Estudio prospectivo y multicéntrico de la epidemiología de las infecciones del sistema nervioso central (meningitis y encefalitis) en los servicios de urgencias hospitalarios: análisis de subgrupo del estudio INFURG-SEMES / Multicentre prospective study of the epidemiology of central nervous system infections (meningitis and encephalitis) in emergency departments: subgroup analysis from the INFURG-SEMES Study

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Possible case of peripheral osmotic demyelination syndrome.

Central pontine myelinolysis (CPM) is an uncommon neurological syndrome that is usually related to the rapid restoration of a previous hyponatraemia. Although the most frequent location of CPM injury is the pons, it is now designated osmotic demyelination syndrome (ODS) because, as well as in the brainstem, these injuries can be observed in other parts of the central nervous system (CNS)-for example, the thalamus, subthalamic nucleus, external geniculate body, putamen, globus pallidum, internal capsule, white matter of cerebellum and the deep layers of the brain cortex. However, an exhaustive search of the literature (MEDLINE 1967-2007) has revealed no case report of peripheral nervous system (PNS) demyelination secondary to severe hyponatraemia.

[Trigeminal-autonomic cephalalgias: International Headache Society diagnostic criteria does not allow for a correct classification]. / Cefaleas trigeminoautonómicas: los criterios diagnósticos de la International Headache Society no permiten su clasificación correctamente.

INTRODUCTION: The International Classification of Headache Disorders only recognizes the following as trigeminal- autonomic cephalalgias (TAC): cluster headache, paroxysmal hemicrania and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome. Nevertheless, there are other types of TAC that still have not been incorporated into the International Classification of Headache Disorders although they have been before reported in the literature. We present the results of the analysis of a series of 94 cases of TAC. METHODS: We performed a retrospective study of 2,132 patient who attended a general neurology consultation due to headache between January 1997 and June 2006. Those patients with unilateral headache (orbital, supraorbital y/o temporal) accompanied of some ipsilateral autonomic sign were selected. We investigated the etiology of all cases and made a nosology classification according with two types of criteria: IHS (International Headache Society) strict criteria as well as other criteria (IHS plus) including migraine with ipsilateral autonomic signs and hemicrania continua, thus following Goabsby. RESULTS: A total of 94 cases of TAC (4.4% of all the headaches) were found, 89 of which had a primary etiology and 5 secondary etiology. Cluster headache was the most frequent TAC in our series (29%), independently of the criteria used. However, hemimigraine with ipsilateral autonomic signs had a similar frequency (28%) according to IHS plus criteria. CONCLUSIONS: a) In our series TAC have constituted an not very frequent entity, there being a subgroup of secondary cases among them, and b) the current International Classification of the Headache Disorders has some limitations as an instrument for the nosology classification of TAC.

Cefaleas trigeminoautonómicas: los criterios diagnósticos de la International Headache Society no permiten su clasificación correctamente / Trigeminal-autonomic cephalalgias: International Headache Society diagnostic criteria does not allow for a correct classification

Introducción. La Clasificación Internacional de las Cefaleasde la International Headache Society (IHS) sólo reconoce comocefaleas trigeminoautonómicas (CTA) la cefalea en racimos, lahemicránea paroxística y la cefalea SUNCT (short-lasting unilateralneuralgiform headache attacks with conjunctival injectionand tearing). No obstante, existen otras cefaleas hemicranealescon signos autonómicos ipsilaterales aún no incluidas a pesar dehaber sido ampliamente descritas en la literatura. Se presentanlos resultados del análisis de una serie de 94 casos de CTA.Métodos. Estudio retrospectivo de 2.132 pacientes queacudieron por cefalea a una consulta de neurología generalentre enero de 1997 y junio de 2006. Se seleccionaron aquellospacientes que sufrían una cefalea unilateral de localizaciónorbitaria, supraorbitaria y/o temporal acompañada de algúnsigno autonómico ipsilateral. Se realizó una clasificaciónetiológica de todas las CTA y una nosológica de las primarias.Esta última se llevó a cabo de acuerdo con dos tipos de criterios:IHS estricta e IHS plus (incluyendo migraña con signosautonómicos y criterios de Goabsby para hemicránea continua).Resultados. Encontramos 94 casos de CTA (4,4 % deltotal de cefaleas), 89 de etiología primaria y 5 secundaria.La cefalea en racimos fue la CTA más frecuente en nuestraserie (29 %), independientemente de los criterios utilizados,aunque la hemicránea continua y la migraña con signosautonómicos presentaron una frecuencia similar (28%) cuandose aplicaron los criterios IHS plus.Conclusiones. a) En nuestra serie las CTA han constituidouna entidad poco frecuente, existiendo entre ellas unsubgrupo de casos secundarios, y b) la actual ClasificaciónInternacional de las Cefaleas de la IHS tiene limitaciones paradistribuir nosológicamente las CTA (AU)

Introduction. The International Classification ofHeadache Disorders only recognizes the following as trigeminal-autonomic cephalalgias (TAC): cluster headache,paroxysmal hemicrania and short-lasting unilateralneuralgiform headache attacks with conjunctival injectionand tearing (SUNCT) syndrome. Nevertheless, thereare other types of TAC that still have not been incorporatedinto the International Classification of HeadacheDisorders although they have been before reported in theliterature. We present the results of the analysis of a seriesof 94 cases of TAC.Methods. We performed a retrospective study of2,132 patient who attended a general neurology consultationdue to headache between January 1997 and June2006. Those patients with unilateral headache (orbital,supraorbital y/o temporal) accompanied of some ipsilateralautonomic sign were selected. We investigated theetiology of all cases and made a nosology classificationaccording with two types of criteria: IHS (InternationalHeadache Society) strict criteria as well as other criteria(IHS plus) including migraine with ipsilateral autonomicsigns and hemicrania continua, thus following Goabsby.Results. A total of 94 cases of TAC (4.4 % of all theheadaches) were found, 89 of which had a primary etiologyand 5 secondary etiology. Cluster headache was themost frequent TAC in our series (29 %), independently ofthe criteria used. However, hemimigraine with ipsilateralautonomic signs had a similar frequency (28 %) accordingto IHS plus criteria.Conclusions. a) In our series TAC have constitutedan not very frequent entity, there being a subgroup ofsecondary cases among them, and b) the current InternationalClassification of the Headache Disorders has somelimitations as an instrument for the nosology classificationof TAC (AU)

[Advances in our knowledge of the causation and pathophysiology of reflex epilepsies]. / Avances en el conocimiento de la etiología y la fisiopatología de las epilepsias reflejas.

AIMS: In recent years our knowledge of the pathophysiology and aetiology of reflex seizures and epilepsies has advanced significantly due to the contributions made by modern functional neuroimaging and neurophysiology techniques, as well as the findings of research being conducted in molecular biology. The aim of our review is to summarise and integrate these contributions in order to describe the current state of our knowledge on the subject. DEVELOPMENT: The fundamental pathophysiological pattern is common to all types of reflex epilepsy and is based on the existence of a hyperexcitable cortical or subcortical neuronal area that can respond to a physiological stimulus in an exaggerated manner. This focal hyperexcitability may derive from a predisposing genetic substrate (idiopathic reflex epilepsy) or from a focal cortical lesion (lesional reflex epilepsy). Some genetic substrates have been reported in isolated families and there are many candidate genes, but we still do not know enough about the genetic base. The lesion-based aetiology is very heterogeneous, but disorders affecting cortical development are the lesions with the greatest capacity to cause reflex epilepsy. Clinical expression is conditioned by the topography of the lesion and not by the type of underlying lesion. CONCLUSIONS: Future characterisation of reflex epileptic seizures and syndromes must include a diagnosis centred around three axes, that is, topographic, lesional and genetic.

Avances en el conocimiento de la etiología y la fisiopatología de las epilepsias reflejas / Advances in our knowledge of the causation and pathophysiology of reflex epilepsies

Objetivo. En los últimos años nuestro conocimiento dela fisiopatología y la etiología de las crisis y epilepsias reflejas haavanzado sustancialmente gracias a las aportaciones de las modernastécnicas de neuroimagen funcional y neurofisiología, asícomo de las investigaciones en biología molecular. El objetivo denuestra revisión es realizar una síntesis e integración de estasaportaciones, dibujando el estado actual de nuestros conocimientosen este tema. Desarrollo. El esquema fisiopatológico básico escomún para todos los tipos de epilepsias reflejas y se basa en laexistencia de un área neuronal hiperexcitable, cortical o subcortical,capaz de responder de forma exagerada a un estímulo fisiológico.Esta hiperexcitabilidad focal puede derivar de un sustratogenético predisponente (epilepsia refleja idiopática) o de una lesióncortical focal (epilepsia refleja lesional). Se han descrito algunossustratos genéticos en familias aisladas y hay muchos genescandidatos, pero nuestro conocimiento de la base genética es aúninsuficiente. La etiología lesional es muy heterogénea, pero lostrastornos del desarrollo cortical son las lesiones con mayor capacidadepileptogénica refleja. La expresión clínica está condicionadapor la topografía lesional y no por el tipo de lesión subyacente.Conclusión. La caracterización de las crisis y los síndromes epilépticosreflejos en el futuro debe incluir un diagnóstico en tres ejes:topográfico, lesional y genético

Aims. In recent years our knowledge of the pathophysiology and aetiology of reflex seizures and epilepsies hasadvanced significantly due to the contributions made by modern functional neuroimaging and neurophysiology techniques, aswell as the findings of research being conducted in molecular biology. The aim of our review is to summarise and integratethese contributions in order to describe the current state of our knowledge on the subject. Development. The fundamentalpathophysiological pattern is common to all types of reflex epilepsy and is based on the existence of a hyperexcitable corticalor subcortical neuronal area that can respond to a physiological stimulus in an exaggerated manner. This focal hyperexcitabilitymay derive from a predisposing genetic substrate (idiopathic reflex epilepsy) or from a focal cortical lesion(lesional reflex epilepsy). Some genetic substrates have been reported in isolated families and there are many candidate genes,but we still do not know enough about the genetic base. The lesion-based aetiology is very heterogeneous, but disordersaffecting cortical development are the lesions with the greatest capacity to cause reflex epilepsy. Clinical expression isconditioned by the topography of the lesion and not by the type of underlying lesion. Conclusions. Future characterisation ofreflex epileptic seizures and syndromes must include a diagnosis centred around three axes, that is, topographic, lesional andgenetic

[Acute disseminated encephalomyelitis due to hymenoptera stings]. / Encefalomielitis aguda diseminada por picadura de himenóptero.

INTRODUCTION: Hymenoptera stings can give rise to a variety of conditions. The most common is a self-limiting local allergic reaction, but occasionally they may produce potentially serious systemic reactions. Neurological complications are rare, but very varied and of differing degrees of severity. Some such consequences are those of a demyelinating nature, which can affect the central and/or peripheral nervous system. Their exact pathogenesis remains unknown but an autoimmune mechanism has been suggested. CASE REPORT: We report the case of a 28-year-old female who had a clinical picture compatible with acute disseminated encephalomyelitis ten days after being stung several times by a bumblebee. The diagnosis was based on findings from magnetic resonance imaging and on the clinical-radiological course after a one-year follow-up. The patient was treated with massive doses of intravenous corticosteroids and immunoglobulins, which brought about partial recovery from the neurological deficit. CONCLUSIONS: Hymenoptera stings can produce demyelinating neurological complications, presumably related to the autoimmune system, which could benefit from treatment with immunomodulators such as corticosteroids, immunoglobulins or plasmapheresis.

Encefalomielitis aguda diseminada por picadura de himenóptero / Acute disseminated encephalomyelitis due to hymenoptera stings

Introducción. Las picaduras por himenópteros pueden producir diversas manifestaciones. La más común es una reacción alérgica local autolimitada, pero inusualmente pueden dar lugar a reacciones sistémicas potencialmente graves. Las complicaciones neurológicas son raras aunque muy variadas y de diversa consideración. Entre ellas de encuentran aquellas de naturaleza desmielinizante que pueden afectar al sistema nervioso central y/o periférico. Su patogenia no se conoce aunque se ha postulado un mecanismo autoinmune. Caso clínico. Mujer de 28 años que presentó unc uadro compatible con una encefalomielitis aguda diseminada diez días después de sufrir varias picaduras de abejorro. El diagnóstico se basó en los hallazgos de resonancia magnética y en la evolución clínico-radiológica al cabo de un año de seguimiento. Se trató con megadosis intravenosas de corticoesteroides e inmunoglobulinas obteniendo una recuperación parcial del déficit neurológico. Conclusiones. Las picaduras de himenóptero pueden producir complicaciones neurológicas desmielinizantes, de presumible naturaleza autoinmune, que se podrían beneficiar de tratamientos inmuno-moduladorestales como corticoesteroides, inmunoglobulinas o plasmaféresis (AU)

Introduction. Hymenoptera stings can give rise to a variety of conditions. The most common is a self-limiting local allergic reaction, but occasionally they may produce potentially serious systemic reactions. Neurological complications are rare, but very varied and of differing degrees of severity. Some such consequences are those of a demyelinating nature, which can affect the central and/or peripheral nervous system. Their exact pathogenesis remains unknown but an autoimmune mechanism has been suggested. Case report. We report the case of a 28-year-old female who had a clinical picture compatible with acute disseminated encephalomyelitis ten days after being stung several times by a bumblebee. The diagnosis was based on findings from magnetic resonance imaging and on the clinical-radiological course after a one-year follow-up. The patient was treated with massive doses of intravenous corticosteroids and immunoglobulins, which brought about partial recovery from the neurological deficit. Conclusions. Hymenoptera stings can produce demyelinating neurological complications, presumably related to the autoimmune system, which could benefit from treatment with immunomodulators such as corticosteroids, immunoglobulins or plasmapheresis (AU)