[Model of analysis in two halves for semantic fluency tasks]. / Modelo de análisis en dos mitades para tareas de fluidez semántica.

INTRODUCTION: Semantic verbal fluency (SVF) tasks involve the activation of language, semantic memory, working memory and the executive functions. The second half of the test increases the demands on working memory and search for lexical items, which can make it more difficult than the first half. AIM: To conduct a pilot study in order to standardise the two halves of the SVF test ('animals' category) in adults over 50 years of age. SUBJECTS AND METHODS: Two verbal fluency tasks (semantic and phonological) and the Spanish version of the Mini-Mental State Examination with working memory were applied in 122 controls (range: 50-89 years), who were distributed according to age and schooling. RESULTS: There is a significant difference between the two halves of the test adjusted for age and schooling (p < 0.0001). Scoring in the second half showed a higher correlation with age, schooling and working memory than the first half. Performance in the two halves was standardised for three age groups (50-59, 60-79 and 80-89 years) and two levels of schooling (high and low). CONCLUSIONS: The number of names in the second half of the SVF test was lower than in the first half in all the groups, which supports the hypothesis of a greater cognitive demand, probably on working memory and the search for lexical items in semantic memory. Having normative values available for the two halves of the SVF test allows simultaneous and independent interpretation of the performance at two levels of intra-task difficulty. This model of analysis complements the traditional assessment and can easily be applied in day-to-day clinical practice.

[Facial diplejia: a regional variant of Guillain-Barré syndrome]. / Diplejia facial: variante regional del síndrome de Guillain-Barré.

Facial palsy is a uncommon clinical manifestation that it can be caused by different etiologies. We show a patient with a chronic periodontal disease who presented a sudden facial palsy, initially in left-face but it becomes bilateral quickly. In few days he associated paresthesias in his right arm and hyporreflexia. These clinical findings with albumin-cytological dissociation, they had allowed to establish the diagnosis of regional variant of Guillain-Barré Syndrome (SGB). Facial diplejia is an idiopathic manifestation in 25% of patients, and this is the most common cause. However, facial diplejia can be secondary to many etiologies as SGB. The affectation of facial nerve associated to other motor symptoms in SGB is frequent, but it is not frequent the presentation as facial diplejia alone. In conclusion, it is necessary a high clinical suspicion to do a lumbar punction (PL) and MRI to reject neoplasic pathologies and to obtain a diagnosis and an adequate treatment.

[Neuronal migration disorders: a cause of curable medication resistant epilepsy]. / Alteraciones de la migración neuronal: una causa de epilepsia farmacorresistente curable.

INTRODUCTION: Focal cortical dysplasia is a neuronal migration disorder that appears with bouts of epileptic seizures that are usually medication resistant. The improvements introduced into neuroimaging techniques in recent years have made it possible to diagnose and offer surgical treatment to certain patients who have been under polytherapy for many years with poor control over their seizures. CASE REPORT: We report the case of a 22-year-old male with epileptic fits since the age of 18 months, who was refractory to multiple combinations of oral antiepileptic drugs (AEDs) with electroencephalograms that displayed frontal, fast spike-wave discharges, with greater expression on the left side and findings from neuroimaging techniques that were repeatedly normal. After being readmitted to hospital because his seizures had got worse, the neuroimaging study was repeated and left frontal focal cortical dysplasia was observed, which led us to start considering neurosurgery. At present, six months after the intervention, the patient has had no further convulsive fits and therapy with oral AEDs is being reduced. CONCLUSIONS: Neuroimaging studies must be repeated in patients that have already been examined because in this way it may be possible to save a group of patients (who would otherwise have to resign to poor control over their seizures as well as the side effects of many AEDs) from becoming medication resistant.

[Language and cognitive impairment: a semiological study into visual naming]. / Lenguaje y deterioro cognitivo: un estudio semiológico en denominación visual.

INTRODUCTION: The presence of anomia and/or paraphasias in patients with cognitive impairment suggests an associated deterioration of their language. Visual naming makes it possible to examine these signs in a controlled manner as the target word is already known. AIM. To conduct a semiological study of naming in normal aging, cognitive impairment and language impairment conditions. SUBJECTS AND METHODS: The study consisted in an analysis of nine types of signs (correct response, increased latencies, circumlocutions, absence of response, semantic verbal paraphasias, verbal paraphasias with a similar form, unrelated verbal paraphasias, phonemic paraphasias and neologisms) in a visual naming task (6 items), in two groups with normal language--controls > 70 years and patients with Alzheimer's disease (AD) with onset of amnesia--and two groups with language impairment--vascular aphasia and anomic AD-. RESULTS: Patients failed to perform naming correctly in 4.2% of the responses in controls, 10% in patients with amnesic AD, 30% in patients with vascular aphasia and 50% in patients with anomic AD. Semantic paraphasias were observed in the two groups with normal language abilities (controls and amnesic AD), although frequencies were low. Signs that suggest difficulties in accessing/retrieving lexical items were more frequent in patients with AD and ran parallel to the degree of anomia. One notable finding was the absence of signs of phonological dysfunction in the two groups with AD, regardless of the degree of anomia. CONCLUSIONS: Semiological quantification makes it possible to distinguish differences in the degree of anomia and in the pattern of errors both in controls and in patients with amnesic onset AD and among patients with vascular aphasia and neurodegenerative anomia.

[Progressive myoclonic cerebellar ataxia as a manifestation of Creutzfeldt-Jakob disease]. / Ataxia cerebelosa mioclónica progresiva como manifestación de la enfermedad de Creutzfeldt-Jakob.

INTRODUCTION: Progressive myoclonic cerebellar ataxia is a clinical entity with an important spectrum of possible diagnoses that requires a complex and exhaustive differential diagnosis. CASE REPORT: A 53-year-old male patient with no relevant medical history who was admitted to hospital because of an unstable gait, together with mild bilateral dysymmetry and the progressive and insidious widening of the base of support. The patient's symptoms then became more pronounced and included myoclonus and a deterioration of the higher functions. The patient died four months after the onset of the symptoms. The explorations that were conducted included a pathological study of the brain, which confirmed the diagnosis of classical spongiform encephalopathy (Creutzfeldt-Jakob disease). CONCLUSIONS: Creutzfeldt-Jakob disease must be included in the differential diagnosis of progressive cerebellar ataxias.

[Study into what the population knows about strokes and vascular risk factors]. / Estudio sobre el conocimiento de la población acerca del ictus y de los factores de riesgo vascular.

INTRODUCTION: The application of treatments in the acute phase of a stroke makes it necessary to reduce as far as possible the time required for patients to reach hospital. One of the factors associated with delayed care is the population's scant knowledge about the disease. SUBJECTS AND METHODS: Our aim is to analyse what the population knows about strokes and vascular risk factors, as well as their attitude towards it. To do so, we interviewed both patients who visited neurology departments and their relatives. RESULTS: Altogether 386 interviews were carried out, with 158 females and 228 males and a mean age of 52 years. Two hundred and thirty subjects (59%) were unfamiliar with the Spanish term ictus, although they had heard of others, such as infarto or embolia. One hundred and seventy-five (45%) did not know any symptoms of a completed stroke, or answered incorrectly. The most widely recognised symptom (86%) is (partial) paralysis in one side of the body. Almost a third (32%) of the interviewees would not take a correct attitude if faced with symptoms of a stroke and if it were a transient ischaemic attack, 25% would not think it was serious. One hundred and ninety-eight (51%) did not know any vascular risk factors. Of those who answered correctly, the most frequently named factor is arterial hypertension. The factors related independently with a better knowledge of the disease are a high educational level and being young. CONCLUSIONS: The general population knows little about strokes and vascular risk factors. Information campaigns are needed to improve the population's attitude towards this disease.

[Ischaemic stroke in a patient with giant aneurysms in the internal carotid]. / Ictus isquémico en un paciente conaneurismas gigantes de carótida interna.

INTRODUCTION: Presentation of signs and symptoms of haemorrhage and/or ischaemia associated to an intracranial dissecting aneurysm is quite frequent. CASE REPORT: A 77-year-old male with a history of a stroke probably due to a cardioembolic causation, and consequently anticoagulation therapy was established. Ten years later, a tomography scan performed because of persistent headaches revealed the presence of fusiform mirror aneurysms in both supraclinoid carotids and early stages of development in the two middle cerebral arteries with predominance of the left-hand side. The anticoagulation therapy was withdrawn. Said aneurysmal alterations did not exist in the previous study, and so they are thought to have originated due to spontaneous dissection. A month later the patient suffered a stroke in the territory of the right middle cerebral artery, caused by partial occlusion of the aneurysm by a thrombus that gave rise to turbulent flow; distal micro-embolisms were also detected in the right middle cerebral artery. Our aetiological hypothesis, in view of the way events progressed, is an arterio-arterial embolism from the aneurysmal thrombus. Supported by data from the specialised literature available on the matter, we decided to implement surgical treatment, although this possibility was rejected by the family; the decision was thus taken to establish an antiaggregating treatment regimen and follow-up. CONCLUSIONS: Giant aneurysms are a potential source of haemorrhagic events, but we must not forget that secondary ischaemic events may also appear; more especially, the aneurysmal lumen can become partially occluded by thrombi and these then become the focus point for embolic events. A complete neuro-ultrasonographic study would be a very appropriate option with which to tailor the therapeutic decision to each patient.

[Contribution of the set-test to the diagnosis of Alzheimer's disease]. / Aportaciones del set-test al diagnóstico de la enfermedad de Alzheimer.

The categoric verbal fluency (colors, animals, fruits and cities) was prospectively evaluated with the set-test in a group of 71 patients with dementia (Alzheimer's disease 38, vascular dementia 19, mixed dementia 2, other dementias 12). In all types of dementia, greater decreases in the categoric verbal fluency were found with greater degrees of severity. However, for equal degree of deterioration, patients with Alzheimer's disease had the smallest scores in the set-test as compared with other types of dementia. 90% of the percentile distribution of patients with Alzheimer's disease had set-test scores below 25. The hypothesis that a set-test score lower than 25 is diagnostic for Alzheimer's disease has a 87% sensitivity and a 67% specificity. It is concluded that in patients with criteria of probable Alzheimer's disease a set-test score lower than 25 supports the diagnosis.

[Giant aneurysms of the basilar artery. Six cases]. / Aneurismas gigantes del tronco basilar. A propósito de 6 casos.

The authors describe 6 cases of giant aneurysm of the basilar artery diagnosed in the Neurology Department of the Hospital Clínico Universitario of Zaragoza over the last 8 years by cerebral CT, arteriography and NMR with the clinical, diagnostic, and evolutive features of the same being reported. In 2 cases the clinical manifestations initiated as subarachnoid hemorrhage, in another two as AIT, in the fifth case as cerebral infarction and in the sixth case with pseudotumoral clinical manifestations with a necropsy study being available in the latter case. Three of the 6 patients died and of the remaining three only one was, successfully operated on. Anatomopathologic, clinical, therapeutic and diagnostic aspects of giant aneurysms of the basilar trunk are also reviewed with NMR being recommended as the technique of choice in diagnosis.

[The set-test for diagnosis of dementia]. / El set-test en el diagnóstico de la demencia.

The set-test evaluates the verbal fluency in 4 categories: colors, animals, fruits and cities. It has been proposed as a diagnostic aid in dementia in elderly patients. Therefore, we have evaluated its yield in a normative control reference group of 79 individuals and in 56 patients with possible dementia. It had a positive correlation of 73 with minimental test. The cut-off value was 29 in adults an 27 in elderly people. A lower score is indicative of dementia. Sensitivity was 79% and specificity 82%, with 20% of incorrectly classified patients. Considering the short duration of the test, we think that it is very useful for the evaluation of patients with dementia.

[Magnetic resonance in Hallevorden-Spatz disease]. / Resonancia magnética en la enfermedad de Hallevorden-Spatz.

The Hallevorden-Spatz disease should be suspected in children or young people with motor, pyramidal or extrapyramidal symptomatology and deterioration of superior functions specially upon the existence of family history. Pathognomonic diagnosis may only be obtained by necropsy but magnetic resonance images, although not pathognomic, are sufficiently characteristics to strongly support the diagnosis. Four cases of Hallevorden-Spatz disease are presented with special emphasis on the MR images characteristic of this disease.

Diplejia facial: variante regional del síndrome de Guillian-Barré / Facial diplejia: a regional variant of Guillian-Barre syndrome

Diplejia facial es una manifestación clínica poco frecuente que puede presentarse como consecuencia de múltiples etiologías. Presentamos un paciente con una infección periodontal crónica que sufrió de forma brusca una parálisis facial inicialmente izquierda, que rápidamente se hizo bilateral. La aparición posterior de parestesias distales en extremidad superior derecha e hiporreflexia, junto con la disociación albúmino-citológica en el LCR, permitieron establecer el diagnóstico de variante regional del Síndrome de Guillian-Barré (SGB). La diplejia facial es idiopática en el 25% de casos, siendo la causa más frecuente. Sin embargo, existe un elevado porcentaje de casos secundarios a múltiples etiologías como el SGB donde es frecuente la afectación del nervio facial habitualmente asociado a otros trastornos motores siendo infrecuente la diplejia facial aislada. Concluimos la necesidad de sospecha clínica para realización de punción lumbar y RNM para descartar procesos neoplásicos y poder determinar la etiología responsable para establecer un adecuado abordaje terapéutico

Facial palsy is a uncommon clinical manifestation that it can be caused by different ethiologies. We show a patient with a chronic periodontal disease who presented a sudden facial palsy, initially in left-face but it becomes bilateral quickly. In few days he associated paresthesias in his right arm and hiporreflexia. These clinical findings with albumin-citological dissociation, they had allowed to establish the diagnosis of regional variant of Guillain-Barré Syndrome (SGB). Facial diplejia is an idiopathic manifestation in 25% of patients, and this is the most common cause. However, facial diplejia can be secondary to many ethiologies as SGB. The affectation of facial nerve associated to other motor symptoms in SGB is frequent, but it is not frequent the presentation as facial diplejia alone. In conclusion, it is necessary a high clinical suspicion to do a lumbar punction (PL) and MRI to reject neoplasic pathologies and to obtain a diagnosis and an adequatted treatment