Attachment goes to court: child protection and custody issues.

Attachment theory and research are drawn upon in many applied settings, including family courts, but misunderstandings are widespread and sometimes result in misapplications. The aim of this consensus statement is, therefore, to enhance understanding, counter misinformation, and steer family-court utilisation of attachment theory in a supportive, evidence-based direction, especially with regard to child protection and child custody decision-making. The article is divided into two parts. In the first, we address problems related to the use of attachment theory and research in family courts, and discuss reasons for these problems. To this end, we examine family court applications of attachment theory in the current context of the best-interest-of-the-child standard, discuss misunderstandings regarding attachment theory, and identify factors that have hindered accurate implementation. In the second part, we provide recommendations for the application of attachment theory and research. To this end, we set out three attachment principles: the child's need for familiar, non-abusive caregivers; the value of continuity of good-enough care; and the benefits of networks of attachment relationships. We also discuss the suitability of assessments of attachment quality and caregiving behaviour to inform family court decision-making. We conclude that assessments of caregiver behaviour should take center stage. Although there is dissensus among us regarding the use of assessments of attachment quality to inform child custody and child-protection decisions, such assessments are currently most suitable for targeting and directing supportive interventions. Finally, we provide directions to guide future interdisciplinary research collaboration.

Toxoplasmosis en sistema nervioso central: revisión sobre la patología, abordaje diagnóstico y tratamiento / Toxoplasmosis in the central nervous system: Review of the pathology, diagnostic approach and treatment

RESUMEN La toxoplasmosis es una de las infecciones más comunes en humanos. Debido a la prevalência de la coinfección con VIH, conlleva un alto impacto en los sistemas de salud. Los seres humanos pueden infectarse de toxoplasma al consumir carne mal cocinada de cordero o cerdo que contenga los quistes tisulares, o al consumir agua o alimentos contaminados con heces de gatos. Una vez presente en el humano, el T. gondii se multiplica en los enterocitos y se disemina por el torrente sanguíneo o linfático, parasitando las células musculares, de la retina y frecuentemente el sistema nervioso central. La técnica más usada para la detección de IgG o IgM contra toxoplasma es la técnica de Elisa. Los anticuerpos IgG pueden estar elevados sin tener una infección activa, por lo que el diagnóstico con IgM y posteriormente con test de avidez es fundamental. El líquido cefalorraquídeo muestra pleocitosis leve e hiperproteinorraquia. Las neuroimágenes son de alta utilidad, ya que usualmente la punción lumbar puede estar contraindicada por lesiones que producen efecto de masa. Idealmente, los pacientes deben ser valorados con resonancia magnética en la que típicamente se observan lesiones eccéntricas en ganglios basales con realce en anillo, posteriormente a la aplicación de contraste. Se debe considerar el linfoma del sistema nervioso central como diagnóstico diferencial. El tratamiento suele iniciarse de manera empírica con pirimetamina, sulfadiazina y ácido folínico, con evaluación de la mejoría imagenológica a los 10-14 días. Si no se encuentra disponible, es posible el tratamiento con trimetoprin-sulfametoxazol. El inicio temprano del tratamiento antibiótico es fundamental para el buen pronóstico; en cuatro meses se observa recuperación neurológica completa en menos del 20 % de los casos y a los tres años de seguimiento en aproximadamente el 30 % de los pacientes.

SUMMARY Toxoplasmosis is one of the most common infections in humans. Due to the prevalence of coinfection with HIV, it carries a high impact on health systems. Humans can become infected with toxoplasma by consuming undercooked lamb or pork meat that contains tissue cysts, or by consuming water or food contaminated with cat feces. Once present in humans, T. gondii multiplies in enterocytes and spreads through the blood or lymphatic stream, parasitizing muscle cells, the retina, and frequently the Central Nervous System. The most used technique for the detection of IgG or IgM against toxoplasma is the ELISA technique. IgG antibodies can be elevated without having an active infection, so diagnosis with IgM and later with avidity test is essential. Cerebrospinal fluid shows mild pleocytosis and hyperprotein spinal cord. Neuroimaging is highly useful, since lumbar puncture can usually be contraindicated due to lesions that produce a mass effect. Ideally, patients should be evaluated with magnetic resonance imaging, which typically shows eccentric lesions in the basal ganglia with ring enhancement after the application of contrast. Central Nervous System lymphoma should be considered as a differential diagnosis. Treatment is usually started empirically with pyrimethamine, sulfadiazine, and folinic acid, with evaluation of imaging improvement at 10-14 days. If not available, treatment with trimethoprine-sulfamethoxazole is possible. The early initiation of antibiotic treatment is essential for a good prognosis, in 4 months complete neurological recovery is observed in less than 20 % of cases and after 3 years of follow-up in approximately 30 % of patients.

Coexistencia de lupus eritematoso sistémico y miastenia gravis. Una expresión infrecuente de poliautoinmunidad / Coexistence of systemic lupus erythematosus and myasthenia gravis: an unusual case of polyautoimmunity

La relevancia clínica de la poliautoinmunidad, definida como la presencia de 2o más enfermedades autoinmunes en un mismo individuo, es uno de los temas aun sin dilucidar en la práctica médica. La coexistencia entre miastenia gravis (MG) y lupus eritematoso sistémico (LES) supone un reto clínico por los posibles diagnósticos diferenciales dados en el momento de abordar el compromiso muscular en pacientes con LES. Presentamos el caso de una paciente que consultó a urgencias del Hospital Universitario San Ignacio de Bogotá, Colombia, con diagnóstico previo de LES, que desarrolla un síndrome de debilidad aguda en el contexto de una infección sistémica, haciendo diagnóstico clínico y electrofisiológico de MG

The relevance of polyautoimmunity, defined as the presence of 2or more autoimmune diseases in the same individual, is one of the issues not yet elucidated in medical practice. The coexistence of myasthenia gravis (MG) and systemic lupus erythematosus (SLE) is a clinical challenge due to the possible differential diagnoses of muscle involvement in patients with SLE. We present the case of a patient who came to the emergency room of Hospital Universitario San Ignacio in Bogotá, Colombia, with a previous diagnosis of SLE, who developed acute weakness in the context of a systemic infection, with a clinical and electrophysiological diagnosis of MG

Coexistence of Systemic Lupus Erythematosus and Myasthenia Gravis: An Unusual Case of Polyautoimmunity. / Coexistencia de lupus eritematoso sistémico y miastenia gravis. Una expresión infrecuente de poliautoinmunidad.

The relevance of polyautoimmunity, defined as the presence of 2or more autoimmune diseases in the same individual, is one of the issues not yet elucidated in medical practice. The coexistence of myasthenia gravis (MG) and systemic lupus erythematosus (SLE) is a clinical challenge due to the possible differential diagnoses of muscle involvement in patients with SLE. We present the case of a patient who came to the emergency room of Hospital Universitario San Ignacio in Bogotá, Colombia, with a previous diagnosis of SLE, who developed acute weakness in the context of a systemic infection, with a clinical and electrophysiological diagnosis of MG.

Clinical and neurophysiological characteristics of patients with Guillain-Barré syndrome at Hospital Universitario San Ignacio, Bogotá, Colombia between 2009 and 2017.

Guillain-Barré syndrome (GBS) is the most common acute peripheral polyneuropathy in the world. The estimated incidence in Colombia is 1.2-1.7 cases per 100 000 inhabitants, although during 2016 an increase in the incidence of the disease was documented, apparently associated with an epidemiological peak of the Zika virus. We conducted to describe the clinical and neurophysiological characteristics of adult patients with GBS treated at Hospital Universitario San Ignacio, Bogota, Colombia, between 2009 and 2017. An observational, descriptive, cross-sectional study was designed.

Neutral lipid storage disease with myopathy and dropped head syndrome. Report of a new variant susceptible of treatment with late diagnosis.

Neutral lipid storage disease with myopathy (NLSDM) is characterized by the accumulation of cytoplasmic triglyceride droplets in various tissues; this very rare condition is caused by mutations in the PNPLA2 gene, susceptible to specific pharmacological management that decreases clinical progression. We describe the clinical and biochemical characteristics of a Colombian patient with a previously unreported homozygous mutation in the PNPLA2 gene with a difficult to manage disease, who was diagnosed late by advances in molecular techniques.

Miastenia gravis seronegativa: revisión de la literatura / Seronegative Myasthenia Gravis: A Literature Review

RESUMEN En 1976 se identificaron varios anticuerpos dirigidos contra el receptor de acetil colina en el suero de los pacientes con miastenia gravis (MG). Sin embargo, luego de unos años, se evidenció que aproximadamente el 20 % de los pacientes con MG generalizada y con evidencia electrofisiológica de un trastorno de la unión neuromuscular, no expresan dichos anticuerpos por radioinmunoensayo (RIA); éstos constituyen los casos de miastenia gravis seronegativa (MGSN). El diagnóstico en estos pacientes es difícil, dada la ausencia de autoanticuerpos detectables en suero y la falta de estudios neurofisiológicos sensibles. Recientemente un nuevo método basado en ensayos celulares muestra un aumento significativo en la detección de miastenia seropositiva, en casos diagnosticados previamente como seronegativos. Este artículo pretende dar un abordaje sobre la fisiopatología de la miastenia gravis seronegativa, así como una actualización de los últimos avances sobre su diagnóstico. También busca hacer una revisión sobre el contexto general actual de esta patología en Colombia.

SUMMARY In 1976 antibodies against acetyl-choline receptor were identified on the serum of patients with myasthenia gravis. However, some years later, it became clear that about 20% of patients with generalized MG and an electro physiologic disorder on the neuromuscular junction did not express these antibodies by radioimmunoassay (RIPA). These cases represent seronegative myasthenia gravis (SNMG). The diagnosis of these patients is difficult, given the absence of detectable autoantibodies on serum and the lack of sensitive neurophysiologic tests. Recently, a new method based on cellular assays shows an increase on detection of seropositive MG from cases, which were initially diagnosed as seronegative. This article reviews the physiopathology of seronegative MG and gives an update on the latest advances concerning its diagnosis. It also hopes to approach the current general context of the illness in Colombia.

[The cost-effectiveness of interferon beta treatment in patients with a clinically isolated syndrome in Colombia]. / Costo-efectividad del tratamiento con interferón beta en pacientes con síndrome clínico aislado de alto riesgo en Colombia.

INTRODUCTION: Approximately 85% of patients with multiple sclerosis have an initial demyelinating event. Treatment with interferon beta delays the progression of multiple sclerosis for nearly two years in patients with a clinically isolated syndrome. In Colombia, interferon is very expensive when compared to other countries. OBJECTIVE: We sought to determine the cost-effectiveness of a two-year interferon beta treatment within Colombia in patients with a clinically isolated syndrome. MATERIALS AND METHODS: Based on patient and society perspectives, a cost-effectiveness analysis was conducted using a decision tree. A variety of probabilities were defined after a systematic review of the available literature. The disease costs were calculated by reviewing medical charts at the Hospital San Ignacio University and surveys completed by multiple sclerosis patients. To control for uncertainty in these data, analysis of approximately one-thousand patients was performed using Monte Carlo methods. RESULTS: The two-year treatment cost per patient exceeds Col$ 95,000,000 (US$ 50,000). Approximately 80 % of this cost corresponds to medications (US$ 40,500). The price of relapse and indirect costs totals Col$ 41,632,149 (US$ 21,744) and Col$ 11,656,389 (US$ 6,088), respectively. Treatment represents an increase of 0.06 quality-adjusted life years (QALY). The incremental cost-effectiveness ratio exceeds the threshold, regardless of the use of Monte Carlo methods for analysis. CONCLUSION: Administering interferon beta over the course of two years to high-risk patients with a clinically isolated syndrome is not cost-effective within Colombia.

Costo-efectividad del tratamiento con interferón beta en pacientes con síndrome clínico aislado de alto riesgo en Colombia / The cost-effectiveness of interferon beta treatment in patients with a clinically isolated syndrome in Colombia

Introducción. En 85 % de los pacientes con esclerosis múltiple se presenta como manifestación inicial un primer evento desmielinizante o síndrome clínico aislado. En estos casos, el tratamiento con interferón beta retrasa hasta dos años la progresión a esclerosis múltiple. Sin embargo, en Colombia este medicamento es costoso. Objetivo. Determinar si el tratamiento del síndrome clínico aislado con interferón beta es costo-efectivo al retrasar la esclerosis múltiple en dos años. Materiales y métodos. Se realizó un análisis de costo-efectividad empleando un árbol de decisiones basado en la perspectiva del paciente y la sociedad. A partir de una revisión sistemática de la literatura y de conceptos de expertos se definieron las diversas probabilidades. Los costos de la enfermedad se calcularon por medio de la revisión de historias y la aplicación de encuestas a los pacientes atendidos en el Hospital Universitario San Ignacio. Para controlar la incertidumbre se realizó un análisis de sensibilidad mediante una simulación de Monte Carlo con mil pacientes. Resultados. El costo del tratamiento con interferón sobrepasa los Col$ 95´000.000 (US$ 50.000) por paciente durante los dos años. Aproximadamente, 80 % corresponde a los costos del medicamento. El costo de la recaída se acerca a Col$ 39´139.200 (US$ 21.744), y los costos indirectos corresponden a Col$ 10´958.400 (US$ 6.088). La tasa representativa del mercado fue de Col$ 1.800. Con el tratamiento se ganan sólo 0,06 años de vida ajustados por discapacidad (AVAD) adicionales. La razón de costo-efectividad ‘incremental´ (sic.) supera el umbral, incluso en el análisis de sensibilidad. Conclusión. La administración de interferón beta en pacientes con síndrome clínico aislado de alto riesgo en los primeros dos años no es costo-efectiva en Colombia.

Introduction: Approximately 85% of patients with multiple sclerosis have an initial demyelinating event. Treatment with interferon beta delays the progression of multiple sclerosis for nearly two years in patients with a clinically isolated syndrome. In Colombia, interferon is very expensive when compared to other countries. Objective: We sought to determine the cost-effectiveness of a two-year interferon beta treatment within Colombia in patients with a clinically isolated syndrome. Materials and methods: Based on patient and society perspectives, a cost-effectiveness analysis was conducted using a decision tree. A variety of probabilities were defined after a systematic review of the available literature. The disease costs were calculated by reviewing medical charts at the Hospital San Ignacio University and surveys completed by multiple sclerosis patients. To control for uncertainty in these data, analysis of approximately one-thousand patients was performed using Monte Carlo methods. Results: The two-year treatment cost per patient exceeds Col$ 95,000,000 (US$ 50,000). Approximately 80 % of this cost corresponds to medications (US$ 40,500). The price of relapse and indirect costs totals Col$ 41,632,149 (US$ 21,744) and Col$ 11,656,389 (US$ 6,088), respectively. Treatment represents an increase of 0.06 quality-adjusted life years (QALY). The incremental cost-effectiveness ratio exceeds the threshold, regardless of the use of Monte Carlo methods for analysis. Conclusion: Administering interferon beta over the course of two years to high-risk patients with a clinically isolated syndrome is not cost-effective within Colombia.

Leucoencefalitis hemorrágica aguda: manifestaciones clínicas, hallazgos radiológicos y neuropatológicos / Acute hemorrhagic leukoencephalitis: clinical presentation, radiologic and neuropathologic features

La Leucoencefalitis Hemorrágica Aguda o enfermedad de Hurst es una enfermedad rara, caracterizada clínicamente por inicio súbito, curso clínico severo, usualmente fatal que se presenta posterior a una infección viral o vacunación. Patológicamente se caracteriza por desmielinización perivenular y necrosis hemorrágica difusa del sistema nervioso central. Se considera que representa una forma hiperaguda y severa de la Encefalomielitis Aguda Diseminada, la cual es una entidad inflamatoria con una base fisiopatológica autoinmune postinfecciosa. A continuación, se expone el caso de una paciente adulta, que ingresó al servicio de urgencias con cuadro clínico típico de migraña y antecedente de cefaleas previas de similares características. Quien doce horas posterior a su ingreso desarrolló de forma rápidamente progresiva depresión del estado de conciencia, signos neurológicos focales y signos de hipertensión de fosa posterior, que llevaron a desenlace fatal en tan solo 96 horas del inicio del cuadro clínico con hallazgos patológicos postmortem que confirman leucoencefalitis hemorrágica aguda. Se revisan las características clínicas, los hallazgos radiológicos y patológicos de esta entidad clínico-patológica poco común.

Acute hemorrhagic leukoencephalitis or Hurst disease is a rare disorder characterized by its severe neurological involvement, rapid progression and fatal outcome in a few days. The disease is usually a post infectious condition. Under microscope, it is identified by a perivenular demyelination and a diffuse hemorrhagic necrosis. This entity is thought to represent a hyperacute severe form of acute disseminated encephalomyelitis, which is an inflammatory autoimmune post infectious disorder. We describe the case of an adult woman, who visits the emergency room with migraine-like symptoms and a previous clinical history of similar headaches. Twelve hours later she developed focal neurologic findings, stupor and signs of endocraneal hypertension, her clinical status continued to worsen and in 96 hours she succumbed. The autopsy confirm acute hemorrhagic Leukoencephalitis. Reviewed clinical, radiological and pathological characteristics of this uncommon disease.

Sistema trigémino vascular y cefalea / Trigeminovascular System and Primary Headache

La fisiopatología de las cefaleas primarias es compleja e incluye un sinnúmerode interacciones que regulan el proceso nociceptivo. Dentro de los principalesresponsables de generar el dolor se encuentra el sistema trigémino vascular, que esun conjunto de estructuras que integran vías tanto centrales corticosubcorticales comoperiféricas, que desempeñan un papel activo no solo en la génesis del dolor, sino enlas manifestaciones autonómicas y visuales que acompañan la cefalea. Así mismo, estesistema es el responsable de los mecanismos de sensibilización central característicosdel dolor. En el artículo se desarrollan brevemente las principales estructuras queparticipan en la génesis de las cefaleas primarias y sus interacciones en las diferentespartes del sistema nervioso...

The pathophysiology of primary headache iscomplex and it includes several interactionsthat regulate the nociceptive process. The trigeminal-vascular system is perhaps one of theprincipal structures that generate pain due tothe integration of several pathways both centraland peripheral. In addition to this, the trigeminalvascular system also plays a central role inthe autonomic and visual symptoms that affectindividuals with headache and in the centralsensitization process. In this article we brieflydiscuss the main structures that participate in thepathophysiology of primary headaches and theirinteractions in the different levels of the centralnervous system...

Síndrome de opsoclonus-mioclonus-ataxia (OMA) parainfeccioso secundario a infección por citomegalovirus / Opsoclonus myoclonus ataxia syndrome secondary to a parainfectious process by cytomegalovirus

A continuación, se presenta un paciente de 66 años con alteración en los movimientos oculares, asociado a mioclonías, disartria y ataxia cerebelosa, secundario a un proceso parainfeccioso por citomegalovirus. Posteriormente, se revisa la fisiopatología del síndrome de opsoclonus – mioclonus – ataxia, las etiologías del mismo y las opciones terapéuticas disponibles. Esta es una etiología poco frecuente del síndrome, pues sólo se encontró un caso reportado de opsoclonus mioclonus ataxia asociado a citomegalovirus.