Monuments to Cajal in Madrid, Spain: Rejection of public tributes.

During Santiago Ramón y Cajal's lifetime, two monuments to him were erected in Madrid. However, Cajal excused himself from attending their inaugurations for reasons that have so far remained unexplained. The present report has therefore investigated the political context and personal circumstances that might account for his behavior. The first monument is a fountain in El Retiro Park, the work of sculptor Victorio Macho, inaugurated in 1926 during a major confrontation between progressive intellectuals and physicians against the dictatorship of Miguel Primo de Rivera. An official press release warned of a prison sentence for those who attempted a second (illegal) inauguration. The second monument appeared in 1931, barely a month after the establishment of the Second Spanish Republic. This full-body statue, standing>3m (around 10ft) high on a narrow pedestal, was financed by medical students and sculpted by Lorenzo Domínguez, a Chilean medical student. Its unlikely height and thinness earned it the nickname 'The Pencil'. At present, it flanks the entrance to Cajal's old classroom at the Madrid College of Physicians. Closer inspection reveals fractures as evidence of its having been broken into pieces at some point, presumably during bombings that took place in 1936. The calcareous Novelda stone used in its construction and its exposure to the elements may also partly explain its deterioration. A few metres away, a second sculpture, apparently a replica of the original, was inaugurated in 1998. Cajal's excuses for not attending the inaugurations of both his monuments may have different explanations. Regarding the fountain, it was probably a gesture of solidarity against those (many of whom were doctors) opposing the dictatorship whereas, when The Pencil was inaugurated, Cajal was 79 years old, and his physical limitations might have accounted for his inability to attend the ceremony. Thus, given the different political context in which each inauguration took place, Cajal's attitude was presumably in line with his politics, but also the result of his age-related infirmities.

[Epileptic encephalopathies in infancy. How do we treat them? Does the aetiology influence the response to treatment?] / Encefalopatias epilepticas del lactante. Como las tratamos? Influye la etiologia en la respuesta al tratamiento?

INTRODUCTION: Resistance to treatments is a common feature of Ohtahara, Aicardi, West and Dravet syndromes, as well as malignant migrating epilepsy in infancy. AIMS: To update the therapeutic management and to analyse whether the aetiology somehow determines the treatment. DEVELOPMENT: Convulsive seizures in the first year of life may be due to a potentially treatable aetiology, which makes it essential to carry out a complete evaluation so as to be able to begin, as early as possible, the most suitable and the non-specific symptomatic treatments to control the seizures, which prevents or minimises their deleterious effects. Metabolic disease must be ruled out and it is also essential to try a therapeutic regimen of vitamins and cofactors, as well as antiepileptic drugs. In Ohtahara and Aicardi syndromes, the first-order treatment is phenobarbital and phenytoin, and the most commonly used second-order drugs are midazolam, levetiracetam, lidocaine and valproate. In West's syndrome, the first-order treatment consists of adrenocorticotropic hormone and vigabatrine for the case of tuberous sclerosis; if there is no response, other pharmaceuticals, a ketogenic diet and surgery must be considered. For Dravet's syndrome, the main treatment consists in valproate with clobazam and stiripentol, and as the second order, other drugs and a ketogenic diet should be considered. In epilepsy with migrating seizures, the most effective treatment is with bromides, stiripentol, clonazepam and levetiracetam. CONCLUSIONS: Today there is little consensus on the therapeutic approach to be able to establish specific indications. The aetiology has an influence on the treatment, both in cases in which a curative treatment exists (metabolic diseases) and in the symptomatic management with antiepileptic drugs or other treatments (ketogenic diet or surgery).

TITLE: Encefalopatias epilepticas del lactante. Como las tratamos? Influye la etiologia en la respuesta al tratamiento?Introduccion. La refractariedad es una caracteristica comun del tratamiento de los sindromes de Ohtahara, Aicardi, West, Dravet y epilepsia maligna del lactante con crisis migrantes. Objetivo. Actualizar el manejo terapeutico y analizar si la etiologia determina de alguna manera el tratamiento. Desarrollo. Las crisis convulsivas en el primer año de vida pueden deberse a una etiologia potencialmente tratable, por lo que es imperativo una completa evaluacion para instaurar de manera precoz el tratamiento adecuado y el sintomatico no especifico para el control de las crisis, que evite o minimice el efecto deletereo de estas. Es obligado hasta descartar metabolopatia y ensayar pauta de vitaminas y cofactores, ademas de antiepilepticos. En los sindromes de Ohtahara y Aicardi, la primera linea es fenobarbital y fenitoina, y en segunda linea, los mas habituales son midazolam, levetiracetam, lidocaina y valproato. En el sindrome de West, la primera linea la constituye la hormona adrenocorticotropa y la vigabatrina para el caso de esclerosis tuberosa; si no hay respuesta, considerar otros farmacos, dieta cetogenica y cirugia. Para el sindrome de Dravet, los principales son valproato con clobazam y estiripentol, y de segunda linea, considerar otros farmacos y dieta cetogenica. En la epilepsia con crisis migrantes, los mas eficaces son bromuros, estiripentol, clonacepam y levetiracetam. Conclusiones. Actualmente existe poco consenso en el abordaje terapeutico para establecer indicaciones taxativas. La etiologia influye en el tratamiento, tanto en el caso de disponer de tratamiento curativo (metabolopatias) como en el abordaje sintomatico con antiepilepticos u otros tratamientos (dieta cetogenica o cirugia).

Cajal's unbearable cephalalgias: The consequences of a misdiagnosis.

Around the age of 66 Cajal consulted neurologist Nicolas Achúcarro complaining of "unbearable cephalalgias". He had not ever suffered from headaches. The diagnosis of early arteriosclerosis that was considered at the time a physiological, irreversible ailment of ageing had a strong emotional impact on Cajal. Comorbid depression, insomnia and self-treatment with escalating doses of Veronal® (barbital), a short-acting barbiturate, presumably aggravated the situation. Exposure to warm environments and being involved in tense discussions were identified as triggering factors of the headaches. Achúcarro and Cajal were probably assuming scientific concepts at the time, such as cerebral congestion, increased temperature at the cerebral cortex during mental activity and vasoconstriction and vasodilatation phenomena. Up to his death aged 82, no evidence was discovered of any organic nervous system disorder. Cajal remained anxious up to the end of his life fearing an impending cerebral haemorrhage. The diagnosis was followed by profound lifestyle changes such as social isolation, which forced him to leave his laboratory. Cajal's cephalalgias marked in some way the end of the Spanish school of neurohistology.

Azañón's disease. A 19th century epidemic of neurolathyrism in Spain.

The cultivation and consumption of grasspea (Lathyrus sativus) in Spain probably dates back centuries, especially during times of famine when the neurotoxic potential of this legume was expressed in the form of a spastic paraparesis known as neurolathyrism. Little known outside the country, the epidemic of neurolathyrism in the years following the Spanish Civil War (1936-1939) came to affect more than a thousand people. In late 1872, during the Six Years Revolutionary Term, young Alejandro San Martín Satrústegui (1847-1908), then editor of the popular weekly El Siglo Médico, travelled to Azañón, a remote village in the province of Guadalajara, to clarify a so-far unknown disease. We analysed the original article published in 1873 by San Martin, as well as communications sent by El Siglo Médico readers reporting similar cases in many other Castilian provinces. San Martín's neurological findings in seven personally examined cases were astonishingly accurate; he concluded the subjects' neurological deficits resulted from injury to the lateral columns in the lower portion of the spinal cord. Description of the clinical findings provided both by San Martín, and by the readers of El Siglo Médico, leave no doubt as to the diagnosis of neurolathyrism. However, none suspected the patient's staple food was the determinant cause of the disease. San Martín proposed the eponym Azañón's disease for lack of a better name the same year (1873) in which Cantani in Italy introduced the term lathyrism. The epidemic of neurolathyrism that affected many Castilian towns represents one of the best-documented in Europe during the last third of the 19th century.

A case report of recurrent adult-onset xanthogranuloma: is the radiotherapy a treatment option?

Adult-onset xanthogranuloma (AOX) is one of the four uncommon syndromes called adult xanthogranulomatous disease (AXD), which is diagnosed by characteristic histopathology. AXD is rare and heterogeneous group of entities that can affect multiple organ systems. Orbital involvement is included in the xanthogranulomatous disease although less prevalent. This work focuses on the use of external beam radiotherapy in the control of local symptoms of periocular manifestation of AOX as case report and literature review.

The Madrid School of Neurology (1885-1939).

The emergence of neurology in Madrid between 1885 and 1939 had well-defined characteristics. On foundations laid by Cajal and Río-Hortega, pioneers combined clinical practice with cutting-edge neurohistology and neuropathology research. Luis Simarro, trained in Paris, taught many talented students including Gayarre, Achúcarro and Lafora. The untimely death of Nicolás Achúcarro curtailed his promising career, but he still completed the clinicopathological study of the first American case of Alzheimer's disease. On returning to Spain, he studied glial cells, including rod cells. Rodríguez Lafora described progressive myoclonus epilepsy and completed experimental studies of corpus callosum lesions and clinical and neuropathology studies of senile dementia. He fled to Mexico at the end of the Spanish Civil War (1936-1939). Sanchís Banús, a sterling clinical neurologist, described the first cluster of Huntington's disease in Spain, and he and Río-Hortega joined efforts to determine that pallidal degeneration underlies rigidity in advanced stages of the disease. Just after the war, Alberca Llorente eruditely described inflammatory diseases of the neuraxis. Manuel Peraita studied "the neurology of hunger" with data collected during the siege of Madrid. Dionisio Nieto, like many exiled intellectuals, settled in Mexico DF, where he taught neurohistological methods and neuropsychiatry in the tradition of the Madrid School of Neurology.

Reacciones adversas a medicamentos con compromiso mucocutáneo notificadas al Instituto de Salud Pública de Chile durante el año 2013 / Adverse reactions to medicinal products with mucocutaneous involvement reported to the Chilean Public Health Institute during the year 2013

Las reacciones adversas a medicamentos (RAM) se encuentranen forma habitual en la práctica clínica, representando hasta el2% del total de consultas dermatológicas. Dentro de las RAM reportadas,hasta el 30% corresponderían a RAM mucocutáneas(RAM- MC). El Programa de Farmacovigilancia se implementó enChile el año 1995. Desde el año 2011 se encuentra normado,siendo de carácter obligatorio la notificación al Subdepartamentode Farmacovigilancia del Instituto de Salud Pública (ISP). El objetivode este trabajo es describir retrospectivamente las notificacionesde sospechas de RAM- MC realizadas al ISP durante el año2013. Se obtuvo un total de 113 notificaciones, la mayoría de ellasen mujeres (59,3%). Las RAM- MC más frecuentemente notificadasfueron erupción eritematosa (47,8%), hiperpigmentación dela piel (23,9%), síndrome de Stevens Johnson (8,8%) y anafilaxia(8,8%). Los fármacos involucrados con mayor frecuencia fueronlos quimioterapéuticos (28,3%), antibióticos (19,5%), otros (15%)y anticonvulsivantes (8%).

Adverse drug reactions (ADRs) are seen routinely in clinicalpractice, representing up to 2% of dermatology consultations.Among the reported ADRS, up to 30% correspond to mucocutaneousADRS-MC. The Pharmacovigilance Programmewas implemented in Chile in 1995. Since 2011 it notificationis mandatory to the Subdepartment of Pharmacovigilance ofthe Public Health Institute (ISP). The aim of this study is todescribe retrospectively the reports of suspected ADRS-MCmade to the ISP during 2013. A total of 113 notifications wereobtained, most of them in women (59.3%). The RAM-MC morefrequently reported were erythematous rash (47.8%), skin hyperpigmentation(23.9%), Stevens Johnson syndrome (8.8%)and anaphylaxis (8.8%).The drugs most often involved were chemotherapeutics(28.3%), antibiotics (19.5%), others (15%) and anticonvulsants(8%).

Halitosis management by the general dental practitioner--results of an international consensus workshop.

Clinical investigations on patients suffering from halitosis clearly reveal that in the vast majority of cases the source for an offensive breath odor can be found within the oral cavity (90%). Based on these studies, the main sources for intra-oral halitosis where tongue coating, gingivitis/periodontitis or a combination of the two. Thus, it is perfectly logical that general dental practitioners (GDPs) should be able to manage intra-oral halitosis under the conditions found in a normal dental practice. However, GDPs who are interested in diagnosing and treating halitosis are challenged to incorporate scientifically based strategies for use in their clinics. Therefore, the present paper summarizes the results of a consensus workshop of international authorities held with the aim to reach a consensus on general guidelines on how to assess and diagnose patients' breath odor concerns and general guidelines on regimens for the treatment of halitosis.

La Salpetrière antes de Charcot: una visita de Pedro González Velasco / La Salpetrière Hospital before Charcot: A visit described by Pedro Gonzalez Velasco

Introducci¨®n: Bajo su direcci¨®n, Charcot fue capaz de convertir La Salpetri¨¨re en centro puntero de la neurolog¨ªa mundial. Sin embargo, es escasa la informaci¨®n sobre las condiciones asistenciales que pudo encontrar a su llegada en 1862. Desarrollo: El art¨ªculo publicado en 1860 por el español Pedro Gonz¨¢lez Velasco tras visitar al famoso hospicio representa un valioso testimonio sobre los cuidados que recib¨ªan los pacientes poco antes de la llegada de Charcot. Aunque esencialmente encomi¨¢stico, el art¨ªculo describe tambi¨¦n la situaci¨®n, bastante menos satisfactoria, de pacientes con trastornos mentales graves, epilepsia y par¨¢lisis confinados en jaulas, con un simple lecho de paja sobre el suelo como ¨²nica cama y un desag¨¹e para recoger sus deyecciones. Velasco no era alienista, sino un cirujano de prestigio y defensor apasionado del positivismo. Como amigo personal, compañero de hospital y correligionario pol¨ªtico de Jos¨¦ Mar¨ªa Esquerdo, conoc¨ªa de primera mano la lucha de este por mejorar la asistencia neuropsiqui¨¢trica en Madrid. Conclusiones: El art¨ªculo de Velasco, en ¨²ltima instancia, sirve de pretexto para denunciar las deplorables condiciones asistenciales de esta clase de pacientes en el Hospital General de Madrid. Por otro lado, Charcot tendr¨¢ que afrontar no solo el desarrollo de la neurolog¨ªa, sino mejorar tambi¨¦n las condiciones de vida de los enfermos en La Salpetri¨¨re(AU)

Introduction: Under Charcot's leadership, La Salpetri¨¨re was transformed into one of the world's top neurology centres. However, there is little information regarding the patient care facilities which Charcot would have encountered upon his arrival in 1862. Development: A paper published in 1860 by Spanish physician Pedro Gonz¨¢lez Velasco following a visit to that famous hospital is a valuable testimony to the quality of patient care just prior to Charcot¡äs arrival. Although it essentially praises the institution, the article also describes the largely unsatisfactory conditions endured by patients with severe mental disorders, epilepsy and paralysis, who were locked inside cages with simple straw pallets on the floor for beds and open holes for toilets. Rather than an alienist, Velasco was a well-known surgeon and passionate advocate of positivism. As a personal friend and hospital fellow of Jose Maria Esquerdo's, with similar political affiliations, he had first-hand knowledge of the struggle to improve neuropsychiatric care in Madrid. Conclusions: Publishing his paper ultimately provided Velasco with a pretext for denouncing the deplorable care conditions endured by similar patients in Hospital General de Madrid. Meanwhile, Charcot would go on to improve the living conditions of inpatients at La Salpetri¨¨re and found the specialty of neurology(AU)

La Salpêtrière Hospital before Charcot: a visit described by Pedro González Velasco.

INTRODUCTION: Under Charcot's leadership, La Salpêtrière was transformed into one of the world's top neurology centres. However, there is little information regarding the patient care facilities which Charcot would have encountered upon his arrival in 1862. DEVELOPMENT: A paper published in 1860 by Spanish physician Pedro González Velasco following a visit to that famous hospital is a valuable testimony to the quality of patient care just prior to Charcot's arrival. Although it essentially praises the institution, the article also describes the largely unsatisfactory conditions endured by patients with severe mental disorders, epilepsy and paralysis, who were locked inside cages with simple straw pallets on the floor for beds and open holes for toilets. Rather than an alienist, Velasco was a well-known surgeon and passionate advocate of positivism. As a personal friend and hospital fellow of Jose Maria Esquerdo's, with similar political affiliations, he had first-hand knowledge of the struggle to improve neuropsychiatric care in Madrid. CONCLUSIONS: Publishing his paper ultimately provided Velasco with a pretext for denouncing the deplorable care conditions endured by similar patients in Hospital General de Madrid. Meanwhile, Charcot would go on to improve the living conditions of inpatients at La Salpêtrière and found the specialty of neurology.

¿Cuándo derivar un paciente al cirujano pediátrico? / When should a patient be referred to the pediatric surgeon?

Introducción. Las derivaciones incorrectas de los pacientes a consulta de cirugía pediátrica tienen como resultado una saturación de estas y malestar en el paciente y personal sanitario, provocando un deterioro en la calidad de atención. Objetivos. 1) Evaluar las derivaciones de pacientes nuevos a consulta de cirugía pediátrica (CCP) desde atención primaria, considerando indicación médica (IM) y calendario quirúrgico (CQ) e identificar los fallos más comunes. 2) Analizar los inconvenientes de una derivación incorrecta tanto para el paciente como para el personal sanitario. 3) Proponer medidas para disminuir los pacientes incorrectamente derivados. Material y Método. Realizamos un estudio observacional retrospectivo de cohortes, revisando historias clínicas de pacientes nuevos atendidos en CCP desde septiembre de 2010 a enero de 2012. Se recogieron variables de edad, domicilio, diagnóstico, indicación médica y centro de derivación. Resultados. Se revisaron 2.430 historias clínicas de pacientes nuevos, encontrándose una derivación incorrecta en un 44,28% (2,37 niños/día). De estos pacientes, el 18,59% de los errores se relacionaban con CQ y el 81,41% con IM. Los errores más comunes relacionados con la IM fueron: fimosis (91,45% n= 113) y criptorquidia (85,29% n= 122);en relación al CQ fueron: hernia umbilical (88,46% n= 21), hipospadias (72,22% n= 31) e hidrocele (70,83% n=12). La media de kilómetros (..) (AU)

Introduction. An incorrect transfer of patients to paediatric surgery clinic results in clinic saturation and both patient and medical staff discomfort. As a consequence the quality of medical care is deteriorated. Aim. 1) To evaluate the transfer of new patients to paediatric surgery clinic from primary paediatric care considering the medical diagnosis and the surgical calendar, looking for the most common mistakes. 2) To analyze the inconvenience of a wrong indication for both our patients and the medical staff. 3) To propose new measures to decrease the rate of patients incorrectly transferred. Material and Method. An observational retrospective study was performed based on medical records of new patients evaluated in paediatricsurgery clinic between september 2010 and january 2012. Age, address, diagnosis, transfer indication and referring centre were collected. Results. Two thousand four hundred thirty medical records were reviewed from new patients finding an incorrect transfer in 44.28% of them (2.37 children/day). From these cases wrong transfer was related to surgical calendar in 18.59% and wrong diagnosis in 81.41% of them. Most common mistakes due to wrong diagnosis were Phimosis (91.45%n= 113) and Cryptorquidism (85.29% n= 122); mistakes related to surgical calendar were Umbilical Hernia (88.46%), Hypospadias (72.22%n= 31) and Hydrocele (70.83% n= 12). Mean distance of transfer for (..) (AU)

Neuroimágenes sin evidencia de déficit dopaminérgico (SWEDD) / SWEDDs: scans without evidence of dopaminergic deficit

Introducción. Aproximadamente un 10% de los pacientes diagnosticados inicialmente de Enfermedad de Parkinson (E.P.), no presentan alteraciones en la vía dopaminérgica nigroestriatal en su vertiente presináptica; se engloban bajo el acrónimo SWEDDs (Scans without evidence of dopaminergic deficit) [1]. Objetivo. Revisar aquellos aspectos clínicos que pueden ayudar al diagnóstico diferencial de los SWEDDs, así como las bases del tratamiento. Desarrollo. Las técnicas complementarias empleadas en el diagnóstico de la E.P. como el DAT-SCAN, han favorecido que se establezca un diagnóstico diferencial entre estas dos entidades: E.P. y SWEDDs, cuya evolución, pronóstico y tratamiento será diferente. Conclusiones. El término SWEDDs incluye pacientes con temblor de reposo asimétrico y ausencia de disfunción de la vía dopaminérgica nigroestriatal. En casos dudosos la prueba complementaria a realizar es el DAT-SCAN. La hipótesis de que el temblor en SWEDDs pueda ser una forma de distonía primaria debe ser contrastada en futuros estudios [1]. El tratamiento de los pacientes con SWEDDs se basa en fármacos anticolinérgicos (AU)

Introduction. Approximately 10% of all patients initially diagnosed with Parkinson's disease (PD) do not present any alterations in the presynaptic nigrostriatal dopaminergic pathway; they are classified under the acronym SWEDDs (Scans without evidence of dopaminergic deficit). Aims. Our aim is to review those clinical aspects that can be of use in the differential diagnosis of the SWEDDs, as well as the bases of treatment. Development. The complementary techniques employed in the diagnosis of PD, like DAT-SCAN, have made a valuable contribution to establishing a differential diagnosis between these two conditions, i.e. PD and SWEDDs, whose development, prognosis and treatment will be different. Conclusions. The term SWEDDs includes patients with asymmetrical tremor at rest and absence of dysfunction of the nigrostriatal dopaminergic pathway. In doubtful cases, the complementary test to be conducted is DAT-SCAN. The hypothesis that claims that the tremor in SWEDDs may be a form of primary dystonia must be tested in future studies. The treatment of patients with SWEDDs is based on anticholinergic drugs (AU)

Long-term results with oral fluoropyrimidines and oxaliplatin-based preoperative chemoradiotherapy in patients with resectable rectal cancer. A single-institution experience

INTRODUCTION: Neoadjuvant 5-FU-based chemoradiotherapy in resectable rectal cancer (RC) is a standard of treatment. The use of oral fluoropyrimidines and new agents such as oxaliplatin may improve efficacy and tolerance. MATERIAL AND METHODS: Between 1999 and 2009, 126 RC patients with T3-T4 and/or N+ disease were given three successive protocols: UFT (32), UFT-oxaliplatin (75) and capecitabine-oxaliplatin (19), alongside 45 Gy of radiotherapy; with surgery 4-6 weeks after. Adjuvant treatment was given in all patients. The primary objective was pathologic complete response (pCR). RESULTS: Preoperative therapy was well tolerated, with no toxic deaths and a 15% grade 3-4 toxicity rate. Eighty-five percent of patients received the full chemotherapy dose, 56% had an abdominoperineal resection, 6% reinterventions and 57% received the full adjuvant chemotherapy planned. The pCR rate was 13%. The downstaging rate was 80%; 8% had progression of disease. The relapse rate was 20%, with local relapse in 6%. By 5 years of followup, 92% of relapses had occurred. Median follow-up was 73 months, 5- and 10-year disease-free survival rates were 75% and 50%, and 5- and 10-year overall survival rates were 79% and 66% respectively. There was no benefit from the use of oxaliplatin regarding survival or pCR rates. Older patients had worse long-term outcomes. CONCLUSIONS: Neoadjuvant chemoradiotherapy with oral fluoropyrimidines and oxaliplatin is feasible and well tolerated. The risk of early progression is low. However, there was no added benefit with the use of oxaliplatin. There were no relapses in patients with pCR. The role of adjuvant chemotherapy is unclear (AU)

[When should a patient be referred to the pediatric surgeon?]. / Cuándo derivar un paciente al cirujano pediátrico?

INTRODUCTION: An incorrect transfer of patients to paediatric surgery clinic results in clinic saturation and both patient and medical staff discomfort. As a consequence the quality of medical care is deteriorated. AIM: 1) To evaluate the transfer of new patients to paediatric surgery clinic from primary paediatric care considering the medical diagnosis and the surgical calendar, looking for the most common mistakes. 2) To analyze the inconvenience of a wrong indication for both our patients and the medical staff. 3) To propose new measures to decrease the rate of patients incorrectly transferred. MATERIAL AND METHOD: An observational retrospective study was performed based on medical records of new patients evaluated in paediatric surgery clinic between september 2010 and january 2012. Age, address, diagnosis, transfer indication and referring centre were collected. RESULTS: Two thousand four hundred thirty medical records were reviewed from new patients finding an incorrect transfer in 44.28% of them (2.37 children/day). From these cases wrong transfer was related to surgical calendar in 18.59% and wrong diagnosis in 81.41% of them. Most common mistakes due to wrong diagnosis were Phimosis (91.45% n= 113) and Cryptorquidism (85.29% n = 122); mistakes related to surgical calendar were Umbilical Hernia (88.46%), Hypospadias (72.22% n= 31) and Hydrocele (70.83% n = 12). Mean distance of transfer for patients wrongly referred outside the metropolitan area was 35.92 km (Range: 7.1-129). CONCLUSIONS: We have detected major mistakes in patient transfer from primary paediatric care. The knowledge of surgical calendar and accurate diagnosis should avoid these problems. These mistakes result in deterioration of medical care quality, patient discomfort and clinic saturation. It is necessary to propose and develop measures that improve the knowledge of surgical calendar and to make more accurate surgical diagnosis in primary paediatric care.

Posible origen en la arteria carótida de un infarto vertebrobasilar / No disponible

No disponible

[Digestive disorders in Parkinson's disease: dysphagia and sialorrhea]. / Trastornos digestivos en la enfermedad de Parkinson: disfagia y sialorrea.

INTRODUCTION: The non-motor symptoms of Parkinson's disease are a frequent and often under-diagnosed disorder. Two of the most significant non-motor symptoms are perhaps dysphagia and sialorrhea (which are relatively common in advanced stages of the disease) owing to their important functional repercussions and to the associated comorbidity. DEVELOPMENT AND CONCLUSIONS: In recent years, different evaluation scales have been developed for clinical use and in screening the aforementioned symptoms. Of the different therapeutic options available, botulinum toxin represents the preferred treatment for sialorrhea. In contrast, speech therapy and an optimisation of the antiparkinsonian therapy are generally useful measures to treat dysphagia, percutaneous endoscopic gastrostomy being reserved for patients suffering from Parkinson who have severe dysphagia.

Clinical experience with miglustat therapy in pediatric patients with Niemann-Pick disease type C: a case series.

Niemann-Pick disease type C (NP-C) is an inherited neurovisceral lysosomal lipid storage disease characterized by progressive neurological deterioration. Different clinical forms have been defined based on patient age at onset: perinatal, early-infantile (EI), late-infantile (Li), juvenile and adult. We evaluated the efficacy and tolerability of miglustat in 16 symptomatic NP-C patients, with comparative reference to one neurologically asymptomatic, untreated patient. All patients were categorized according to age at neurological disease onset, and were assessed using a standardized clinical assessment protocol: disability and cognitive function scales, positron emission tomography (PET), and biochemical markers. PET and disability scale evaluations indicated that cerebral hypometabolism and neurological symptoms were stabilized during treatment in juvenile-onset NP-C patients. EI and Li NP-C patients, who had higher disease severity at baseline (treatment start), showed increased disability scores and progressive cerebral hypometabolism during follow up. Similarly, while cognitive scale scores remained relatively stable in patients with juvenile NP-C, cognition deteriorated in EI and Li patients. Plasma chitotriosidase (ChT) activity was lower in the juvenile NP-C subgroup than in EI and Li patients, and generally increased in patients who discontinued treatment. Plasma CCL18/PARC and ChT activities indicated greater macrophagic activity in EI and Li patients versus juveniles. Miglustat was generally well tolerated; frequent adverse events included diarrhea and flatulence, which were managed effectively by dietary modification and loperamide. Overall, miglustat appeared to stabilize neurological status in juvenile-onset NP-C patients, but therapeutic benefits appeared smaller among younger patients who were at a more advanced stage of disease at baseline.