1st Post-European Headache Federation Meeting: a review of the latest developments presented at the 2020 European Headache Federation Congress. / I Reunión Post-European Headache Federation: revisión de las novedades presentadas en el Congreso de la European Headache Federation de 2020.

INTRODUCTION: After the European Headache Federation (EHF) Congress, renowned Spanish neurologists specialised in migraine presented the most significant latest developments in research in this field at the Post-EHF Meeting. DEVELOPMENT: The main data presented concerning the treatment of chronic and episodic migraine were addressed, with attention paid more specifically to those related to preventive treatments and real-life experience in the management of the disease. An important review was carried out of the new therapeutic targets and the possibilities they offer in terms of understanding the pathophysiology of migraine and its treatment. An update was also presented of the latest developments in the treatment of migraine with fremanezumab, a monoclonal antibody recently authorised by the European Medicines Agency. Participants were also given an update on the latest developments in basic research on the pathology, as well as an overview of the symptoms of migraine and COVID-19. Finally, the repercussions of migraine in terms of its burden on the care and economic resources of the health system were addressed, along with its impact on society. CONCLUSIONS: The meeting summarised the content presented at the 14th EHF Congress, which took place in late June/early July 2020.

TITLE: I Reunión Post-European Headache Federation: revisión de las novedades presentadas en el Congreso de la European Headache Federation de 2020.Introducción. Tras la celebración del congreso de la European Headache Federation (EHF), reconocidos neurólogos españoles expertos en el tratamiento de la migraña expusieron en la Reunión Post-EHF las principales novedades presentadas en el congreso y relacionadas con ese ámbito. Desarrollo. Se abordan los principales datos presentados relacionados con el tratamiento de la migraña crónica y episódica; concretamente, los relacionados con los tratamientos preventivos y la experiencia en vida real en el manejo de la enfermedad. Se hizo una importante revisión de las nuevas dianas terapéuticas y las posibilidades que ofrecen en cuanto al conocimiento de la fisiopatología de la migraña y su tratamiento. Asimismo, se hizo una actualización de las novedades presentadas en el tratamiento de la migraña con fremanezumab, anticuerpo monoclonal recientemente autorizado por la Agencia Europea de Medicamentos. Se hizo una actualización de las novedades en investigación básica en la patología, así como una relación de los síntomas de migraña y COVID-19. Finalmente, se abordaron las implicaciones de la migraña en la carga sanitaria asistencial y económica, y su impacto en la sociedad. Conclusiones. En la reunión se hizo un resumen del contenido presentado en el 14 Congreso de la EHF, que tuvo lugar a finales de junio y principios de julio de 2020.

I Reunión Post-European Headache Federation: revisión de las novedades presentadas en el Congreso de la European Headache Federation de 2020 / 1st Post-European Headache Federation Meeting: a review of the latest developments presented at the 2020 European Headache Federation Congress

Introducción: Tras la celebración del congreso de la European Headache Federation (EHF), reconocidos neurólogos españoles expertos en el tratamiento de la migraña expusieron en la Reunión Post-EHF las principales novedades presentadas en el congreso y relacionadas con ese ámbito. Desarrollo: Se abordan los principales datos presentados relacionados con el tratamiento de la migraña crónica y episódica; concretamente, los relacionados con los tratamientos preventivos y la experiencia en vida real en el manejo de la enfermedad. Se hizo una importante revisión de las nuevas dianas terapéuticas y las posibilidades que ofrecen en cuanto al conocimiento de la fisiopatología de la migraña y su tratamiento. Asimismo, se hizo una actualización de las novedades presentadas en el tratamiento de la migraña con fremanezumab, anticuerpo monoclonal recientemente autorizado por la Agencia Europea de Medicamentos. Se hizo una actualización de las novedades en investigación básica en la patología, así como una relación de los síntomas de migraña y COVID-19. Finalmente, se abordaron las implicaciones de la migraña en la carga sanitaria asistencial y económica, y su impacto en la sociedad. Conclusiones: En la reunión se hizo un resumen del contenido presentado en el 14 Congreso de la EHF, que tuvo lugar a finales de junio y principios de julio de 2020.(AU)

Introduction: After the European Headache Federation (EHF) Congress, renowned Spanish neurologists specialised in migraine presented the most significant latest developments in research in this field at the Post-EHF Meeting. Development: The main data presented concerning the treatment of chronic and episodic migraine were addressed, with attention paid more specifically to those related to preventive treatments and real-life experience in the management of the disease. An important review was carried out of the new therapeutic targets and the possibilities they offer in terms of understanding the pathophysiology of migraine and its treatment. An update was also presented of the latest developments in the treatment of migraine with fremanezumab, a monoclonal antibody recently authorised by the European Medicines Agency. Participants were also given an update on the latest developments in basic research on the pathology, as well as an overview of the symptoms of migraine and COVID-19. Finally, the repercussions of migraine in terms of its burden on the care and economic resources of the health system were addressed, along with its impact on society. Conclusions: The meeting summarised the content presented at the 14th EHF Congress, which took place in late June/early July 2020.(AU)

Brain iron accumulation in a blood donor family with restless legs syndrome. / Aumento de los depositos cerebrales de hierro en una familia de donantes de sangre con sindrome de piernas inquietas.

INTRODUCTION: The pathophysiology of restless legs syndrome (RLS) is complex. Secondary RLS with iron deficiency -which suggests disturbed iron homeostasis- remains to be elucidated. CASE REPORTS: We report the findings from a unique blood donor family with RLS. Three blood donors family members were diagnosed with RLS defined by the International RLS Study Group and without history of neurologic diseases and RLS symptoms in the last 3-5 years (range of blood donation: 10-40 years). The neurological examination and electromyographies were normal. A polisomnography showed disturbed nocturnal sleep with a reduction in sleep efficiency and an increased periodic limbs movement index. The cranial MRI showed brain iron deposits in basal ganglia, substantia nigra, red nuclei and dentate nuclei. Phenotypic and genotypic studies rule out genetic haemochromatosis or iron overload. CONCLUSION: The abnormal iron accumulation in the basal ganglia indicated a complex iron metabolism disorder of the central nervous system. Further studies are warranted to confirm our findings and its role in the pathophysiology of RLS.

TITLE: Aumento de los depositos cerebrales de hierro en una familia de donantes de sangre con sindrome de piernas inquietas.Introduccion. La fisiopatologia del sindrome de piernas inquietas (SPI) es compleja. El mecanismo a traves del cual la ferropenia favorece el desarrollo del SPI no esta esclarecido, aunque se sugiere la presencia de una alteracion en la homeostasis cerebral del hierro. Casos clinicos. Se presentan los hallazgos inusuales en una familia de donantes de sangre con SPI. Tres miembros de la misma familia fueron diagnosticados de SPI, cumpliendo los criterios definidos por el grupo internacional para el estudio del SPI (International Restless Legs Syndrome Study Group). Todos eran donantes de sangre habituales (rango de donacion: 10-40 años) y los sintomas de SPI tenian un curso de 3-5 años. La exploracion general y neurologica fue normal en todos los casos, asi como los electromiogramas. El estudio fenotipico y genotipico descarto la presencia de hemocromatosis y otras causas geneticas de sobrecarga cerebral de hierro. Los estudios polisomnograficos mostraron sueño nocturno perturbado, con reduccion de su eficiencia, y un aumento del indice de movimientos periodicos de las piernas. La resonancia magnetica craneal evidencio un aumento de los depositos cerebrales de hierro en los ganglios basales, la sustancia negra, el nucleo rojo y los dentados. Conclusion. Este aumento patologico de los depositos cerebrales de hierro sugiere la presencia de un complejo trastorno del metabolismo cerebral del hierro en nuestros pacientes. Futuros estudios deben confirmar estos hallazgos y profundizar en el estudio de su relacion con la fisiopatologia del SPI.

Transcriptional Regulation of Glutamate Transporters: From Extracellular Signals to Transcription Factors.

Glutamate is the predominant excitatory neurotransmitter in the mammalian CNS. It mediates essentially all rapid excitatory signaling. Dysfunction of glutamatergic signaling contributes to developmental, neurologic, and psychiatric diseases. Extracellular glutamate is cleared by a family of five Na(+)-dependent glutamate transporters. Two of these transporters (GLAST and GLT-1) are relatively selectively expressed in astrocytes. Other of these transporters (EAAC1) is expressed by neurons throughout the nervous system. Expression of the last two members of this family (EAAT4 and EAAT5) is almost exclusively restricted to specific populations of neurons in cerebellum and retina, respectively. In this review, we will discuss our current understanding of the mechanisms that control transcriptional regulation of the different members of this family. Over the last two decades, our understanding of the mechanisms that regulate expression of GLT-1 and GLAST has advanced considerably; several specific transcription factors, cis-elements, and epigenetic mechanisms have been identified. For the other members of the family, little or nothing is known about the mechanisms that control their transcription. It is assumed that by defining the mechanisms involved, we will advance our understanding of the events that result in cell-specific expression of these transporters and perhaps begin to define the mechanisms by which neurologic diseases are changing the biology of the cells that express these transporters. This approach might provide a pathway for developing new therapies for a wide range of essentially untreatable and devastating diseases that kill neurons by an excitotoxic mechanism.

[Quality of neurological care in the emergency services: a study from the community-hospital]. / Calidad de la asistencia neurológica en urgencias: un estudio desde el área.

INTRODUCTION: The medical conditions shared by hospital emergency services and community-hospital neurology clinics (CHNC) have not been described, and the quality of the medical care received in these conditions has not been evaluated in our environment. METHODS: Over a 2 month period, those patients presenting at any of the seven CHNC in a Health Care Area 1 of Madrid due to previously attended medical conditions in the emergency services were systematically registered. The area neurologists of the CHNC collected administrative and clinical variables and made a judgment on the medical care (primary outcome measure) and diagnoses (secondary outcome measure) received. RESULTS: A total of 181 patients were included (mean age: 58 years; 60% women). The inclusion rate was one patient per working day, and 31% of patients were visited out of the established quota number of patients for the clinic. The most frequent reasons for visiting the emergency room were: headache (20%), focal neurological syndrome (16%) and loss of consciousness (14%). The most frequent diagnoses at the CHNC were: primary headache (19 %), stroke (11%) and epilepsy (9 %). Emergency care was deemed correct in 56 % of patients. When the patients with intervention were compared to those with no intervention, participation of the neurology service in the emergency room was associated to a greater percentage of correct diagnoses (59% vs. 41%; p=0.019) and care (69% vs. 47%; p=0.003). CONCLUSIONS: The medical conditions shared with the emergency services represent a small but relevant proportion of the patients assisted in the CHNC. Some of these conditions (primary headaches, syncopes) should be canalized into primary health care. Others (epilepsy) require a circuit between emergency room and CNNC, but the appointment system should be adapted. The intervention of a neurologist in the emergency room raises the quality of the care.

Calidad de la asistencia neurológica en urgencias: un estudio desde el área / Quality of neurological care in the emergency services: a study from the community-hospital

Introducción. Los procesos compartidos por los servicios hospitalarios de urgencias y las consultas de neurología del área sanitaria (CNA) no han sido descritos y su calidad asistencial no ha sido evaluada en nuestro medio. Métodos. Durante 2 meses se registraron todos los pacientes que acudieron a las siete CNA 1 de Madrid debido a procesos médicos que habían sido previamente atendidos en urgencias. Los neurólogos del área sanitaria recogieron variables organizativas y clínicas y emitieron un juicio acerca de la asistencia (variable de efecto primaria) y del diagnóstico (variable de efecto secundaria) realizados. Resultados. Fueron incluidos 181 pacientes (edad media: 58 años; 60% mujeres), a un promedio de un paciente por día de consulta. El 31% estaban citados fuera del cupo. Los motivos más frecuentes de la visita a urgencias habían sido: cefalea (20%), focalidad neurológica (16 %) y pérdida de conciencia (14 %). Los diagnósticos más frecuentes en la CNA fueron: cefalea primaria (19%), accidente cerebrovascular (11%) y epilepsia (9 %). La asistencia en urgencias fue correcta en el 56% de los pacientes. Comparado con los procesos en que no intervino, la participación de neurología se asoció a un mayor porcentaje de diagnóstico y de asistencia correctos (59 frente a 41%; p=0,019, y 69 frente a 47%; p=0,003, respectivamente). Conclusiones. Los procesos compartidos con urgencias suponen una proporción pequeña pero no desdeñable del total de pacientes atendidos en las CNA. Algunos de estos procesos (cefaleas primarias, síncopes) deberían canalizarse hacia la asistencia primaria. Otros (epilepsia) precisan del circuito urgencias-CNA, pero el sistema de citaciones debe adaptarse. La actuación del neurólogo en urgencias eleva la calidad asistencial (AU)

Introduction: The medical conditions shared by hospital emergency services and community-hospital neurology clinics (CHNC) have not been described, and the quality of the medical care received in these conditions has not been evaluated in our environment. Methods: Over a 2 month period, those patients presenting at any of the seven CHNC in a Health Care Area 1 of Madrid due to previously attended medical conditions in the emergency services were systematically registered. The area neurologists of the CHNC collected administrative and clinical variables and made a judgment on the medical care (primary outcome measure) and diagnoses (secondary outcome measure) received. Results: A total of 181 patients were included (mean age: 58 years; 60% women). The inclusion rate was one patient per working day, and 31% of patients were visited out of the established quota number of patients for the clinic. The most frequent reasons for visiting the emergency room were: headache (20%), focal neurological syndrome (16%) and loss of consciousness (14%). The most frequent diagnoses at the CHNC were: primary headache (19 %), stroke (11%) and epilepsy (9 %). Emergency care was deemed correct in 56 % of patients. When the patients with intervention were compared to those with no intervention, participation of the neurology service in the emergency room was associated to a greater percentage of correct diagnoses (59% vs. 41%; p=0.019) and care (69% vs. 47%; p=0.003). Conclusions: The medical conditions shared with the emergency services represent a small but relevant proportion of the patients assisted in the CHNC. Some of these conditions (primary headaches, syncopes) should be canalized into primary health care. Others (epilepsy) require a circuit between emergency room and CNNC, but the appointment system should be adapted. The intervention of a neurologist in the emergency room raises the quality of the care (AU)

Nummular headache secondary to an intracranial mass lesion.

Nummular headache is a coin-shaped, chronic cephalalgia usually considered to stem from epicranial tissues. We describe a patient complaining of circumscribed pain in the head as the only symptom of a subtentorial meningioma. This observation underlines the need to revise the concept of circumscribed, referred pains in the head arising from pain-sensitive intracranial structures.

[Long-term risk of relapses in Tolosa-Hunt syndrome]. / Riesgo de recaídas a largo plazo en el síndrome de Tolosa-Hunt.

INTRODUCTION: The reviewed diagnostic criteria of Tolosa- Hunt síndrome (THS) by the International Classification of Headache Disorders (ICHD-II, 2004) includes demonstration of inflammatory changes by magnetic resonance imaging (MRI) in the cavernous sinus region as an alternative to biopsy. Careful follow-up is required to exclude other possible causes of painful ophthalmoplegia. It remains unclear for how long such close observation should be extended to assure the diagnosis nor whether THS represents a self-limited condition in the long-term. CASE REPORT: An observational study along 13 years of a patient fulfilling current ICHD-II criteria for THS to clarify the natural history of the disorder. RESULTS: We witnessed three episodes, the presenting one causing severe left orbital pain, ipsilateral abducens palsy , and hypoesthesia in the territory of the ophthalmic branch of the trigeminal nerve. Gadolinium MRI showed enhancement of tissue infiltrating the lateral wall of the carotid sinus. A relapse 21 months later caused involvement of left oculomotor and abducens nerves in addition to orbital pain which responded rapidly to steroids. Ensuing remission maintained for up to 11 years concluded in a new relapse again characterized by severe ipsilateral orbital pain and frontal numbness, but no ophthalmoplegia. A gadolinium-MRI did not show enhancement on this occasion despite prompt response to steroids. CONCLUSION: The risk of relapses in THS and its response to steroids may be maintained for as long as 13 years.

Riesgo de recaídas a largo plazo en el síndrome de Tolosa-Hunt / Long-term risk os relapses in Tolosa-Hunt syndrome

Introducción. Los criterios revisados por la International Classification of Headache Disorders (ICHD-II, 2004) para el diagnóstico del síndrome de Tolosa-Hunt (STH) exigen demostrar cambios inflamatorios en el seno cavernoso mediante imágenes por resonancia magnética (RM) como alternativa a la biopsia, pero se requiere un seguimiento cuidadoso del paciente que permita excluir otras causas de oftalmoplegía dolorosa. No está clara su duración ni si el proceso es autolimitado en el tiempo. Caso clínico. Estudio observacional a lo largo de 13 años de una paciente que cumplía criterios ICHD-II a fin de clarificar la historia natural del proceso. Resultados. Observamos tres episodios: el primero iniciado con intenso dolor orbitario izquierdo, seguido de parálisis ipsolateral del nervio motor ocular externo e hipoestesia en el territorio de la rama oftálmica del trigémino. Una RM con gadolinio mostró captación por un tejido que infiltraba la pared lateral del seno carotídeo. Sufrió una recaída 21 meses después; ocasiona ahora parálisis de los nervios motor ocular común y motor ocular externo izquierdo, además de dolor orbitario, con respuesta rápida al tratamiento esteroideo. Tras una remisión prolongada 11 años, una nueva recaída se caracterizó nuevamente por intenso dolor orbitario y acorchamiento frontal ipsolateral izquierdos, pero no oftalmoplegía. Una RM con gadolinio no mostró realce en esta ocasión pese a una pronta respuesta a los corticoides. Conclusiones. El riesgo de recaídas en el STH se prolonga al menos durante 13 años, manteniéndose una respuesta favorable a los corticoides (AU)

Introduction: The reviewed diagnostic criteria of Tolosa- Hunt síndrome (THS) by the International Classification of Headache Disorders (ICHD-II, 2004) includes demonstration of inflammatory changes by magnetic resonance imaging (MRI) in the cavernous sinus region as an alternative to biopsy. Careful follow-up is required to exclude other possible causes of painful ophthalmoplegia. It remains unclear for how long such close observation should be extended to assure the diagnosis nor whether THS represents a self-limited condition in the long-term. Case report: An observational study along 13 years of a patient fulfilling current ICHD-II criteria for THS to clarify the natural history of the disorder. Results: We witnessed three episodes, the presenting one causing severe left orbital pain, ipsilateral abducens palsy , and hypoesthesia in the territory of the ophthalmic branch of the trigeminal nerve. Gadolinium MRI showed enhancement of tissue infiltrating the lateral wall of the carotid sinus. A relapse 21 months later caused involvement of left oculomotor and abducens nerves in addition to orbital pain which responded rapidly to steroids. Ensuing remission maintained for up to 11 years concluded in a new relapse again characterized by severe ipsilateral orbital pain and frontal numbness, but no ophthalmoplegia. A gadolinium-MRI did not show enhancement on this occasion despite prompt response to steroids. Conclusion: The risk of relapses in THS and its response to steroids may be maintained for as long as 13 years (AU)

Frequency and significance of anti-Ro (SS-A) antibodies in multiple sclerosis patients.

OBJECTIVE: To determine the frequency and significance of antinuclear (ANA), anticardiolipin (ACA) and anti-Ro (SS-A) antibodies in multiple sclerosis (MS) patients. METHODS: ANA (indirect immunofluorescence), ACA and anti-Ro (SS-A) antibodies (ELISA) were tested in sera of 42 patients with Poser defined MS and 50 healthy individuals. RESULTS: High levels of anti-Ro (SS-A) antibodies were found in 3 patients (7%) (vs 0 in the control group). Two of them had normal salivary gland biopsy. Clinical MS form was chronic-progressive in 2 cases and relapsing-remitting in the third one. Ten patients (23%) had low levels of ANA (vs 4%), none of them positive for anti-Ro (SS-A) antibodies. Only 1 patient (2%) with RR clinical form had ACA (vs 0). No clinical or neuroradiological differences with conventional MS patients were observed. CONCLUSIONS: ANA, ACA and anti-Ro (SS-A) antibodies in MS patients indicate an underlying autoimmune disease but our series suggests that they are an epiphenomenon of a more diffuse immunological dysfunction.

In vitro dialyzability of zinc from different salts used in the supplementation of infant formulas.

Seven zinc salts--acetate, chloride, lactate, sulfate, citrate, gluconate, and oxide--were added to milk--and soy-based infant formulas to estimate possible differences in zinc availability depending on the type of salt used. For this purpose, an in vitro method that estimates the dialyzability of the element (i.e., the fraction available for absorption) was applied. Zinc dialyzability is always higher in milk-based products than in soy products, even when the total zinc contents are higher in the latter. The salts can be classified according to the zinc dialyzability in the two types of formulas as follows: oxide > gluconate = chloride = lactate > citrate = acetate > sulfate. Therefore, according to the dialysis percentage, oxide and gluconate are the compounds of choice for zinc supplementation of infant formulas.

[Approach to quality of life changes in patients with multiple sclerosis]. / Una aproximación sobre la calidad de vida en pacientes con esclerosis múltiple.

INTRODUCTION AND OBJECTIVES: Multiple sclerosis (MS), as a chronic, incurable and often severely disabling disease, greatly affects patients normal life. The objectives of the study are to analyze which aspects of daily life are more affected by the disability and to investigate whether the degree of support demanded by patients is related to their level of incapacity. PATIENTS AND METHODS: Group of 60 ambulatory patients with Poser defined MS, mean age of 44.5 years, Expanded Disability Status Scale (EDSS) of 3.1, and average period of disease evolution 13.2 years. Test instrument was a structured 12 items questionnaire given during a visit to the attending physician and regarding social and family aspects, financial needs, professional life and emotional well being. RESULTS: 58.3% of the professionally active patients had to give up their jobs; 8.3% experienced changes in their marital status and general family life somewhat changed in 41.7% of the cases due to either physical or psychical disease consequences. Social relationships were altered in 68.2% of the cases; 43.3% required some level of economical support, and 80% went through episodes of emotional distress. CONCLUSION: Statistically significant relationships exist between level of incapacity and professional and social life changes but not with family life changes, emotional alterations and level of support required.

Una aproximación sobre la calidad de vida en pacientes con esclerosis múltiple / Approach to quality of life changes in patients with multiple sclerosis

Introducción y objetivos. La esclerosis múltiple (EM) es una enfermedad inflamatoria de la mielina del sistema nervioso central, de causa desconocida, crónica, que afecta a jóvenes y que en su evolución a menudo produce déficit motores, sensitivos, autonómicos y psíquicos, con la consiguiente perturbación en la calidad de vida. Nuestro objetivo es analizar las implicaciones de la discapacidad causadas por la enfermedad en varios aspectos de la vida diaria y conocer si éstos se relacionan con el grado de discapacidad. Pacientes y métodos. Estudiamos un grupo 60 pacientes afectados de EM definida por los criterios de Poser, ambulantes, con una media de edad de 44,5 años, cuta puntuación en la escala del estado de discapacidad ampliada (EDSS, Expanded Disability Status Scale) era de 3,1 y un período medio de evolución de 13,2 años. Mediante entrevista directa, se realizó a todos los pacientes un cuestionario con 12 ítems relativos a diferentes limitaciones en la vida diaria ocasionadas por la enfermedad. Resultados. Encontramos que la EM produjo perturbaciones en el trabajo remunerado en el 58,3 por ciento y cambios en el estado marital en el 8,3 por ciento; el 41,7 por ciento requerían ayuda externa o familiar, el 68,2 por ciento sufrían limitaciones sociales, el 43,32 por ciento demandaban necesidad de recursos económicos y el 80 por ciento referían haber padecido problemas emocionales, aunque sólo un 7 por ciento eran graves. La gravedad de la enfermedad medida por la escala EDSS se correlacionaba con todos los aspectos a excepción de la demanda económica y emocional. Conclusión. Proponemos que debería tenerse en consideración un mejor conocimiento y tratamiento de estos problemas en la práctica clínica y en la planificación de los recursos sociosanitarios (AU)