[Controversies about new drugs for the treatment of dementia. An unfavourable view]. / Controversias en torno a los nuevos fármacos en el tratamiento de la demencia. Postura desfavorable.

Currently, the important part played by the senile plaque and neurofibrillary tangles puts amyloid protein and tau hyperphosphorylation at the centre of a new direction in the investigation of the molecular biology and treatment of Alzheimer s disease. OBJECTIVE. To consider the scientific evidence regarding the limitations of anticholinesterase treatment. DEVELOPMENT. Most of the clinical trials with anticholinesterase inhibitor drugs have been too short (3-6 months in a disease lasting an average of 8.5 years). There is slight clinical improvement with a definite therapeutic ceiling. A small proportion of patients respond, but there is no way of knowing who these will be. Improvement occurs during the early stages of the illness. This means that when the illness is advanced or does not respond initially, treatment should be reconsidered. It is necessary for consensus to be reached to obtain uniformity of objectives and methodology in clinical trials, which have been heterogeneous until now, incorporating the results of epidemiological studies. CONCLUSION. Initial expectations of an analogy between the cholinergic deficit of Alzheimer s disease and the dopaminergic deficit of Parkinson s disease, with the effects on treatment implied by this, has been proved false with the passage of time and clinical, therapeutic and scientific experience.

[Epidemiological features of epilepsy in adults requiring hospital admission]. / Caracterización epidemiológica de la epilepsia del adulto subsidiaria de ingreso hospitalario.

INTRODUCTION AND OBJECTIVES: There are few epidemiological studies specifically directed towards finding the features of the adult epileptic population requiring hospital admission. We proposed to analyze these features in a consecutive series of patients admitted to our department with epilepsy and find the prevalence of diagnoses classified as syndromes and epileptic seizures according to the classification of ILAE and their distribution according to age. PATIENTS AND METHODS: We made a retrospective classification of the characteristics of the patients admitted to our department with the diagnosis of epilepsy during 1999. We divided the sample into three age groups: 18 30, 31 60 and >60 years. We calculated the absolute prevalence of the different types of crises and epileptic syndromes, the specific relative prevalence according to age groups and diagnostic category expressed with confidence intervals of 95%. The degree of statistical significance was obtained using the chi squared test of Mantel Haenszel. RESULTS: The sample group contained 96 patients (51% men and 49% women). There were 65.62% partial seizures, 21.87% generalized seizures and 12.5% pseudoseizures. The syndromes found included 60.7% of epilepsies related to site, 5.95% generalized epilepsies, 7% unclassified syndromes and 26.19% special syndromes. Of the symptomatic epilepsies, the commonest etiology was vascular, followed by toxic, tumoral and degenerative. The evolution of the relative prevalence according to age corresponded to three different patterns: ascending (partial crises and special syndromes), descending (pseudocrises and generalized epilepsy) and stable with peaks in middle age (generalized seizures, partial with generalization, unclassified syndromes, symptomatic and cryptogenic partial epileptic syndromes). CONCLUSIONS: The distribution according to seizures is similar to that reported in population studies. Special syndromes are much commoner than that reported in population studies, especially in epilepsies conditioned by the environment. The diagnosis of pseudoseizures are significantly commoner in the group of young adults. Partial seizures and special syndromes tended to be more frequent in older patients, whilst pseudocrises and generalized epilepsies were commoner in younger adults. The remaining seizures and syndromes predominated in the 18 30 year age group.

[Malignant middle cerebral artery infarction: medical or surgical treatment?]. / Infarto maligno de la arteria cerebral media: tratamiento médico o quirúrgico?

INTRODUCTION: Post-ischemic brain edema occurs in 10-20% of cases of infarction of the middle cerebral artery and is the main cause of early death following a completed stroke. This complication, which is known as malignant middle cerebral artery infarction (MMCI), has a mortality rate of 78% when treated medically and thus requires a different management, such as a decompressive craniectomy. The main aim of this study is to review this procedure. DEVELOPMENT: We conducted a search in the literature published over the last 20 years on this subject. Most of the studies are series of clinical cases with very favourable surgical outcomes. In non-random case-control studies the mortality rate was seen to decrease in the surgical group, and more so if the intervention was carried out early, as compared to the group that underwent medical treatment. Another study that compared decompressive craniectomy with hypothermia showed a higher survival rate in the surgical group. The post-surgery morbidity rate has not been determined, although it seems to be lower in infarction of the non-dominant hemisphere and in younger patients. CONCLUSIONS: The low degree of conclusiveness of the studies published to date only enables us to offer one practical opinion concerning this issue: decompressive craniectomy should be evaluated on an individual basis in patients with MMCI who do not respond to medical treatment. The final decision and the most appropriate moment to operate on the patient following the stroke must be based on the family's opinion and on the clinical features of the patient.

[Inflammatory pseudotumor: differential diagnosis of tumors of the 4th ventricle]. / Pseudotumor inflamatorio: diagnóstico diferencial de los tumores del IV ventrículo.

INTRODUCTION: An inflammatory pseudotumour is a condition of unknown origin and inflammatory nature. It is rarely found in the CNS. We report a case of inflammatory pseudotumour localized to the IV ventricle and review the clinical characteristics of previously reported cases in order to outline the clinical profile of this condition. CLINICAL CASE: A 40 year old man was admitted to hospital complaining of a subacute condition involving difficulty in speaking and in moving his right arm and leg. On examination he had ocular deviation on initial gaze, a complete right Horner's syndrome, right supranuclear facial palsy, dyssynergy-asymmetry on the right finger-nose test and a dissociated sensitivity disorder of the left arm. On MR of the brain there was a space occupying lesion, nodular in form and fixed to the roof of the fourth ventricle. The histopathological report on the specimen removed by surgery stated it to be an inflammatory pseudotumour. On a MEDLINE search for reported cases of inflammatory pseudotumour of the CNS, 27 were found since 1967. Four cases, including ours, involved masses growing into the interior of the fourth ventricle. CONCLUSIONS: Inflammatory pseudotumour of the CNS is a condition affecting young adults, with a slight male predominance and some association with clinical and analytical data suggesting autoimmune dysfunction. The intraventricular site, particularly within the fourth ventricle, is relatively common (4/28) and is usually associated with clinical features of dysfunction of the posterior fossa and/or intracranial hypertension. We consider that inflammatory pseudotumour should be included in the differential diagnosis of tumours of the fourth ventricle.

[Parry-Romberg progressive facial hemiatrophy: pathogenic and evolutionary considerations with regards to a case followed up for a long period of time]. / Hemiatrofia facial progresiva de Parry-Romberg: consideraciones patogénicas y evolutivas a propósito de un caso con seguimiento prolongado.

INTRODUCTION: The Parry-Romberg syndrome is a clinical condition in which there is progressive facial hemiatrophy which may be associated with neurological, ocular, cutaneous and auto-immune disorders. It is of unknown aetiology, although many theories have been put forward. CLINICAL CASE: We present a case of left facial hemiatrophy associated with ipsilateral lingual hemiatrophy and signs of cortico-cerebellar dysfunction, the neuroimaging findings (NMR) and clinico-radiological follow-up covered a period of over 10 years. CONCLUSION: The data on the clinical course and neuroimaging, together with our review of the literature, suggest that the Parry-Romberg syndrome may be considered to be a dysgenetic process which may originate during the first stages of CNS embryogenesis.

Clúster-tic como comienzo clínico de una fístula dural carótido cavernosa / Cluster-tic syndrome as the first clinical manifestation of a dural carotid-cavernous fistula

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Possible case of peripheral osmotic demyelination syndrome.

Central pontine myelinolysis (CPM) is an uncommon neurological syndrome that is usually related to the rapid restoration of a previous hyponatraemia. Although the most frequent location of CPM injury is the pons, it is now designated osmotic demyelination syndrome (ODS) because, as well as in the brainstem, these injuries can be observed in other parts of the central nervous system (CNS)-for example, the thalamus, subthalamic nucleus, external geniculate body, putamen, globus pallidum, internal capsule, white matter of cerebellum and the deep layers of the brain cortex. However, an exhaustive search of the literature (MEDLINE 1967-2007) has revealed no case report of peripheral nervous system (PNS) demyelination secondary to severe hyponatraemia.

[Trigeminal-autonomic cephalalgias: International Headache Society diagnostic criteria does not allow for a correct classification]. / Cefaleas trigeminoautonómicas: los criterios diagnósticos de la International Headache Society no permiten su clasificación correctamente.

INTRODUCTION: The International Classification of Headache Disorders only recognizes the following as trigeminal- autonomic cephalalgias (TAC): cluster headache, paroxysmal hemicrania and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome. Nevertheless, there are other types of TAC that still have not been incorporated into the International Classification of Headache Disorders although they have been before reported in the literature. We present the results of the analysis of a series of 94 cases of TAC. METHODS: We performed a retrospective study of 2,132 patient who attended a general neurology consultation due to headache between January 1997 and June 2006. Those patients with unilateral headache (orbital, supraorbital y/o temporal) accompanied of some ipsilateral autonomic sign were selected. We investigated the etiology of all cases and made a nosology classification according with two types of criteria: IHS (International Headache Society) strict criteria as well as other criteria (IHS plus) including migraine with ipsilateral autonomic signs and hemicrania continua, thus following Goabsby. RESULTS: A total of 94 cases of TAC (4.4% of all the headaches) were found, 89 of which had a primary etiology and 5 secondary etiology. Cluster headache was the most frequent TAC in our series (29%), independently of the criteria used. However, hemimigraine with ipsilateral autonomic signs had a similar frequency (28%) according to IHS plus criteria. CONCLUSIONS: a) In our series TAC have constituted an not very frequent entity, there being a subgroup of secondary cases among them, and b) the current International Classification of the Headache Disorders has some limitations as an instrument for the nosology classification of TAC.

[Vasomotor reactivity throught the voluntary apnea test can be measured without calculating the apnea index]. / La reactividad vasomotora mediante el test de la apnea voluntaria puede determinarse sin calcular el índice de apnea.

INTRODUCTION: Vasomotor reactivity (VR) is the capability of the cerebral arterioles to dilate and to constrict in response to determined stimuli. Using transcranial doppler (TCD), there are various test to evaluate it, including the breath holding test. With this test, VR can be calculated two ways: using the increment percentage of the mean velocity with respect to the basal velocity (PIV) or by quantifying the apnea time to obtain what is call the breath holding index (PIV/apnea seconds). The objective of our study is to study the linear correlation between the PIV and the breath holding index. METHODS: This was an observational study performed in a neurology ambulatory setting. A total of 128 subjects, age 16 to 50 years old, were included. We assessed VR using the breath holding test described by Markus and Harrison on the middle cerebral artery. The linear correlation between the two quantitative variables was analysed using Pearson's correlation coefficient. RESULTS: Pearson's correlation coefficient between the PIV and the breath holding index was 0.75 for a significance level of p < 0.0001. CONCLUSION: Given the good linear correlation between these two methods for calculating the VR using the breath holding test, we consider it is not necessary to calculate breath holding test time when using this test.

[Descriptive analysis of the e-mail list "Neurología-RedIRIS" (http://listserv.rediris.es/neurologia.html): an active, plural instrument having scientific quality at the reach of the Spanish speaking neurology sector]. / Análisis descriptivo de la lista de correo electrónica "Neurología-RedIRIS" http://listserv.rediris.es/neurologia.html): un instrumento activo, plural y de calidad científica al alcance de la neurología hispanohablante.

INTRODUCTION: In recent years, professional mailing-lists (PML) have experienced wide diffusion in the field of the medicine thanks to their educational and academic potential. In neurology and in Spanish, Neurología list belonging to RedIRIS has played the lead in the history of PML. Since the year 1997, it has maintained approximately 200 persons related with neurology in communication and has exceeded more of 7,300 messages. MATERIAL AND METHODS: We analyzed retrospectively the activity recorded in the Neurología PML between January and December 2003, recording variables related to the activity and scientific quality of the PML and to the origin and content of the messages. RESULTS: 758 messages were registered (2.07 messages/day; 3.73 messages/subscriber). 93 of 203 subscribers (45.81 %) participated on at least one occasion. 83 % of the messages came from Spain, there being an inverse relationship between the neurologists relative endowment and degree of participation in the PML. The most frequent messages referred to clinical cases followed by debate of topics of management and scientific consultations. The scientific quality of Neurología PML is comparable to other English language based PML analyzed based on the number of publications in Index Medicus and of the impact factor of its subscribers. CONCLUSION: Neurología PML has reached a level of maturity and enjoys a critical mass and a number of subscribers of high scientific level that assures its immediate future. Besides its educational and consultation functions, it may become an opinion forum for the Spanish neurologists without their losing their independence or Latin-American vocation.

Déficit de cianocobalamina que simula una enfermedad de Creutzfeldt-Jakob / A cyanocobalamin deficiency that simulates Creutzfeldt-Jakob disease

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Cefaleas trigeminoautonómicas: los criterios diagnósticos de la International Headache Society no permiten su clasificación correctamente / Trigeminal-autonomic cephalalgias: International Headache Society diagnostic criteria does not allow for a correct classification

Introducción. La Clasificación Internacional de las Cefaleasde la International Headache Society (IHS) sólo reconoce comocefaleas trigeminoautonómicas (CTA) la cefalea en racimos, lahemicránea paroxística y la cefalea SUNCT (short-lasting unilateralneuralgiform headache attacks with conjunctival injectionand tearing). No obstante, existen otras cefaleas hemicranealescon signos autonómicos ipsilaterales aún no incluidas a pesar dehaber sido ampliamente descritas en la literatura. Se presentanlos resultados del análisis de una serie de 94 casos de CTA.Métodos. Estudio retrospectivo de 2.132 pacientes queacudieron por cefalea a una consulta de neurología generalentre enero de 1997 y junio de 2006. Se seleccionaron aquellospacientes que sufrían una cefalea unilateral de localizaciónorbitaria, supraorbitaria y/o temporal acompañada de algúnsigno autonómico ipsilateral. Se realizó una clasificaciónetiológica de todas las CTA y una nosológica de las primarias.Esta última se llevó a cabo de acuerdo con dos tipos de criterios:IHS estricta e IHS plus (incluyendo migraña con signosautonómicos y criterios de Goabsby para hemicránea continua).Resultados. Encontramos 94 casos de CTA (4,4 % deltotal de cefaleas), 89 de etiología primaria y 5 secundaria.La cefalea en racimos fue la CTA más frecuente en nuestraserie (29 %), independientemente de los criterios utilizados,aunque la hemicránea continua y la migraña con signosautonómicos presentaron una frecuencia similar (28%) cuandose aplicaron los criterios IHS plus.Conclusiones. a) En nuestra serie las CTA han constituidouna entidad poco frecuente, existiendo entre ellas unsubgrupo de casos secundarios, y b) la actual ClasificaciónInternacional de las Cefaleas de la IHS tiene limitaciones paradistribuir nosológicamente las CTA (AU)

Introduction. The International Classification ofHeadache Disorders only recognizes the following as trigeminal-autonomic cephalalgias (TAC): cluster headache,paroxysmal hemicrania and short-lasting unilateralneuralgiform headache attacks with conjunctival injectionand tearing (SUNCT) syndrome. Nevertheless, thereare other types of TAC that still have not been incorporatedinto the International Classification of HeadacheDisorders although they have been before reported in theliterature. We present the results of the analysis of a seriesof 94 cases of TAC.Methods. We performed a retrospective study of2,132 patient who attended a general neurology consultationdue to headache between January 1997 and June2006. Those patients with unilateral headache (orbital,supraorbital y/o temporal) accompanied of some ipsilateralautonomic sign were selected. We investigated theetiology of all cases and made a nosology classificationaccording with two types of criteria: IHS (InternationalHeadache Society) strict criteria as well as other criteria(IHS plus) including migraine with ipsilateral autonomicsigns and hemicrania continua, thus following Goabsby.Results. A total of 94 cases of TAC (4.4 % of all theheadaches) were found, 89 of which had a primary etiologyand 5 secondary etiology. Cluster headache was themost frequent TAC in our series (29 %), independently ofthe criteria used. However, hemimigraine with ipsilateralautonomic signs had a similar frequency (28 %) accordingto IHS plus criteria.Conclusions. a) In our series TAC have constitutedan not very frequent entity, there being a subgroup ofsecondary cases among them, and b) the current InternationalClassification of the Headache Disorders has somelimitations as an instrument for the nosology classificationof TAC (AU)

Evaluación de la fiabilidad del EUROTEST / Reliability assessment of the EUROTEST

Introducción. El EUROTEST es un instrumento válido y útil para la detección de deterioro cognitivo y demencia. Nuestro objetivo fue evaluar la fiabilidad test-retest (FTR) y la fiabilidad interobservador (FIO) de este instrumento. Métodos. La FTR se evaluó mediante un diseño transversal de medidas repetidas en una muestra de 30 sujetos sin deterioro cognitivo; la FIO se determinó mediante la evaluación a ciegas de 10 aplicaciones del test por 20 observadores independientes; en ambos casos se utilizó el coeficiente de correlación intraclase y los resultados se compararon con los obtenidos con las mismas muestras y los mismos procedimientos por el Test de Fluidez Verbal semántica (TFVs). Resultados. No hay diferencia significativa en las características de los sujetos de este estudio y las de estudios previos del EUROTEST. La FTR del EUROTEST (0,94 [IC 95%: 0,87-0,97]) fue significativamente superior a la del TFVs (0,51 [IC 95 %: 0,17-0,74]); no hubo diferencia significativa, en cambio, entre la FIO del EUROTEST (0,91 [IC 95 %: 0,82- 0,97]) y la del TFVs (0,96 [IC 95%: 0,91-0,99]). Conclusiones. El EUROTEST tiene altas FTR y FIO; por tanto, es un instrumento adecuado para seguir evolutivamente a sujetos con demencia y evaluar su respuesta al tratamiento

Introduction. The EUROTEST is a valid and useful instrument for detecting cognitive impairment and dementia. Our aim was to assess the test-retest reliability (TRTR) and the inter-rater reliability (IRR) of this instrument. Methods. We assessed the TRTR by means of a cross-sectional repeated measures design in 30 subjects with no cognitive impairment. The IRR was determined through the blind assessment of 10 test administrations by 20 independent observers. In both instances we calculated the intra-class correlation coefficient and we compared those coefficients with the ones of the semantic Verbal Fluency Test (sVFT) obtained with the same samples and procedures. Results. There were not significant differences concerning the characteristics of the subjects included in this study and those of previous studies on the EUROTEST. The EUROTEST showed a significantly higher TRTR (0.94 [95% CI: 0.87-0.97]) than the sVFT (0.51 [95% CI: 0.17- 0.74]), but there was not significant difference in IRR between the EUROTEST (0.91 [95% CI: 0.82-0.97]) and the sVFT (0.96 [95% CI: 0.91-0.99]). Conclusions. The EUROTEST shows high TRTR and IRR; therefore, this instrument is appropriate for the longitudinal assessment of subjects with dementia and their response to treatment

Análisis descriptivo de la lista de correo electrónica "Neurología-RedIRIS" (http://listserv.rediris.es/neurologia.html): un instrumento activo, plural y de calidad científica al alcance de la neurología hispanohablante / Descriptive analysis of the e-mail list "Neurología-RedIRIS" (http://listserv.rediris.es/neurología.html): an active, plural instrument having scientific quality at the reach of the Spanish speaking neurology sector

Introducción. En los últimos años las listas de correo profesionales (LCP) han encontrado amplia difusión en el campo de la medicina gracias a su potencial docente y académico. En neurología y en castellano, la historia de las LCP ha estado protagonizada por la lista Neurología, dependiente de RedIRIS, que desde el año 1997 mantiene comunicadas a unas 200 personas relacionadas con la neurología y ha superado los 7.300 mensajes. Material y métodos. Se analizó de forma retrospectiva la actividad registrada en la LCP Neurología en el período comprendido entre los meses de enero y diciembre de 2003, registrando variables relativas a la actividad y calidad científica de la LCP y a la procedencia y contenido de los mensajes. Resultados. Se registraron 758 mensajes (2,07 mensajes/día; 3,73 mensajes/suscriptor). Noventa y tres de los 203 suscriptores (45,81 por ciento) participaron al menos en una ocasión. El 83 por ciento de los mensajes procedieron de España, existiendo una relación inversa entre la dotación relativa de neurólogos y el grado de participación en la LCP. Los mensajes más frecuentes fueron referidos a casos clínicos, temas de gestión y consultas médicas. El análisis del número de publicaciones de los suscriptores y de su factor de impacto permite afirmar que la calidad científica de la lista es equiparable a otras LCP de ámbito anglosajón. Conclusión. La actividad, número de suscriptores y calidad científica de la lista Neurología permite afirmar que ha alcanzado su madurez y que tiene asegurado su futuro inmediato. Además de su función docente y de consulta puede convertirse en un foro de opinión para los neurólogos españoles sin perder su independencia ni su vocación latinoamericana (AU)