimoto disease (KFD) is a benign and self-limiting condition of unknown etiology. Neurologic manifestations involving the brachial plexus is extremely rare with only two cases reported in adults. We hereby present the first case in pediatrics and review the literature on the subject. Case: A 14 years old female of Iraqi descent presented with right neck swelling and pain for 3 weeks duration but no fevers. There was limited range of motion of the neck as well as significant persistent tender lymphadenopathy in the right post-auricular and posterior cervical chains. The rest of the physical examination was unremarkable. …

Efficacy comparison between plate fixation with in situ reinforcing ulnar nerve and anterior transposition for humeral intercondylar fracture

Objective To investigate the clinical efficacy of plate fixation with in situ reinforcing ulnar nerve and anterior transposition by olecranon osteotomy approach in the treatment of type C2 and C3 humeral intercondylar fractures. Methods A retrospective case control study was conducted to analyze the clinical data of 23 patients with humeral intercondylar fractures admitted to the Affiliated Hospital of Hangzhou Normal University from June 2014 to January 2017. There were 17 males and six females, aged 19-56 years, with an average of 41.2 years. According to AO classification, there were 15 patients with type C2 and eight with type C3. All patients were treated with plate internal fixation by olecranon osteotomy approach. The patients were divided into anterior group (11 patients) which was treated with ulnar nerve release anterior transposition and in situ reinforcing group (12 patients) which was treated with ulnar nerve release in situ. The operation time, intraoperative blood loss, fracture healing time, elbow flexion and extension range, elbow joint Mayo score, and excellent and good rate were compared between the two groups. Postoperative complications were recorded. Results All patients were followed up for 15-43 months, with an average of 26.7 months. All patients had no need of blood transfusion. There were no significant differences between anterior group and in situ reinforcing group in operation time [(219.1±15.1)minutes vs. (211.2±17.1)minutes], intraoperative blood loss [(263.6±35.3)ml vs. (237.5±25.6)ml] and fracture healing time [(18.2±2.4)weeks vs. (18.9±1.7)weeks] (P>0.05). No significant differences were found between the two groups in elbow flexion [(120.3±7.1)° vs. (120.3±4.1)°], straightness [(3.7±2.7)° vs. (4.1±2.4)°], Mayo score [(89.09±9.17)points vs. (86.67±10.29)points] and excellent and good rate [ 91% (10/12) vs. 83% (10/12)] (P>0.05). After operation, no complications such as infection, heterotopic ossification, ulnar neuritis, elbow valgus deformity, or internal fixation failure occurred. Conclusions For humeral intercondylar fracture, the plate fixation with in situ reinforcing ulnar nerve and anterior transposition by olecranon osteotomy approach both have advantages such as small surgical trauma, strong fixation, fewer complications, satisfactory recovery of elbow function. Ulnar nerve anterior transposition or not does not affect the occurrence of ulnar neuritis, and both can obtain satisfactory clinical efficacy. Key words: Humeral fractures; Intra-articular fractures; Fracture fixation, internal; Ulnar nerve

Comparison of MRI features of first-episode optic neuritis with positive AQP4 and MOG antibodies

Objective To explore the MRI features of acute optic neuritis with positive aquaporin-4 (AQP4) antibodies and myelin oligodendrocyte glycoprotein (MOG) antibodies during the first attack. Methods Eighty five patients (105 affected nerves) with first-episode AQP4-seropositive optic neuritis (AQP4-ON; n=58; 64 affected nerves) and MOG-seropositive optic neuritis (MOG-ON; n=27; 41 affected nerves) diagnosed by our hospital Neuro-ophthalmology Department between April 2014 and December 2017 were retrospectively analyzed. Double seronegative patients or double seropositive patients were not included. All patients underwent orbital conventional MRI; 29 patients also underwent RESOLVE-DWI and 55 underwent brain T2 fluid-attenuated inversion recovery (T2FLAIR).Theclinical features (age, gender, disease duration) and MRI features (T2WI, bilateral involvement, the degree and extent of enhancement, the extent of involvement, the affected segment and ADC values of nerve; demyelinating lesions of brain) were analyzed by 2 doctors. The qualitative parameters were compared with χ2 test or Fisher exact test and the quantitative parameters were compared with two independent sample t test (normal distribution) or the Mann-Whitney U test (skewed distribution). The Kappa test was used to test the consistency of the qualitative characteristics of the images evaluated by the 2 doctors. The interclass correlation coefficient (ICC) was used to test the consistency of the ADC obtained by the 2 doctors. Results There were no significant differences in the distributions of age and disease duration between 2 groups (t=1.911, Z=-1.054, P>0.05). AQP4-ON had a higher female proportion (χ2=13.911, P 0.05). There was a higher probability of optic nerve swelling, long segmental lesions and optic nerve head involvement in MOG-ON(χ2=16.845, 13.525, 13.264, P<0.05). Moreover, the rate of obvious enhancement and the involvement of optic chiasm was higher in AQP4-ON(χ2=7.152, 5.088, P<0.05). RESOLVE-DWI showed the ADC values of the AQP4-ON were significantly lower than those of MOG-ON (t=-3.300, P<0.05). The qualitative features of image evaluated by the 2 doctors were consistent well (Kappa=0.712-0.932, P<0.001) .The ADC values obtained by the 2 doctors were consistent well (ICC=0.942, P<0.001). Conclusions For the first-episode acute optic neuritis, AQP4-ON hasobvious female predilection and the trend of unilateral involvement and obvious enhancement, while MOG-ON isoften involved bilaterally with longitudinally extensive lesions.The ADC values of the AQP4-ON are significantly lower than those of MOG-ON and AQP4-ON are more vulnerable to involve the optic chiasm. Conventional MRI and RESOLVE-DWI are helpful for differentiating AQP4-ON from MOG-ON during the early stage of the disease course. Key words: Optic neuritis; Magnetic resonance imaging; Antibodies; Aquaporin 4; Myelin oligodendrocyte glycoprotein

Current research situation of the characteristics of recurrent optic neuritis and the pathogenesis of optic neuritis

Optic neuritis (ON) is one of the symptom of a central nervous system demyelinating, systemic or infectious disease. The pathogenetic mechanism of ON is still not completely clear, and its core is inflammation and immune that occurred in the optic nerve axons, and apoptosis of RGC was induced. Few patients experience recurrent episodes after treatment, presenting a remission - recurrence course of polyphasic disease, named recurrent ON (RON). Two forms of RON have been assigned: recurrent isolated optic neuritis, which is a chronic corticosteroid-dependent optic neuropathy with intermittent acute relapses, and recurrent isolated optic neuritis, which is a non-progressive relapsing ON without steroid dependence. Recurrence of ON causes cumulative damage to the optic nerve lesions and impaired visual signal transmission, thus causing irreversible damage to vision. Therefore, it is very important to have a deep understanding of the pathogenesis of ON and the clinical characteristics of RON, so as to better conduct clinical treatment. Key words: Optic neuritis/etiology; Review; Recurrent optic neuritis

Analysis of clinical characteristics of optic nerve metastases

Objective To explore the clinical features, primary lesions and prognosis of optic nerve metastases. Methods Seven patients (11 eyes) with optic nerve metastatic tumor diagnosed by the examinations of ophthalmology, laboratory and pathology in Chinese PLA General Hospital from April 2015 to September 2017 were included in this study. All patients underwent BCVA, flash VEP, OCT, orbital MRI, serum tumor marker, cerebral spinal fluid detection and PET-CT. Histopathological examination of primary or near superficial metastases was performed. The follow-up period was ranged from 16 to 44 months, with the mean of 23.0±10.9 months. The clinical characteristics, primary tumor, imaging features, treatment and clinical prognosis in the patients were analyzed. Results Among 7 patients, there were 5 males and 2 females, with the mean age of 53.90±14.99 years; 3 patients with unilateral optic nerve involvement, 4 patients with bilateral optic nerve involvement; 5 patients (71.4%) first diagnosed in ophthalmology. Five patients (45.5%) were misdiagnosed as optic neuritis, optic disc edema in 6 eyes (54.5%). All of them appear loss of visual acuity, including 8 eyes (72.7%) with BCVA 0.5. MRI results show that 1 patient with intraorbital segment, 1 patient with internal segment of optic canal, 4 patients with intracranial segment, 1 patient with intracranial segment and optic chiasma involved simultaneously, 4 patients involving surrounding tissue. There were 4 patients (57.1 %) with lung cancer, 2 patients (28.6%) with kidney cancer, 1 patient (14.3%) with gastric cance; 6 patients (85.7%) with metastasis from other sites, 2 patients with brain metastasis (1 patient with meningitis carcinomatosa). There were 2 patients(28.6%) with previous primary cancer surgery. After diagnosis, 1 patient received chemotherapy, 1 patient received radiotherapy, 5 patients gave up treatment. At the end of follow-up, 1 patient (1 eye) of chemotherapy with BCVA increased by 2 line; 1 patient (2 eyes) of radiotherapy with no change in BCVA; of the 5 patients who gave up treatment, 1 patient died of disease, 1 patient lost follow-up, and 3 patients (4 eyes) had no change in BCVA. Conclusions With atypically clinical manifestations, the optic nerve metastases easily misdiagnosed as optic neuritis, and with poor therapeutic effect. Primary lesions are mostly found in lung cancer. Key words: Optic nerve neoplasms; Neoplasm metastasis; Lung neoplasms; Disease attributes

Effects of APELIN-13 on the expression of IL-6, TNF-α, and IFN-γ in rats with experimental autoimmune neuritis.

The objective of this paper was to study the effects of PYR-ARG-PRO-ARG-LEU-SER-HIS-YSGLY-PRO-MET-PRO-PHE-OH (APELIN-13) on the expression of inflammatory factors interleukin-6 (IL-6), tumor necrosis factor-α (TNF-α), and interferon-γ (IFN-γ) in rats with experimental autoimmune neuritis (EAN). A total of 30 rats were divided into a control group, an EAN group, and an APELIN-13 group. Enzyme-linked immunosorbent assay (ELISA) was used to detect the levels of IL-6, TNF-α, and IFN-γ in rat plasma. Real-time quantitative Polymerase Chain Reaction (PCR) and Western blot were used to detect the protein and mRNA expression of IL-6, TNF-α, and IFN-γ in rat lymph nodes. In the EAN group, the infiltration of various types of inflammatory cells and focal demyelination were observed near the nerve fascicles of sciatic nerves. Compared with the EAN group, the infiltration of inflammatory cells and demyelination in the APELIN-13 group decreased significantly. The levels of plasma IL-6, TNF-α, and IFN-γ in the EAN group were significantly higher than those in the control group (P < 0.05) but significantly lower than those in the APELIN-13 group (P < 0.05). Compared with the control group, the mRNA and protein expression of IL-6, TNF-α, and IFN-γ increased significantly (P < 0.05) in the EAN group but decreased significantly in the APELIN-13 group (P < 0.05). In conclusion, APELIN-13 exerted a protective effect against EAN in rats.

Related research progress of neuromyelitis optica

Neuromyelitis optica (NMO) is an autoimmune inflammatory diseases of the central nervous systems (CNS) mainly affecting the optic nerves and spinal cord. It has the characteristics of high recurrence rate and poor prognosis. NMO related optic neuritis is a common neuro-ophthalmic disease which often results in permanent visual loss or even blindness. Aquaporin 4 (AQP4) antibody is a specific and pathogenic autoantibody in NMO patients. Although AQP4 is expressed in multiple tissues, NMO pathology is remarkably limited to the CNS. Corticosteroids and other immunosuppressive drugs are the standard managements for NMO patients, in order to reduce the relapses and the severity of the acute attack. Multiple avenues of investigation in the laboratory have significantly advanced our understanding of NMO pathophysiology, which is helpful for our understanding of immunologic and nonimmunologic mechanisms. Many offer significant means for NMO therapy by selectively targeting pathways. In the future, moving these agents from the bench to the bedside offers the opportunity to identify safe and effective therapies that limit CNS injury and preserve visual function. Key words: Neuromyelitis optica; Aquaporin 4; Optic neuritis; Review

Recent developments in neuro-ophthalmology

Some recent advances in neuro-ophthalmology including clinical trials, diagnostic testing and newly elucidated disease entitites were summarized in present paper, such as neuromyelitis optica spectrum disorder.The review goes into great detail regarding recent clinical trials in neuro-ophthalmology.These trials have investigated the management of idiopathic intracranial hypertension as well as treatment of Leber hereditary optic neuropathy and optic nerve glioma.The potential gene therapy for Leber hereditary optic neuropathy seems very promising and may have a significant clinical impact within the next few years.This review also includes an extensive discussion about the application of optical coherence tomography (OCT) and OCT-angiography (OCTA) in neuro-ophthalmology.OCT has important clinical relevance not only for the diagnosis and management of optic neuropathies, such as optic neuritis, ischemic optic neuropathy and chiasmal compression, but also for retro-chiasmal injury.In terms of OCTA, recent studies have demonstrated increased vessel density in acute stage of ischemic optic neuropathy (NAION) and optic neuritis, but decreased vessel density in severe papilloedema and late stage of optic atrophy of any cause.These serve as the potential foundation for future clinical applications.Ophthalmologists should pay more attentions to these recent progress in neuro-ophthalmology. Key words: Neuro-ophthalmology; Clinical trials, intracranial hypertension; Leber's optic neuropathy; Optical coherence tomography; Optical coherence tomography angiography; Optic neuritis; Ischemic optic neuropathy

Clinical significance of bedside dynamic visual acuity test

Objective: Dynamic visual acuity (DVA) is defined as the visual acuity when there are relative movements between subjects and visual targets. The purpose of this study was to discuss the correlation between bedside DVA test and other examinations of vestibular function, and to assess the value of DVA test for clinical diagnosis. Methods: Retrospective analysis of 323 cases with peripheral vestibular disorder, and analyzing the correlation between bedside DVA results and caloric test were performed. Results: Out of these 323 cases, 113 cases showed positive results of DVA.Among these 113 cases with positive DVA test, 109 cases were bilateral or unilateral vestibular function loss according to the results of caloric test or VEMP. The disease with the highest positive rate of DVA was bilateral vestibulopathy(BVP), followed by vestibular neuritis (VN) and profound sudden sensorineural hearing loss (pSSNHL). Conclusions: Bedside DVA is effective to determine the cases with BVP and severe unilateral vestibular function loss.

Clinical and imaging characteristics of pediatric neuromyelitis optica spectrum disorders

Objective To analyze the clinical and imaging characteristics of pediatric neuromyelitis optica spectrum disorders(NMOSD) in children. Methods The clinical data, imaging manifestations and follow-up data of 16 NMOSD patients at Department of Pediatric Neurology, Shandong Provincial Hospital Affiliated to Shandong University between July 2013 and September 2017 were respectively analyzed. Results In 16 patients, initial presentations included optica neuritis(ON) in 5 cases, longitudinally extensive transverse myelitis(LETM) in 6 cases, and among them there were 2 cases with acute disseminated encephalomyelitis and 3 cases with both ON and LETM. Eleven cases received aquaporin-4(AQP4) antibody examination and 4 cases were found seropositive.One case out of 7 detected cases was found AQP4 antibody positive in cerebrospinal fluid.Eleven cases received optica magnetic resonance imaging (MRI), and 8 cases were found abnormal signals in optic nerve and optica chiasma.The spinal cord MRI showed 13 cases with LETM manifestations, and abnormal signals were found in vertebral segments(5-13), and among them 1 case had cervical cord, 3 cases were thoracic cord and 9 cases were both of the above.Lesions in the cervical cord in 2 cases were extended upward to the medulla.Fifteen cases received brain MRI and all of them had brain lesions, which were mainly involved in the central and subcortical white matter, thalamus, corpus callosum, brainstem, the junction of spinal cord and medulla, cerebellum, and so on.All patients received treatment for acute attacks with high-dose Methylprednisolone and/or gamma globulin and got obvious relief.Two cases with recurrent ON received treatment of Rituximab and their vision became improved.Fifteen patients were followed up, and 2 cases had limb disorders and 4 cases had visual impairment, other patients had no clinical symptoms. Conclusions Pediatric NMOSD has a diverse clinical pre-sentation at the onset disease.Those who are initial diagnosed acute myelitis, ON and acute disseminated encephalomyelitis should be considered the possibility of NMOSD.Antibody to AQP4 testing can assist the diagnosis.The typical imaging characters of NMOSD children are abnormal signals in the high expression area of AQP4.Intracranial lesions are more common in children.The acute treatment includes the high-dose Methylprednisolone and gamma globulin.Rituximab can be used for the recurrent patients. Key words: Neuromyelitis optica spectrum disorders; Aquaporin-4; Child

Efficacy and safety analysis of VCD and VD regimens for treatment of newly diagnosed patients with multiple myeloma

Objective To compare the clinical efficacy and safety of bortezomib, cyclophosphamide, dexamethasone (VCD) regimen and bortezomib dexamethasone (VD) regimen in the treatment of the patients with newly diagnosed multiple myeloma (NDMM). Methods The clinical data of 73 patients with NDMM in Shanxi Dayi Hospital from January 2013 to January 2016 were retrospectively analyzed. According to the chemotherapy regimen, the patients were divided into VCD group (41 cases) and VD group (32 cases). The efficacy and adverse reactions of the two groups were evaluated. Results The overall response rate of VCD group and VD group was 80.5% (33/41) and 78.1% (25/32) respectively, and the difference was not statistically significant (χ2= 0.061, P= 0.804); and complete remission (CR) rate was 36.6% (15/41) and 15.6% (5/32) respectively, and the difference was statistically significant (χ2= 3.970, P= 0.046); the median progression-free survival (PFS) was 27 months and 24 months respectively, and the median overall survival (OS) was 35 months and 33 months respectively, and the differences were not statistically significant (all P > 0.05). Most adverse reactions occurred in grade 1-2, in which peripheral polyneuropathy and thrombocytopenia were the most common. Peripheral neuritis was the most common finding among the adverse reactions of grade 3. The incidence of adverse reactions in both groups was not statistically significant (P > 0.05). Conclusions VCD and VD regimen could be recommended as a better induction therapy for NDMM patients. Compared with VD scheme, VCD scheme has a higher CR rate. Key words: Multiple myeloma; Therapy; Adverse events

Clinical analysis of 426 emmetropia patients with unilateral rhegmatogenous retinal detachment and retinal lesions in the contralateral eyes

Objective To summarize the clinical features and visual outcome of posterior scleritis presented with symptoms involving affected optic nerve. Methods Retrospective case series study. Twelve eyes of 12 female patients with posterior scleritis were included in this study. The average age was 35.2±14.31 years old. The patients got diagnosed with an average of 24.75±22.91 days. Ocular pain was complained in all patients, and blurred vision in 11 patients. The best corrected visual acuity (BCVA), intraocular pressure (IOP), slit lamp microscope examination, B-scan ultrasound, optical coherence tomography (OCT), fundus photography, fundus fluorescein angiography (FFA) and ocular wall thickness measurement were performed in all patients. Nine eyes received visual field examination. All patients received systemic corticosteroid and steroidal eye drops for 3 months. Clinical features and outcome were retrospectively studied. Results Before treatment, the BCVA was from 0.8. There were 3 eyes with scleral hyperemia, 3 eyes with anterior chamber flares, 12 eyes with papilledema and different degrees of retinal vein dilatation, 3 eyes with star-shaped macular exudates and 2 eyes with macular retinal pigment epithelium detachment. B-scan ultrasound demonstrated that the ocular walls were thickening in all eyes with typical T-sign, and the average thickness was 2.76±0.68 mm. OCT demonstrated optic disc swelling, and the macular retinal detachment in 2 eyes. In the FFA examination, the fluorescein leakage of the disc was enhanced with time. In the Humphrey test, the value of mean deviation (MD) was 12.56±5.73 dB and pattern standard deviation (PSD) was 8.15±4.23 dB in 9 eyes before the treatment. After treatment for 3 months, the symptoms were attenuated and the visual acuity was obviously improved with BCVA>0.1 in all eyes. Scleral hyperemia and anterior chamber flares were only found in 1 eye. The optic disc edema gradually faded away. The ocular wall thickness in the poster part of the eyeball decreased, and the T-sign disappeared in all eyes, the average thickness was 1.53±0.41 mm. Compared with parameters before the treatment, the difference was statistically significant (t=0.003 5, P<0.05). OCT demonstrated the recovery of the macular retinal detachment. There was no abnormal leakage evidenced in FFA in the optic disc and macular. After treatment, the value of MD and PSD was 5.19±4.82 dB and (4.33±3.76) dB, respectively. The difference of MD value between before and after the treatment was significant (t=0.026, P<0.05). Conclusions Posterior scleritis with an initial symptom of optic nerve was tend to affect middle-aged patients, with clinical manifestations of anterior segment signs in some patients and optic disc swelling with retinal vein dilatation in all patients. B ultrasound examination showed typical T sign. Systemic corticosteroid treatment always obtained remission of the ocular inflammatory activity, and could achieve favorable visual outcome. Key words: Scleritis/diagnosis; Scleritis/therapy; Optic neuritis; Disease attributes

Etiological factor distribution in elderly outpatients with vertigo/dizziness

Objective To clarify the distribution of etiological factors in elderly versus non-elderly outpatients with vertigo/dizziness for optimizing the diagnosis and therapy. Methods We retrospectively analyzed data of outpatients with vertigo/dizziness in Shaanxi Provincial People′s Hospital from April 2015 to April 2017 and conducted diagnoses in accordance with the currently wide-accepted diagnostic criteria. Results A total of 3 356 patients with chief complains of vertigo/dizziness were recruited, and their top seven etiological factors were benign paroxysmal positional vertigo(n=1 320, 39.3%), chronic subjective dizziness(n=680, 20.3%), vestibular migraine(n=386, 11.5%), posterior circulation ischemia(n=213, 6.4%), Meniere′s disease(n=138, 4.1%), vestibular neuritis(n=121, 3.6%), and vestibular paroxysmia(n=76, 2.3%). The top four etiological factors for the elderly patients(n=1 255)were benign paroxysmal positional vertigo(n=498, 39.7%), chronic subjective dizziness(n=279, 22.2%), posterior circulation ischemia(n=161, 12.8%), and vestibular migraine(n=73, 5.8%); while the top four etiological factors for non-elderly patients(n=2 101)were benign paroxysmal positional vertigo(n=822, 39.1%), chronic subjective dizziness(n=401, 19.1%), vestibular migraine(n=313, 14.9%), and vestibular neuritis(n=105, 5.0%). The detection rate in elderly patients versus non-elderly patients was significantly higher in chronic subjective dizziness(22.2% vs.19.1%, P=0.032)and in posterior circulation ischemia(12.8% vs.2.5%, P=0.000), and was significantly lower in vestibular neuritis(1.3% vs.5.0%, P= 0.000), in vestibular migraine(5.8% vs.19.4%, P=0.000)and in other causes(1.0% vs.2.7%, P=0.002). Conclusions The ratio of posterior circulation ischemia is markedly higher in elderly outpatients than in non-elderly outpatients, whereas the ratios of vestibular migraine and vestibular neuritis in elderly patients are lower than in non-elderly outpatients. Key words: Vertigo; Dizziness; Etiological factor