Incidence and clinical characteristics of Guillain-Barré syndrome in Osona (Barcelona, Spain), 2003-2016.

INTRODUCTION: According to most studies, the incidence of Guillain-Barré syndrome increases with age, with a peak incidence occurring between 70 and 80 years of age. The objective of this study is to describe the incidence (overall and by sex and age group) and clinical characteristics of Guillain-Barré syndrome in Osona (Barcelona, Spain). METHODS: We performed a retrospective, descriptive, population-based study covering the period 2003 to 2016. RESULTS: The global incidence of Guillain-Barré syndrome is 2.07 cases per 100000 person-years. Incidence increases with age, except for small peaks during childhood and between 40 and 50 years, and reaches a maximum of 6.26 cases per 100000 person-years above the age of 80. The incidences of the different variants were: AIDP, 72.1%; AMAN, 16.3%; ANSAN, 4.7%; and Miller Fisher syndrome, 4.7%. A total of 41.9% of patients had a history of respiratory tract infections, and 20.9% had a history of gastrointestinal infections. Protein in the cerebrospinal fluid was found in 76.7%. EMG findings suggested demyelination in 73.7% of the patients and axonal degeneration in 26.3%. A total of 20.9% of patients needed ventilatory support. Six-month mortality was 9.3%. Variables associated with worse prognosis were age over 80 years, delay in admission, previous gastrointestinal infection, and AMAN variant. CONCLUSIONS: The incidence observed in our study is in the upper range of estimated incidence rates reported in European and North American studies. The syndrome may be underdiagnosed in elderly patients; physicians must be vigilant to the possibility of the disease, which is associated with a high mortality rate if it is not treated early.

Incidencia y características clínicas del síndrome de Guillain-Barré en la comarca de Osona (Barcelona, España) (2003-2016) / Incidence and clinical characteristics of Guillain-Barré syndrome in Osona (Barcelona, Spain), 2003-2016

Introducción: La mayoría de los estudios muestran que la incidencia del síndrome de Guillain-Barré aumenta con la edad, con un máximo entre los 70 y los 80 años y un descenso posterior. El objetivo del estudio es describir la incidencia global y específica por sexo y grupos de edad y las características clínicas del síndrome de Guillain-Barré en la comarca de Osona (Barcelona, España).MétodosEstudio descriptivo retrospectivo de base poblacional en el periodo 2003-2016.ResultadosLa incidencia global es de 2,07/100.000 habitantes-año. La incidencia aumenta con la edad, salvo un pequeño pico en la infancia y entre los 40 y los 50 años, alcanzando la máxima de 6,26/100.000 habitantes-año pasados los 80 años. Los porcentajes de las variantes fueron: AIDP (72,1%), AMAN (16,3%), ANSAN (4,7%) y síndrome de Miller-Fisher (4,7%). Presentaron infección previa de vías respiratorias el 41,9% e infección gastrointestinal el 20,9%. Se halló proteinorraquia en el 76,7%. El EMG mostraba un predominio desmielinizante en el 73,7% y axonal en el 26,3%. Necesitaron soporte ventilatorio el 20,9%. La mortalidad a los 6 meses fue del 9,3%. Las variables que se asociaron a un peor pronóstico fueron la edad superior a los 80 años, la demora en el ingreso, presentar infección gastrointestinal previa y la variante AMAN.ConclusionesLa incidencia descrita en nuestro estudio se encuentra en el rango más alto de las estimadas en Europa y Estados Unidos. En ancianos pudiera estar infradiagnosticado y se requeriría de una mayor alerta ante una enfermedad con alta mortalidad si no se trata de forma precoz. (AU)

Introduction: According to most studies, the incidence of Guillain-Barré syndrome increases with age, with a peak incidence occurring between 70 and 80 years of age. The objective of this study is to describe the incidence (overall and by sex and age group) and clinical characteristics of Guillain-Barré syndrome in Osona (Barcelona, Spain).MethodsWe performed a retrospective, descriptive, population-based study covering the period 2003 to 2016.ResultsThe global incidence of Guillain-Barré syndrome is 2.07 cases per 100,000 person-years. Incidence increases with age, except for small peaks during childhood and between 40 and 50 years, and reaches a maximum of 6.26 cases per 100,000 person-years above the age of 80. The incidences of the different variants were: AIDP, 72.1%; AMAN, 16.3%; ANSAN, 4.7%; and Miller Fisher syndrome, 4.7%. A total of 41.9% of patients had a history of respiratory tract infections, and 20.9% had a history of gastrointestinal infections. Protein in the cerebrospinal fluid was found in 76.7%. EMG findings suggested demyelination in 73.7% of the patients and axonal degeneration in 26.3%. A total of 20.9% of patients needed ventilatory support. Six-month mortality was 9.3%. Variables associated with worse prognosis were age over 80 years, delay in admission, previous gastrointestinal infection, and AMAN variant.ConclusionsThe incidence observed in our study is in the upper range of estimated incidence rates reported in European and North American studies. The syndrome may be underdiagnosed in elderly patients; physicians must be vigilant to the possibility of the disease, which is associated with a high mortality rate if it is not treated early. (AU)

[Scoping review on formaldehyde toxicity in occupationally exposed workers]. / Toxicidad del formaldehido en trabajadores profesionalmente expuestos. Revisión bibliográfica.

The presence of formaldehyde at workplace remains significant. Exposure to it results in local irritation of the eye, nose and upper respiratory tract mucous membranes, and it has been chronically related to a higher risk of cancer development at the paranasal sinuses, naso-oropharynx and lungs. The aim of our work has been the updating of the bibliography and the categorization of the most up to date scientific evidence of formaldehyde effects on human body. Bibliographic search on the electronic database Medline / PubMed, restricted to the last 10 years through a combination of free and controlled language.Review of 185 scientific articles, finally including 54 due to duplicity, language, and inclusion criteria. We find among the main results a major evidence regarding genotoxicity; limited, inconsistent, and contradictory evidence regarding various neoplastic pathologies; and lack of evidence regarding bronchial asthma. Further studies have to be carried out, especially longitudinal studies and greater epidemiological power, to generate new knowledge about the behavior of this toxic.

La presencia del formaldehido en el ámbito laboral sigue siendo importante. La exposición produce irritación local de mucosas oculares, nasales y del tracto respiratorio superior, y crónicamente se ha asociado con mayor riesgo de desarrollar cáncer a nivel de senos paranasales, naso-orofaringe y pulmón. Esta revisión tiene por objetivo actualizar la bibliografía y categorizar la evidencia científica más actualizada de los efectos que el formaldehido produce sobre el organismo humano. Búsqueda bibliográfica en la base de datos electrónica Medline/PubMed, limitada a los últimos 10 años mediante combinación de lenguaje libre y controlado. Se revisaron 185 artículos con inclusión final de 54 tras descartar por duplicidad, idioma y criterios de inclusión. Se observa un elevado grado de evidencia respecto a la genotoxicidad, evidencia contradictoria, inconsistente o limitada respecto a patologías neoplásicas de origen hematopoyético, laringe, naso-sinusales o de pulmón y falta de evidencia sobre la relación con el asma bronquial. Es preciso efectuar nuevos estudios, especialmente con carácter longitudinal y mayor potencia epidemiológica, para generar nuevo conocimiento sobre el comportamiento de este tóxico.

[Prevalence of physical activity in primary health care workers of Catalonia]. / Prevalencia de actividad física en profesionales de atención primaria de Cataluña.

INTRODUCTION: Physical inactivity is the fourth leading risk factor for global mortality, and a significant percentage of the world population does not perform the necessary physical activity for health benefits. Certain professional groups are seen as an example for the general population with whom they interact. Prevalence of physical activity in health workers, one of these reference groups, is mainly unknown. The aim of this study has been to assess the prevalence of physical activity levels in Primary Health Care professionals. MATERIAL AND METHODS: A study was conducted on the physical activity levels in Primary Health Care workers who came voluntarily for a medical examination in 2014, and completed the short version of the International Physical Activity Questionnaire activity. RESULTS: A low level of physical activity was reported by 26.5% of those taking part, with 31.5% of the medical group indicating a low level of activity, followed by support staff (28.1%), nurses (24.7%), and finally the administrative staff (19.0%). CONCLUSIONS: The physical activity levels of Primary Health Care staff are significantly different from those of the general reference population. The latter has a higher percentage of physical activity of mild to moderate intensity, and below the level of physical activity of high intensity. Although there seems to be a tendency to significantly lower physical activity in other health groups, we do not have sufficiently reliable data to compare them.

Prevalence of myasthenia gravis in the Catalan county of Osona. / Prevalencia de miastenia gravis en la comarca de Osona (Barcelona, Cataluña).

INTRODUCTION: The reported prevalence of myasthenia gravis ranges between 5 and 24 cases per 100,000, and people over 65years account for less than 50% of all cases. The prevalence and clinical characteristics of myasthenia gravis in the county of Osona were studied in patients younger and older than 65. METHODS: The study draws from the county-based prospective myasthenia gravis register implemented by the Neurology Department at Hospital General de Vic in 1991. RESULTS: The prevalence of myasthenia gravis was 32.89×105 inhabitants (95%CI, 23.86-41.91). The standardized prevalence (European population) was 35.47×105 inhabitants (95%CI, 26.10-44.84). The ratio of women to men was 1.3. Overall, the group of patients older than 65 accounted for 62.75% of all cases. The prevalence of myasthenia gravis increased considerably in older age groups. No cases were registered among patients under 25years old, prevalence was 21.87×105 in the 25 to 64 age group, and prevalence in patients over 65 years increased to 122.35×105. The clinical characteristics prior to treatment and at the cut-off date are similar (P>.05) in patients younger than 65 and those aged 65 and older. CONCLUSIONS: These figures show the highest prevalence rate reported to date. This high prevalence is due to the rate observed among patients older than 65. These results provide a new warning that myasthenia gravis may be underdiagnosed in the elderly population.

Occupational exposure to volatile anaesthetics: a systematic review.

BACKGROUND: The effects of long-term occupational exposure to small concentrations of volatile anaesthetics on health professionals are still uncertain despite the research literature available on this subject. AIMS: To analyse the existing literature on the health effects of volatile anaesthetics on exposed health professionals. METHODS: We performed a systematic review from March 2013 to January 2014. The literature was searched in the Medline and Cochrane libraries using the following keywords: 'Anaesthetics AND occupational health', 'Volatile anaesthetics AND occupational health', 'Sevoflurane AND occupational health' and 'Occupational surveillance AND anaesthetics', with no time limit. We used the Scottish Intercollegiate Guidelines Network to evaluate the quality of studies and to grade the recommendations. RESULTS: From 1429 articles retrieved from the databases and an additional 20 obtained from secondary sources, we excluded 1391 (95.9%). After excluding duplicate publications, we finally included 17 articles in the review. CONCLUSIONS: Evidence for adverse effects of volatile anaesthetics on exposed personnel is scarce and inconsistent, but there is no evidence of adverse effects when environmental levels are kept within legal threshold values. Further studies are needed to improve our knowledge of the effects of occupational exposure to volatile anaesthetics. New surveillance methods that include systematic data collection, clinical signs and biomarkers of exposure are required to formulate consistent and reproducible surveillance criteria for exposed personnel.

Myasthenia gravis: a higher than expected incidence in the elderly.

This 10-year (1991 to 2000) prospective study of MG in the county of Osona (Barcelona, Spain) reveals an annual incidence rate of 21.27 cases per million inhabitants (95% CI 13.89 to 31.16). Incidence increased from 5.03 x 10(6) in the age group of 0 to 14 years to 14.68 x 10(6) in the age group of 15 to 64 years and to 63.38 x 10(6) in the older population. These results, the highest reported to date, may be explained by the population aging.

[Painful bilateral lumbar radiculoplexopathy associated with Epstein-Barr virus infection]. / Radiculoplexopathie lombaire douloureuse bilatérale associée à une infection par le virus d'Epstein-Barr.

We report a case of polyneuropathy caused by primary Epstein Barr virus (EBV) infection in a 57-year-old patient. The primary EBV infection was confirmed by serology tests and EBNA (Epstein-Barr nuclear antigen) seroconversion. The main clinical sign was a highly painful subacute, bilateral lumbar radiculoplexopathy with amyotrophy which responded to corticosteroids and complement treatment within a few months. The association of left facial paralysis, neurophysiological signs of polyradiculopathy and elevated protein levels in the cerebrospinal fluid demonstrate the variability of peripheral neurological involvement in the same subject with EBV infection. This case also demonstrates the poorly limits of dysimmune polyneuropathies, including lumbar radiculoplexopathy which can be considered as an exceptional variant.

[Deletion of 17p11.2 chromosome in Spanish families with hereditary neuropathy and abnormal sensitivity to pressure]. / Deleción del cromosoma 17p11.2 en familias españolas con neuropatía hereditaria por vulnerabilidad excesiva a la presión.

Hereditary neuropathy with abnormal liability to pressure palsies (HNPP) is a dominant autosomally transmitted disease that gives rise to foci of peripheral nerve myelination, reducing conduction and leading to episodes of palsy and sensory changes that are all linked to sensitivity to pressure and traction on the affected nerve roots. The molecular basis of HNPP has been identified as a submicroscopic deletion of the 17p11.2 chromosome in exactly the same region that it is duplicated in Charcot-Marie-Tooth disease, type 1A (CMT1A). We report genetic analyses of 13 patients (belonging to 3 families) diagnosed of HNPP by means of physical examination and electrophysiologic and morphologic tests (the last in 3 cases only). Inter- and intrafamilial variation in symptomatology was studied. Some patients presented the usual clinical signs, such as recidivating brachial plexus palsy, permanent sensory polyneuropathy, foot deformities and others that might also be found in patients with CMT1A. All the patients showed electrophysiologic signs of underlying demyelinating polyneuropathy. Genetic study centered on detecting the deletion of 17p11.2 by segregation analysis with the polymorphic markers VAW409R3a (D17S122) and EW401HE (D17S61). Our results confirmed deletion at the CTM1A location of chromosome 17p11.2 in all 13 patients examined. These data suggest that the deletion of 17p11.2 plays a causal role in HNPP and that it is the most prevalent mutation in this disease; our findings constitute new evidence of the importance of the CMT1A/HNPP locus in the formation and control of peripheral myelin and in the ultimate functioning of peripheral nerves.

Migraine: an alternative in the diagnosis of unclassified vertigo.

Vertigo and unsteadiness are frequent reasons for medical consultation. In some cases, these symptoms remain unclassified. The association of equilibrium disorders with migraine is often mentioned in literature. Seventy-two cases of unclassified vertigo were studied in order to ascertain the prevalence of migraine in patients affected by recurring episodes of vertigo from undetermined causes, and attempting to establish a possible relationship with migraine. Characteristics of the vertigo and the headache, were evaluated by clinical history and examination, electronystagmogram (ENG), electroencephalogram (EEG), computerized tomography scanning (CT) and/or magnetic resonance imaging (MRI). In the group of patients studied 50% suffered from headache, and 32.8% fulfilled the diagnostic criteria of migraine. Results suggest that only a thorough clinical history would be able to give enough information to establish the diagnosis of migraine in these patients. These observations imply an alternative diagnosis to be taken into account when a case of unclassified vertigo is evaluated.

[Lumbosacral spinal cord infarction. Data from magnetic resonance imaging]. / Infarctus de la moelle lombosacrée. Données de l'imagerie par résonance magnétique.

A 77-year old woman and a 61-year old man with lumbosacral spinal cord ischaemic lesions and paraplegia presented with dissociated or partial forms of the lumbar enlargement artery (Adamkiewicz's artery) syndrom. Magnetic resonance imaging (MRI) was performed in both patient with an 0.15 Tesla resistive magnet. A hypersignal on T2-weighted images was present in both cases, which corresponded to the infarction affecting the lumbosacral part of the cord. MRI provided accurate delineation of the lesions and clinicoradiological correlations in both patients. The hypersignal occupied the lumbar cord in case 1 and the conus medullaris in case 2. The clinical signs and location of the lesions seemed to be determined by the different patterns of lumbosacral arterial supply. Since such images could be confused with tumoral images, surgical exploration was carried out in both patients; it failed to demonstrate any tumour or cyst and only showed a pale cord. Surgical cord biopsy in case 2 demonstrated ischaemic tissue. We conclude that ischaemia of the spinal cord can be demonstrated by MRI, but owing to the low specificity of this imaging method aedema cannot be distinguished from necrosis, gliosis or demyelination.

The infrapiriformis foramen syndrome resulting from intragluteal injection.

The authors describe a syndrome resulting from a lesion at the level of the infrapiriform foramen due to intramuscular injection, with involvement of the sciatic nerve, the inferior gluteal nerve, the posterior femoral cutaneous nerve and the pudendal nerve. This appears to be the first case of a complete syndrome of the infrapiriform foramen to be reported. In a second case the pudendal nerve was not involved and a releasing neurolysis was performed with remarkable postoperative relief. All the reported cases with involvement of the inferior gluteal nerve are associated with a more or less complete injury of the sciatic nerve. Conversely, the cases of injection injury to the superior gluteal nerve are rarely associated with injury of the sciatic nerve. The anatomic reasons that might explain both kinds of lesion are given. Finally some aspects of 131 cases of sciatic paralysis resulting from injection are also examined.