[Multidrug resistant narcolepsy]. / Narcolepsia multirresistente.

INTRODUCTION: Narcolepsy type 1 is a focal degenerative disease of the hypothalamus that selectively affects orexin (hypocretin)-producing neurons. It presents multiple clinical manifestations, both in wakefulness and in sleep. The symptoms are often so disruptive that they cause enormous suffering and impair patients' quality of life. Although a non-pharmacological approach is sometimes sufficient, the vast majority of patients need medication for adequate clinical management. CASE REPORT: A male who, at 43 years of age, began to present acutely with excessive daytime sleepiness and episodes of cataplexy. After a thorough examination, he was diagnosed with narcolepsy type 1. Throughout the course of the disease, he was prescribed antidepressants, neurostimulants and sodium oxybate, in monotherapy or in combination. The response to pharmacological treatment was insufficient and accompanied by numerous side effects. Following the introduction of pitolisant, there was a marked improvement in his symptoms and a reduction in the dose of the other drugs and their adverse effects was achieved. CONCLUSION: A number of measures are now available to address the cardinal symptoms of the disease, although there are still cases that are resistant to anti-narcoleptic treatment. Drugs with mechanisms of action that act upon receptors in the histaminergic system can be very useful in these cases.

TITLE: Narcolepsia multirresistente.Introducción. La narcolepsia de tipo 1 es una enfermedad degenerativa focal del hipotálamo que afecta selectivamente a las neuronas productoras de orexina (hipocretina). Presenta múltiples manifestaciones clínicas, tanto en vigilia como en sueño. Con frecuencia, los síntomas son tan disruptivos que ocasionan enorme sufrimiento y deterioro de la calidad de vida de los pacientes. Aunque en ocasiones es suficiente con un abordaje no farmacológico, la gran mayoría de los enfermos necesita medicación para un adecuado control clínico. Caso clínico. Varón que a los 43 años comenzó a presentar de forma aguda excesiva somnolencia diurna y episodios de cataplejía. Tras un exhaustivo estudio se le diagnosticó narcolepsia de tipo 1. A lo largo de la evolución de la enfermedad se le prescribieron antidepresivos, neuroestimulantes y oxibato sódico, en monoterapia o en combinación. La respuesta al tratamiento farmacológico fue insuficiente y se acompañó de numerosos efectos secundarios. Tras la introducción de pitolisant se objetivó una franca mejoría de los síntomas, y se consiguió reducir la dosis de los otros fármacos y de sus efectos adversos. Conclusión. Son numerosas las medidas disponibles en la actualidad para abordar los síntomas cardinales de la enfermedad, aunque siguen existiendo casos resistentes al tratamiento antinarcoléptico. Los fármacos con mecanismos de acción sobre receptores del sistema histaminérgico pueden resultar de gran utilidad en estos casos.

RSV infection in a macrophage-cell line activates the non-canonical NF-κB pathway and induces pro-inflammatory cytokine expression.

Respiratory syncytial virus (RSV) is a highly prevalent infectious agent that causes severe respiratory tract illnesses in infants and children worldwide. Children who have suffered severe RSV infections during infancy are prone to develop recurrent episodes of wheezing and asthma that may be associated with viral persistence. RSV infections in humans and animal models are characterized by extensive inflammatory responses. Epithelial cell lines acutely infected by RSV have shown activation of the NF-κB signaling through two independent pathways: the canonical pathway, mediated by RelA and p50 subunits, and the non-canonical pathway, mediated by the subunits RelB and p52. Herein, we investigated the state of activation of the canonical and non-canonical NF-κB signaling pathways in macrophages either acutely or persistently infected by RSV and examined the expression of pro-inflammatory mediators. Activation of NF-κB subunits was analyzed through Western blot assays using acutely RSV-infected epithelial cells as a control. The expression levels of two pro-inflammatory cytokines and a chemokine were determined by quantitative RT-PCR and through immunobead assays. The results showed that p52 was abundant during acute and persistent RSV infection, indicating that macrophages predominantly activate the non-canonical pathway. We also observed activation of IL-1ß, TNF-α and CCL5/RANTES transcription, though at higher levels in persistently infected macrophages than in acutely infected macrophages. In contrast, the protein levels of these cytokines/chemokine did not correlate with their mRNA transcription, as quantitation displayed higher levels during acute infection than in persistent infection, suggesting post-transcriptional regulation by RSV persistence.

[Postural control related to age in patients with benign positional paroxysmal vertigo]. / Control postural según la edad en pacientes con vértigo posicional paroxístico benigno.

OBJECTIVE: To study the relationship between age and posturography in patients with benign positional paroxysmal vertigo. MATERIAL AND METHODS: Prospective study performed in 65 patients with benign positional paroxysmal vertigo (BPPV) in any variant. Sensory Organization Test outcomes were recorded and compared with their equivalents in a control group by means of scatter-plot diagrams and regression line. For statistical study, Mann-Whitney's U-test was used. RESULTS: Slope for regression lines in composite was -0.0934 in group NORMAL; in group BPPV it was -0.4284. This difference is due to conditions 5 and 6. Results were statistically significative. CONCLUSIONS: BPPV patients have a worse postural control than control group. The difference is bigger the older the patient is. It is due to a failure in conditions 5 and 6, so it should be from a vestibular origin.

Control postural según la edad en pacientes con vértigo posicional paroxístico benigno / Postural control related to age in patients with benign positional paroxysmal vertigo

Objetivo: Estudiar la relación entre la edad y el control postural en pacientes con vértigo posicional paroxístico benigno. Material y método: Estudio prospectivo realizado en 65 pacientes diagnosticados de vértigo posicional paroxístico benigno (VPPB) en cualquiera de sus variantes. Se miden los resultados del Test de Organización Sensorial, comparándolas con sus equivalentes en un grupo control mediante nube de puntos y recta de regresión. Para el estudio estadístico se usó el test U de Mann-Whitney. Resultados: En el grupo NORMAL, la recta de regresión del resultado global tiene una pendiente de –0,0934; en el grupo VPPB, de –0,4284. Esta diferencia se debe fundamentalmente a las condiciones 5 y 6, siendo los resultados estadísticamente significativos. Conclusiones: Los pacientes con VPPB tienen un peor control postural que el grupo control. Esta diferencia es mucho más acusada cuanto mayor es la edad del paciente, y es de origen vestibular

Objective: To study the relationship between age and posturography in patients with benign positional paroxysmal vertigo. Material and methods: Prospective study performed in 65 patients with benign positional paroxysmal vertigo (BPPV) in any variant. Sensory Organization Test outcomes were recorded and compared with their equivalents in a control group by means of scatter-plot diagrams and regression line. For statistical study, Mann-Whitney’s U-test was used. Results: Slope for regression lines in composite was –0.0934 in group NORMAL; in group BPPV it was –0.4284. This difference is due to conditions 5 and 6. Results were statistically significative. Conclusions: BPPV patients have a worse postural control than control group. The difference is bigger the older the patient is. It is due to a failure in conditions 5 and 6, so it should be from a vestibular origin

Postural control according to the age in patients with benign paroxysmal positional vertigo.

OBJECTIVES: To study the relationship between age and postural control in patients with benign paroxysmal positional vertigo. MATERIAL AND METHODS: Prospective study performed in 65 diagnosed patients with benign paroxysmal positional vertigo (BPPV) in any of its variants. The results of the Sensory Organization Test are compared with their equivalent in a control group by means of scatterplot and regression lines. For the statistical study the Mann-Whitney's U test was used. RESULTS: In the NORMAL group, the regression line for composite has a 0.0934 slope; in the BPPV group, 0.4284. This difference is due to conditions 5 and 6 fundamentally, the results being statistically significant. CONCLUSIONS: The patients with BPPV have a worse postural control than the group control. This difference is much more pronounced the older the patient, and is of vestibular origin.

Resultados del tratamiento del cáncer laríngeosupraglótico mediante cirugía endoscópica con láser CO2 / Treatment of supraglottic cancer by means of CO2 laser endoscopic surgery: results

Hemos estudiado una serie de 253 pacientes con cáncer de laringe, tratados mediante cirugía endoscópica con láser CO2.Cincuenta pacientes presentaban una localización supraglótica. Tras analizar diversos parámetros, se concluye que la cirugía endoscópica láser en los carcinomas supraglóticos tiene una tasa de supervivencia y un tiempo libre de enfermedad muy similares a la cirugía por vía externa. El tiempo de ingreso es mucho más reducido, el riesgo de complicaciones menor y el coste por proceso más bajo que con la cirugía convencional. Se evitó la traqueotomía en la mayor parte de los pacientes. Las recidivas pueden tratarse de nuevo por vía endoscópica, pero, la necesidad de realizar una laringectomía total tras una recidiva, se considera un factor de mal pronóstico (AU)

A group of 253 patients with laryngeal carcinoma was treated by endoscopic laser-CO2 surgery, 50 of them presented a supraglottic localization. The follow up period ranged from 1 to 10 years. From the analysis of different parameters (age, site, stage, histopathology, etc) it could be concluded that survival and free-symptoms-time rates in patients treated with laser endoscopy were similar to those obtained in patients treated with conventional external surgery. However, the endoscopic laser-CO2 surgery reported several advantages such as: no pharyngostoma, less complications, shorter stay in hospital and, consequently, lower health costs. In addition, tracheotomy was almost never necessary. Recurrencies can be also treated endoscopically, but, when total laryngectomy is needed following recurrency, this is considered to be a bad prognostic factor (AU)

[Results of supraglottic laryngeal cancer treatment with endoscopic surgery using CO2 laser]. / Resultados del tratamiento del cáncer laríngeo supraglótico mediante cirugía endoscópica con láser CO2.

A group of 253 patients with laryngeal carcinoma was treated by endoscopic laser-CO2 surgery, 50 of them presented a supraglottic localization. The follow up period ranged from 1 to 10 years. From the analysis of different parameters (age, site, stage, histopathology, etc) it could be concluded that survival and free-symptoms-time rates in patients treated with laser endoscopy were similar to those obtained in patients treated with conventional external surgery. However, the endoscopic laser-CO2 surgery reported several advantages such as: no pharyngostoma, less complications, shorter stay in hospital and, consequently, lower health costs. In addition, tracheotomy was almost never necessary. Recurrencies can be also treated endoscopically, but, when total laryngectomy is needed following recurrency, this is considered to be a bad prognostic factor.

[Heat and vestibular stimulation]. / Calor y estimulación vestibular.

In 1905 Barany analyzed the nystagmus that appears during the irrigation of the ear with cold and hot water, justifying the phenomenon due to convectives currents in the horizontal semicircular canal. This hypothesis has been widely accepted until doubt was casted on it by the experiences carried out in the Skylab. Of the various mechanisms that could be responsible for the vestibular stimulation in the caloric tests, those which entail convection currents or endolymph expansion would need--we think--thermal gradients very superior to those occurring during these tests, that according to our calculations--always pro such hypothesis--seem insufficient.

[Polymorphic reticulosis of the rhinopharynx]. / Reticulosis polimorfa de rinofaringe.

"Malignant centrofacial granulomatosis" is an unusual but devastating spectrum of lymphoproliferative disorders that is now thought to include several entitites, such as polymorphic reticulosis or peripheral T-cell lymphoma. Characteristically there are destructive centrofacial lesions which often produce difficulties for the diagnostic to the clinician and pathologist. The case of a 71-year-old man diagnosed in our Department as polymorphic reticulosis sitting on the rhinopharynx is reported.

[Bimastoid leads and bilateral stimulation in the recording of auditory-evoked brain-stem potentials]. / La derivación bimastoidea y estimulación bilateral en el registro de potenciales evocados auditivos de tronco cerebral.

The recording of auditory brain stem potentials (ABR) with bimastoid leads and bilateral stimulation makes it possible to evaluate relative cochlear function and the type of hearing loss. The theoretical background for this technique and the results obtained in patients with disease of the internal-middle ear, cochlea, and auditory nerve are discussed.

The association of human papillomavirus type 16 E6 and E7 antibodies with stage of cervical cancer.

OBJECTIVE: Previous investigators have reported higher HPV type 16 antibody positivity among cervical cancer patients than among healthy women. The objective of this study was to determine the association of HPV 16 antibody levels with the stage of cervical cancer. METHODS: Pretreatment tumor biopsies and sera were obtained from 137 newly diagnosed cervical cancer patients residing in Mexico. Using peptide ELISA and radioimmunoprecipitation assay (RIPA), HPV 16 E6- and E7-specific antibodies were measured. RESULTS: By ELISA, elevated antibody titers to HPV 16 E6 and E7 were detected in 16.8 and 32.8% of the women, respectively. While sera positivity did not differ by disease stage, the mean absorbance in the E7-positive sera was 0.42, 0.62, 0.91, and 0.81 for stages I to IV, respectively. Using RIPA, anti-E6 and E7 positivity was demonstrated in 46.7 and 38.7% of the females, respectively. Although no difference across disease stage was detected for E6, increasing proportions of positivity to E7 with stage of disease was detected. The rates for increasing disease stage were 0.14, 0.37, 0.40, and 0.67. Sera from the 6-month postradiation follow-up examinations of a small group of patients demonstrated a statistically significant decrease in antibody positivity from pretreatment positivity to HPV 16 E6 (n = 14; P = 0.01) and HPV 16 E7 (n = 20; P = 0.0001) using ELISA. CONCLUSIONS: These data suggest that HPV 16 E7 antibody positivity may be associated with stage of cervical cancer. Such immune parameters may be applicable to disease staging, monitoring of recurrence and, perhaps, diagnosis. Further investigation into the relationship of HPV 16 E6 and E7 antibodies with stage of cervical cancer and response to therapy is warranted.

[Ectopic primitive thyroid papillary carcinoma: report of a fatal case and review of literature]. / Carcinoma papilar primitivo de quiste tirogloso: aportación de un caso letal y revisión de la literatura.

One percent of excised thyroglossal duct remnants exhibit histologic malignancy, a thyroid papillary carcinoma in 80% of the cases. The prognosis is excellent except for anaplastic types. We report the case of a 44-year-old woman with primitive thyroid papillary carcinoma diagnosed histologically as thyroglossal cyst. Its unusual aggressiveness produced local and regional spread, metastases and the patient's death in spite of treatment: Sistrunk operation, local excision of a recurrence and laryngothyroidectomy. Thyroid histology revealed the absence of carcinomatous foci. We discuss the most noteworthy aspects of the case in relation to other reports.

[Radiotherapy of cancer of the endometrium. Analysis of 53 cases]. / Radioterapia en cáncer de endometrio. Análisis de 53 casos.

Fifty three patients with endometrial carcinoma received radiotherapy from 1986 to 1987, at the Hospital de Oncologia Centro Medico Nacional. Radiotherapy was given preoperatively in five patients, postoperatively in thirty nine patients, and radical in nine cases. Obesity, Hypertension and Diabetes were present in 60%. The patients have been in control from 3 to 44 months, with average of 18 months. Diagnosis was realized for genital bleeding 45/53 (85%), and increased uterine size 6/53 (11%). There were stage I 24/53 (45%), stage II 13/53 (24%) patients. Non classified eight cases, five of them were without tumoral activity at initial valoration, and three had tumor present. We analyzed stage treatment utilized, correlated with morbidity, tumoral response, free survival. We concluded that staging surgery is effective to chose the type of treatment.

A remotely programmable insulin delivery system. Successful short-term implantation in man.

A remotely controlled, programmable insulin delivery system was implanted in a diabetic man and the feasibility of the technique was examined. Specific problems included (1) development of an appropriate surgical approach, (2) identification of methods to assess the integrity of the insulin delivery system following implantation, and (3) assessment of plasma glucose and free-insulin profiles obtained with the implanted system. The insulin pump was implanted submuscularly through a midline abdominal incision. The insulin reservoir was placed subcutaneously to allow percutaneous refilling. The insulin delivery catheter terminated in the peritoneal space. No postoperative wound infection occurred and rapid healing of the surgical site ensued. In vivo assessment of the system included (1) dye contrast roentgenography, (2) vasopressin stimulation, and (3) reservoir volume monitoring. Short-acting insulin was then placed in the implanted reservoir and delivered by the system for one month. Mean plasma glucose concentration declined to normal levels, as did glycosylated hemoglobin. Plasma insulin profiles were normalized with appropriate insulin peaks with each meal. We conclude that implantation of a remotely programmable insulin pump is feasible in type I diabetic man. Additional studies are necessary to define which patients will benefit from this type of insulin delivery system.