Epidemiología de la transfusión sanguínea en los Servicios de Medicina Intensiva en España: «Transfusion Day» / Epidemiology of blood transfusion in the Spanish Critical Care Units: «Transfusion Day»

Objective: To describe the transfusion practice in the ICUs in Spain, according to national and international recommendations (guidelines). Design: Prospective, cross-sectional, multi-centre study. Scope: Data collection was carried out by means of a questionnaire sent electronically to the Heads of Service of 111 ICUs in Spain. Participants: 1,448 patients were included, aged 61.8 (SD 15.7) years, 66.2% male, with an SOFA of 4.7 ± 3.8 and average stay of 10.62 ± 17.49 days. Variables: Demographic and clinical variables of the patients were collected, as well as variables related to the transfusion act. Results: Of the 1,448 patients, 9.9% received al least one transfusion of any blood product, 3.7% fresh plasma, 3.9% platelets and 8.9% red blood cell concentrate, mainly by analytical criteria (36.2%). Hemoglobin had a mean of 7.8 g/dL (95% CI: 6-9-8.5) and 9.8 g/dL (95% CI: 8.5–11.2) before and after the transfusion, respectively, p < 0.001. The transfusion units had a mean of 2.5 ± 2.4 per patient. The most commonly used blood product was red blood cell concentrate (CH) (90.2%). Patients admitted for surgery had a higher transfusion rate (14.4%) than those admitted for medical pathology (8.9%) (p = 0.006). 5.4% (7/129) of patients who received CH died compared to 2.4% (31/1302) who did not (p = 0.04). Mortality of transfused patients was higher. The transfusion rate in most of hospitals was 5% to 20%, with 18 hospitals (16.21%) having transfusion rates between 20% and 50%. Hospitals with PBM programs and mass transfusion programs had a lower transfusion rate, although not statistically significant. Conclusions: In this multicenter cross-sectional study, a transfusion prevalence of 9.9% was observed in Spanish Critical Care Units. The most frequent blood product transfused was red blood cells and the main reasons for transfusion were acute anemia with hemodynamic impact and analytical criteria. Mortality of transfused patients was higher (AU)

Objetivo: Describir la práctica transfusional en las UCIs de España, acorde con recomendaciones (guidelines) nacionales e internacionales. Diseño: Estudio prospectivo, transversal y multicéntrico. Ámbito: La recogida de datos se realizó mediante una encuesta enviada electrónicamente a los médicos intensivistas de 111 UCIs de España. Participantes: Se incluyeron 1.448 pacientes, de 61,8 (DE 15,7) años, el 66,2% varones, con un SOFA de 4,7 ± 3,8 y estancia media de 10,62 ± 17,49 días. Variables: Se recogieron variables demográficas y clínicas de los pacientes, así como variables relacionadas con el propio acto transfusional. Resultados: De los 1.448 pacientes, el 9,9% recibieron al menos una transfusión de cualquier hemocomponente, 3,7% de plasma fresco, 3,9% de plaquetas y 8,9% de concentrado de hematíes, siendo la causa principal el umbral transfusional basado en la hemoglobina (36,2%). La hemoglobina tuvo una media de 7,8 g/dL (IC 95%: 6,9–8,5), y de 9.8 g/dl (IC95%: 8,5–11,2) antes y después de la transfusión respectivamente (p < 0,001). Las unidades transfundas tuvo una media por paciente de 2,5 ± 2,4 por paciente. El hemoderivado más utilizado fue el concentrado de hematíes (CH) (90,2%). Los pacientes ingresados por motivos quirúrgicos tuvieron una tasa de transfusión mayor (14,4%) respecto a los ingresados por patología médica (8,9%) (p = 0,006). El 5,4% (7/129) de los pacientes que recibieron CH fallecieron respecto el 2,4% (31/1302) que no lo recibieron (p = 0,04). La tasa de transfusión en la mayor parte de hospitales fue de 5% al 20%, habiendo 18 hospitales (16.21%) con tasas de transfusión entre el 20% y el 50%. Los hospitales con programas PBM y programas de transfusión masiva tuvieron una menor tasa de transfusión, aunque sin ser significativa. Conclusiones: En este estudio multicéntrico de corte transversal se observó una prevalencia transfusional en las unidades de críticos españolas del 9,9% (AU)

Epidemiology of blood transfusion in the Spanish Critical Care Units: «Transfusion Day¼.

OBJECTIVE: To describe the transfusion practice in the ICUs in Spain, according to national and international recommendations (guidelines). DESIGN: Prospective, cross-sectional, multi-centre study. SCOPE: Data collection was carried out by means of a questionnaire sent electronically to the Heads of Service of 111 ICUs in Spain. PARTICIPANTS: 1,448 patients were included, aged 61.8 (SD 15.7) years, 66.2% male, with an SOFA of 4.7 ±â€¯3.8 and average stay of 10.62 ±â€¯17.49 days. VARIABLES: Demographic and clinical variables of the patients were collected, as well as variables related to the transfusion act. RESULTS: Of the 1,448 patients, 9.9% received al least one transfusion of any blood product, 3.7% fresh plasma, 3.9% platelets and 8.9% red blood cell concentrate, mainly by analytical criteria (36.2%). Hemoglobin had a mean of 7.8 g/dL (95% CI: 6-9-8.5) and 9.8 g/dL (95% CI: 8.5-11.2) before and after the transfusion, respectively, p < 0.001. The transfusion units had a mean of 2.5 ±â€¯2.4 per patient. The most commonly used blood product was red blood cell concentrate (CH) (90.2%). Patients admitted for surgery had a higher transfusion rate (14.4%) than those admitted for medical pathology (8.9%) (p = 0.006). 5.4% (7/129) of patients who received CH died compared to 2.4% (31/1302) who did not (p = 0.04). Mortality of transfused patients was higher. The transfusion rate in most of hospitals was 5% to 20%, with 18 hospitals (16.21%) having transfusion rates between 20% and 50%. Hospitals with PBM programs and mass transfusion programs had a lower transfusion rate, although not statistically significant. CONCLUSIONS: In this multicenter cross-sectional study, a transfusion prevalence of 9.9% was observed in Spanish Critical Care Units. The most frequent blood product transfused was red blood cells and the main reasons for transfusion were acute anemia with hemodynamic impact and analytical criteria. Mortality of transfused patients was higher.

Epidemiology of blood transfusion in the Spanish Critical Care Units: «Transfusion Day¼. / Epidemiología de la transfusión sanguínea en los Servicios de Medicina Intensiva en España: «Transfusion Day¼.

OBJECTIVE: To describe the transfusion practice in the ICUs in Spain, according to national and international recommendations (guidelines). DESIGN: Prospective, cross-sectional, multi-centre study. SCOPE: Data collection was carried out by means of a questionnaire sent electronically to the Heads of Service of 111 ICUs in Spain. PARTICIPANTS: 1,448 patients were included, aged 61.8 (SD 15.7) years, 66.2% male, with an SOFA of 4.7±3.8 and average stay of 10.62±17.49 days. VARIABLES: Demographic and clinical variables of the patients were collected, as well as variables related to the transfusion act. RESULTS: Of the 1,448 patients, 9.9% received al least one transfusion of any blood product, 3.7% fresh plasma, 3.9% platelets and 8.9% red blood cell concentrate, mainly by analytical criteria (36.2%). Hemoglobin had a mean of 7.8g/dL (95% CI: 6-9-8.5) and 9.8g/dL (95% CI: 8.5-11.2) before and after the transfusion, respectively, p<0.001. The transfusion units had a mean of 2.5±2.4 per patient. The most commonly used blood product was red blood cell concentrate (90.2%). Patients admitted for surgery had a higher transfusion rate (14.4%) than those admitted for medical pathology (8.9%) (p=0.006). 5.4% (7/129) of patients who received red blood cell concentrate died compared to 2.4% (31/1302) who did not (p=0.04). Mortality of transfused patients was higher. The transfusion rate in most of hospitals was 5% to 20%, with 18 hospitals (16.21%) having transfusion rates between 20% and 50%. Hospitals with PBM programs and mass transfusion programs had a lower transfusion rate, although not statistically significant. CONCLUSIONS: In this multicenter cross-sectional study, a transfusion prevalence of 9.9% was observed in Spanish critical care units. The most frequent blood product transfused was red blood cells and the main reasons for transfusion were acute anemia with hemodynamic impact and analytical criteria. Mortality of transfused patients was higher.

Main Contributory Factors on Asthma Control and Health-Related Quality of Life in Elderly Asthmatics.

OBJECTIVE: To assess the main factors involved in asthma control and health-related quality of life in elderly asthmatic patients. METHODS: We performed a retrospective case-control study nested in a historical cohort that compared patients who had partly controlled or uncontrolled asthma (Asthma Control Test [ACT] score ≤19) (cases) with patients who had well-controlled asthma (ACT ≥20) (controls). Clinical data were collected from medical records. Outcomes included ACT score and health-related quality of life (Asthma-Specific Quality of Life Questionnaire [AQLQ]). Pulmonary function was determined by spirometry. RESULTS: We evaluated 209 asthma patients (151 women) aged ≥65 years. Mean age was 73.55 years. Most patients had persistent moderate (47.60%) or severe (47.12%) asthma. A total ACT score ≤19 was obtained in 64 (30.62%) patients. Lack of adherence to treatment and presence of severe exacerbations were risk factors for partly controlled/uncontrolled asthma (OR, 8.33 and 5.29, respectively). In addition, for each additional unit score in the AQLQ, the risk of poor control increased by 1.51. The factors influencing the AQLQ score were asthma control (ACT) and presence of comorbidities such as depression, gastroesophageal reflux disease, and osteoporosis. CONCLUSIONS: Despite receiving antiasthma therapy, almost one-third of elderly patients had uncontrolled asthma, possibly as a result of poor adherence, exacerbations, and reduced health-related quality of life. Nonrespiratory comorbid conditions in older patients do not seem to be associated with worse control of asthma symptoms, although their effect on health-related quality of life could indirectly affect asthma control.

Transfusions in the Emergency department: More than a blood transfusion. / Transfusión en Urgencias: algo más que una transfusión de sangre.

Increasing haemoglobin and haematocrit levels with blood transfusions has been the gold standard for treating severe anaemia; however, the indication for transfusing concentrated red blood cells is based merely on a few laboratory markers, such as haemoglobin and haematocrit levels, rather than based on the symptoms according to clinical practice guidelines, the implementation of legal regulations and the consensus achieved by the hospitals' transfusion committees. The aim of this multicentre study was to reassess the suitability of the indication for transfusing concentrated red blood cells and the volumes transfused in emergency departments. We established an observational, multicentre, cross-sectional design with 2 participating centres: the La Paz University Hospital and the Hospital of Salamanca. In total, we obtained data from 381 patients, 220 (57.74%) of whom were men with an average age of 71.4±14.0 years and 161 (42.26%) of whom were women with an average age of 75.3±15.3 years (P<.001). The most prevalent underlying diseases in the patients who underwent transfusions were heart disease, which included haemorrhaging due to antiplatelet or anticoagulant therapy (57.7%), haemato-oncologic (15.3%) diseases and neurological disease. Only 54.9% (209/381) of the prescriptions for transfusion were considered appropriate, with significant differences according to the indication.

Impact on patient outcome of emergency department length of stay prior to ICU admission.

OBJECTIVE: The favorable evolution of critically ill patients is often dependent on time-sensitive care intervention. The timing of transfer to the intensive care unit (ICU) therefore may be an important determinant of outcomes in critically ill patients. The aim of this study was to analyze the impact upon patient outcome of the length of stay in the Emergency Care Department. DESIGN: A single-center ambispective cohort study was carried out. SETTING: A general ICU and Emergency Care Department (ED) of a single University Hospital. PATIENTS: We included 269 patients consecutively transferred to the ICU from the ED over an 18-month period. INTERVENTIONS: Patients were first grouped into different cohorts based on ED length of stay (LOS), and were then divided into two groups: (a) ED LOS ≤5h and (b) ED LOS >5h. VARIABLES: Demographic, diagnostic, length of stay and mortality data were compared among the groups. RESULTS: Median ED LOS was 277min (IQR 129-622). Patients who developed ICU complications had a longer ED LOS compared to those who did not (349min vs. 209min, p<0.01). A total of 129 patients (48%) had ED LOS >5h. The odds ratio of dying for patients with ED LOS >5h was 2.5 (95% CI 1.3-4.7). Age and sepsis diagnosis were the risk factors associated to prolongation of ED length of stay. CONCLUSIONS: A prolonged ED stay prior to ICU admission is related to the development of time-dependent complications and increased mortality. These findings suggest possible benefit from earlier ICU transfer and the prompt initiation of organ support.

[Factors associated with adherence in HIV patients]. / Factores relacionados con la adherencia en pacientes infectados por el virus de la inmunodeficiencia humana.

OBJECTIVE: To establish the relationship between the adherence to ARVT and the clinical situation and detect those factors which relate to the lack of adherence. METHOD: Observational study on HIV patients who had attended the Pharmacy Service in Navarra Hospital between February and May 2005. The SMAQ questionnaire and pharmacy dispensing records were used to assess adherence to treatment. Socio-demographic variables and other factors which could influence adherence were recorded. Statistical analysis was carried out using the SPSS programme, version 14.0. RESULTS: No concordance was noted between the two measurements of adherence, although there was an association between the viral load and compliance, irrespective of the method used. The questionnaire recorded a higher percentage of non-adhering female patients, substance users and psychiatric patients. Non-adhering patients indicated more frequently factors which made taking the medication difficult. The multivariate analysis showed that the lack of a suitable social-familial environment negatively influenced the adherence level, according to the SMAQ questionnaire, and that the high number of tablets per dose was related to the lack of adherence according to the pharmacy dispensing records. CONCLUSIONS: Patients who adhere to ARVT have a lesser risk of virological failure. An unsuitable social-familial environment and the complexity of treatment are associated with a lack of adherence. The method of using dispensing records should be combined with a patient interview to define the factors which reduce adherence and to propose intervention strategies.

Epidemiological assessment of levodopa use in Cuba: 1993-1998.

PURPOSE: The purpose of this study is to describe and evaluate the use of levodopa in Cuba in order to provide a basis for intervention aimed at improving pharmacological treatment of individuals presenting with Parkinson's disease (PD). METHODS: We studied the amount of levodopa, both plain and combined, distributed by the central laboratory to hospital and community pharmacies in Cuba in the period 1993-1998. An internationally established drug-classification system and a reported method for epidemiological assessment of levodopa sales were applied. Sweden in 1994 served as the reference population. RESULTS: National crude rates of levodopa use basically remained stable since 1994, and in 1998 stood at 0.11 defined daily dose (DDD) per 1000 inhabitants/day, approximately 15 times lower than the corresponding figure for the reference population. Annual provincial use of levodopa showed considerable geographical variation, with the lowest rates in the eastern provinces and the highest rates in Havana City (Ciudad de La Habana). Adjustment for age reduced such differences by approximately 50%. CONCLUSIONS: Levodopa use in Cuba is low and consistent with the reported low prevalence of the diagnosis of PD. Results suggest that the diagnosis and treatment of PD can be improved, with emphasis on better detection of PD.

[Specific alterations in attention in the early stages of Parkinson's disease]. / Alteraciones específicas de la atención en estadios tempranos de la Enfermedad de Parkinson.

INTRODUCTION: The cognitive disorders presented by patients with Parkinson s disease (PD) have drawn attention to the role played by the basal ganglia in cognition. It has been suggested that through a system of cortico subcortical circuits they monitor the work done by the frontal regions by guiding the anterior and posterior attentional systems, whose functioning is necessary for the so called executive functions to be carried out. PATIENTS AND METHODS: We studied 10 patients with PD in developmental stages I and II according to the scale of Hoehn and Yahr, and 10 healthy paired controls. All of them were administered a test for simple sustained attention, complex sustained attention and attentional shift. RESULTS: In the simple sustained attention task there were no significant differences between groups. In the complex sustained attention test the patients committed more mistakes, and in the attentional shift task they committed more omissions and there was a significant increase in reaction time. CONCLUSIONS: Even in the early stages of the disease, patients suffering from Parkinson present cognitive disorders. These are concentrated in the detection and evaluation of new information, in the capability to shift attention between different spatial regions in a flexible manner and in choosing, inhibiting and activating motor programmes. In order to detect these alterations more sensitive and complex tests must be used.

Alteraciones específicas de la atención en estadios tempranos de la enfermedad de Parkinson / Specific alterations in attention in the early stages of Parkinson´s disease

Introducción. Los trastornos cognitivos que presentan los pacientes con enfermedad de Parkinson (EP) han llamado la atención sobre la función de los ganglios basales en la cognición. Se ha propuesto que monitorizan el trabajo de las regiones frontales mediante un sistema de circuitos cortico subcorticales, al guiar a los sistemas atencionales anterior y posterior, cuyo funcionamiento se necesita para el desarrollo de las llamadas funciones ejecutivas. Pacientes y métodos. Se estudiaron 10 pacientes con EP en estadios evolutivos I y II según la escala de Hoehn yYahr y 10 controles sanos pareados. A todos se les aplicó una prueba de atención sostenida simple, atención sostenida compleja y de cambio atencional. Resultados. En la prueba de atención sostenida simple no hubo diferencias significativas entre los grupos. En la de atención sostenida compleja los pacientes cometieron una mayor cantidad de errores, y en la de cambio atencional cometieron más omisiones y aumentaron significativamente su tiempo de reacción. Conclusiones. Los pacientes parkinsonianos que se encuentran en estadios tempranos de la enfermedad presentan alteraciones cognitivas. Éstas se concentran en la detección y evaluación de nueva información, en la posibilidad de cambiar de manera flexible la atención entre regiones espaciales diferentes y en elegir, inhibir y activar programas motores. Para detectar estas alteraciones se precisa de pruebas más sensibles y complejas (AU)

Switching from bromocriptine to ropinirole in patients with advanced Parkinson's disease: open label pilot responses to three different dose-ratios.

Newly introduced dopamine agonists, such as ropinirole, may offer advantages compared to such older drugs as bromocriptine in patients with advanced Parkinson's disease (PD) with response oscillations or waning efficacy. Dose equivalence of these two drugs, however, has not been well established, which may complicate switching in clinical practice. In 23 such patients with advanced PD no longer satisfactorily responsive to prolonged bromocriptine therapy (mean dose: 18.9 +/- 6.5 mg/d), we prospectively switched the medication to ropinirole administered at three different dose-ratios (5:1, 3:1, and 2:1), increased at monthly intervals. Selegiline remained unmodified in all 17 patients receiving this medication. A dose-ratio of bromocriptine to ropinirole of close to 2:1 (1.87; mean ropinirole dose: 10.1 +/- 2.5 mg/d) was the only dose that significantly reduced mean motor Unified Parkinson's Disease Rating Scale (UPDRS) scores ( p = 0.030, analysis of variance). Individually considered, however, four patients (21%) scored worse even at this dose-ratio when compared to baseline assessment on bromocriptine. "Off" time was reduced by 57.3% in fluctuating patients, and the dyskinesia score decreased by 53.8%, although the changes were not statistically significant. Higher bromocriptine to ropinirole dose ratios (i.e., 5:1 and 3:1) resulted in "off"-time increases in half of the patients with fluctuations, and two previously stable patients developed a wearing-off effect and one other patient experienced off-time dystonia. One patient developed dose-dependent dopaminomimetic psychotic symptoms with ropinirole. In conclusion, "off"-time motor scores and possibly "off"-time duration, and severity of dyskinesias in patients with advanced PD with prolonged bromocriptine therapy may improve in a majority of cases by switching to ropinirole, provided that the latter drug is administered at a dose ratio of 2:1 compared to bromocriptine. Higher dose ratios are often ineffective or may even cause a clinical worsening of symptoms in some patients.

[Prevalence of Parkinson disease in an urban area of the Ciudad de La Habana province, Cuba. Door-to-door population study]. / Prevalencia de la enfermedad de Parkinson en un área urbana de la provincia Ciudad de La Habana, Cuba. Estudio poblacional "puerta a puerta".

OBJECTIVE: To determine the prevalence of Parkinson's disease in an urban area of Havana City province, Cuba. BACKGROUND: Parkinson's disease is one of the most frequent chronic neurodegenerative diseases in the elderly. Prevalence varies widely between different geographic areas and type of studies. To the author's knowledge, there are not epidemiological data on Parkinson's disease in Cuban population that allows a real estimation of the true magnitude of the disease. PATIENTS AND METHODS: A "door-to-door" population study was carried out between November and December 1997. The area total population aged 15 years and over (n = 17.784) was interviewed and examined during the first phase of the study. This phase was performed by the 33 family doctors practicing in that area. An experienced neurologist previously trained the family doctors on making Parkinson's disease diagnosis. Diagnosis was based on the Brain Bank Society criteria. In order to make a definitive diagnosis of Parkinson's disease every subject that received such a diagnosis during the first phase was re-evaluated by two experienced neurologists. Those subjects with final diagnosis of Parkinson's disease were asked about family history of the disease. Prevalence rates were calculated according to sex, age group, color of the skin and smoking status. RESULTS: A total of 24 subjects received the final diagnosis of Parkinson's disease, yielding a population prevalence rate of 135 x 100,000 inhabitants. Eight subjects (33.3%) received a diagnosis of Parkinson's disease for the first time (de novo cases). Subjects with white color of the skin and non-smoking subjects showed a statistically significant higher prevalence of Parkinson's disease than subjects with non-white color of the skin and non-smokers respectively. Only 3 (12.5%) subjects reported a family history of Parkinson's disease. CONCLUSION: The population of this area has a low prevalence rate of Parkinson's disease compared to that reported in other populations. The observed low frequency of family history of the disease suggests that the main determinants of Parkinson's disease are environmental factors yet to be identified.

Prevalencia de la enfermedad de parkinson en un área urbana de la provincia ciudad de la habana, cuba. estudio poblacional "puerta a puerta" / Prevalence of Parkinson disease in an urban area of the Ciudad de La Habana, province in Cuba. Door-to-door populational study

Objetivo: Determinar la prevalencia de la enfermedad de parkinson en un área urbana de la provincia Ciudad de La Habana, Cuba. Fundamento: La enfermedad de Parkinson es una de las enfermedades crónicas neurodegenerativas más frecuentes del anciano. La prevalencia de dicha enfermedad varia notablemente entre las distintas áreas geográficas y los estudios realizados. Hasta el momento no existen datos epidemiológicos sobre la verdadera magnitud de la enfermedad de Parkinson en población cubana. Pacientes y método: Se realizó un estudio poblacional de tipo "puerta a puerta". En una primera etapa se entrevistó y examinó a toda la población mayor de 15 años de edad (17.784 habitantes) por parte de los médicos de la familia que brindan servicios a la población residente en el área de salud del Policlínico Corintia. Dichos médicos habían recibido con anterioridad un entrenamiento estandarizado por parte de un médico especialista en neurología. El diagnóstico de enfermedad de Parkinson se realizó según los criterios clínicos de la Sociedad del Banco de Cerebros del Reino Unido. Todos los sujetos con diagnóstico de enfermedad de Parkinson en la primera etapa fueron evaluados en una segunda etapa por dos expertos neurólogos para confirmar dicho diagnóstico. Se indagó sobre la historia familiar de enfermedad de Parkinson y se calculó la tasa de prevalencia de la enfermedad según grupo de edad, sexo, color de la piel y hábito tabáquico. Resultados: Un total de 24 sujetos recibieron el diagnóstico definitivo de enfermedad de Parkinson para una tasa de prevalencia poblacional de 135 * 100.000 habitantes. Ocho casos (33,3 por ciento) recibieron por primera vez dicho diagnóstico (casos de novo). En los sujetos de piel blanca y los no fumadores se apreciaron tasas de prevalencia de enfermedad de Parkinson significativamente más elevadas que los sujetos de piel no blanca y los no fumadores, respectivamente. Sólo tres (12,5 por ciento) de los 24 casos diagnosticados presentaban historia familiar de enfermedad de Parkinson. ConclusiÓn: La población cubana residente en esta área posee una baja tasa de prevalencia de enfermedad de Parkinson comparada con la hallada en poblaciones de otros países. En esta población existe una mayor frecuencia de enfermedad de Parkinson en individuos de color de la piel blanca y en fumadores. La observada baja frecuencia de historia familiar de enfermedad de Parkinson sugiere que los principales determinantes de esta enfermedad son factores ambientales aún por identificar (AU)

[Progressive myoclonic epilepsy: clinical characteristics of 18 patients]. / Epilepsia mioclónica progresiva: caracterización clínica de 18 pacientes.

INTRODUCTION: The term progressive myoclonic epilepsy (PME) includes a groups of heterogeneous conditions, with genetic causes, characterized by having different types of seizures, basically myoclonic, and other neurological findings due to a progressive lesion of the central nervous system. OBJECTIVE: To demonstrate the aetiology and clinico-encephalographic changes seen in patients with PME. PATIENTS AND METHODS: A retrospective, descriptive study was done of patients attended for PME in the Instituto de Neurología y Neurocirugía de Cuba between 1990 and 1995. Eighteen patients were included. All were interviewed and had a physical examination, EEG and the specific complementary tests for each aetiology. RESULTS: There was a predominance of neural ceroid lipofuschinosis in 10 patients (55.5%), and in 9 of these the illness started before the age of 9 years. The second most frequent condition was myoclonic epilepsy with red-torn fibres (16.6%) and Unverricht-Lundborg disease (16.6%). The latter began in late childhood or adolescence. The most marked clinical characteristics were epilepsy, which was difficult to control and intellectual deterioration in 100%, followed by cerebellar signs in 88.8%. Myoclonias were the commonest type of seizures (94.4%) and many children presented with prior tonic-clonic seizures (88.8%). CONCLUSION: Response to treatment was poor but the best results were obtained using valproate either alone or associated with benzodiazepines.

[Tumors of the posterior fossa in children]. / Tumores de fosa posterior en el niño.

INTRODUCTION: Primary tumours of the CNS form 20% of the neoplasias seen in children. They are the second commonest type of cancer seen in childhood, after leukemia. A significant proportion of paediatric cerebral tumours (> 50%) are intratentorial. OBJECTIVE: To find the frequency and clinicopathological behavior of posterior fossa tumours in children seen at our hospital. PATIENTS AND METHODS: From the hospital records, a descriptive, retrospective study was made of 112 children with tumours of the posterior fossa who were attended at the Institute of Neurology and Neurosurgery between January 1980 and December 1997. The variables: age, sex, clinical findings, site and extent of the tumour and its histological description were recorded. The mean, standard deviation, frequency distribution and chi-squared test were used, depending on the type of variable to be analyzed. RESULTS: The age of the patients when the diagnosis was made was between 8 months and 15 years (mean = 8.32 years). The male/female ratio was 59/53 (1.1/1). The commonest site of the tumours was the cerebellum (58.9%). The predominant histological types were medulloblastoma (33.92%) and astrocytoma (19.64%) in the cerebellum, glioma in the brain stem (10.71%) and ependymoma in the i.v. ventricle (8.09%). CONCLUSIONS: Endocranial hypertension and the cerebellar syndrome were the predominant clinical findings in medulloblastoma, cerebellar astrocytoma and ependymoma. In brainstem gliomas there was often involvement of several cranial nerves and long tracts.