RESUMEN
Cluster headache (CH) is one of the worst primary headaches that remain underdiagnosed and inappropriately treated. There are recent advances in the understanding of this disease and available treatments. This paper aims to review CH's recent clinical and pathophysiological findings, diagnosis, and treatment. We performed a narrative literature review on the socio-demographics, clinical presentations, pathophysiological findings, and diagnosis and treatment of CH. CH affects 0.1% of the population with an incidence of 2.07-9.8/100,00 person-years-habitants, a mean prevalence of 53/100,000 inhabitants (3-150/100,000 inhabitants). The male-to-female ratio remains inconclusive, as the ratio of 4.3:1 has recently been modified to 1.3-2.6, possibly due to previous misdiagnosis in women. Episodic presentation is the most frequent (80%). It is a polygenetic and multifactorial entity that involves dysfunction of the trigeminovascular system, the trigeminal autonomic reflex, and the hypothalamic networks. An MRI of the brain is mandatory to exclude secondary etiologies. There are effective and safe pharmacological treatments oxygen, sphenopalatine, and great occipital nerve block, with the heterogeneity of clinical trial designs for patients with CH divided into acute, transitional, or bridge treatment (prednisone) and preventive interventions. In conclusion, CH remains underdiagnosed, mainly due to a lack of awareness within the medical community, frequently causing a long delay in reaching a final diagnosis. Recent advances in understanding the principal risk factors and underlying pathophysiology exist. There are new therapeutic possibilities that are effective for CH. Indeed, a better understanding of this challenging pathology will continue to be a subject of research, study, and discoveries in its diagnostic and therapeutic approach.
RESUMEN
ABSTRACT The association between memory loss and Hodgkin's lymphoma has been given the eponym of Ophelia syndrome, in memory of Shakespeare's character in The Tragedy of Hamlet, Prince of Denmark. Nevertheless, there are differences between the disease and the character. Objective: To review the origins and uses of the eponym through an original article by pathologist Ian Carr, its relation to the character Ophelia, and the related autoantibodies. Methods: Historical narrative review. Results: Besides an eloquent description in the original article, Carr presaged the presence of autoantibodies, before they had been thoroughly researched. Since then, five different autoantibodies (mGluR5, Hu, NMDAR, SOX, PCA2) have been associated with Hodgkin's disease. It is interesting to note the divergent outcomes of Shakespeare's character and the patient in the original description by Carr, the latter recovering to lead a normal life, and the former deceased. Conclusions: Although there is little relationship between the fictional character and the syndrome, both imply the unintentional trigger of self-harm (suicide in one case, autoimmunity in the other), thus remaining associated.
RESUMO El síndrome de Ofelia describe la asociación entre pérdida de memoria y enfermedad de Hodgkin, en memoria del personaje de La Tragedia de Hamlet, Príncipe de Dinamarca, de William Shakespeare. Sin embargo, existen diferencias entre ambos. Objetivo: Revisar los orígenes y usos del epónimo a través del artículo original, su relación con el personaje y los autoanticuerpos relacionados. Métodos: Revisión narrativa histórica. Resultados: Además de una descripción elocuente, el artículo original prefigura los autoanticuerpos, cuando no se buscaban de rutina. Desde entonces, cinco distintos (mGluR5, Hu, NMDAR, SOX, PCA2) han sido asociados. Cabe destacar, que el desenlace del personaje y del paciente fueron diametralmente opuestos, el primero falleció y el segundo se recuperó, llevando una vida normal. Conclusiones: A pesar de la poca relación entre el personaje y el síndrome, ambos implican el desencadenamiento no intencional de daño auto-inflingido (suicidio en un caso, autoinmunidad en el otro), manteniendo así la adecuacía.
Asunto(s)
Humanos , Masculino , Femenino , Historia del Siglo XX , Enfermedad de Hodgkin/inmunología , Medicina en la Literatura , Trastornos de la Memoria/inmunología , Autoanticuerpos , Síndrome , Encefalitis LímbicaRESUMEN
Se informa de un paciente femenina de cuatro años de edad, con diagnóstico de Síndrome de Miller-Fisher. Requirió ventilación mecánica y fue tratada con esquema de inmunoglobulina, obteniéndose recuperación completa pocas semanas después. Se concluye que la inmunoglobulina es un tratamiento seguro y efectivo en niños que presentan este diagnóstico, coincidiendo con los resultados obtenidos por otros investigadores