Auxiliary Examination Findings Research Articles

Clinical, radiological, therapeutic and prognostic differences between MOG-seropositive and MOG-seronegative pediatric acute disseminated encephalomyelitis patients: a retrospective cohort study.

This study aimed to compare the clinical, radiological, therapeutic, and prognostic differences between pediatric patients showing acute disseminated encephalomyelitis (ADEM) with and without myelin oligodendrocyte glycoprotein (MOG) antibodies. We retrospectively collected all available data of children diagnosed with ADEM and tested for serum MOG antibodies at the Children's Hospital of Chongqing Medical University from January 2017 to May 2021. A total of 62 patients were included in our cohort, of which 35 were MOG-seropositive and 27 were MOG-seronegative. MOG-seropositive ADEM children presented with significantly lower rates of seizures (P = 0.038) and cranial nerve (III-XII) palsy (P = 0.003). Isolated leukocytosis in the blood was more common in ADEM children with MOG antibodies (P < 0.001). The two groups showed no significant differences in the distributions and extent of the MRI lesions as well as the appearance of typical/atypical magnetic resonance imaging (MRI) features. MOG-seropositive children were more likely to relapse (P = 0.017) despite having slower oral prednisolone tapering after acute treatments (P = 0.028). In scoring performed on the basis of two neurological function scoring systems, MOG-seropositive children showed milder neurological disability status at onset (P = 0.017 and 0.025, respectively) but showed no difference during follow-up. In summary, the differences in the clinical manifestations and auxiliary examination findings for MOG-seropositive and MOG-seronegative ADEM children lacked significance and specificity, making early identification difficult. MOG-seropositive children were more likely to relapse and showed slower steroid tapering. Moreover, MOG-seronegative children tended to have more severe neurological impairments at onset with no difference during follow-up.

Electroencephalogram Features of Anti-N-Methyl-D-Aspartate Receptor Encephalitis and Their Value for Clinical Assessment

To analyze the electroencephalogram (EEG) features of anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDARE) and to study the clinical assessment value of the degree of EEG background slowing and the presence of δ brush. We enrolled 52 patients with anti-NMDARE and collected their clinical data, including age, sex, form of disease onset, status of tumor comorbidity, auxiliary examination findings (cerebrospinal fluid [CSF] anti-methyl-D-aspartate receptor antibody titers, magnetic resonance imaging [MRI] reports, and EEG results), treatment status, and follow-up after discharge. The degree of EEG background abnormality and the presence of δ brush in the EEG of patients with different clinical features were analyzed. Among the 52 patients, 7 (14%) had normal EEG, and 45 (87%), abnormal EEG, including 25 (48%) with mild abnormalities, 11 (21%) with moderate abnormalities, and 9 (17%) with severe abnormalities. δ brush was seen in 6 (12%) patients. At the time of EEG, 32 (62%) patients were in the mild condition group and 20 (38%) patients were in the severe condition group. After 1 year of follow-up, there were 45 (86%) patients in the good prognosis group and 7 (14%) patients in the poor prognosis group. The exacerbation of EEG background abnormalities and the presence of δ brush were indications for an increase in the proportion of patients who were in severe condition, who needed ICU admission, and who had poor prognosis ( P<0.01). The worse the EEG background abnormalities, the higher the proportion of CSF antibody titers>1∶10 ( P=0.035), and the higher the proportion of patients initiating second-line immunotherapy ( P=0.008). The δ brush was seen a higher proportion in patients with comorbid tumors ( P=0.012). The probability of δ brush presence was higher in the first-time diagnosis cases than that in recurrent cases ( P=0.023). The degree of EEG slowing and the presence of δ brush have shown consistent performance in assessing patients' condition and predicting prognosis. The slower the EEG, the more severe the disease, and the worse the prognosis. The presence of δ brush indicates severe disease and poor prognosis. EEG slowing is correlated with the immune status of patients with anti-NMDARE. The slower the EEG, the more severe the immune abnormalities. In clinical practice, patient EEG should be under dynamic monitoring in order to evaluate the effect of immunotherapy. If EEG slowing is not improved, enhanced immunotherapy should be considered as early as possible. The δ brush is seen at a higher proportion in patients with comorbid tumors. Therefore, active efforts should be made to screen for tumors when δ brush is present.

Analysis of clinical characteristics and prognostic factors in 110 patients with nitrous oxide abuse.

PurposeTo review the clinical symptoms, auxiliary examination findings, and outcomes of patients with nitrous oxide (N2O) abuse, and analyze the factors that affect outcomes.MethodsPatients with N2O abuse treated in the Department of Neurology between January 2018 and December 2020 were included. The clinical data of these patients were collected, and follow‐up was conducted to determine the outcomes.ResultsThe average age of the 110 patients with N2O abuse was 21.4 ± 4.2 years (range: 14–33 years). Clinical presentation primarily included neurological symptoms, such as limb numbness and/or weakness (97%), psychiatric symptoms, changes in appetite, and skin hyperpigmentation. Laboratory test results were characterized by vitamin B12 deficiency (60%, 34 out of 57 cases) and high homocysteine level (69%, 31 out of 45 cases). Electromyography indicated mixed axonal and demyelination injury (92%, 80 out of 87 cases). Motor and sensory nerves were simultaneously involved, and injury primarily involved the lower limbs. One hundred and seven (97%) patients were clinically diagnosed with peripheral neuropathy, of whom 26 (24%) exhibited spinal abnormalities on magnetic resonance imaging, supporting a diagnosis of subacute combined degeneration. Treatment included N2O withdrawal and vitamin B12 supplementation. Reexamination of six patients indicated that treatment was effective. Follow‐up was completed for 51 patients. Thirty‐four patients (67%) recovered completely, 17 patients (33%) had residual limb numbness, and only one patient experienced relapse. Sex was an independent prognostic factor; the outcomes of female patients were better than that of male patients.ConclusionThe recreational use of N2O has largely expanded among youth in recent decades, which has become a growing public health concern in China. It highlights the importance of the recognition of various clinical symptoms, particularly limb numbness and/or weakness related to the cases of N2O abuse. The therapeutic administration of vitamin B12 supplementation and N2O withdrawal can make the overall prognosis good, especially for female patients.

Clinical manifestations and genetic analysis of 4 children with chronic granulomatous disease.

Pediatricians are unfamiliar with chronic granulomatous disease (CGD) because of its rarity and paucity of available data, potentially leading to misdiagnosis, late treatments, and mortality. The main purpose of this study was to summarize the clinical manifestations and auxiliary examination findings of four children with CGD confirmed by genetic testing.This was a case series study of children hospitalized at the Pediatric Respiratory Department of Shandong Provincial Hospital. The clinical, laboratory, treatment, and prognosis data were analyzed.All 4 children were boys. Two were brothers. The children's age was from 34 days to 3 years and 2 months at disease onset. The manifestations were repeated pulmonary infection, lymphadenitis, skin infection, and granuloma formation. Pulmonary infections were common. Abnormal responses were common after BCG vaccination. Thoracic computed tomography (CT) mainly showed nodules and masses, while the consolidation area in CT images reduced slowly. No abnormalities in cellular immune functions and immunoglobulin were found. The disease in all four children was confirmed by genetic testing. Long-term antibiotics and anti-fungal drugs were needed to prevent bacterial and fungal infections.CGD should be considered in children with repeated severe bacterial and fungal infections. Abnormal responses after BCG vaccination and nodular or mass-shaped consolidation in thoracic CT images should hint toward CGD. Gene sequencing could provide molecular evidence for diagnosis. The treatments of CGD include the prevention and treatment of infections and complications. Immunologic reconstitution treatment is currently the only curative treatment for CGD.

Comparison of Clinical Features and Visual Outcome between Sympathetic Ophthalmia and Vogt–Koyanagi–Harada Disease in Chinese Patients

To characterize the clinical features of sympathetic ophthalmia (SO) and compare SO and Vogt-Koyanagi-Harada (VKH) disease in Chinese patients. Retrospective case series. A total of 131 consecutive SO and 500 VKH disease patients randomly selected from among those referred to our uveitis center from April 2008 through June2018. History, extraocular and ocular findings, best-corrected visual acuity (BCVA), auxiliary examination findings, complications, and therapeutic effects were analyzed retrospectively in SO and VKH disease patients. Visual outcome, extraocular and ocular findings, and therapeutic effects. Sympathetic ophthalmia manifested as posterior uveitis (68.8%) within 2 weeks and equal involvement of anterior and posterior segment (44.4%), respectively, was observed between 2 weeks and 2 months after disease onset. Two months after disease onset, SO patients showed sunset glow fundus (51.2%) and granulomatous anterior uveitis (27.3%). Vogt-Koyanagi-Harada disease patients mainly showed posterior uveitis (100%), anterior segment involvement (92.4%) associated with posterior uveitis (84.9%), and granulomatous anterior uveitis (97.4%) accompanying sunset glow fundus (91.5%) in the 3 periods mentioned above. The frequencies of extraocular manifestations were lower in SO patients (24.4%) as compared with VKH disease patients (84.8%; P < 0.001). Best-corrected visual acuity of SO patients improved from 0.68±0.86 logarithm of the minimum angle of resolution (logMAR) to 0.47±0.78 logMAR (P= 0.01), and BCVA of VKH disease patients improved from 0.67±0.79 logMAR to 0.24±0.53 logMAR (P < 0.001) at 12 months of follow-up. A worse BCVA was noted in SO patients compared with VKH disease patients after treatment (P= 0.003). Kaplan-Meier survival analysis showed that the risk of loss of useful vision in SO patients was significantly higher than that of VKH disease patients (P < 0.001). Chinese SO and VKH disease patients have a different evolutionary process. The frequency of extraocular manifestations in SO patients is much lower as compared with VKH disease patients. Visual outcome is worse in SO as compared with VKH disease.

Evaluation of visceral involvement and detection of autoantibodies in patients with systemic sclerosis

Objective To investigate clinical characteristics of systemic sclerosis (SSc) , and to explore the correlation between visceral involvement and autoantibodies. Methods Clinical data, laboratory examination and auxiliary examination findings were collected from 63 patients with SSc in the Department of Dermatology of Zhongshan Hospital affiliated to Fudan University between 2012 and 2015, and the correlation of autoantibodies with visceral involvement and clinical phenotypes was analyzed. Results The 63 patients with SSc included 29 with diffuse SSc, 29 with limited SSc and 5 with overlap syndrome. Of the 63 patients, 30 had pulmonary interstitial fibrosis, 32 had lung function impairment which manifested as reduced diffusing capacity for carbon monoxide, and 12 had increased pulmonary arterial pressure. Among 29 patients with anti- DNA topoisomerase Ⅰ (SCL-70) antibody, 19 (65.52%) had pulmonary interstitial fibrosis, while 11 (32.35%) of 34 patients without anti-SCL-70 antibody had pulmonary interstitial fibrosis. Pulmonary interstitial fibrosis also occurred in 13 (68.42%) of 19 patients with anti-Sjogren′s syndrome antigen A (SSA) antibody, as well as in 17 (38.64%) of 44 patients without anti-SSA antibody. The incidence of pulmonary interstitial fibrosis was significantly higher in the patients with anti-SCL-70 or anti-SSA antibody than in the patients without anti-SCL-70 or anti-SSA antibody respectively (χ2= 6.901, 4.720, respectively, both P < 0.05) . Among the 63 patients, 22 had fingertip ulcers, and 16 (72.73%) of the 22 patients had pulmonary interstitial fibrosis, while 14 (34.15%) of 41 patients without fingertip ulcers had pulmonary interstitial fibrosis, and the incidence of pulmonary interstitial fibrosis significantly differed between the two groups (χ2= 8.544, P < 0.01) . Of the 63 patients, 50 (79.40%) initially presented with Raynaud′s phenomenon, and the average duration between the occurrence of Raynaud′s phenomenon and the diagnosis of SSc was 48.3 months. Conclusions Patients with SSc usually present with a high incidence of pulmonary interstitial fibrosis, which is complicated by lung function impairment and pulmonary hypertension. Raynaud′s phenomenon usually occurs as the initial symptom. There is a high incidence of pulmonary interstitial fibrosis in the patients with fingertip ulcers and anti-SCL-70 and anti-SSA antibodies. Key words: Scleroderma, systemic; Lung diseases, Interstitial; Autoantibodies; Hypertension, pulmonary

Clinical analysis of eight cases of ulcerative colitis complicated by pyoderma gangrenosum

Objective To investigate clinical manifestations, histopathological features and treatment of ulcerative colitis (UC) complicated by pyoderma gangrenosum (PG) . Methods Data on clinical manifestations, auxiliary examination findings, treatment and prognosis were collected from 8 inpatients with UC complicated by PG in Peking Union Medical College Hospital between July 2009 and July 2016, and analyzed retrospectively. Results Of the 8 cases, 5 were male, and 3 were female. The average age of onset was 30.6 years and 35.1 years in males and females respectively. All the patients developed intestinal symptoms of UC before the onset of PG with an average time interval of 5 years. Moreover, all the patients had active total colonic type UC at the onset of PG, including 6 with moderately active UC and 2 with mildly active UC. PG manifested as painful ulcers in 8 patients, and affected lower limbs in 7 patients. Histopathological examination of skin lesions showed typical characteristics of vasculitis in 5 patients. Five patients were complicated by arthralgia. All the patients were treated with glucocorticoids and 5-aminosalicylic acid agents as the basic therapy, 3 patients received extra treatment with immuno-suppressive agents or minocycline, and 2 with infliximab. Conclusions PG is a severe skin manifestation of UC, commonly occurs in patients with total colonic type UC in the active stage, and mostly affects the lower limbs, with typical histopathological features of vasculitis. Key words: Pyoderma gangrenosum; Colitis, ulcerative; Disease attributes; Treatment outcome; Inflammatory bowel disease

Clinical Features and Complications of Scleritis in Chinese Patients

ABSTRACTPurpose: To characterize the clinical features of scleritis in Chinese patients.Methods: The history, demographics, ocular findings, auxiliary examination findings, complications, systemic diseases and therapeutic effects were analyzed retrospectively.Results: The study included 124 male and 169 female patients with scleritis. Anterior and posterior scleritis were, respectively, found in 243 and 42 patients. The other eight patients had both anterior and posterior involvement. The mean age of scleritis onset was 39.4 years. Systemic diseases associated with scleritis mainly included rheumatoid arthritis (RA) (4.4%), ankylosing spondylitis (AS) (3.4%), and tuberculosis (1.7%). Anterior uveitis (23.7%), complicated cataract (16.7%), and intraocular hypertension (12.6%) were common complications of scleritis. Scleritis was controlled in 94.6% of the patients treated with corticosteroids combined with immunosuppressive agents.Conclusions: Diffuse anterior scleritis was the most common scleritis entity in China. RA and AS were relatively common diseases associated with scleritis. Corticosteroids combined with immunosuppressive agents effectively controlled the disease.

Uveitis in Chinese Patients with Psoriasis

ABSTRACTPurpose: To investigate the clinical features of 51 uveitis patients with psoriasis in China.Methods: The psoriasis type, demographics, ocular findings, auxiliary examination findings, complications, and therapeutic effects were analyzed.Results: A total of 37 male and 14 female uveitis patients with psoriasis were classified into four groups: psoriasis vulgaris (29 cases); psoriatic arthritis (15 cases); psoriatic erythroderma (6 cases); and pustular psoriasis (1 case). The onset age of psoriasis was younger than for uveitis (p < 0.001). Anterior uveitis, panuveitis, and posterior uveitis was observed in 58.8%, 35.3%, and 5.9% of the patients, respectively. Hypopyon was more frequently noted in the group with psoriatic arthritis (p = 0.007). Optic disc staining was more frequently noted in the group with psoriatic erythroderma (p = 0.029). Significant visual improvement was observed in 17 patients.Conclusions: Uveitis can be associated with various types of psoriasis in China, but was most frequently observed in patients with psoriasis vulgaris and psoriatic arthritis.

The analysis on 12 cases of acute disseminated encephalomyelitis

Objective To study the clinical features and treatment of acute disseminated encephalomyelitis (ADEM). Methods All patients admitted with ADEM during May 1990 to Dec 2010 were included in the study. Clinical data of 12 cases with ADEM were reviewed and analysed. The diagnosis of ADEM was made based on the clinical presentation, suggestive MRI and auxiliary examination findings. All patients were treated with intravenous steroids or immunoglobulins (IVIg). Results The sample consisted of 10 men and 2 women. The oldest patient was 69 years old and the youngest was 6 years old. Six patients had definite upper respiratory tract infection preceded the onset of neurological symptoms, 3 patients had non⁃specific fever, 1 patient had measles vaccination, 1 patient had measles prior to the onset 4 months ago. No preceding illness and vaccination occurred in 1 patient. The common presenting symptoms were fever, nausea, vomiting, headache. Neurological manifestations included cranial nerve involvement (the abducent nerve was the most common cranial nerve involved), paralysis (include hemiplegia, quadriplegia, paraplegia), altered sensorium, bladder involvement (both incontinence and retention), meningeal irrigation sign and conscious disturbance. Conclusion Despite the serious manifestation, ADEM in patient has good immediate outcome. Early diagnosis and treatment should be emphasized. DOI：10.3969/j.issn.1672⁃6731.2012.02.015

Vogt-Koyanagi-Harada disease presenting as acute angle closure glaucoma at onset

To investigate the clinical features of Vogt-Koyanagi-Harada (VKH) disease presenting as acute angle closure glaucoma at onset. Retrospective non-comparative case series. Four hundred and eighty-six VKH patients seen from February 2001 to March 2010. The history and clinical findings of all patients were reviewed. Auxiliary examinations, including ultrasound biomicroscopy, fundus fluorescein angiography and optical coherence tomography, were performed in certain cases. Corticosteroids with or without cyclosporine A were used to treat these patients. Patients' demographics, clinical presentation and auxiliary examination findings. Eight out of 486 VKH patients were misdiagnosed as acute angle closure glaucoma. The mean age of these eight patients was 55.6 years. Six patients were female. The mean intraocular pressure (IOP) at disease onset was 32.9 mmHg. All of these patients had a shallow anterior chamber and a narrow or closed angle at their first visit. The complaints of these patients were mostly headache and sudden decreased vision in both eyes. Alterations shown on ultrasound biomicroscopy included detachment of the ciliary body and peripheral choroid. The increased IOP did not respond to anti-glaucoma therapy, but resolved following treatment with corticosteroids. The eye of one patient was enucleated after failed trabeculectomies prior to referral to our uveitis centre. VKH disease presenting with a bilateral increased IOP mostly occurs in older women. The strikingly decreased visual acuity associated with mild to moderate increased IOP is a clue to the diagnosis. The increased IOP responded well to corticosteroids but not to anti-glaucoma treatment.

Clinical Features of Chinese Patients with Behçet’s Disease

To characterize the clinical features of Behçet's disease in Chinese patients. Retrospective noncomparative case series. Seven hundred seventy-five eyes of 437 patients with Behçet's disease initially examined from August, 1995, through June, 2006. The history, demographic parameters, and clinical findings of all consecutive Behçet's patients referred to the uveitis study center of Sun Yat-sen University were reviewed. Laser flare-cell photometry (LFCM), fundus fluorescein angiography (FFA), optical coherence tomography (OCT), ultrasound biomicroscopy (UBM), and B-scan ultrasonography were performed in certain cases. Most patients were treated by immunosuppressive agents combined with oral corticosteroids. Patient demographics, clinical presentation, and auxiliary examination findings. Four hundred thirty-seven patients were diagnosed with Behçet's disease. There were 319 male and 118 female patients. Panuveitis was the most common type of uveitis in both genders, although anterior uveitis was seen more frequently in females. Retinal vasculitis, vitreitis, and retinitis were the most common ocular manifestations in these patients. Cataract and macular edema were the most common complications. Oral aphthae were the most frequent extraocular manifestation, followed by dermatologic lesions, and genital ulcers. The results of FFA, OCT, B-scan ultrasonography, LFCM, and UBM generally were in accordance with or comparable with clinical observations. At the final visit, uveitis was well controlled in 86.2% of patients. However, 20.4% of eyes became legally blind despite aggressive treatment. Kaplan-Meier analysis estimated the risk of losing useful vision (0.05) at 5 and 10 years for males and females as 29% versus 6% and 65% versus 33%, respectively. Behçet's disease in Chinese patients mainly manifests as nongranulomatous uveitis frequently associated with oral aphthae and dermatologic lesions. Fundus fluorescein angiography, B-scan ultrasonography, LFCM, UBM, and OCT may provide much information about pathophysiologic hallmarks of Behçet's disease. A combination of corticosteroids with other immunosuppressive agents is effective for most patients with Behçet's disease. Males had a more severe course and were at higher risk for losing vision than females.