Exploration of correlation between multiple subjective and objective evaluation indicators in early stage with recovery time in combination of acupuncture and medication for treating facial neuritis / 重庆医学

Objective To analyze the correlation between the multiple evaluation indicators in the early stage and the cure time(needed time from treatment to cure)of the patients with facial neuritis treated by ac-upuncture combined with medication,and to optimize the subjective and objective indicators enable predicting the cure time of facial neuritis in early stage.Methods All patients were treated by acupuncture and medica-tion combination.The research subjects were 64 patients with facial neuritis from the outpatient of cupuncture and moxibustion department of this hospital.The correlation between the grade of facial nerve paralysis,sur-face electromyography related data,scores of self-made symptom scoring scale,Sunnybrook Facial Grading System(SFGS)score,Facial Disability Index-Physical(FDIP)score,Facial Disability Index-Social(FDIS)score on 7 d of onset with the cure time was analyzed.Results The cure time was positively correlated with the grade of facial nerve paralysis and FDIS score on 7 d of onset(P＜0.01),amd negatively correlated with the scores of self-made symptom scoring scale,FDIP score,SFGS score and the affected side to healthy ratio of CMAP amplitude of buccal temporal branch of facial nerve(P＜0.01 or P＜0.05);the cure time had no sig-nificant correlation with the ratio of affected side and healthy side of CMAP amplitude in zygomatic branch of facial nerve,the ratio of affected side and healthy side of CMAP latent period of temporal branch,buccal branch and zygomatic branch of facial nerve and F wave output rate(P＞0.05).Conclusion In the early stage subjective indicators of the acupuncture combined with medication for treating facial neuritis,grade of facial nerve paralysis,self-made symptom scoring scale,scores of self-made symptom scoring scale,FDIP and FDIS scores and the ratio of affected side to healthy side of CMAP amplitude of the buccal branch,temporal branch of the facial nerve in sEMG in the objective indicators could be used to predict the cure time,better guide the treatment and have more effective and accurate comunication with the patients.

Preliminary analysis of serum metabolomics in patients with optic neuritis based on liquid chromatography-mass spectrometry / 中华检验医学杂志

Objective:To investigate the serum metabolites and their metabolic characteristics of patients with optic neuritis.Methods:Case-control study. From January 2021 to January 2022, 9 serum specimens of diagnosed patients with optic neuritis were collected in Department of Neurology from Beijing Tongren Hospital and 9 healthy subjects as the control. Among them, there were 5 females and 4 males in the optic neuritis group, aged (35.8±12.9) years; there were 5 females and 4 males in the healthy control group, aged (32.6±8.6) years. Liquid chromatography-mass spectrometry was used to detect metabolites in serum of healthy control and patients with optic neuritis. The principal component analysis (PCA) and orthogonal partial least-squares discriminination analysis (OPLS-DA) were used to analyze the differential metabolites . The variable importance projection value of OPLS-DA model and the P value of t-test was applied to find the different metabolites. Results:Thirty-seven metabolites were finally identified from serum samples. Four metabolites with variable important in projection (VIP) values larger than 1 and P values less than 0.05 were teased out, three metabolites, LysoPC (P-16∶0), LysoPC (16∶0), LysoPC (P-18∶0) belonge to phospholipid and one metabolite was L-Threonine, they were all down-regulated. The area under curve were 0.951, 0.889, 0.963 and 0.944, respectively. Conclusion:Based on metabonomic analysis, some metabolites in serum have changed, which can provide basis for biomarkers screening of optic neuritis.

Quantitative analysis of vessel density in the optic disc and macular of patients with idiopathic optic neuritis / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To assess the changes of vessel density in the optic disc and macular of the affected eye and the uninvolved contralateral eye in patients with idiopathic optic neuritis(ON)and to provide clinical guidance for the treatment and follow-up of idiopathic ON.METHODS: A total of 16 patients with first-episode monocular idiopathic ON ≤3 mo diagnosed between December 2019 and December 2021 were included in this cross-sectional study. The eye of patients was divided into 16 eyes in the affected eye group and 16 eyes in the uninvolved contralateral eye group, and 20 healthy age-matched eyes(n=20)served as controls. Optical coherence tomography angiography(OCTA)was performed in all eyes at 4.5 mm×4.5 mm region of the optical disc and 6 mm×6 mm region of the macular, and blood flow indicators were collected and compared. RESULTS: Compared with the control group and the uninvolved contralateral eye group, the density of all vessels and capillary were reduced in the whole area of optic disc, and all subdivisions of the peripapillary region in the ON group(all P<0.05). Compared with the uninvolved contralateral eye group, the density of superficial capillary plexus(SCP)was significantly lower in the whole area of macular and perifovea region, and its all subdivisions of the ON eye, as well as in the superior-hemi and superior subdivision of the parafovea region(all P<0.05). Compared with the control group, the density of SCP in the inferior-hemi, nasal, and inferior perifovea region was significantly reduced in the ON affected eye group(all P<0.05). Compared with the control group, the whole area of macular and its subdivisions in the uninvolved contralateral eye group showed an increase in the density of SCP(P<0.05)and an increase in the density of SCP in the parafovea region(P<0.05), but no significant change in the inferior-hemi and nasal subdivisions; the increase in the density of SCP in the perifovea region was only significant in the superior-hemi and superior subdivisions(P<0.05).CONCLUSION: Patients with ON in the duration of ≤3 mo may showed a decreased vessel density in all peripapillary subdivisions, and a decreased density of SCP in some subdivisions of the perifovea region, accompanied by an increased density of SCP in some subdivisions of the macular region of the contralateral eyes.

Encefalomielite aguda disseminada com provável etiologia de Bartonella henselae / Acute disseminated encephalomyelitis with probable Bartonella henselae etiology

RESUMO A encefalomielite aguda disseminada é uma doença rara, aguda, inflamatória e desmielinizante do sistema nervoso central, presumivelmente associada, em mais de três quartos dos casos, a infecções (virais, bacterianas ou inespecíficas) e imunizações ou sem qualquer antecedente indentificável. Habitualmente, apresenta um curso monofásico com início de sintomas inespecíficos na fase prodrómica, podendo evoluir com alterações neurológicas multifocais e até à perda total da acuidade visual. Descrevemos o caso de um menino de 9 anos com quadro inicial de edema de papila causado por encefalomielite aguda disseminada devido a Bartonella henselae. Apesar da gravidade da doença, o diagnóstico e o tratamento precoce proporcionaram bons desfechos.

ABSTRACT Acute disseminated encephalomyelitis is a rare, acute, inflammatory, and demyelinating disease of the central nervous system. Presumably associated in more than three quarters of cases by infections (viral, bacterial, or nonspecific) and immunizations or without any identifiable antecedent. It usually presents a monophasic course with onset of nonspecific symptoms in the prodromal phase and may evolve with multifocal neurological changes and even visual acuity loss. We describe a case of a 9-year-old boy with an initial picture of papillary edema caused by acute disseminated encephalomyelitis due to Bartonella henselae. Despite the severity of the disease, early diagnosis and treatment provided good outcomes.

Enfermedad asociada a anticuerpos contra la glicoproteína de mielina de oligodendrocitos / Myelin oligodendrocyte glycoprotein antibody disease

Introducción: La enfermedad asociada a anticuerpos contra la glicoproteína de mielina del oligodendrocito (MOGAD, por sus siglas en inglés) es una entidad clínica recientemente identificada. La frecuencia de presentación del MOGAD es desconocida, pero se considera baja con respecto a otras enfermedades inflamatorias desmielinizantes. Materiales y métodos: Revisión narrativa de la literatura. Resultados: Las manifestaciones clínicas de esta condición son heterogéneas e incluyen neuritis óptica, mielitis, desmielinización multifocal del sistema nervioso central y encefalitis cortical. Se han descrito algunos hallazgos radiológicos que aumentan la sospecha diagnóstica, como el realce perineural del nervio óptico, el signo de la H en el cordón espinal y la resolución de lesiones T2 con el tiempo. El diagnóstico se basa en la detección de inmunoglobulinas G específicas contra MOG, en el contexto clínico adecuado. El tratamiento consiste en manejo de los ataques agudos con dosis altas de corticoides y en algunos casos se deberá considerar la inmunosupresión crónica, considerar la inmunosupresión crónica en pacientes con recurrencia o con discapacidad severa residual tras el primer evento. Conclusiones: En esta revisión narrativa se resumen los aspectos clave con respecto a la fisiopatología, las manifestaciones, el diagnóstico y el tratamiento de la MOGAD.

Introduction: The disease associated with antibodies against the myelin oligodendrocyte glycoprotein (MOGAD) is a recently identified clinical entity, with unknown frequency, but is considered low compared to other demyelinating inflammatory diseases. Materials And Methods: Narrative review. Results: The clinical manifestations are heterogeneous, ranging from optic neuritis or myelitis to multi-focal CNS demyelination or cortical encephalitis. There have been described characteristic MRI features that increase the diagnostic suspicion, such as perineural optic nerve enhancement, spinal cord H-sign or T2-lesion resolution over time. The diagnosis is based on the detection of specific G- immunoglobulins against MOG, in the suggestive clinical context. Acute treatment is based on high dose steroids and maintenance treatment is generally reserved for relapsing cases or patients with severe residual disability after the first attack. Conclusions: In this narrative review, fundamental aspects of pathophysiology, clinical and radiological manifestations, diagnosis and treatment of MOGAD are discussed.

Clinical spectrum of myelin oligodendrocyte glycoprotein antibody-associated disease in Brazil: a single-center experience / Espectro clínico de doença associada com anticorpos contra a glicoproteína de oligodendrócito de mielina no Brasil: uma experiencia de centro único

Abstract Background Anti-myelin oligodendrocyte glycoprotein (anti-MOG) antibody-associated disease (MOGAD) is an immune-mediated neurological disorder with a broad spectrum of clinical presentation that is often difficult to distinguish from other demyelinating diseases, such as multiple sclerosis and neuromyelitis optica spectrum disorder. Objective To describe the clinical and paraclinical characteristics of MOGAD in a Brazilian tertiary center. Methods We retrospectively reviewed the records of adult and pediatric patients who tested positive for anti-MOG antibodies and presented with clinical and radiological diseases compatible with MOGAD. Results Forty-one patients (10 children) were included: 56% female, 58% Caucasian, mean age at onset 31 years (range 6-64), with a mean disease duration of 59.6 months (range 1-264 months). The most frequent onset presentation was optic neuritis (68%), acute disseminated encephalomyelitis (ADEM, 12%), and myelitis (10%). A monophasic disease course was observed in 49%. EDSS median was 2.1 at the last visit. Most patients (83%) were under continuous immunosuppressive treatment. Azathioprine was the first-line treatment in 59%. In all ADEM cases, conus, and root involvement was radiologically observed on MRI. Conclusion Brazilian MOGAD patients presented with a similar spectrum of previously reported MOGAD phenotypes. Conus and spinal root involvement seems to be frequently present in MOGAD-ADEM and could serve as radiologic characteristics of this clinical entity.

Resumo Antecedentes A doença associada ao anticorpo da glicoproteína da mielina de oligodendrócitos (anti-MOG; MOGAD) é uma doença neurológica imunomediada com um amplo espectro de apresentações clínicas que muitas vezes é difícil de distinguir de outras doenças desmielinizantes, como a esclerose múltipla e o distúrbio do espectro da neuromielite óptica. Objetivo Descrever as características clínicas e paraclínicas da MOGAD em um centro terciário brasileiro. Métodos Revisamos retrospectivamente os prontuários dos pacientes adultos e pediátricos que testaram positivos para anticorpos anti-MOG e apresentaram um quadro clínico e radiológico compatível com MOGAD. Resultados Quarenta e um pacientes (10 crianças) foram incluídos: 56% do sexo feminino, 58% caucasianos, idade média de início da doença foi 31 anos (intervalo de 6-64), com duração média da doença de 59,6 meses (intervalo de 1-264 meses). A apresentação inicial mais frequente foi neurite óptica (68%), seguida pela encefalomielite disseminada aguda (ADEM, 12%) e mielite (10%). Um curso monofásico da doença foi observado em 49%. EDSS foi de 2,1 na última visita. A maioria dos pacientes (83%) estava sob tratamento imunossupressor contínuo. Azatioprina foi o tratamento de primeira linha em 59%. Em todos os casos de ADEM, o envolvimento do cone medular e das raízes espinhais foi observado radiologicamente na ressonância magnética. Conclusão Os pacientes brasileiros com MOGAD apresentam um espectro clínico e radiológico semelhante aos fenótipos de MOGAD relatados anteriormente. O envolvimento do cone e das raízes espinhais parece estar frequentemente presente no MOGAD-ADEM e poderia servir como característica radiológica nesta entidade.

Clinical features and visual outcomes of pediatric optic neuritis in the Indian population: A prospective study

Purpose: Optic neuritis, defined as inflammation of the optic nerve, is the most common optic neuropathy affecting adults. Various studies in Southeast Asia have shown that the clinical profile of optic neuritis might differ in these regions from that reported in the western literature. Through this study, we evaluate the clinical profile of pediatric optic neuritis (PON) in the Indian population. Methods: This was a hospital?based prospective observational study. Patients with optic neuritis younger than 16 years who attended the neuro?ophthalmology clinic from May 2016 to April 2017 were included in the study. Results: This study included 54 eyes of 38 patients. The mean age of presentation was 10.6 years. Unilateral disease (58%) was found to be more common, and a slight female preponderance (58%) was noted. The most common feature was visual loss (96.3%). Pupillary light reflex abnormality was seen in most patients. Fundus examination revealed disk edema (77.7%) to be the most common feature. Neuroimaging was performed in 34 patients, and multiple sclerosis was diagnosed in four patients. At 3 months follow?up after treatment, 89% of eyes had best correct visual acuity of 6/9 or better (P < 0.001). Conclusion: In our study, we found the clinical profile of PON to be similar to that seen in western studies as well as those done previously in the Indian population, although with a few differences

Myelin oligodendrocyte glycoprotein antibody-associated optic neuritis: an update / Neurite óptica associada com anticorpo contra a glicoproteína oligondendrócita da mielina: uma breve atualização

ABSTRACT Myelin oligodendrocyte glycoprotein-immunoglobulin G (IgG)-associated optic neuritis has been established as a new entity of immune-mediated optic neuropathy. Patients usually present with recurrent optic neuritis, often bilaterally with initially severe vision loss and optic disc edema. However, in contrast to aquaporin 4-IgG-seropositive neuromyelitis optica spectrum disorder, visual recovery tends to be more favorable, with good response to steroid treatment. Another important differential diagnosis of myelin oligodendrocyte glycoprotein-IgG--associated optic neuritis is multiple sclerosis. Close monitoring for signs of relapse and long-term immunosuppression may be considered to maintain optimal visual function. The diagnosis can be made on the basis of the presence of a specific, usually serological, antibody against myelin oligodendrocyte glycoprotein (IgG; cell-based assay), and a demyelinating event (optic neuritis, myelitis, brainstem syndrome, or cortical lesions with seizures). The clinical spectrum of this newly recognized inflammatory demyelinating disease is expanding rapidly. We briefly review the epidemiological characteristics, clinical manifestations, diagnostic considerations, and treatment options of myelin oligodendrocyte glycoprotein-IgG-associated optic neuritis.

RESUMO A neurite óptica associada à glicoproteína de oligodendrócito de mielina-IgG foi estabelecida como uma nova entidade de neuropatia óptica imunomediada. Tipicamente os pacientes apresentam neurite óptica recorrente, muitas vezes bilateral, com perda de visão frequentemente severa e alta prevalência de edema do disco óptico na fase aguda. No entanto, em contraste com neuromyelitis optica spectrum disorder associada com presença de anticorpo contra aquaporina 4, a recuperação visual tende a ser mais favorável e responde bem ao tratamento com corticoide em altas doses. A esclerose múltipla representa outro importante diagnóstico diferencial de glicoproteína de oligodendrócito de mielina-IgG. O diagnóstico pode ser feito com base na presença de um anticorpo específico, geralmente sorológico contra glicoproteína de oligodendrócito de mielina (IgG, ensaio baseado em células), e presença de evento desmielinizante (neurite óptica, mielite, síndrome do tronco cerebral, lesões corticais com convulsões). O espectro clínico desta doença desmielinizante inflamatória recém-reconhecida está se expandindo rapidamente. Faremos uma breve revisão das características epidemiológicas, manifestações clínicas, considerações diagnósticas e opções de tratamento da neurite óptica associada à glicoproteína de oligodendrócito de mielina-IgG.

Intoxicación por metanol por consumo de bebidas alcohólicas adulteradas. Efectos en el sistema nervioso. Reporte de un caso / Methanol Poisoning by Consumption of Adulterated Alcoholic Beverages. Effects on the Nervous System. A Case Report

Resumen La intoxicación por metanol puede ocurrir de forma inadvertida por la ingesta de bebidas alcohólicas adulteradas. Se trata una entidad poco frecuente, sin embargo, se ha reportado un aumento en la incidencia durante la pandemia de COVID-19. La intoxicación con metanol representa una urgencia médica, ya que puede provocar daño severo en el sistema nervioso central y periférico, además de acidosis metabólica, daño renal agudo e incluso la muerte. En este artículo se presenta el caso de un paciente que cursó con intoxicación por metanol de manera inadvertida al consumir bebidas alcohólicas presumiblemente adulteradas. En el encéfalo se demostró necrosis hemorrágica de ambos núcleos putamen, además de cursar con neuritis óptica bilateral y polineuropatía periférica. Fue manejado con pulsos de esteroides intravenosos, con lo cual, mejoró significativamente su función visual, sensitiva y motora. En el presente caso no existieron complicaciones fatales y presentó una buena respuesta al tratamiento, sin embargo, el caso pone de relieve la necesidad de una mejor regulación en la producción y comercialización de bebidas alcohólicas en nuestro país, y, por otro lado, permite hacer a un llamado a los consumidores a tomar más precauciones en el consumo de bebidas alcohólicas de dudosa calidad o procedencia.

Abstract Methanol poisoning can occur unnoticed, by the ingestion of adulterated alcoholic beverages. In general, it is a rare entity, however, an increase in incidence has been reported during the SARS-CoV-2 pandemic. Methanol poisoning represents a medical emergency as it can cause severe damage to the central and peripheral nervous systems, as well as metabolic acidosis, acute kidney injury, and even death. This article presents the case of a patient who inadvertently developed methanol intoxication after consuming presumably adulterated alcoholic beverages. In the brain, hemorrhagic necrosis of both putamen nuclei was demonstrated, in addition to presenting with bilateral optic neuritis and peripheral polyneuropathy. He was managed with intravenous steroid pulses, which significantly improved his visual, sensory, and motor function. In the present case there were no fatal complications and presented a good response to treatment, however, the case highlights the need for better regulation in the production and marketing of alcoholic beverages in our country, and on the other hand, to invite consumers to take more precautions in the consumption of alcoholic beverages of dubious quality or origin.

Clinical characteristics and analysis of 49 misdiagnosed nonarteritic anterior ischemic optic neuropathy patients / 中华眼底病杂志

Objective:To observed and analyze the clinical features of patients with nonarteritic anterior ischemic optic neuropathy (NAION) causes of misdiagnosis.Methods:A retrospective case study. From November 2014 to July 2022, 49 NAION patients with 49 eyes diagnosed in Department of Ophthalmology, The First People’s Hospital of Lanzhou were included in the study. All patients were misdiagnosed with other eye diseases at first diagnosis. All eyes were examined by best corrected visual acuity (BCVA), relative afferent pupil defect (RAPD), orbital magnetic resonance imaging (MRI), visual field, optical coherence tomography (OCT), and graphic visual evoked potential (P-VEP). Fluorescein fundus angiography (FFA) was performed in 32 eyes. Clinical and MRI, visual field, P-VEP、FFA features of the patients were retrospectively analyzed.Results:There were 31 males and 18 females among the 49 patients. All cases were monocular. Age was (59.3±7.8) years. All of them complained of painless visual acuity loss or occlusion sensation in one eye. There were 12 (24.5%, 12/49) and 37 (75.6%, 37/49) cases with disease duration >2 months and ≤2 months, respectively. In 49 eyes, misdiagnosed as optic neuritis, normal tension glaucoma (NTG) or suspected glaucoma, optic disc vasculitis, cataract, diabetic retinopathy, traumatic optic neuropathy and toxic optic neuropathy were 28 (57.1%, 28/49), 11 (22.4%, 11/49), 5 (10.2%, 5/49), 2 (4.1%, 2/49), 1 (2.0%, 1/49), 1 (2.0%, 1/49), 1 (2.0%, 1/49) eyes. 24 (49.0%, 24/49), 16 (32.7%, 16/49) and 9 (18.4%, 9/49) eyes had BCVA<0.1, 0.1-0.5 and> 0.5, respectively. RAPD was positive in 45 eyes (91.8%, 45/49). There were 37 (75.6%, 37/49) and 12 (24.5%, 12/49) eyes with and without optic disc edema, respectively. Bleeding was observed on and around the optic disc in 15 eyes (30.6%, 15/49). MRI examination showed no obvious abnormality in the optic nerve segments of all affected eyes. OCT showed an increase in retinal nerve fiber layer thickness (307.1±62.1) μm in 37 patients with optic disc edema. The visual field examination showed that 24 eyes (49.0%, 24/49) had typical lower visual field defect connected with the physiological blind spot and circumvented the central fixation point, 6 eyes (12.2%, 6/49) had limited visual field defect connected with the physiological blind spot, and 19 eyes (38.8%, 19/49) had diffuse visual field defect. By P-VEP examination, the amplitude of P100 wave decreased moderately to severely in all affected eyes. There were 24 eyes (49.0%, 24/49) with mild peak delay and 11 eyes (22.4%, 11/49) with moderate peak delay. In 32 eyes examined by FFA, the arteries had early peridisk limitation or diffuse delayed filling, and mid-course fluorescein leakage in the corresponding area.Conclusions:The main symptoms of NAION patients are painless visual acuity loss in one eye or occlusion of vision. The main clinical features of NAION patients are visual field defect, retinal nerve fiber layer thickening and visual electrophysiological abnormalities. NAION patients with acute or subacute visual loss accompanied by optic disc edema and/or bleeding are often misdiagnosed as optic neuritis, optic neurovasculitis and other types of optic neuropathy. NAION patients with a disease course of >2 months are easily misdiagnosed as NTG.

Observation of 4 patients with positive anti-glial fibrillary acidic protein antibody / 中华眼底病杂志

Objective:To observe the clinical characteristics of the patients with positive anti-glial fibrillary acidic protein (GFAP) antibody.Methods:A retrospective study. From January 2017 through December 2021, 4 patients with positive anti-GFAP antibodies hospitalized in Departments of Ophthalmology and Neurology of Xijing Hospital, Air Force Medical University were included in this study. There were 3 patients with optic neuritis (ON) and 1 patient with the spinal and cerebral lesions. All patients were female, with an average age of 35 years. Three patients with ON received the examinations of best corrected visual acuity (BCVA), optical coherence tomography, visual evoked potential and magnetic resonance imaging (MRI) for the head and orbital. Another 1 patient with the spinal and cerebral lesions underwent MRI for the head, cervical and thoracic vertebras. All patients were tested for demyelinating ON-related antibodies in the serum, and the patient with the spinal and cerebral lesions for the antibodies in both serum and cerebrospinal fluid. Patients with ON received intravenous infusion of methylprednisolone sodium succinate in the acute stage, while the patients with spinal cord and brain lesions were given glucocorticoid and immunosuppressive therapy.Results:The initial symptoms of the patients with ON were sudden blurred vision in the right eye together with a pain when the eye rotated. BCVA were hand moving/in-front, 0.2 and 0.12, respectively. The serum anti-GFAP antibodies were positive. MRI showed a rough and thickened optic nerve in 1 patient. For patients with BCVA of hand moving/in-front, the BCVA was increased to counting fingers/30 cm on discharge; while the other 2 patients had no changes for BCVA. When followed up on phone 2-3 years after discharge, BCVA of the patients with ON increased to higher than 0.6. No ocular symptoms occurred in the patient with spinal and cerebral lesions and his initial symptoms were numbness, weakness and convulsions of limbs, accompanied by slurred speech. His anti-GFAP antibodies in the serum were negative but positive in the cerebrospinal fluid. MRI showed enhanced cerebellum and spinal dura mater. The initial symptoms were relieved on discharge, and vanished when followed up on phone after discharge.Conclusions:The patients with positive anti-GFAP antibodies are more common in young and middle-aged women. Monocular optic neuritis is more often seen in the form of sudden blurred vision with an eye-rotating pain. Anti-GFAP antibodies in the serum are positive, and a few patient show a rough and thickened optic nerve. They are sensitive to glucocorticoid therapy with a satisfied prognosis.

Neuromyelitis optica spectrum disorders: a review with a focus on children and adolescents / Espectro da neuromielite óptica: uma revisão com ênfase em crianças e adolescentes

Abstract Neuromyelitis optica spectrum disorder (NMOSD) is a rare and severe inflammatory disorder of the central nervous system (CNS). It is strongly associated with anti-aquaporin 4 antibodies (AQP4-IgG), and it mainly affects young women from non-white ethnicities. However, ~ 5 to 10% of all cases have onset during childhood. Children and adolescents share the same clinical, radiologic, and laboratory presentation as adults. Thus, the same NMOSD diagnostic criteria are also applied to pediatric-onset patients, but data on NMOSD in this population is still scarce. In seronegative pediatric patients, there is a high frequency of the antibody against myelin oligodendrocyte glycoprotein (MOG-IgG) indicating another disease group, but the clinical distinction between these two diseases may be challenging. Three drugs (eculizumab, satralizumab, and inebilizumab) have been recently approved for the treatment of adult patients with AQP4-IgG-positive NMOSD. Only satralizumab has recruited adolescents in one of the two pivotal clinical trials. Additional clinical trials in pediatric NMOSD are urgently required to evaluate the safety and efficacy of these drugs in this population.

Resumo O espectro da neuromielite óptica (ENMO) é uma rara e grave doença inflamatória do sistema nervoso central (SNC), fortemente associada ao anticorpo anti-aquaporina 4 (AQP4-IgG) e que afeta preferencialmente mulheres jovens de etnias não-caucasianas. No entanto, aproximadamente de 5 a 10% de todos os casos se iniciam na infância. Crianças e adolescentes compartilham as mesmas características clínicas, radiológicas e laboratoriais dos adultos. Além disso, o mesmo critério diagnóstico de ENMO é aplicado para pacientes com início na infância. No entanto, dados da população pediátrica são escassos. Em pacientes pediátricos soronegativos, existe uma alta frequência de positividade ao anticorpo contra a glicoproteína na mielina do oligodendrócito (MOG-IgG), indicando outra patologia; porém, a distinção clínica entre as duas doenças é desafiadora. Três medicações (eculizumabe, inebilizumabe e satralizumabe) foram recentemente aprovadas para pacientes adultos com AQP4-IgG. Apenas um dos ensaios pivotais do satralizumabe recrutou adolescentes. Novos ensaios clínicos em pacientes pediátricos com ENMO são necessários para avaliar a segurança e eficácia destas drogas nesta população.

A comparative study of detection methods for assessing superior and inferior vestibular nerve damages in patients with vestibular neuritis / 临床耳鼻咽喉头颈外科杂志

Objective:This study aims to compare the examination results of the vestibular evoked myogenic potential（VEMP） and video head impulse testing（vHIT） in patients with vestibular neuritis（VN）, thus exploring the methods to distinguish superior and inferior vestibular nerve damages in VN patients, and their feasibility. Methods:A total of 25 patients with unilateral VN treated in the Otology Department of the First Hospital of Qinhuangdao from May 2018 to July 2021 were recruited. They were respectively tested for ocular VEMP（oVEMP）, cervical VEMP（cVEMP） and vHIT, and the examination results were analyzed. Results:Examination results of oVEMP showed that 96%（24/25） patients had one-ear abnormalities with the amplitude decline or no waveform introduced, and 4%（1/25） patient had no waveform introduced of both ears. The overall abnormal rate examined by oVEMP was 100%（26/26）. Examination results of cVEMP showed that 36%（9/25） patients had one-ear abnormalities with the amplitude decline or no waveform introduced, and 4%（1/25） patients had no waveform introduced of both ears. The overall abnormal rate examined by cVEMP was 40%（10/25）, and 60%（15/25） patients had normal waveforms of both ears. Examination results of vHIT showed that 100%（25/25） patients had semicircular canal gain decline of one side, 92%（23/25） had anterior Semicircular canal decline of one side, and 36%（9/25） had posterior semicircular canal decline of one side. VEMP and vHIT results were compared. Examination results of VEMP showed that 60%（15/25） VN patients had superior vestibular nerve damage, and 40%（10/25） had both superior and inferior vestibular nerve damages. Examination results of vHIT showed that 64%（16/25） VN patients had superior vestibular nerve damage, and 36%（9/25） had both superior and inferior vestibular nerve damages. There was no significant difference in the ratio of VN patients with superior and inferior vestibular nerve damages examined by VEMP or vHIT（χ²=0.085, P>0.05）. The matching ratio of VEMP and vHIT results was 80%（20/25）, and the non-matching ratio was 20%（5/25）. Conclusion:Consistent results obtained from both VEMP and vHIT can preliminarily identify the type of vestibular nerve damage. If their results are not consistent, it is recommended not to identify the scope of the vestibular nerve damage.

Neuromielitis óptica y lupus eritematoso sistémico: caso clínico

La neuromielitis óptica (NMO) es una enfermedad desmielinizante del sistema Nervioso Central, con morbilidad, mortalidad alta, con respuesta favorable al tratamiento inmunosupresor y asociación infrecuente a enfermedades inmunológicas como Lupus Eritematoso Sistémico. Se reporta caso de un adolescente con diagnóstico de Neuromielitis óptica que presento durante su evolución Lupus Eritematoso Sistémico. Su evolución fue adecuada por la respuesta favorable a terapia inmunosupresora.

Optic neuromyelitis (NMO) is a demyelinating disease of the Central Nervous System, with morbidity, high mortality, favorable response to immunosuppressive treatment and infrequent association to immunological diseases such as Systemic Lupus Erythematosus. We report the case of a teenager with a diagnosis of Optic Neuromyelitis who presented Systemic Lupus Erythematosus during his evolution. Its evolution was adequate due to the favorable response to immunosuppressive therapy.

Effectiveness of neurolysis as a treatment for complications of leprosy neuritis: a systematic review

ABSTRACT Background: Complications of leprosy neuritis are considered serious and apparent, with the potential to disable and/or limit individuals. These complications affect not only a patient's physical functioning, but also their family and social lives, while directly impacting the ability to work and/or maintain financial independence, subsequently interfering with their overall quality of life. The present review, therefore, aimed to analyze the effectiveness of neurolysis as an alternative treatment for the complications associated with leprosy neuritis. Methods: The present review was performed based on the Joanna Briggs Institute methodology, in an effort to answer the following research question: what is the effectiveness of neurolysis as a treatment for leprosy neuritis complications? This research question was defined using the patient-intervention-outcome (PIO) framework, where leprosy represents 'P', neurolysis for 'I', and neuropathic pain/motor function/sensorial function/physical disability/quality of life for 'O'. Randomized and non-randomized clinical trials and prospective observational cohort studies were included in the present review, with no time or date restrictions. Results: The present review included 1 randomized clinical trial and 10 prospective studies, published between 1976 and 2020. All of the outcomes showed improvement, with relief from neuropathic pain being the primary finding. Conclusions: The evidence obtained in the present review suggested that neurolysis is an effective alternative for the treatment of physical disabilities, the recovery of sensory and motor function, the restoration of quality of life, and neuropathic pain relief.

Application of vestibular function examination in the analysis of damaged site in patients with acute vestibular neuritis / 临床耳鼻咽喉头颈外科杂志

Objective:To analyze the site of vestibular nerve damaged in patients with acute vestibular neuritis. Methods:Fifty-seven patients with acute vestibular neuritis were recruited, and each patient underwent caloric irrigation test, video head impulse test（vHIT） and vestibular evoked myogenic potentials（VEMPs）. The results were further analyzed. Results:Analysis of abnormal rates of different vestibular function tests: the abnormal rate of caloric irrigation test, horizontal semicircular canal vHIT, anterior semicircular canal vHIT, and posterior semicircular canal vHIT were 92.98%, 92.98%, 92.98%, and 52.63%, respectively. The abnormal rate of cervical vestibular evoked myogenic potentials（cVEMP） and ocular vestibular evoked myogenic potentials（oVEMP） were 52.63% and 89.47%. The abnormal rate of caloric irrigation test, horizontal semicircular canal vHIT, anterior semicircular canal vHIT, and oVEMP were significantly higher than posterior semicircular canal vHIT and cVEMP（P<0.01）. Combination analysis of different vestibular function tests: there are twenty-six patients（45.61%, superior and inferior vestibular nerve） with abnormal caloric irrigation test, video head impulse test, and VEMPs. There are twenty-five patients（43.86%, superior vestibular nerve） with abnormal caloric irrigation test, horizontal semicircular canal vHIT, anterior semicircular canal vHIT, and oVEMP. There are 4 patients（7.02%, inferior vestibular nerve） with abnormal posterior semicircular canal vHIT and cVEMP. There are two patients（3.51%, ampullary vestibular nerve） with abnormal caloric irrigation test, horizontal semicircular canal vHIT, and anterior semicircular canal vHIT. The rate of superior and inferior vestibular neuritis and superior vestibular neuritis were significantly higher than inferior vestibular neuritis and ampullary vestibular neuritis（P<0.01）. Conclusion:Acute vestibular neuritis subtypes can be divided into four categories: superior and inferior vestibular neuritis, superior vestibular neuritis, inferior vestibular neuritis, and ampullary vestibular neuritis. Video head impulse test can accurately assess the site of vestibular nerve damage in patients with acute vestibular neuritis. In addition, vHIT combined with VEMPs can provide objective evidence for the diagnosis of ampullary vestibular neuritis.

Dental erosion caused by glucocorticoid therapy in a patient with optic neuritis: a case report / 华西口腔医学杂志

Dental erosion is characterized by progressively destroyed teeth, which has no relation to bacteria but to chemicals. Some internal factors, such as gastroesophageal reflux induced by bulimia, anorexia, gastrointestinal diseases, or drugs, and external factors, such as diet, drugs, and occupational acid exposure, are considered promotive factors for this disease. This article presents a patient suffering from severe dental erosion in the whole dentition, especially in the maxillary teeth, due to gastroesophageal reflux induced by glucocorticoid therapy for optic neuritis. This article discusses the mechanism between optic neuritis glucocorticoid therapy and dental erosion.

Optic neuritis in Guillain-Barré syndrome: a case report and literature review / 中华神经科杂志

Objective:To summarize the clinical characteristics of patients with Guillain-Barré syndrome (GBS) complicated with optic neuritis (ON).Methods:The clinical data of a patient with GBS complicated with ON, who admitted to the Department of Neurology, the First Hospital of Shanxi Medical University in December 2021, were collected, including demographic characteristics, clinical symptoms and signs, laboratory and electrophysiological data, and results of fundus color films. The patients with GBS complicated with ON reported in the literature were also reviewed.Results:A 40-year-old female patient with GBS was diagnosed by the results of electromyography and cerebrospinal fluid tests combining with the history and signs, who was treated with intravenous immunoglobulin on the 3rd day after onset. On the 8th day, her muscle strength improved significantly. However, on the 12th day, the visual field darkened, and on the 19th day, the vision decreased significantly (oculus dexter visual acuity 0.2, oculus sinister visual acuity 0.1 +1) with bilateral papilloedema, a relative afferent pupillary defect and delayed P100 response of the visual evoked potential. Obvious abnormality was not noted in optic nerve magnetic resonance imaging. Thus ON was diagnosed and treated with pulse methylprednisolone therapy. After 8 days of treatment, the visual acuity was completely recovered and there was no abnormality in the ocular fundus. A total of 28 cases of GBS complicated with ON (including the present patient) were reported in the literature. The age of onset was mostly 20-60 years, and there was no gender preference. Mycoplasma pneumoniae was the most common premorbid pathogen and was identified in 7 of the 10 cases in which the causative agent was described. ON usually involved both sides, and 21 of 28 patients had bilateral optic nerves involved. GBS preceded ON or both occurred simultaneously in the majority of patients; GBS preceded ON in 14 of 28 patients, and both occurred simultaneously in 10 of 28 patients. All patients responded well to immunotherapy, and vision was completely recovered in 20 patients. Conclusions:GBS complicated with ON is rare. Attention should be paid to the loss of vision in patients with GBS. Relevant examinations should be completed as soon as possible and immunotherapy should be given.

Screening and diagnostic system construction for optic neuritis and non-arteritic anterior ischemic optic neuropathy based on color fundus images using deep learning / 中华眼底病杂志

Objective:To construct and evaluate a screening and diagnostic system based on color fundus images and artificial intelligence (AI)-assisted screening for optic neuritis (ON) and non-arteritic anterior ischemic optic neuropathy (NAION).Methods:A diagnostic test study. From 2016 to 2020, 178 cases 267 eyes of NAION patients (NAION group) and 204 cases 346 eyes of ON patients (ON group) were examined and diagnosed in Zhongshan Ophthalmic Center of Sun Yat-sen University; 513 healthy individuals of 1 160 eyes (the normal control group) with normal fundus by visual acuity, intraocular pressure and optical coherence tomography examination were collected from 2018 to 2020. All 2 909 color fundus images were as the data set of the screening and diagnosis system, including 730, 805, and 1 374 images for the NAION group, ON group, and normal control group, respectively. The correctly labeled color fundus images were used as input data, and the EfficientNet-B0 algorithm was selected for model training and validation. Finally, three systems for screening abnormal optic discs, ON, and NAION were constructed. The subject operating characteristic (ROC) curve, area under the ROC (AUC), accuracy, sensitivity, specificity, and heat map were used as indicators of diagnostic efficacy.Results:In the test data set, the AUC for diagnosing the presence of an abnormal optic disc, the presence of ON, and the presence of NAION were 0.967 [95% confidence interval ( CI) 0.947-0.980], 0.964 (95% CI 0.938-0.979), and 0.979 (95% CI 0.958-0.989), respectively. The activation area of the systems were mainly located in the optic disc area in the decision-making process. Conclusion:Abnormal optic disc, ON and NAION, and screening diagnostic systems based on color fundus images have shown accurate and efficient diagnostic performance.

Changes of corneal nerve fiber and the correlation analysis with visual acuity in demyelinating optical neuritis patients / 中华眼底病杂志

Objective:To investigate the changes in the nerve fiber layer of the cornea in patients with demyelinating optic neuritis (DON) and its correlation with visual acuity.Methods:A cross-sectional study. From March 2021 to July 2022, 27 cases (39 eyes) of DON patients diagnosed in the Department of Neurology and Ophthalmology of Beijing Tongren Hospital Affiliated to Capital Medical University were enrolled in this study. According to the serological test results, the patients were divided into aquaporin 4 antibody associated optic neuritis (AQP4-ON group) and myelin oligodendrocyte glycoprotein antibody associated optic neuritis (MOG-ON group), with 15 cases (19 eyes) and 12 cases (20 eyes) respectively. According to previous history of glucocorticoid treatment, the patients were divided into glucocorticoid treated group and non-glucocorticoid treated group, with 17 cases (27 eyes) and 10 cases (12 eyes) respectively. Twenty healthy volunteers (20 eyes) with age- and gender-matched were selected as the control group. All eyes underwent best corrected visual acuity (BCVA) and in vivo confocal microscopy (IVCM) examinations. BCVA was performed using Snellen's standard logarithmic visual acuity chart, which was converted into logarithmic minimum angle resolution (logMAR) visual acuity during statistics. The corneal nerve fiber length (CNFL), corneal nerve fiber density (CNFD), corneal nerve fiber branch length (CNBL), corneal nerve fiber branch density (CNBD) and the density of corneal dendritic cells (DC) were detected by IVCM examination. Parameter comparison between groups by t-test and Kruskal-Wallis rank sum test. The correlation between logMAR BCVA and pamameters of corneal nerve fibers were analyzed using Spearman analysis. Results:The CNFL, CNFD, and CNBL of the DON group and the control group were (10.67±2.55) mm/mm 2, (57.78±12.35) root/mm 2, (3.27±1.34) mm/mm 2, and (13.74±3.05) mm/mm 2, (70.95±13.14) root/mm 2, and (4.22±1.03) mm/mm 2, respectively; the difference in CNFL, CNFD, and CNBL between the two groups were statistically significant ( t=4.089, 3.795, 2.773; P<0.05). The CNFL, CNBL, and CNBD of the affected eyes in the MOG-ON group and AQP4-ON group were (12.02±2.13) mm/mm 2, (3.80±1.19) mm/mm 2, (47.97±8.86) fibers/mm 2, and (9.25±2.19) mm/mm 2, (2.72±1.19) mm/mm 2, (39.43±13.86) fibers/mm 2, respectively; the differences in CNFL, CNBL, and CNBD between the two groups were statistically significant ( t=-4.002, -2.706, -2.306; P<0.05). The corneal DC density of the patients in the hormone treated group and the non-hormone treated group was (24.43±8.32) and (41.22±9.86) cells/mm 2, respectively. The difference in corneal DC density between the two subgroups was statistically significant ( P<0.001). Correlation analysis showed that there was a significant negative correlation between logMAR BCVA and CNBL and CNFL in patients with DON ( r=-0.422, -0.456; P<0.05). Conclusions:There are different degrees of corneal nerve fiber damage in patients with different types of DON. There was a negative correlation between BCVA and the length of corneal nerve fibers.