A child with resistant Kawasaki disease successfully treated with anakinra: a case report.

BACKGROUND: Kawasaki disease (KD) is an acute self-limited systemic vasculitis of unknown etiology. Intravenous immunoglobulin (IVIG) is an effective treatment and decreases the risk of cardiac complications to less than 5%. In spite of its effectiveness, some children do not respond to this therapy and still develop coronary aneurysms (CAA). The optimal treatment for IVIG non-responsive patients remains controversial although corticoids have been suggested to be an effective treatment in some patients. For those patients still resistant to IVIG and corticoids, interleukin-1 receptor antagonists (IL-1RA) such anakinra could be an alternative. CASE PRESENTATION: We present a 3 year-old Caucasian patient with KD without cardiac complications but with important resistance to treatment. After becoming resistant to IVIG and corticoids, anakinra proved to be an effective treatment. CONCLUSIONS: To our knowledge, this is the first report of the utility of IL-1RA in refractory KD without coronary impairment. The patient fulfilled the classical criteria for KD and, after becoming resistant to first and second line treatments, anakinra proved to be an effective treatment. Further studies are required to determine if this is an effective treatment option for other cases of resistant Kawasaki disease.

Arrhythmogenic right ventricular cardiomyopathy in boxer dogs: a retrospective study of survival.

The aim of the present study was to retrospectively evaluate survival in a population of 62 boxer dogs with arrhythmogenic right ventricular cardiomyopathy (ARVC), without left ventricular systolic failure, based on the following factors: age at diagnosis, presence of syncopal episodes, Holter arrhythmia classification and administered treatment. Medical records of boxer dogs with a diagnosis of ARVC between 2000 and 2010 were reviewed. Results showed that median survival time (MST) was longer in younger ARVC dogs than in the older ones P<0.001). MST was statistically different (P=0.012) between dogs with syncope (365 days) and dogs without syncope episodes (693 days), the probability of death within a year being 4.8 times greater in dogs with syncope (95% CI 1.48 to 15.99) than in dogs without syncope. Regarding Holter classification results, MST was 547.5 days in Holter class-2 dogs and 365 days in Holter class-4 dogs (P=0.030). There were no differences regarding treatment options; MST was 365 days (95% CI 193.615 to 536.4) in the sotalol group, 365 days (95% CI 92.86 to 637.14) in the mexiletine plus atenolol group, and 547.50 days (95% CI 170.45 to 924.55) in the procainamide group (P=0.383). According to this study, the best prognosis is for the younger boxer dog without syncope. There were no differences in survival times in relation to the different treatment options used.

Prevalence of dementia subtypes in El Prat de Llobregat, Catalonia, Spain: the PRATICON study.

BACKGROUND: Studies on dementia subtypes show a wide variation in the prevalence of Alzheimer's disease (AD) and vascular dementia (VD) worldwide. However, studies reporting on Lewy body dementia (LBD) and frontotemporal dementia (FTD) are sparse. AIMS: To describe the prevalence of dementia and subtypes. METHOD: A 34% sample of 5,150 subjects aged 70 years and over in El Prat de Llobregat (Barcelona) were screened by the Mini-Mental State Examination. When scoring <24, participants were assessed to establish a diagnosis. RESULTS: There were 165 subjects diagnosed with dementia (prevalence of 9.4%). Subtypes of dementia were: AD 69.1%, VD 12.7%, LBD 9.1%, FTD 3% and secondary dementia 1.8%. Prevalences were: AD 6.5%, VD 1.2%, LBD 0.9% and FTD 0.3%. CONCLUSIONS: AD and VD were the most common type of dementia. Prevalence of dementia, AD and FTD were similar to those reported, while prevalence of VD and LBD were lower.

[Values of GERMCIDE neuropsychological protocol in a sample of normal subjects]. / Valores del protocolo neuropsicológico GERMCIDE en una muestra de sujetos normales.

INTRODUCTION: The diagnosis of patients with cognitive deterioration or dementia requires a global approach in which the neuropsychological examination is a key piece. As part of the GERMCIDE study (Group for the Study and Multicenter Registry of Incident Cases of Dementia in Spain), a protocol was designed that included an assessment of the different cognitive functions that are most frequently altered in dementias (memory, orientation, speech, praxis, abstraction capacity and executive function). METHODS: In order to obtain data in normal subjects, this neuropsychological protocol was applied to a group of persons over 50 years without cognitive deterioration or dementia. RESULTS: A total of 103 subjects whose ages ranged from 50 to 95 years (mean: 73.5; SD: 9.3 years); 39 (37.9%) men and 64 (62.1%) women were studied. The mean score on the Mini-Mental State Examination (MMSE) was 27/30 (SD: 2.0). In the speech and praxis tests, 90% of the subjects obtained the maximum value, while performances were more unequal in memory, reasoning and programming. Mean score, standard deviation and distribution in percentages for each subtest are presented. CONCLUSIONS: The values obtained in this sample of normal subjects and their distribution in percentages may be very helpful to facilitate the interpretation of the findings of the neuropsychological examination with the GERMCIDE protocol in the general neurology clinic visits and also in the specialized visits in dementia.

Valores del protocolo neuropsicológico GERMCIDE en una muestra de sujetos normales / Values of GERMCIDE neuropsychological protocol in a sample of normal subjects

Introducción. El diagnóstico de los pacientes con deterioro cognitivo o demencia exige un enfoque global en el que la exploración neuropsicológica es una pieza clave. Como parte del estudio GERMCIDE (Grupo para el Estudio y Registro Multicéntrico de Casos Incidentes de Demencia en España) se diseñó un protocolo que incluye una valoración de las diferentes funciones cognitivas que con mayor frecuencia se alteran en las demencias (memoria, orientación, lenguaje, praxias, capacidad de abstracción y función ejecutiva). Métodos. Con el objetivo de obtener datos en sujetos normales este protocolo neuropsicológico se aplicó a un grupo de personas mayores de 50 años sin deterioro cognitivo ni demencia. Resultados. Se estudiaron 103 sujetos con edades comprendidas entre 50 y 95 años (media: 73,5; desviación estándar [DE]: '9,3 años); 39 (37,90J0) hombres y 64 (62,1 %) mujeres. La puntuación media en el Mini-Mental State Examination (MMSE) fue de 27/30 (DE: 2,0). En las pruebas de lenguaje y praxias el 900J0 de los sujetos obtuvieron el valor máximo, mientras que en memoria, razonamiento y programación los rendimientos fueron más dispares. Se presenta la puntuación media, DE y distribución en percentiles para cada subtest. Conclusiones. Los valores obtenidos en esta muestra de sujetos normales y su distribución en percentiles pueden ser de gran ayuda para facilitar la interpretación de los hallazgos de la exploración neuropsicológica con el protocolo GERMCIDE en las consultas de neurología general y también en las consultas especializadas en demencia

Introduction. The diagnosis of patients with cognitive deterioration or dementia requires a global approach in which the neuropsychological examination is a key piece. As part of the GERMCIDE study (Group for the Study and Multicenter Registry of Incident Cases of Dementia in Spain), a protocol was designed that included an assessment of the different cognitive functions that are most frequently altered in dementias (memory, orientation, speech, praxis, abstraction capacity and executive function). Methods. In order to obtain data in normal subjects, this neuropsychological protocol was applied to a group of persons over 50 years without cognitive deterioration or dementia. Results. A total of 103 subjects whose ages ranged from 50 to 95 years (mean: 73.5; SD: 9.3 years); 39 (37.9%) men and 64 (62.1%) women were studied. The mean score on the Mini-Mental State Examination (MMSE) was 27/30 (SD: 2.0). In the speech and praxis tests, 90% of the subjects obtained the maximum value, while performances were more unequal in memory, reasoning and programming. Mean score, standard deviation and distribution in percentages for each subtest are presented. Conclusions. The values obtained in this sample of normal subjects and their distribution in percentages may be very helpful to facilitate the interpretation of the findings of the neuropsychological examination with the GERMCIDE protocol in the general neurology clinic visits and also in the specialized visits in dementia

Banco de tejidos neurológicos destinados a la investigación en neurociencias. Una realidad en Navarra / Neurological tissue bank for research in neuroscience. A reality in Navarre

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Nested PCR for diagnosis of canine leishmaniosis in peripheral blood, lymph node and bone marrow aspirates.

A nested polymerase chain reaction (PCR) assay was developed using primers selected from the genomic DNA of Leishmania infantum and applied to the diagnosis of leishmaniosis in peripheral blood in dogs. Blood of 39 dogs of different breeds, all sampled in Catalonia (Spain), were tested for leishmaniosis by enzyme linked immunosorbent assay (ELISA), western blotting (WB) and peripheral blood mononuclear cell (PBMC) culture and nested PCR. Twenty negative controls (healthy dogs less than 1-year-old that had not been exposed to a sandfly season) were also studied. Nineteen of the 39 dogs studied were positive by ELISA and/or WB, and 18 of these had a positive PBMC nested PCR. PBMC nested PCR was negative in all the remaining animals that were negative by serological examination, including the 20 negative controls. Parasitological examination and nested PCR of bone marrow and lymph node aspirate from the 19 dogs positive by serological examination, were also positive. These results indicate that PBMC nested PCR is a sensitive and specific tool to diagnose leishmaniosis in dogs. The use of PBMC has the advantage over bone marrow and lymph node aspirates in that it is a less invasive sample.

Prolongation of epidural anaesthesia in dogs with bupivacaine in a lipid emulsion.

An aqueous solution and a lipid emulsion of bupivacaine were administered epidurally in doses of 1.8 mg/kg to six beagle dogs following a randomised two-phase crossover design. The aqueous solution was absorbed rapidly and the mean (sd) peak venous plasma concentration of bupivacaine, 1.4 (0.4) microg/ml, was detected after five minutes. After administration of the lipid emulsion, the peak plasma concentration of bupivacaine, 0.6 (0.2) microg/ml, was detected after 30 minutes. The mean (sd) t1/2beta of the aqueous preparation was 149.1 (32.6) minutes, and of the lipid emulsion 119.2 (34.0) minutes. Both preparations had a similar bioavailability. The mean time to the onset of motor block after the administration of the aqueous solution, 2.3 (2.2) minutes, was significantly shorter (P=0.028) than after the administration of the lipid emulsion, 9.4 (1.9) minutes, and the duration of the motor block induced by the lipid emulsion, 217.6 (26.2) minutes, was significantly longer (P=0.043) than for the aqueous solution, 158 (48.8) minutes. During anaesthesia, the plasma concentrations of bupivacaine ranged between 1.3 and 0.2 microg/ml. Non-significant changes in systolic blood pressure and heart rate were observed which coincided with the peak plasma concentrations of bupivacaine.

Deterioro cognitivo y demencia de origen vascular / Cognitive deterioration and dementia of vascular origin

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[Multiple sclerosis and epileptic seizures]. / Esclerosis múltiple y crisis epilépticas.

INTRODUCTION: Although epileptic seizures are uncommon in multiple sclerosis they are more prevalent than in the general population, which supports an aetiological relationship. Similarly in a considerable proportion of patients with multiple sclerosis and epileptic seizures, alterations in magnetic resonance and electroencephalogram studies which could be correlated with the clinical features of epilepsy were observed. Nevertheless, it is difficult to establish definite clinical characteristics in these patients since the underlying pathogenic mechanisms are poorly understood and there is great variability with regard to the type of seizure, point at which this occurs during the course of the disease, degree of recurrence and other aspects. CLINICAL CASE: We report the clinical, electroencephalographical and neuroimaging findings of seven patients with multiple sclerosis who had epileptic seizures and those in whom there was no evidence of other potentially epileptogenic pathology. In two patients the epileptic seizures formed part of the first episode of their illness. One patient presented more than one type of epileptic seizure. These seizures were generalized in two cases, partial sensory and/or motor with secondary generalization in three, simple partial motor in one and partial complex in two. The epileptic seizures coincided with other clinical features of episodes in three cases and the electroencephalogram showed anomalies in five cases. CONCLUSIONS: The findings observed were of a wide variety, as was found in other reported series. We point out certain correlations between the clinical data, magnetic resonance and electroencephalogram which may help to orientate the management of these patients.

Pharmacokinetics and pharmacologic effects of the S(-) isomer of bupivacaine after intravenous and epidural administration in dogs.

OBJECTIVE: To determine pharmacokinetic variables and pharmacologic effects of the S(-) isomer of bupivacaine (S[-]-BPV) in dogs. ANIMALS: 6 adult male Beagles. PROCEDURE: Dogs received S(-)-BPV (1 mg/kg of body weight) i.v., and 15 days later, the same dogs received 1.8 mg/kg epidurally. Pharmacokinetic variables and pharmacologic effects were determined for each route of administration. RESULTS: After i.v. administration, plasma concentration versus time curves were adjusted, using biexponential equations that indicated a rapid distribution phase followed by a slower elimination phase, with a mean +/- SD half-life of 33.5 +/- 17.0 minutes. Mean plasma clearance was 21.0 +/- 10.7 ml/min/kg, and mean volume of distribution at steady state was 0.8 +/- 0.2 L/kg. After i.v. administration, mean peak plasma concentration was 2.6 +/- 0.7 micrograms/ml; after epidural administration, it was 0.9 +/- 0.5 microgram/ml at approximately 3 minutes. Half-life after epidural administration was 5 times longer than that observed after i.v. administration. Motor block began immediately after the end of epidural administration and lasted for 3 to 4 hours. Changes in systolic blood pressure and heart rate after epidural administration were slight but occurred at the same time that plasma concentration peaked. After i.v. administration, motor block or variations in physiologic variables studied were not observed. CONCLUSIONS AND CLINICAL RELEVANCE: In dogs, the pharmacologic behavior of S(-)-BPV was similar to that of the bupivacaine racemate, but motor block attributable to S(-)-BPV lasted longer than that attributable to the racemate, with lower plasma concentrations observed at equivalent sample collection times.

[Variation of Mini-Mental-State examination scores due to age and educational level. Normalized data in the population over 70 years of age in Pamplona]. / Variación de las puntuaciones en el Mini-Mental-State con la edad el nivel educativo. Datos normalizados en la población mayor de 70 años de Pamplona.

The Mini-Mental-State Examination (MMSE) is widely used as a screening tool for dementia in epidemiological studies. Its applicability in population-based studies is nevertheless limited by its low specificity. The effect of age and educational level have been usually ignored when cut-off scores have been selected. The aim of this study was to evaluate the effect of age and educational level on the MMSE scores in a representative sample of subjects older than 70 and provide adjusted normalised data according to these two variables, after excluding subjects with dementia or cognitive decline. Population-based, cross-sectional and longitudinal study of a representative cohort of 1367 subjects older than 70. All subjects with suspected dementia or cognitive decline received a neurological evaluation where clinical and etiological diagnosis were established. Normal MMSE scores, as defined by the 10th percentile, varied significantly across age and educational level groups. Exclusion of demented or cognitively declined patients from the reference population reduced the variability and "range of normality", but this remained excessively high in the older and less educated groups. The use of different cut-off points for each age and educational level groups may improve the specificity and applicability of the MMSE in population-based epidemiological studies. However, the wide amplitude of the range of normality suggests that different approaches, other than this vibariate analysis, may prove more adequate in the selection of cut-off scores for the MMSE.

[Phenotype variability in adrenoleukodystrophy. Presentation of three new cases and a review of literature]. / Variabilidad fenotípica de la adrenoleucodistrofia. Presentación de tres casos y revisión de literatura.

INTRODUCTION: Adrenoleukodystrophy is a hereditary recessive sex-linked disorder with very variable phenotype expression, including classical infantil ALD, adrenomyeloneuropathy (AMN) in adults and sex-linked Addison's disease. Clinical observations. Three affected patients are presented. The first showed signs of myeloneuropathy from the age of 38 and diagnosis was made by showing raised serum and fibroblasts levels of very long chain fatty acids (C26:0). In the second case symptoms started at the age of 13 and cerebral and peripheral nervous system changes developed progressively. This patient's brother was the third case, showing symptoms when he was 21 and developing cerebral, medullary and peripheral nervous system involvement. In the latter two cases, diagnosis was made by showing intracytoplasmatic trilaminary inclusions in the nervous system. CONCLUSIONS: It is important to recognize the different varieties of this disease in view of the possibilities of genetic counselling and of the therapeutic implications which are currently being evaluated.

Bupivacaine disposition and pharmacologic effects after intravenous and epidural administrations in dogs.

Pharmacokinetic variables of bupivacaine (BPV) were determined after IV and epidural administrations in the same 6 dogs. Plasma BPV concentration curves after IV administration were adjusted to biexponential kinetics: a rapid distribution phase was followed by a slower elimination phase, with half-life of 34.5 +/- 7.8 minutes. Mean plasma clearance was 20.2 +/- 7.4 ml/min/kg of body weight, and mean volume of distribution at steady state was 0.7 +/- 0.2 L/kg. After epidural administration, absorption was rapid. Peak plasma concentration, 1.4 +/- 0.4 microgram/ml, was detected approximately 5 minutes after BPV administration. The half-life corresponding to epidural administration (179 +/- 33.6 minutes) was 5 to 6 times longer than that observed after IV administration, possibly because of the slow release of BPV from the epidural space. Induction times were short (2.3 +/- 2.2 minutes); anesthesia quickly began and lasted for more than 2 hours (158 +/- 48.8 minutes). During that period, BPV plasma concentration ranged between 1.4 and 0.2 microgram/ml. Changes in systolic blood pressure and heart rate were correlated to high plasma concentration of BPV. These modifications were observed for the first 30 minutes, reaching baseline values after 60 minutes.

Prevalence of Alzheimer's disease and other dementing disorders in Pamplona, Spain.

We investigated the prevalence of dementing disorders in the city of Pamplona, Spain, using a door-to-door two-phase approach. We first administered the Cambridge Examination of Mental Disorders of the Elderly (CAMDEX) to all survivors, as of March 1, 1991, of a probability sample of the total population identified in 1989 (n = 1,127). Using specified diagnostic criteria, the study neurologists extensively investigated those subjects who screened positive on CAMDEX. We found 194 subjects affected by dementia, 119 had Alzheimer's disease; 51 vascular or mixed dementia, and 16 secondary dementia. The prevalence of both dementia and Alzheimer's disease increased steeply with advancing age and was consistently higher in women. The prevalence of combined vascular and mixed dementia increased less rapidly with age, and was generally higher in men. Alzheimer's disease was the most common type of dementia. Our prevalence figures for dementia and Alzheimer's disease are similar to those previously reported in Europe.

[Multicenter register of index cases of dementia. A study by the Spanish Neurological Society's dementia group]. / Registro multicéntrico de casos incidentes de demencia. Un estudio del grupo de demencias de la Sociedad Española de Neurología.

We report the objectives and case registration methods used by neurologists belonging to the Spanish Neurological Society's dementia group (GERMICIDE). Forerunners of the present project are also described. The group's principal aim, which is to determine the distribution of dementia in the "neurological" environment is pursued by enrolling consecutive index cases (new presentations) arriving at 18 Spanish neurological units over a period of 2 years. A standard protocol was used to study the patients and final diagnoses were agreed upon in keeping with international criteria for dementia and the main neurological diseases (DSM-III-R, CDR and others). A second objective is to study risk factors for the main etiological groups of dementia (Alzheimer's disease and vascular dementia) and to follow each patient in the cohort until death in order to assess the course of disease and its biological markers. The series will be large, as there are already over 800 cases listed in the coordinating centre even though the period of enrollment has not ended.

[Brain photon emission tomography. Value of the corticocerebellar index and gammagraphic patterns in Alzheimer's disease and other diseases]. / Tomografía de emisión de fotones cerebral. Valor del índice corticocerebeloso y patrones gammagráficos en la enfermedad de Alzheimer y otras afecciones.

Brain single photon emission computed tomography (SPECT) with 99mTc-HMPAO is a diagnostic tool for evaluating regional cerebral blood flow. Recently, the diagnostic possibilities of the method are being investigated in some neurologic disorders, such as cerebrovascular accidents, seizures and dementia. This work has been carried out with 54 subjects, 9 healthy volunteers and 45 patients (31 dementia and 14 epileptics), in order to evaluate gammagraphic patterns and the utility of cortico/cerebellar activity indexes. An interesting diagnostic finding is a significant decrease (p less than 0.001) in perfusion of temporoparietal regions in the patients with Alzheimer's disease in relation with the healthy volunteers' group. We have not found significant changes in perfusion in the group of epileptic patients during the interictal phase. We conclude emphasizing the interest of the SPECT in the differential diagnosis of dementia.