The objective of this study is to describe the characteristics of patients with pachymeningitis (PM) in granulomatosis with polyangiitis (GPA) from Latin America, including three young patients. This is a retrospective case series. Patients were classified according to the ACR criteria, the 2012 Chapel Hill Consensus Conference Nomenclature and the EMA algorithm. Demographic, clinical, serological, and neuroimaging characteristics are described. Thirteen patients (nine females, four males) were identified. Mean age ± SD of PM diagnosis was 35.5 ± 20.4 years (median 48, range 8-71 years). Mean time ± SD between GPA first symptom and PM diagnosis was 59.8 ± 70.1 months (median 48, range 2-252 months). An important difference between children and adults was the median time elapsed between first GPA symptoms and PM diagnosis (range 2-4 months vs 5-252 months, respectively). Chronic headache was present in all, followed by intracranial hypertension (n = 5), single cranial nerve palsy and orbital mass (n = 4), seizures (n = 3), cavernous sinus syndrome and multiple cranial nerve palsies (n = 2), and meningism and cerebellar syndrome (n = 1 each). At time of PM diagnosis, mean BVAS/WG (Birmingham Vasculitis Activity Score for Wegener's granulomatosis) was 4 ± 2.4 and mean VDI of 2 ± 1.6, mostly due to ENT damage. Gadolinium-enhanced brain MRI showed dural thickening in 12 patients and leptomeningeal enhancement in one. All received a combination of glucocorticoids plus immunosuppressants, rituximab being used favourably in one refractory case. Improvement was observed in 12 patients. Chronic headache should lead to suspect PM. PM predominates in localised GPA. Children may present it earlier in the disease course than adults. Treatment is non-standardised and remains difficult.
Background:IgG4-related ophthalmic disease (IgG4-ROD) may present as a cause of orbital myositis leading to proptosis and diplopia. This clinical scenario can be mistakenly diagnosed as Graves’ orbitopathy (GO), preventing a timely and adequately treatment.Objectives:To elucidate if there are specific radiological features that might differentiate between IgG4-ROD and GO by imaging.Methods:We included 19 patients with diagnosis of IgG4-related disease (IgG4-RD) according to the Comprehensive Diagnostic Criteria for IgG4-RD, who regularly attended a tertiary referral center in Mexico City. All the patients had ophthalmic involvement and available computed tomography (CT) and/or magnetic resonance imaging (MRI) of the orbits. We also included 32 patients with GO with available CT and/or MRI of the orbits. Imaging studies were evaluated by a blinded head and neck radiologist for the following features: exophthalmos, extraocular muscles (EOM) size and morphology, lacrimal gland enlargement, orbital fat involvement, stretching of the optic nerve (ON), ON sheath thickening and orbital bone changes.Results:Both groups were similar in age (49.1±15.8 vs. 51.6±14.7, p=0.58) and gender (men 58.9% vs. 40.6%, p=0.23). In addition to ophthalmic involvement, 18 (94.7%) IgG4-RD patients had extra-ophthalmic involvement with a median number of organs involved of 7 (1-12), mainly submandibular glands (73.7%), lymph nodes (68.4%), parotid glands (63.2%) and pancreas (47.4%). Three patients were misdiagnosed as GO before IgG4-RD diagnosis. Graves’ disease was the underlying thyroid disorder in 28 (87.5%) GO patients, Hashimoto’s thyroiditis in 2 (6.3%), papillary thyroid carcinoma in one (3.1%) and one patient was euthyroid with positive thyroid stimulating immunoglobulin. The prevalence of exophaltmos (78.9% vs. 93.8%), bilateral involvement (78.9% vs. 87.8%) and overall EOM involvement (47.4% vs. 68.8%) was similar between IgG4-ROD and GO groups. However, IgG4-ROD patients had a higher frequency of lacrimal gland involvement (73.7% vs. 10.7%, p=0.001) and a tendency for the lateral rectus to be the most frequently involved EOM (22.2% vs. 0%, p=0.07); conversely they had a lower prevalence for the inferior rectus to be the most frequently involved EOM, (33.3% vs. 72.7%, p=0.04), orbital fat involvement (47.4% vs. 81.3%, p=0.01), ON stretching (57.9% vs. 87.5%, p=0.02) and orbital bone changes (0% vs. 25%, p=0.02). EOM bellies were involved in all the IgG4-ROD and GO cases, whereas EOM tendon involvement was present in 9% of GO and in none of the IgG4-ROD group. Patients with IgG4-ROD had more frequently the combination of lacrimal gland and lateral rectus (31.6% vs. 3.1%, p=0.008) and less frequently the combination EOM and orbital fat involvement (21.2% vs. 59.4%, p=0.008).At the logistic regression analysis we found an association of lacrimal gland involvement (OR 64.4.0, 95% CI 6.8-609.5, p=0.001) with IgG4-ROD. In a second model including combined variables, the combination of lacrimal gland and lateral rectus involvement was as...
Low-and middle-income countries (LMIC) have suffered from long-term health system deficiencies, worsened by poor living conditions, lack of sanitation, a restricted access to health facilities and running water, overcrowding, and overpopulation. These factors favor human displacement and deepen marginalization; consequently, their population endures a high burden of infectious diseases. In this context, the current epidemiological landscape and its impact on health and economic development are not promissory, despite the commitment by the international community to eradicate neglected tropical infections -especially tuberculosis and malaria, by 2030. Neglected and (re)-emerging infectious diseases affecting the central nervous system (CNS) are a major public health concern in these countries, as they cause a great morbidity and mortality; furthermore, survivors often suffer from severe neurological disabilities. Herein, we present a retrospective review focused on some neglected and (re)-emerging infectious diseases, including neurocysticercosis, malaria, rabies, West Nile virus encephalitis, tuberculosis, neuroborreliosis, and SARS-CoV-2 in LMIC.
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