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Pateira de Fermentelos is one of the largest natural freshwater lagoons in the Iberian Peninsula (approximately 529 ha). It is an important and extensive humid area integrated in the Special Protection Area (SPA) Ria de Aveiro (PTZPE0004) due to its ornithological importance and habitat typology. Water hyacinth ( Eichhornia crassipes) is a major factor threatening the environmental and ecological balance of the lagoon and associated ecosystems. In recent years, the water hyacinth has spread over more than 50% of the lagoon surface, thus contributing to the degradation of the ecological, economical and social conditions and, ultimately, the lagoon system of Ria de Aveiro. In December 2006 the Municipal Authority of Águeda, in association with other public entities, initiated the mechanical removal (using an aquatic-harvester) of the extensive water hyacinth mats that covered the lagoon, with the intention of improving the system in a sustainable manner. Since the beginning of this action, the aquatic-harvester has removed more than 15 500 m3 of vegetal mats from the lagoon, which, in accordance with the current legislation, has been transported to an old inactive quarry site. At present the lagoon water is free from this invasive exotic plant, hence allowing navigation and the maintenance of traditional activities such as fishing and boating. The monitoring of the lagoon water quality is in progress in several locations, to monitor the system evolution, and develop actions and research aiming for sustainable management of the Pateira. [ABSTRACT FROM AUTHOR]

An abstract of the study "Left ventricular function in adults with muscular dystrophies: genotype-phenotype correlations," by E. Martins and colleagues is presented.

Focal myositis is a very rare form of inflammatory myopathy, with unknown etiology. We describe a 44-year-old previously healthy man who noticed a painless swelling on his left forearm, following trauma over the left cubital fossa. The swelling grew progressively over 2 years. He had otherwise no weakness complaints. Physical and neurological examinations were otherwise normal. Creatine kinase and aldolase levels were increased (1,009 U/L and 11.9 U/L, respectively); autoimmunity panel was negative. MRI showed diffuse edema and gadolinium enhancement of muscles innervated by the median nerve. EMG revealed repetitive complex discharges and patterns of continuous muscular activity. The mass was biopsied disclosing findings consistent with focal myositis. Focal myositis with muscular atrophy is a rare situation; the inflammatory mechanism is yet to be defined, but it seems to be a situation with slow progression, with tendency to stabilization.
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Muscle specific kinase (MuSK) myasthenia gravis (MG, MuSK-MG) is a rare subgroup of MG affecting mainly women during childbearing years. We investigated the influence of pregnancy in the course of MuSK-MG and pregnancy outcomes in females with MuSK-MG. A multicentre cohort of 17 women with MuSK-MG was studied retrospectively; 13 of them with ≥1 pregnancy. MuSK-MG onset age was 35,4 years; 23,0% had other autoimmune disorder; 46,2% were treatment refractory. Thirteen women experienced 27 pregnancies, either after MG onset (group I) (n = 4; maternal age at conception = 29.8 years) or before MG onset (group II) (n = 23; maternal age at conception = 26.2 years). In group I pregnancy occurred in average 9.8 years after the MG onset; it occurred in average 17.0 years before MG in group II. In group I, all were on steroids at time of conception, one on azathioprine and another receiving IVIG regularly. There were mild exacerbations that responded to treatment adjustments. There were no relapses in the 12 months following the delivery. There was no pre-eclampsia, birth defects or stillbirths in either group; 3 miscarriages in group II. One case of neonatal MG was recorded. In this small series, pregnancy did not seem to precipitate MuSK-MG or to have a major influence in the MuSK-MG course, and there was no apparent negative impact in pregnancy outcomes in those where pregnancy followed the MG onset. The weight was lower in the newborn of the group I mothers, although none had low birth weight., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Introduction: In this study we estimated the prevalence, incidence, and mortality of myasthenia gravis (MG) in northern Portugal and characterized the clinical features of the patients identified., Methods: We used 2 data sources: clinical records from the hospitals and pyridostigmine prescription registers., Results: On December 31, 2013, we estimated a point prevalence of 111.7 patients per million population. The highest prevalence was observed in the group >65 years of age, especially in men (288.1 per million). During 2013, we estimated an incidence rate of 6.3 per million per year. Among women, the incidence rate was highest in the 15-49-year age group; in men, incidence increased with age up to 22.1 per million in those >65 years old. The MG-related mortality rate was 0.5 per million., Conclusions: These figures are in keeping with similar studies and emphasize the importance of diagnosis and management of MG in elderly populations. Muscle Nerve 54: 413-421, 2016., (© 2016 Wiley Periodicals, Inc.)

Paraneoplastic neurological syndromes are a group of rare and heterogeneous disorders complicating cancer through immune-mediated mechanisms. They typically arise before the diagnosis of malignancy, thus constituting its first clinical manifestation. A thorough search for the underlying tumour is necessary, as adequate tumour management is essential for both neurological prognosis and overall survival. The authors present the case of a 43 year-old woman who presented with a subacute cerebellar syndrome associated with the paraneoplastic anti-Yo antibody. Although paraneoplastic aetiology was immediately suspected, the diagnosis of the underlying tumour was not straightforward, as is often the case. This case report highlights the importance of directing tumour search for the most probable anatomic locations, according to patient demographics and risk factors as well as the type of onconeural antibodies present, and also the need to use the most sensitive diagnostic modalities appropriate for each target organ.

Vasculitic neuropathy corresponds to the occurrence of vasculitis at the level of vasa nervorum, resulting in ischemic damage of the peripheral nerve and axonal degeneration. Vasculitic neuropathy commonly occurs in association with systemic diseases and may be the initial manifestation or arise in the course of established disease. Although rare, vasculitis can be confined to the peripheral nervous system - non-systemic vasculitic neuropathy. This paper aims to review the classification, diagnosis and treatment of vasculitic neuropathy.

Background: Left ventricular dysfunction (LVD) is a clinical manifestation of muscular dystrophy (MD) in adults and an important prognostic factor. However, there is a lack of recommendations concerning cardiac followup of these patients. The aim of this work was to evaluate the prevalence of systolic LVD in adults with MD., Methods: The patients, referred from a Neuromuscular Diseases Unit, underwent clinical evaluation, ECG and transthoracic echocardiogram. Serum troponin I and NT-proBNP were also evaluated. Systolic LVD was defined by an ejection fraction of <0.45 and/or shortening fraction of <0.25., Results: During a one-year period, we evaluated 24 consecutive patients, 16 men, age 33 +/- 12 years (age at myopathy diagnosis 23 +/- 12 years). They had the following MD: dystrophinopathies--four patients (Duchenne: 1, Becker: 3); Steinert myotonic dystrophy--nine patients; limb-girdle myopathies--six patients; facioscapulohumeral (FSH) myopathies--four patients; and one dysferlinopathy (Miyoshi myopathy). The MD diagnosis, based on clinical and histological criteria, was genetically confirmed in 18 cases (75%). Five patients (20.8%) had thoracic pain, four (16.6%) dyspnea and one a history of syncope. ECG abnormalities were present in 14 cases (58.3%). Stolic LVD or left ventricular remodeling were present in six patients (25%): three with dystrophinopathies, one with limb-girdle myopathy, one with FSH dystrophy and one with myotonic dystrophy. Three patients (12.5%) were diagnosed with heart failure according to the European Society of Cardiology criteria. Three of the five patients with left ventricular dysfunction had elevation of NT-proBNP. One patient with Becker dystrophy had slight elevation of troponin I. In patients with systolic LVD or LV remodeling, the mutations identified were: deletion in intron 1 to exon 49 (one Duchenne MD patient) and deletions in exons 45 to 51 (two Becker MD patients) in the dystrophin gene; deletion of D4Z4 repeats in the DFS locus (4q35) (one patient with FSH MD); and 1300-1500 CTG triplet repeats in the DMPK gene (one patient with myotonic dystrophy)., Conclusions: These results, although preliminary, show that a high percentage of patients with genetically determined skeletal myopathies exhibit cardiac myocyte functional impairment, with severe LVD and heart failure, justifying periodic cardiovascular evaluation. In the future, phenotype-genotype correlations could help to determine the best cardiac surveillance in MD patients.