Objective: To compare disease course in patients with Guillain-Barré syndrome (GBS) with a poor prognosis who were treated with one or with two intravenous immunoglobulin (IVIg) courses.
Methods: From the International GBS Outcome Study, we selected patients whose modified Erasmus GBS Outcome Score at week 1 predicted a poor prognosis. We compared those treated with one IVIg course to those treated with two IVIg courses. The primary endpoint, the GBS disability scale at 4 weeks, was assessed with multivariable ordinal regression.
Results: Of 237 eligible patients, 199 patients received a single IVIg course. Twenty patients received an 'early' second IVIg course (1-2 weeks after start of the first IVIg course) and 18 patients a 'late' second IVIg course (2-4 weeks after start of IVIg). At baseline and 1 week, those receiving two IVIg courses were more disabled than those receiving one course. Compared with the one course group, the adjusted OR for a better GBS disability score at 4 weeks was 0.70 (95%CI 0.16 to 3.04) for the early group and 0.66 (95%CI 0.18 to 2.50) for the late group. The secondary endpoints were not in favour of a second IVIg course.
Conclusions: This observational study did not show better outcomes after a second IVIg course in GBS with poor prognosis. The study was limited by small numbers and baseline imbalances. Lack of improvement was likely an incentive to start a second IVIg course. A prospective randomised trial is needed to evaluate whether a second IVIg course improves outcome in GBS.
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http://dx.doi.org/10.1136/jnnp-2019-321496 | DOI Listing |
Zhonghua Er Ke Za Zhi
February 2025
Department of Gastroenterology and Infectious Diseases, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200062, China.
To summarize the clinical features, laboratory findings, treatment and prognosis of children confirmed as -induced rash and mucositis (MIRM) in children. This retrospective study concluded 6 children diagnosed as MIRM in Department of Gastroenterology and Infectious Diseases, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University from August 2023 to April 2024. This paper described the characteristics of MIRM and analyzed the therapeutic strategy and prognosis.
View Article and Find Full Text PDFNeurol Neuroimmunol Neuroinflamm
March 2025
Department of Neurology, Mayo Clinic, Rochester, MN.
Background And Objectives: While it is well characterized in adults, little is known about the clinical features of neurofascin 155-IgG4 autoimmune nodopathy (NF155-IgG4 AN) in the pediatric population. In this study, we aimed to describe the clinical features and treatment outcomes in children diagnosed with neurofascin 155-IgG4 autoimmune nodopathy (NF155-IgG4 AN).
Methods: Pediatric and adult patients with NF155-IgG4 AN were identified retrospectively through the Mayo Clinic Neuroimmunology Laboratory database.
Neurol Sci
January 2025
Department of Infectious Diseases, Shenzhen Nanshan People's Hospital, Shenzhen University Medical School, Shenzhen, 518052, China.
Background: Glial fibrillary acidic protein (GFAP) astrocytosis is a rare autoimmune encephalitis discovered in the last decade. The diagnosis depends on clinical symptoms, imaging, and antibody testing. However, most cases require several months or even longer to make a definite diagnosis.
View Article and Find Full Text PDFDermatologie (Heidelb)
January 2025
Klinik für Dermatologie, Allergologie und Venerologie, Universitätsklinikum Leipzig, Philipp-Rosenthal-Str. 23, 04103, Leipzig, Deutschland.
Painful ulcerations developed in a 33-year-old woman with anti-NXP-2-positive dermatomyositis in the facial and trunk areas and a 67-year-old woman with TIF1-gamma-positive dermatomyositis on the hands, while undergoing systemic therapy with azathioprine or low-dose methylprednisolone and cyclic administration of intravenous immunoglobulins (IVIG), respectively. In the laboratory workup, the anti-MDA‑5 antibody status remained negative and the creatine kinase (CK) normal in both patients, while histopathological examinations were nonspecific. Intensive topical class 4 corticosteroid therapy and continuation of the immunosuppressive or immunomodulating therapy led to healing of the ulcerative skin lesions.
View Article and Find Full Text PDFCureus
December 2024
Neurology, Tecnológico de Monterrey, Monterrey, MEX.
Isolated ophthalmoplegia as an anti-glutamic acid decarboxylase 65 (anti-GAD65) antibody-associated neurological syndrome is rare. We present a case of a 22-year-old pregnant Hispanic female patient who presented initially with a left oculomotor nerve palsy following an emergency department (ED) visit for migraine headache. Brain imaging was done with no important findings.
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