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The neurological involvement by IgG4‐related disease is not completely understood. We report the case of a patient that developed progressive paraparesis and urinary retention. The spinal MRI revealed a longitudinal extensive transverse myelitis, and the CSF was inflammatory. Anti‐AQP4 and anti‐MOG antibodies were negative in a live cell‐based assay. IgG4 level was 231 mg/dL in serum, twice the upper limit of normal. Later, mediastinal lymphadenopathies and intermediate uveitis were discovered, which are features of IgG4‐related disease leading to the diagnosis. This case highlights that IgG4‐related disease should be included in the differential diagnosis of spinal cord syndromes. [ABSTRACT FROM AUTHOR]

Purpose: Multiple sclerosis (MS) is a long-lasting disabling disease with psychological implications. Hospital Anxiety and Depression Scale (HADS) is a questionnaire developed to screen anxiety and depression among patients in hospital settings. The objective of this report was to study the metric properties of the Portuguese version of HADS regarding MS. Materials and methods: This was a cross-sectional study. Three hundred and eighty individuals with a diagnosis of MS, according to the revised McDonald criteria, were recruited from an outpatient Neuroimmunology Clinic of the city of Porto. Participants had a mean age of 40.04 years (SD =10.86), 63.9% of them were female. HADS consists of two subscales – anxiety and depression – scored separately, with seven items each. Results: The results of our study show the appropriateness of HADS for the identification of mood changes in people with MS and show good metric properties. Conclusion: It seems to be a useful tool for the scrutiny of mood disorders in Portuguese patients with MS. The questionnaire is also sensitive to disease when compared to people with other severe illnesses, and with people without the disease. [ABSTRACT FROM AUTHOR]

Introduction: We evaluated the clinical course during pregnancy and neonatal outcomes of a cohort of Portuguese women with myasthenia gravis (MG).Methods: Retrospective study.Results: Twenty-five patients with 30 pregnancies were included. Mean maternal age was 32.4 ± 4.1 years. Miscarriage rate was 6.7%, with delivery of 28 newborns. Deterioration in MG during pregnancy occurred in 43.3%, and 46.4% occurred at postpartum. Eighty percent were medicated with pyridostigmine, 43.3% with corticosteroids, and 40% with intravenous immunoglobulin. There were no maternal or neonatal deaths. Mean gestational time at delivery was 38.2 weeks. No cases of fetal growth restriction, preeclampsia, preterm delivery, or fetal demise were observed. Global cesarean rate was 64.3%. Two newborns developed transient neonatal myasthenia.Conclusions: A high rate of clinical worsening of MG in the mother was observed in this retrospective study, which highlights the importance of a multidisciplinary approach for avoiding maternal adverse outcomes. Muscle Nerve 54: 715-720, 2016. [ABSTRACT FROM AUTHOR]

Introduction: Behçet's disease (BD) is a multisystem inflammatory disease of unknown etiology that may affect the CNS - Neuro-Behçet (NB). Our aim was to evaluate the frequency of neurological involvement and characterize a cohort of our NB patients. Methods: We retrospectively revised the clinical, laboratory and imaging data of a cohort of BD patients, followed in our hospital outpatient clinic. Results: We identified 138 BD patients. Twenty-five out of 138 had NB (15 female). Four patients presented with neurological symptoms. We identified a total of 37 attacks. Twenty-one attacks were classified as parenchymatous, four non-parenchymatous and 12 as other syndromes. Seventeen patients had CSF analysis performed (20 samples). Five samples were normal, 15 showed CSF pleocytosis. The most frequent finding on MRI performed in the acute phase was extensive lesions involving the brainstem. Two patients died due to the neurological involvement of BD. Conclusion: We found 18.1% prevalence of NB and a higher female-to-male ratio in our group than in other series. Gastrointestinal and vascular involvement was more frequent in the NB group. The fact that neurological involvement may be the first manifestation of BD with therapeutic implications and associated morbidity points out the relevance of an early diagnosis. © 2015 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background: Neuromyelitis Optica (NMO) is an inflammatory demyelinating disease of the CNS. There have been few epidemiologic studies on NMO, none in Portugal. Objective: To analyze the clinical, biological and MRI characteristics from a cohort of Portuguese patients who fulfilled the Wingerchuk 2006 NMO/NMOSD criteria. To identify and characterize those who had concomitant autoimmune disease or circulating autoantibodies. Methods: We performed an observational, retrospective, multicenter study in 5 Hospital Centers in Portugal. Results: Sixty-seven patients fulfilled the inclusion criteria. They were mainly Caucasian, 55 female. Median age at onset was 32.0 years and mean follow-up 7.4 ± 6.0 years. Twenty-one patients were definite NMO and optic neuritis (ON) the most frequent initial presentation. Forty-six were classified as NMO spectrum disorders. The main subtypes were recurrent ON and single longitudinally extensive transverse myelitis. Twenty-four patients had positive AQP4-IgG. Twenty-three had other circulating autoantibodies. Fifteen out of 67 patients had concomitant autoimmune disease. There was a significant correlation between the presence of autoimmune disease and the positivity for AQP4-IgG. Five patients died, all definite NMO. Conclusion: This is the first study about this rare disease in Portugal. Demographic features were similar to other studies. The existence of concomitant autoimmune disease was significantly associated with seropositivity for AQP4-IgG. [ABSTRACT FROM AUTHOR]