Cerebellar Cognitive Affective Syndrome in Costa da Morte Ataxia (SCA36).

SCA36 is an autosomal dominant spinocerebellar ataxia (SCA) affecting many families from Costa da Morte, a northwestern region of Spain. It is caused by an intronic GGCCTG repeat expansion in NOP56. In order to characterize the cognitive and affective manifestations of this cerebellar disease, a group of 30 SCA36 mutation carriers (11 preataxic and 19 ataxic patients) were assessed with a comprehensive battery of standardized tests. Phonological verbal fluency - but not semantic fluency - was already mildly impaired in preataxic subjects. In ataxic patients, both phonological and semantic fluencies were significantly below normal. Depression, while more frequent and prominent in ataxic patients, was also often present in the preataxic stage. This is the first systematic study supporting the presence of a mild cerebellar cognitive and affective syndrome in SCA36. Routine evaluation of cognitive and emotional spheres in SCA36 patients as well as asymptomatic mutation carriers should allow early detection and timely therapeutic intervention.

Benign paroxysmal positional vertigo of the posterior semicircular canal: efficacy of Santiago treatment protocol, long-term follow up and analysis of recurrence.

OBJECTIVES: To evaluate the efficacy of the Santiago treatment protocol for benign paroxysmal positional vertigo of the posterior semicircular canal, to analyse recurrence and to establish prognostic factors. MATERIAL AND METHODS: Four hundred and twelve patients with unilateral benign paroxysmal positional vertigo of the posterior semicircular canal were treated with the Semont manoeuvre and, if symptoms did not resolve, successive application of three Epley manoeuvres plus Brandt-Daroff exercises. RESULTS: Symptoms resolved in 404 patients (98.1 per cent); a single Semont manoeuvre was sufficient in 334 (81.2 per cent). Aetiology had no impact on resolution of symptoms or number of manoeuvres required. The estimated likelihood of recurrence was 14 per cent in the first year and 27 per cent after 10 years. The only factor indicating a worse prognosis was recurrence. CONCLUSION: In unilateral benign paroxysmal positional vertigo of the posterior semicircular canal, the above treatment protocol cured 98 per cent of patients. More than half of recurrences occurred in the first year. None of the analysed factors increased the likelihood of recurrence.

[Rehabilitation treatment of the balance: design of a general protocol with dynamic computerized posturography]. / Tratamiento rehabilitador de los trastornos del equilibrio. Diseño de un protocolo general con la posturografía dinámica computerizada.

The dynamic posturography (PD) is an amply diffused method for the study of balance. Its principal utility is the design of therapeutic strategies based on the vestibular rehabilitation. In determined patients and with the help of other elements of rehabilitation the PD favour the compensation of the degradation ofthe secondary balance to determinated pathologic entities that affect the vestibular peripheric or central system. Normally individualized strategies for each patient are employed and although this is the more correct, we consider necessary to propose global mesures that facilitate the rehabilitation program and the comparison of the results of different authors.

Tratamiento rehabilitador de los trastornos del equilibrio. Diseño de un protocolo general con la posturografía dinámica computerizada / Rehabilitation treatment of the balance: design of a general protocol with dynamic computerized posturography

La posturografía dinámica (PD) es un método ampliamente difundido para el estudio del equilibrio. Su principal utilidad es el diseño de estrategias terapéuticas basadas en la rehabilitación vestibular. En determinados pacientes y con apoyo de otros elementos de rehabilitación la PD favorece la compensación del deterioro secundario a determinadas entidades patológicas que afectan al sistema vestibular periférico o central. Normalmente se emplean estrategias individualizadas por cada paciente, y aunque esto es lo más correcto, consideramos necesario proponer unas pautas globales que faciliten la programación de la rehabilitación y la comparación de los resultados obtenidos por distintos autores

The dynamic posturography (PD) is an amply diffused method for the study of balance. Its principal utility is the design of therapeutic strategies based on the vestibular rehabilitation. In determined patients and with the help of other elements of rehabilitation the PD favour the compensation of the degradation of the secondary balance to determinated pathologic entities that affect the vestibular peripheric or central system. Normally individualized strategies for each patient are employed and although this is the more correct, we consider necessary to propose global measures that facilitate the rehabilitation program and the comparison of the results of different authors

[Our experience in the treatment of the tumors of parotid gland]. / Nuestra experiencia en el tratamiento de los tumores de glándula parótida.

We analyse our results in the treatment of the tumors of the parotid gland, diagnosticated from 1981 till end of 2004. We saw 132 patients with tumors of the parotid, being 41 of them malignant. The surgical technique more used was the total parotidectomy with conservation of the facial nerve. The global survival obtained in the malignant tumors was 60% after five years. Surgery is the treatment of election in the tumors of epitelial origin of the parotid gland. The derived of the lymphoid tissue must be treated by other ways and are inheritance of the hematologic oncology.

Nuestra experiencia en el tratamiento de los tumores de glándula parótida / Our experience in the treatment of the tumors of parotid gland

Analizamos nuestros resultados en el tratamiento de los tumores de la glándula parótida diagnosticados desde 1981 hasta finales del 2004. Vimos 132 pacientes con tumores de la parótida, siendo 41 de ellos malignos. La técnica quirúrgica más usada fue la parotidectomía total con conservación del nervio facial. La supervivencia global que obtuvimos en los tumores malignos fue del 60% a los cinco años. La cirugía es el tratametno de elección de los tumores de estirpe epitelial de la glándula parótida. Los derivados del tejido linfoide deben tratarse por otros medios y son patrimonio de la oncología hematológica

We analyse our results in the treatment of the tumors of the parotid gland, diagnosticated from 1981 till end of 2004. We saw 132 patients with tumors of the parotid, being 41 of them malignant. The surgical technique more used was the total parotidectomy with conservation of the facial nerve. The global survival obtained in the malignant tumors was 60% after five years. Surgery is the treatment of election in the tumors of epitelial origin of the parotid gland. The derived of the lymphoid tissue must be treated by other ways and are inheritance of the hematologic oncology

[Epidemiological notes on nasal bleeding]. / Notas epidemiológicas sobre el sangrado nasal.

The incidence of epistaxis is difficult to calculate because only a 10% of them look for medical attention. In this study the nasal bleeding means the 13.3% of the ENT emergencies (279 patients). Its incidence was 6 cases/10,000/year. The majority were over 50 years old (57.3%). There was season variations. The anterior epistaxis were more frequent but he posterior ones increased over the 40. An 18% had rebleeding more significatively with age and localization. We discuss the mechanisms that can explain the male dominance, the season incidence, and the old ages of the patients with hospital epistaxis.

[Pleomorphic adenoma of the tongue base. A new case of big size]. / Adenoma pleomorfo de base de lengua. Un nuevo caso de gran tamaño.

The pleomorphe adenomas (PA) of the tongue base are rare (there are only 6 cases published until now) We present one case of a male 42 years old with a PA of the tongue base very big but with few symptoms. The FNAB and MRI were the key for the diagnosis and therapy. The treatment was surgical with a trans oral resection. The patient is free of tumor after seven years.

Notas epidemiológicas sobre el sangrado nasal / Epidemiologic notes on nasal bleeding

La incidencia de la epistaxis es difícil de calcular porque sólo un 10 por ciento buscan atención médica. En el presente estudio prospectivo el sangrado nasal supone el 13,3 por ciento de las urgencias ORL (279 pacientes), su incidencia fue 6 casos/10.000/año. Los mayores de 50 años fueron mayoría (57,3 por ciento), y existió variación estacional. Las epistaxis anteriores fueron más frecuentes aunqeu las posteriores aumentaron tras los 40. Resangraron un 18 por ciento teniendo significación la edad y la localización. Discutimos los mecanismos que expliquen la predominancia masculina, la incidencia estacional, y la edad avanzada de los pacientes con epistaxis hospitalarias (AU)

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Adenoma pleomorfo de base de lengua. Un nuevo caso de gran tamaño / Pleomorphe adenoma of the tongue base. A new case of big size

Los adenomas pleomorfos (AP) localizados en la base de lengua, constituyen un hallazgo inusual (sólo han sido publicados seis casos hasta la fecha). El presente art´ciulo presenta el caso de un varón de 42 años que presentó un AP de base de lengua muy voluminoso aunque paucisintomático. La PAAF y la RM fueron determinatnes en el diagnóstico y plan terapéutico. El tratamiento fue quirúrgico mediante resección transoral. El paciente está libre de tumor después de siete años (AU)

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[An immunohistochemistry study of p53 protein in squamous cell carcinoma of the larynx]. / Proteína P53 mediante inmunohistoquímica en el carcinoma epidermoide de laringe.

P53 protein detects damages in DNA, stopping the cellular cycle to repair it and stimulates "apoptosis" or programmed death cell when it cannot do it. We present an immunohistochemistry study of 58 squamous cell carcinomas of larynx operated between 1991 and 1996 and in which we detected the overexpression of P53 in 31 cases (53.44%). We did not find a statistic relationship between survival and the detection of P53 protein using Log Rank statistic analysis; the survival media for the wild-type protein cases was 64 months (very similar to native cases).

[Epidemiological aspects of otosclerosis (1). Its frequency in comparison with other ear pathologies, incidence and prevalence]. / Aspectos epidemiológicos de la otosclerosis (I). Frecuencia en comparación a otras patologías otológicas, incidencia y prevalencia.

A planned study was made with the group of patients seen for first time at Otologic External Consulting Department of ENT Service of the universitary Hospital Complex of Santiago de Compostela. The total amount 1,050 patients, 83 of them suffering otosclerosis. Most frequently diagnosed was sensorineural deafness (29.1%), in second place were chronic middle otitis (27.8%) and as last one was otosclerosis (8.7%). Clinic otosclerosis amounted for 19.20 cases of each 100,000 inhabitants yearly and stapedectomies performed 7.78. Prevalence was evaluated at 7.13 by 1,000 (10.86 women and 3.14 men). Higher value compared with other series published previously are linked to differences on sanitary development as well as health concept and the type of community where people lived through.

[Epidemiological aspects of otosclerosis (II): sex, hereditary factors, its course before diagnosis, age at diagnosis and its beginning]. / Aspectos epidemiológicos de la otosclerosis (II). Sexo, factores hereditarios, tiempo de evolución hasta el diagnóstico, edad de diagnóstico y de comienzo.

Retrospective study done with a group of 401 patients enduring otosclerosis. Seventy percent were women. 59% with positive familial antecedents. Illness was recognized as midpoint, at 44.5 years and symptomatology started at 35.3 years. Lasting time of condition till diagnosis assured was 8.5 years (higher than 5 years the half of cases and between 1 an 5 years the third part of them), these numbers considerably decreasing regarding other older series. Figures lower in those cases without familiar otosclerotic antecedents, the same than in unilateral specimen. The only significative influence of sex where tinnitus aurium among women (70.7% to face up 51.7%).

[Surgical treatment of laryngotracheal stenoses. Our experience]. / Tratamiento quirúrgico de las estenosis laringotraqueales: nuestra experiencia.

Laryngo-tracheal stenoses (LTS) treatment is a problem of difficult management for the otolaryngologist. There are many and varied techniques in order to solve the conundrum. The aim of this article is to analyze retrospectively the outcomes of surgical treatment of LTS in our Institution. We have checked 11 cases (9 men and 2 women, arithmetic mean 30.7 years) operated through surgical procedures during a 6 years lapse of time. All patients were endotracheally intubated prior the stenosis appearance. Stenosis location most encountered were subglottic-tracheal combination. To the end of the study 44 percent of the series was successfully cured and the 33 percent only partially recovered. The employed techniques (endoscopic or open surgery) are revised at the light of today's literature.

Proteína P53 mediante inmunohistoquímica en el carcinoma epidermoide de laringe / An inmunohistochemistry study of P53 protein in squamous cell carcinoma of the larynx

La proteína P53 actúa detectando el ADN celular dañado, deteniendo el ciclo celular para repararlo y estimulando la "apoptosis" o muerte celular programada cuando no lo consigue. Realizamos un estudio mediante inmunohistoquímica de 58 carcinomas epidermoides de laringe intervenidos de su patología entre 1991 y 1996 siendo la proteína mutada positiva en 31 casos (53,44 por ciento). No encontramos una relación estadísticamente significativa mediante el análisis estadístico de Log Rank entre la supervivencia y la positividad para la proteína P53, siendo la supervivencia media para los casos negativos para la proteína de 64 meses de media, muy similar a los casos que presentan la proteína mutada (AU)

P53 protein detects damages in DNA, stopping the cellular cycle to repair it and stimulates «apoptosis» or programmed death cell when it cannot do it. We present an immunohistochemistry study of 58 squamous cell carcinomas of larynx operated between 1991 and 1996 and in which we detected the over expression of P53 in 31 cases (53.44%). We did not find a statistic relationship between survival and the detection of P53 protein using Log Rank statistic analysis; the survival media for the wild-type protein cases was 64 months (very similar to native cases) (AU)

Aspectos epidemiológicos de la otosclerosis (I). Frecuencia en comparación a otras patologías otológicas, incidencia y prevalencia / Epidemiological aspects of otosclerosis (I). Its frequency comparatively with other otic conditions, incidence and prevalence

Se ha realizado un estudio prospectivo de los pacientes atendidos por primera vez en las consultas externas de Otología del Servicio de Otorrinolaringología del Complejo Hospitalario Universitario de Santiago de Compostela. En total han sido incluidos 1.050 pacientes, 83 de ellos con otosclerosis. El diagnóstico más frecuente fue la hipoacusia neurosensorial (29,1 por ciento), en segundo lugar la otitis media crónica (27,8 por ciento) y en tercero la otosclerosis con el 8,7 por ciento. La incidencia de otosclerosis clínica fue de 19,20 casos por 100.000 habitantes y año y la de estapedectomías de 7,78. La estimación de la prevalencia fue de 7,13 por 1.000 (10,86 para mujeres y 3,14 para hombres). Los valores superiores respecto a otras series publicadas previamente probablemente responsan a diferencias en el desarrollo sanitario, así como al concepto de salud y el tipo de sociedad en que se viva. (AU)

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Aspectos epidemiológicos de la otoesclerosis (II). Sexo, factores hereditarios, tiempo de evolución hasta el diagnóstico, edad de diagnóstico y de comienzo / Epidemiological aspects of otoesclerosis (II): sexe, hereditary factors, development course till diagnosis. Age of diagnose and start of the illnes

Se ha realizado un estudio retrospectivo de 401 pacientes diagnosticados de otosclerosis. El 70 por ciento eran del sexo femenino. Existieron antecedentes familiares positivos en el 50 por ciento. La edad del diagnóstico fue de media 44,3 años y la de inicio de los síntomas 35,3 años. El tiempo de evolución hasta el diagnóstico fue de media 8,5 años (en caso la mitad, superior a 5 años, y en la tercera parte entre 1 y 5 años) si bien ha disminuido considerablemente respecto a otras series más antiguas. Ha sido inferior en aquellos sin antecedentes familiares de otosclerosis, al igual que en las formas unilaterales. La única influencia significativa del sexo en las otras variables estudiadas ha sido en la frecuencia de acúfenos, que fueron más frecuentes en las mujeres (70,7 por ciento frente a 51,7 por ciento) (AU)

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Tratamiento quirúrgico de las estenosis laringotraqueles: nuestra experiencia / Surgical treatment of laryngo tracheal stenoses. Our experience

El tratamiento de las estenosis laringotraqueales (ELT) es un problema de difícil manejo para el otorrinolaringólogo. Para su resolución existen numerosas y variadas técnicas. EL objetivo de este estudio es analizar retrospectivamente los resultados del tratamiento quirúrgico de las ELT en nuestra institución. Revisamos 11 pacientes () varones y 2 mujeres, edad media de 30,7 años) en los que se realizaron 20 procedimientos quirúrgicos en un período de 6 años. Todos los pacientes estuvieron intubados endotraquealmente antes de la formación de la estenosis. La localización de la obstruciión más frecuente fue la subglototraqueal combinada. Al final del estudio el 44 por ciento de la serie fue tratado con éxito y en un 33 por ciento adicional se obtuvo un éxito parcial. Las técnicas empleadas (endoscópicas o quirúrgicas abiertas) son revisadas a la luz de la literatura (AU)

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Factores pronósticos del resultado anatómico y funcional de las miringoplastias / Pronostic factors that influence the anatomic and functional outcome in myringoplasties

Los objetivos de la miringoplastia son esencialmente dos: restablecer la integridad de la membrana timpánica y mejorar la audición. Hemos realizado un estudio retrospectivo de las miringoplastias realizadas en nuestro servicio, entre 1996 y 2000, correspondiendo a 197 intervenciones quirúrgicas, en 162 pacientes. Las características más frecuentes de los oídos intervenidos fueron localización central de la perforación, oído seco, buena ventilación tubárica y colocación del injerto de fascia del temporal underlay. El abordaje endo o retroaural fue similar en número. Obtuvimos un 73,6% de éxito anatómico, aunque en algunos casos fue necesario reintervenir al paciente. Al analizar los factores de riesgo en el resultado anatómico, observamos que únicamente influye la localización de la perforación, con mayor porcentaje de éxito en las perforaciones posteriores y menor en las subtotales. Se analizó el resultado funcional en los oídos con éxito anatómico, encontrando un 75,6% de mejoría de los umbrales auditivos con un gap menor de 20 dB. Los factores que influyeron en la mejoría de la audición fueron la experiencia del cirujano y la presencia de alteraciones de oído medio (AU)

The objectives of myringoplasty are essentially two: restore the eardrum integrity and improve hearing level. We have carried out a retrospective study of myringoplasties performed in our Department, between 1996 to 2000, corresponding to 197 surgical interventions, in 162 patients. The characteristics more frequently seen for operating ears were central localization of tympanic membrane perforation, dry ear, good eustaquian tube function and underlay technique placing fascia graft. Endaural or retroauricular approach were similar in number. We obtained 73.6% of anatomic success, though in some cases we needed to reoperate the patient. When we analysed risk factors of anatomic success, we noticed than only the tympanic perforation site had influence, with better rate in posterior perforations and lower rate in subtotal perforations. We analysed functional results in ears with anatomic success, finding 75.6% of hearing level improvement with a gap of less than 20 dB. Factors influencing hearing improvement were surgeon experience and the presence of middle ear alterations (AU)

[Possible factors influencing rehabilitation of the total laryngectomy patient using esophageal speech]. / Estudio de posibles factores que influyen en la rehabilitación del laringuectomizado total mediante voz esofágica.

One of the handicaps of patients that have had a total laryngectomy is the loss of oral speech. There are three possibilities to rehabilitate these patients; surgery, voice prosthesis or esophageal speech. This last one appears as an inexpensive, non-invasive rehabilitation method; it does not need a complex learning and in spite of new technics, it continues to be an effective method in the social rehabilitation of laryngectomized patients. We have made a retrospective study of patients who underwent TL between 1992-1998 and that were rehabilitated by esophageal voice learning. Three factors have been evaluated: first, those related to the patient (gender, age, study level...); second, those related to the treatment itself [TL, pharyngolaryngectomy (PTL), TL plus radiotherapy...] and third, those related to the rehabilitation (technics, delayed on starting, number of sessions...). Data were compared with rehabilitation outcome: good (usually using esophageal voice), medium (speaking sometimes) and bad (not speaking). 74.1% had an acceptable outcome and they use esophageal voice with more or less difficulty to communicate, and this outcome increases to 95.8% when we do not take into account 7 patients who gave-up rehabilitation before finishing the first five sessions.