Clinical application of the new classification criteria in children with Miller Fisher syndrome and clinical characteristics analysis of different types of Miller Fisher syndrome / 中华实用儿科临床杂志

Objective:To investigate the clinical application of the new classification criteria in children with Miller Fisher syndrome (MFS), and to analyze clinical characteristics of different types of MFS.Methods:Clinical data of MFS patients hospitalized in the Department of Neurology, Beijing Children′s Hospital, Capital Medical University from January 2015 to December 2019 were collected and analyzed retrospectively, including demographic characteristics, clinical symptoms, neurological examination findings, laboratory examination data, diagnosis and treatment, and prognosis.The counting data was described by percentage, and the measurement data was described by median.Results:A total of 23 patients were included in the research, including 14 males and 9 females, with a median age of 4 years and 8 months.There were 3 cases of pure MFS; 5 cases of incomplete MFS, including 1 case of acute ptosis and 4 cases of acute ataxia neuropathy; 15 cases of overlapping syndrome, including 13 cases of MFS/Guillain Barre syndrome (GBS), 1 case of MFS/pharyngocervical brachial variant GBS(PCB GBS)and 1 case of MFS/GBS/Bickertaff brainstem encephalitis (BBE). In addition to Ⅲ, Ⅳ and Ⅵ cranial nerve palsy, 11 cases had the involvement of other cra-nial nerves, including 2 cases in pure MFS, 8 cases in MFS/GBS and 1 case in MFS/GBS/BBE.Autonomic nervous dysfunction occurred in 6 cases.Respiratory muscle paralysis occurred in 6 cases, including 5 cases in MFS/GBS and 1 case in MFS/GBS/BBE.Graded by the Hughes scoring system (HG score), 3 cases with pure MFS were graded 4 points; 1 case with acute ptosis was graded 0; 3 cases with acute ataxia neuropathy were graded 2 points, and the other one was graded 3 points; 1 case with MFS/PCB GBS was graded 3 points; 10 cases with MFS/GBS were graded 4 points, 1 case was graded 3 points, and the other 2 cases were graded 2 points; 1 case with MFS/GBS/BBE was graded 4 points.Twenty-two patients were treated with intravenous immunoglobulin.The HG of all patients at discharge decreased at varying degree, which was graded 0 at 6 months of follow-up.Conclusions:The clinical application of the new diagnostic classification method is helpful to the accurate diagnosis of different types of MFS.More than half of MFS cases will develop into the overlapping syndrome.The overlapping of MFS and GBS or BBE is prone to the involvement of cranial nerves except for the external ophthalmic muscles, autonomic nerve dysfunction and respiratory muscle paralysis.The disease course of MFS varies, and its diagnosis should be comprehensively made.All cases of MFS in this study have a satisfactory prognosis.

Analysis of clinical characteristics of patients with anti-myelin oligodendrocyte glycoprotein and anti-N-methyl-D-aspartate receptor antibody positive / 中华神经科杂志

Objective:To analyze the clinical characteristics of patients with double-positive anti-myelin oligodendrocyte glycoprotein (MOG) antibody and anti-N-methyl-D-aspartate receptor (NMDAR) antibody, so as to raise awareness of such diseases and improve the prognosis.Methods:Eighteen patients (double positive group) with positive serum anti-MOG antibody and cerebrospinal fluid anti-NMDAR antibody in Huashan Hospital, Fudan University from March 2017 to March 2020 were retrospectively analyzed. Using the SPSS software for simple random sampling, anti-MOG group(20 cases) and anti-NMDAR group (20 cases) were randomly selected at the same time for comparison. The anti-MOG group referred to the patients only with positive serum anti-MOG antibody. While the anti-NMDAR group referred to the patients whose cerebrospinal fluid anti-NMDAR antibody was positive. The clinical characteristics, laboratory examination results, radiological characteristics and prognosis of the three groups were collected and analyzed.Results:There was no statistically significant difference in demographic data among the three groups ( P>0.05). The symptoms of patients in the double-positive group were divided into two categories by cluster analysis, which corresponded to the symptom groups obtained by cluster analysis of the anti-MOG group and the anti-NMDAR group, and the same result was verified by correspondence analysis. Compared with the anti-MOG group, the incidence of epilepsy (10/18 vs 3/20, P=0.016), psychosis and behavior change (8/18 vs 0/20, P=0.001), visual disturbances (8/18 vs 17/20, P=0.016), dysarthria/dysphagia (8/18 vs 1/20, P=0.007) was significantly different in the double-positive group ( P<0.017). Compared with the anti-NMDAR group, the incidence of ataxia (8/18 vs 19/20, P=0.001), psychosis and behavior change (12/18 vs 1/20, P<0.001) was significantly different in the double-positive group. There was no statistically significant difference in the combination rate of thyroid peroxidase antibody, thyroglobulin antibody and antinuclear antibody between two groups, and the cerebrospinal fluid pressure, white blood cell count, protein, glucose, chloride and positive rate of oligoclonal band were also not statistically different between two groups ( P>0.017; P<0.017 indicates statistically significant difference by Bonferroni corrected multiple comparisons). Compared with the anti-NMDAR group, whether the brain magnetic resonance imaging had lesions was different in double positive group (18/18 vs 8/20, P<0.001). The initial modified Rankin Scale (mRS) scores before treatment were different among the double positive group, anti-MOG group and anti-NMDAR group (3.72±0.96, 2.75±0.97, 3.95±0.76, respectively, F=10.004, P<0.001), but there was no statistically significant difference in the scores after six-month treatment (1.22±1.44, 0.40±0.75, 1.20±1.24, respectively, F=3.153, P=0.051), and the recurrence rate of the disease was different among the three groups (8/18, 14/20, 5/20, respectively, χ2=10.004, P=0.017). Conclusions:Anti-MOG antibodies and anti-NMDAR antibodies could exist at the same time, showing clinical phenotype overlap, which was a new syndrome called the overlapping syndrome of myelin oligodendrocyte glycoprotein antibody-associated disease and NMDAR encephalitis, MNOS. The condition of MNOS patients was more severe than that of patients with MOG antibody-associated disease (MOGAD), but patients with MNOS, MOGAD, and anti-NMDAR encephalitis all responded well to immunosuppressive therapy. It was suggested that early second-line immunotherapy should be given to reduce the recurrence rate in patients with MNOS and MOGAD.

Síndrome de solapamiento: polimiositis-esclerodermia. Presentación de un caso / Polymyosis-Sclerosis Overlap Syndrome. Case report and review of literature

Introducción: en el síndrome de solapamiento o superposición existen simultáneamente manifestaciones clínicas o serológicas de dos o más enfermedades autoinmunes sistémicas. Afectan al 5 por ciento de la población con predominio en mujeres. Presentación del Caso: hombre de 48 años, sin antecedentes patológicos personales. Acude con dolor en ambas manos, inflamación en las articulaciones metacarpo e interfalángicas proximales, bilateral y simétrica, acompañada de rigidez matinal que duraba casi todo el día desde hace 4 meses. Se le diagnostica artritis reumatoidea y se inicia tratamiento que abandonó. Luego aparece un cuadro que es interpretado como un solapamiento por lo que es ingresado con un cuadro clínico florido. En los exámenes de laboratorio: las transaminasas, la creatín fosfoquinasa, el lactato deshidrogenasa, se encontraban elevadas. Por la clínica y los complementarios se diagnosticó Síndrome de Solapamiento de polimiosistis-esclerodermia. Se comenzó tratamiento con inmunosupesores. El paciente evolucionó desfavorablemente y falleció. Discusión: el diagnóstico de este caso se realizó por las manifestaciones clínicas como fascie esclerodérmica, signo de sal y pimienta, fibrosis de predominio distal en ambas manos, telangiectasias peribucales. Los diagnósticos diferenciales son las enfermedades autoinmunes sistémicas cuando se presentan como cuadros únicos. El tratamiento de primera línea y el pronóstico dependen de las enfermedades que se solapen(AU)

Introduction: In the syndrome of overlap or overlap there are simultaneously clinical or serological manifestations of two or more systemic autoimmune diseases. They affect 5 percent of the population with predominance in women. Case Presentation: A 48-year-old man with no personal pathological history. He came with pain in both hands, inflammation in the metacarpal and interphalangeal proximal joints, bilateral and symmetrical, accompanied by morning stiffness that lasted almost all day for 4 months. He was diagnosed with rheumatoid arthritis and started treatment that he abandoned. Then a picture appears that is interpreted as an overlap so it is entered with a florid clinical picture. In laboratory tests: transaminases, creatine phosphokinase, lactate dehydrogenase, were elevated. Clinical and complementary diagnosis of Polymyosis-Sclerosis Overlap Syndrome was diagnosed. Treatment with immunosupers was started. The patient evolved unfavorably and died. Discussion: The diagnosis of this case was made by clinical manifestations such as sclerodermic fascia, salt and pepper sign, predominantly distal fibrosis in both hands, perioral telangiectasias. Differential diagnoses are systemic autoimmune diseases when presented as single frames. First-line treatment and prognosis depend on overlapping diseases(AU)

Efficacy of itopride in functional dyspepsia patients overlapping constipation-predominant irritable bowel syndrome / 中华消化杂志

Objective To assess the efficacy of itopride on treatment of patients with functional dyspepsia (FD) overlapping constipation-predominant irritable bowel syndrome (C-IBS). Methods Patients who met criteria for FD and FD overlapping C-IBS were randomly assigned into FD treatment group (group A), FD control group (group B), FD overlapping C-IBS treatment group (group C) and FD overlapping C-IBS control group (group D). The patients in group A and group C received 100 mg of itopride 3 times daily for 8 weeks. Dyspeptic symptoms including abdominal pain, bloating, early satiety and constipation, were evaluated before and after treatment. Ultrasonic monitoring of gastric emptying function was performed in group A and group C before and two weeks after treatment.ResultsThe symptoms of FD were relieved in both group A and group C (P<0.05), while better results were shown in group C. The significant improvement of constipation was seen in group A and group C. Besides, after medication, gastric emptying was improved in group A and group C in comparison with group B and group D. Conclusion Itopride is an effective therapeutic option in the treatmentping of patients with overlapping of FD and C-IBS.

Carotid Reconstruction for Cerebral Ischemia in Aortitis Syndrome Combined with Ulcerative Colitis / 日本心臓血管外科学会雑誌

We report a case of an overlapping ulcerative colitis and aortitis syndrome. A 28-year-old woman with ulcerative colitis in the region from the rectum to the sigmoid colon developed left anterior cervical pain. A cervical contrast-enhanced computed tomography and an arch aortography showed diffuse narrowing of the left common carotid artery and segmental stenosis of the left subclavian artery involving the orifice of the vertebral artery, which defined the diagnosis as aortitis syndrome. HLA was positive for BW 52, which is frequently found in patient with this type of overlapping syndrome. Treatment with prednisolone was begun at an initial dose of 20mg/day, with gradual tapering to 5mg/day at 11 months later, when her symptoms had subsided and laboratory findings of inflammation had disappeared. One year later, she became apathetic in mental activity, and had a poor memory. She also complained of numbness on the left side of her face. Angiography confirmed the progression of the left common carotid narrowing causing the deterioration of her cerebral ischemic symptom. A saphenous vein bypass graft was placed between the right subclavian artery and the left common carotid artery. The postoperative course was uneventful and she had complete symptomatic relief and recovered active ordinary life. Postoperative angiography revealed the well functioning bypass graft.