[Gastrostomy tubes in patients with amyotrophic lateral sclerosis: indications, safety and experience in a tertiary care centre]. / Sondas de gastrostomía en pacientes con esclerosis lateral amiotrófica: indicaciones, seguridad y experiencia en un centro de tercer nivel.

AIM: The aim of this study is the clinical characterisation of patients diagnosed with amyotrophic lateral sclerosis (ALS), who were indicated for the placement of a gastrostomy tube (G-Tube) in the period 2007-2017 in the Complejo Asistencial Universitario de Salamanca, and to find possible associated prognostic factors. MATERIAL AND METHODS: It is a descriptive study of clinical cases. RESULTS: Between 2007 and 2017, 139 cases of ALS were assessed and a G-Tube was indicated in 29 of them. Of these 29, on two occasions it was not performed, one due to the patient refusing and the other because of clinical deterioration. Of the total number of G-Tubes, 21 were placed by endoscopy, four by interventional radiology and two by surgery. The main indications were: in 10 cases, for severe dysphagia; in eight cases, for weight loss >10%; and in nine cases, for reduced forced vital capacity <50%. Immediate complications (first 24 hours) included: two intraprocedural desaturations, one ostomy infection, one abdominal pain, one paralytic ileus and one respiratory failure resulting in death. Late complications (first month) include pneumonia, intestinal ischaemia, cellulitis and poor control of secretions. The main cause of death was respiratory failure. The median time elapsed between G-Tube placement and death was 7.89 months. An improvement in analytical nutritional parameters was observed, without showing statistical significance, as a prognostic factor. CONCLUSION: G-Tube placement is an effective and safe procedure to ensure enteral nutrition in ALS patients.

TITLE: Sondas de gastrostomía en pacientes con esclerosis lateral amiotrófica: indicaciones, seguridad y experiencia en un centro de tercer nivel.Objetivo. El objetivo es la caracterización clínica de los pacientes diagnosticados de esclerosis lateral amiotrófica (ELA) a los que se les indicó la colocación de una sonda de gastrostomía (SG) en el período 2007-2017 en el Complejo Asistencial Universitario de Salamanca, y encontrar posibles factores pronósticos asociados. Material y métodos. Es un estudio descriptivo de casos clínicos. Resultados. Entre 2007 y 2017 se evaluaron 139 ELA y se indicó SG en 29 casos. De esos 29, en dos ocasiones no se llevó a cabo, en una ocasión por negativa del paciente y en otra por deterioro clínico. Del total de SG, 21 se colocaron por endoscopia; cuatro, por radiología intervencionista; y dos, por cirugía. Las principales indicaciones fueron: en 10 casos, por disfagia grave; en ocho, por pérdida ponderal > 10%; y en nueve, por disminución de la capacidad vital forzada menor de 50%. Entre las complicaciones inmediatas (primeras 24 horas) se observaron: dos desaturaciones intraprocedimiento, una infección de la ostomía, un dolor abdominal, un íleo paralítico y una insuficiencia respiratoria con fallecimiento. Entre las complicaciones tardías (primer mes): una neumonía, una isquemia intestinal, una celulitis y un mal control de las secreciones. La principal causa de fallecimiento fue la insuficiencia respiratoria. La mediana de tiempo desde la colocación de la SG hasta el fallecimiento fue de 7,89 meses. Se constató mejoría de los parámetros analíticos nutricionales, sin demostrarse significación estadística, como factor pronóstico. Conclusión. La colocación de una SG es un procedimiento eficaz y seguro para asegurar la nutrición enteral en los pacientes con ELA.

Sondas de gastrostomía en pacientes con esclerosis lateral amiotrófica: indicaciones, seguridad y experiencia en un centro de tercer nivel / Gastrostomy tubes in patients with amyotrophic lateral sclerosis: indications, safety and experience in a tertiary care centre

Objetivo: El objetivo es la caracterización clínica de los pacientes diagnosticados de esclerosis lateral amiotrófica (ELA) a los que se les indicó la colocación de una sonda de gastrostomía (SG) en el período 2007-2017 en el Complejo Asistencial Universitario de Salamanca, y encontrar posibles factores pronósticos asociados. Material y métodos. Es un estudio descriptivo de casos clínicos. Resultados: Entre 2007 y 2017 se evaluaron 139 ELA y se indicó SG en 29 casos. De esos 29, en dos ocasiones no se llevó a cabo, en una ocasión por negativa del paciente y en otra por deterioro clínico. Del total de SG, 21 se colocaron por endoscopia; cuatro, por radiología intervencionista; y dos, por cirugía. Las principales indicaciones fueron: en 10 casos, por disfagia grave; en ocho, por pérdida ponderal > 10%; y en nueve, por disminución de la capacidad vital forzada < 50%. Entre las complicaciones inmediatas (primeras 24 horas) se observaron: dos desaturaciones intraprocedimiento, una infección de la ostomía, un dolor abdominal, un íleo paralítico y una insuficiencia respiratoria con fallecimiento. Entre las complicaciones tardías (primer mes): una neumonía, una isquemia intestinal, una celulitis y un mal control de las secreciones. La principal causa de fallecimiento fue la insuficiencia respiratoria. La mediana de tiempo desde la colocación de la SG hasta el fallecimiento fue de 7,89 meses. Se constató mejoría de los parámetros analíticos nutricionales, sin demostrarse significación estadística, como factor pronóstico. Conclusión: La colocación de una SG es un procedimiento eficaz y seguro para asegurar la nutrición enteral en los pacientes con ELA.(AU)

Aim: The aim of this study is the clinical characterisation of patients diagnosed with amyotrophic lateral sclerosis (ALS), who were indicated for the placement of a gastrostomy tube (G-Tube) in the period 2007-2017 in the Complejo Asistencial Universitario de Salamanca, and to find possible associated prognostic factors. Material and methods: It is a descriptive study of clinical cases. Results: Between 2007 and 2017, 139 cases of ALS were assessed and a G-Tube was indicated in 29 of them. Of these 29, on two occasions it was not performed, one due to the patient refusing and the other because of clinical deterioration. Of the total number of G-Tubes, 21 were placed by endoscopy, four by interventional radiology and two by surgery. The main indications were: in 10 cases, for severe dysphagia; in eight cases, for weight loss >10%; and in nine cases, for reduced forced vital capacity <50%. Immediate complications (first 24 hours) included: two intraprocedural desaturations, one ostomy infection, one abdominal pain, one paralytic ileus and one respiratory failure resulting in death. Late complications (first month) include pneumonia, intestinal ischaemia, cellulitis and poor control of secretions. The main cause of death was respiratory failure. The median time elapsed between G-Tube placement and death was 7.89 months. An improvement in analytical nutritional parameters was observed, without showing statistical significance, as a prognostic factor. Conclusion: G-Tube placement is an effective and safe procedure to ensure enteral nutrition in ALS patients.(AU)

Estudio controlado del ritmo del habla en la enfermedad de Parkinson / Speech rate in Parkinson's disease: A controlled study

Introducción: Las alteraciones en el habla aparecen en la mayoría de los pacientes con la enfermedad de Parkinson (EP) en el curso del trastorno. Su origen y gravedad son de interés clínico y diagnóstico. Objetivo: Evaluar los patrones de deterioro en el habla en pacientes con la EP, e identificar diferencias en la velocidad de elocución y articulación en comparación con sujetos de control, empleando un método de análisis automático en una tarea de lectura. Pacientes: Participaron 39 pacientes con la EP y 45 controles asintomáticos igualados en sexo y edad. Los pacientes con la EP, en estado on, no presentaban fluctuaciones motoras ni discinesias durante la evaluación del habla. Resultados: El grupo de pacientes con la EP muestran una significativa reducción de la velocidad de elocución y articulación. No se encontraron correlaciones significativas entre los parámetros del habla estudiados y las características de los pacientes, tales como la dosis de L-dopa, duración del trastorno, edad, ni en las puntuaciones en las escalas UPDRS III o Hoehn y Yahr. Conclusiones: Los pacientes con la EP muestran un patrón característico de deterioro del ritmo del habla. Estos resultados indican que las disfluencias en la EP son el resultado de la alteración del movimiento que afecta a la fisiología de los sistemas de producción del habla

Introduction: Speech disturbances will affect most patients with Parkinson's disease (PD) over the course of the disease. The origin and severity of these symptoms are of clinical and diagnostic interest. Purpose: To evaluate the clinical pattern of speech impairment in PD patients and identify significant differences in speech rate and articulation compared to control subjects. Speech rate and articulation in a reading task were measured using an automatic analytical method. Patients: A total of 39 PD patients in the ‘on’ state and 45 age-and sex-matched asymptomatic controls participated in the study. None of the patients experienced dyskinesias or motor fluctuations during the test. Results: The patients with PD displayed a significant reduction in speech and articulation rates; there were no significant correlations between the studied speech parameters and patient characteristics such as L-dopa dose, duration of the disorder, age, and UPDRS III scores and Hoehn & Yahr scales. Conclusion: atients with PD show a characteristic pattern of declining speech rate. These results suggest that in PD, disfluencies are the result of the movement disorder affecting the physiology of speech production systems

Speech rate in Parkinson's disease: A controlled study. / Estudio controlado del ritmo del habla en la enfermedad de Parkinson.

INTRODUCTION: Speech disturbances will affect most patients with Parkinson's disease (PD) over the course of the disease. The origin and severity of these symptoms are of clinical and diagnostic interest. PURPOSE: To evaluate the clinical pattern of speech impairment in PD patients and identify significant differences in speech rate and articulation compared to control subjects. Speech rate and articulation in a reading task were measured using an automatic analytical method. PATIENTS: A total of 39 PD patients in the 'on' state and 45 age-and sex-matched asymptomatic controls participated in the study. None of the patients experienced dyskinesias or motor fluctuations during the test. RESULTS: The patients with PD displayed a significant reduction in speech and articulation rates; there were no significant correlations between the studied speech parameters and patient characteristics such as L-dopa dose, duration of the disorder, age, and UPDRS III scores and Hoehn & Yahr scales. CONCLUSION: Patients with PD show a characteristic pattern of declining speech rate. These results suggest that in PD, disfluencies are the result of the movement disorder affecting the physiology of speech production systems.

Paraoxonase 1 (PON1) polymorphisms and risk for essential tremor.

BACKGROUND: The polymorphic enzyme human serum paraoxonase 1 (PON1), encoded by the gene PON1 (chromosome 7q21.3), plays a major role in the metabolism of organophosphorus compounds. We investigated the possible association between the PON1 genotype and allelic variants of the polymorphisms Leu55Met and Glu192Arg, and the risk for essential tremor (ET). METHODS: We studied the frequency of the PON1 genotypes and allelic variants in 201 patients with ET and 220 healthy controls using a PCR-RLFP method. RESULTS: The frequencies of the PON1 genotypes and allelic variants of the polymorphisms Leu55Met and Gln192Arg did not differ significantly between patients with ET and controls. These polymorphisms were unrelated with the age of onset of ET. CONCLUSIONS: PON1 polymorphisms are not related with the risk for ET.

Glutathione-S-transferase P1 polymorphism and risk for essential tremor.

Glutathione-S-transferases (GST) are polymorphic enzymes that participate in the metabolism of carcinogens (including those of tobacco smoke) and pesticides. We investigated the possible association between the GSTP1 genotype and allelic variants and the risk for essential tremor (ET). We studied the frequency of the GSTP1 genotypes and allelic variants in 200 patients with ET and 220 healthy controls using PCR-RFLP method. The association between GSTP1 polymorphism and the exposure to some environmental factors (agricultural work, pesticides, well-water and smoking-cigarettes habit) was also studied in a subgroup of patients. The frequencies of the GSTP1 genotypes and allelic variants did not differ significantly between patients with ET and controls or between patients with ET exposed to agricultural work, well water and cigarette smoking versus those non-exposed. Mutated allelic variants were significantly more frequent in patients with ET exposed to pesticides versus those non-exposed. GSTP1 polymorphism was unrelated with the age of onset of ET. GSTP1 genotypes and allelic variants were not related with the risk for ET with the possible exception of those patients exposed to pesticides.

[Recent-onset headache is a risk factor of intracranial lesion. A prospective study of 299 patients]. / La cefalea de comienzo reciente es un factor de riesgo de lesión intracraneal. Estudio prospectivo de 299 pacientes.

BACKGROUND: There is some controversy in the medical literature concerning the need to perform neuroimaging studies in neurologically normal patients complaining of headaches. The objective of the study is to determine the detection rate of intracranial abnormalities by computed tomography in patients with different headache durations. METHOD: Consecutive patients with the chief complaint of headache referred for neurological evaluation from January 1996 to April 1997 were studied both clinically and by computed tomography scanning. Brain magnetic resonance imaging was performed in 15 patients. Cerebrospinal fluid and/or blood analyses were performed when clinically indicated to rule out subarachnoid hemorrhage, meningitis or temporal arteritis. RESULTS: 15 (5%) out of the 299 patients available for study had significant intracranial lesion. 3 (1%) out of the 266 patients with headaches lasting for more than 1 month had computed tomography findings considered clinically significant and neurological examination was normal in 2 (0.7%) patients with abnormal scans. Patients with a headache duration of 1 month or less had the following case-finding rate: an overall significant intracranial abnormality of 36% (12/33) and significant intracranial abnormality in neurologically normal patients of 15% (5/33). CONCLUSION: Patients with headache of recent onset (duration of 1 month or less), even with normal neurological examination, are at greater risk of significant intracranial abnormality than patients with long-lasting headaches. These patients at risk should be studied by cranial computed tomography and lumbar puncture if the computed tomography scan is normal and the cause of the headaches cannot be clinically determined.

La cefalea de comienzo reciente es un factor de riesgo de lesión intracraneal. Estudio prospectivo de 299 pacientes / Headache of recent onset is a risk factor for intracranial lesion: a prospective study of 299 patients

Fundamento: La necesidad de realizar o no estudios de neuroimagen a los pacientes neurológicamente normales que consultan por cefaleas es un tema controvertido en las publicaciones médicas. El objetivo de este estudio es determinar la proporción de anormalidades intracraneales detectadas mediante tomografia computarizada en pacientes con cefalea de distinta duración. Método: Se estudiaron clínicamente y con tomografia computarizada los pacientes consecutivos enviados para valoración neurológica por cefalea como síntoma fundamental entre Enero 1996 y Abril 1997. Se practicó estudio con resonancia magnética en 15 pacientes. Se realizó análisis de sangre o de líquido cefalorraquídeo cuando se estimó necesario para descartar hemorragia subaracnoidea, meningitis o arteritis de la arteria temporal. Resultados: 15 (5%) de los 299 pacientes disponibles para el estudio tuvieron alguna lesión intracraneal significativa. 3 (1%) de los 266 pacientes con cefalea de más de 1 mes de duración tuvieron lesiones en la tomografia computarizada consideradas clínicamente significativas y en 2 (0,7%) de estos pacientes la exploración neurológica fue normal. Los pacientes con una duración de la cefalea igual o inferior a 1 mes tuvieron la siguiente proporción de hallazgos: Anormalidad intracraneal significativa global en 36% (12/33) y en los pacientes neurológicamente normales la anormalidad intracraneal significativa fue del 15% (5/33). Conclusión: Los pacientes con cefalea de comienzo reciente (duración igual o inferior a 1 mes), incluso con exploración neurológica normal, tienen un riesgo de albergar una lesión intracraneal significativa mayor que los pacientes con cefaleas de larga evolución. A estos pacientes con aumento de riesgo se les debería practicar una tomografia computarizada craneal y una punción lumbar si la tomografia es normal y el origen de la cefalea no se ha aclarado clínicamente (AU)

[Multiple cranial neuropathy: an atypical variant of the Guillain-Barre syndrome?]. / Neuropatía craneal múltiple: una variante atípica del síndrome de Guillain-Barré?

INTRODUCTION: Multiple cranial neuropathy or polineuritis cranealis is rarely seen in everyday clinical practice. It is considered to be a topographically circumscribed form of the Guillain-Barré syndrome. The cases described have a wide range of clinical and biological characteristics. Some of these may be due to infectious agents. CLINICAL CASE: We present the case of a 50 year old man with acute onset of diplopia, dysphagia, anarthria, cervical and right arm flexor-extensor muscle weakness due to involvement of many motor cranial nerves and superior cervical nerve roots. On neurological examination there was mixed involvement, mainly of the axons of the nerve trunks involved. Studies to determine aetiology did not show any demonstrable agent. DISCUSSION AND CONCLUSIONS: Different topographical varieties of the Guillain-Barré syndrome have been described, including: Fisher's syndrome, pharyngo-cervico-brachial paralysis, arreflexive paraparesia, bilateral facial paralysis with paraesthesias, hyporeflexia and bilateral lumbar polyradiculopathy. We compare the clinical characteristics of our patient with those described in the literature. We found a degree of heterogenicity in the clinical and biological characteristics of the cases described, which may mean that they had different aetiologies. Therefore, we consider that before labelling these conditions as atypical variants of the Guillain-Barré syndrome, a thorough search should be made for a precise aetiology.

[Central motor conduction evaluation in glycogenosis type III]. / Evaluación de la conducción motora central en la glucogenosis tipo III.

We report a 20-year-old man affected by glycogenosis type III with distal muscle weakness, more severe in distal leg muscles. The electromyogram showed myopathic features. Nerve conduction studies and central motor conduction after magnetic stimulation of the brain were normal. Our results suggest that there is no involvement of central motor pathways in this disease.

[Complete axonotmesis of the axillary nerve in relation to microtrauma]. / Axonotmesis completa del nervio axilar en relación con un microtraumatismo.

INTRODUCTION: The axillary nerve is injured in many clinical situations, mainly in major surgical or traumatic lesions of the shoulder. Equally, it may be found in the context of microtraumatisms or compressive mechanisms. Amyotrophic neuralgia is a clinical entity with pain and later atrophy of the muscle which affects various nerves and nerve groups, as shown by neurophysiological studies. CASE REPORT: We present a lesion with complete axonotmesis of the axillary nerve with a time-relationship to microtraumatism. Initially the patient complained of some pain in the shoulder. During follow-up striking atrophy of the deltoid muscle was seen. CONCLUSIONS: We have reviewed the mechanisms described for lesions of the axillary nerve, which do not include the one we found. We discuss the possibility of our case being included in the clinical group described as having amyotrophic neuralgia, although there are some neurophysiological findings which are not typical of this condition. We suggest a review of the many possible trigger factors described in the genesis of amyotrophic neuralgia. Possibly some cases with atypical histories of trauma, and lesions shown by neurophysiological studies to be particularly severe, may be separated from this group.

[The development of a clinical protocol for the study of peripheral facial paralysis. A computerized database version]. / Desarrollo de un protocolo clínico para el estudio de las parálisis faciales periféricas. Versión de captura de datos informatizada.

We present a protocol for the management and follow up of facial palsies, in which, the clinical, diagnosis and therapeutical informations are obtained in order to evaluate the clinical assessment and pathology of this cranial nerve. We also present a computerized database system in order to develop statistical analysis and further studies on this peripheral nerve lesion.

[Ramsay Hunt syndrome. Effects of the treatment with acyclovir (preliminary study)]. / Síndrome de Ramsay Hunt. Efectos del tratamiento con aciclovir (estudio preliminar).

Herpes zoster oticus has a poor prognosis with permanent facial nerve dysfunction. We have studied 11 patients in a total group of 131 facial palsies seen in our service for a 20 month period. Four patients had no treatment. Two were treated with prednisone, and five were infused with acyclovir (10 mg/kg every 8 hours over a 10 day hospitalization period) and prednisone. These treatments were observed for 3 to 6 months. Patients follow up was, acyclovir group, 2 patients have achieved a House grade I, 1 patient grade II, 1 patient grade III and 1 left this treatment because side effects was observed. The other patients, 1 have achieved grade I, 2 patients grade II, 2 patients grade III and 1 patient grade V. The differences and final results are discussed.

[Facial paralysis caused by retroauricular basal cell carcinoma]. / Parálisis facial por extensión de carcinoma basocelular retroauricular.

We make a review of facial palsy of tumoral etiology, including the diagnosis, evolution and treatment. We also present a case of facial palsy caused by retroauricular basal cell carcinoma.

Carpal tunnel syndrome and vitamin B6.

Twelve patients with carpal tunnel syndrome were studied. Clinical and electrophysiological data were obtained and an estimation of vitamin B6 (pyridoxine) status by an assay of erythrocyte aspartate aminotransferase and coenzyme stimulation assay were done. None of the patients was found to have vitamin B6 deficiency. Patients were treated with 150 mg of pyridoxine daily for 3 months. Erythrocyte aspartate aminotransferase increased significantly (p less than 0.001) in all the patients. In 6 patients there were clinical and electrophysiological improvement and erythrocyte aspartate aminotransferase increased more than in the other 6 patients. The data obtained appear to indicate that although vitamin B6 deficiency is not common in carpal tunnel syndrome patients, pyridoxine supplementation can be recommended as adjuvant treatment in those patients undergoing surgery.