[Temporal orientation and cognitive impairment]. / Orientacion en el tiempo y deterioro cognitivo.

INTRODUCTION: Temporal orientation is a component of most screening tests for diagnosing cognitive impairment. Correct temporal orientation involves activating both semantic information (concepts of the calendar date) and episodic information (remembering the current date). AIMS: The aim of this study was to assess the diagnostic usefulness of a technique for evaluating temporal orientation, which was open-ended, and scoring the semantic and episodic information thus generated (0-10 points). SUBJECTS AND METHODS: A total of 24 subjects without impairment, 77 patients with mild cognitive impairment (MCI) and 62 patients with dementia were evaluated by means of a 30-point mini-mental/mini-examination, semantic verbal fluency test, global deterioration scale, mini-mental-type temporal orientation and open-ended temporal orientation tests. The areas under the curve (aROC), sensitivity and specificity for dementia and cognitive impairment at any degree (MCI and dementia) were analysed. RESULTS: Open-ended temporal orientation presented a greater area under the curve (aROC: 0.90) for discrimination between patients with dementia and without dementia (MCI and without impairment) and an aROC of 0.83 for discrimination between patients with MCI or dementia and without impairment. For dementia, with a cut-off point equal to or below 6, sensitivity was 0.96 and specificity was 0.68, and for MCI with dementia, with a cut-off point equal to or below 7, sensitivity was 0.72 and specificity was 0.92. CONCLUSIONS: The usefulness, conciseness and strategic position of this technique in examining mental status make it suitable as an instrument for screening for cognitive impairment. It has a high level of sensitivity with low specificity for dementia and low sensitivity with high specificity for any degree of impairment.

[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.

[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.

[A study of the risk factors in transient global amnesia and its differentiation from a transient ischemic attack]. / Estudio de los factores de riesgo en la amnesia global transitoria y su diferenciación del accidente isquémico transitorio

INTRODUCTION: Transient global amnesia (TGA) is a clinical condition that has been described in many studies, but its pathophysiology is not fully understood. In recent years the theory of valvular insufficiency in the jugular vein has been added to the classical hypotheses that link it to migraine, to epilepsy and -the most widely accepted- to transient ischemic attacks (TIA), although the real origin of the condition has still not been determined. PATIENTS AND METHODS: In a retrospective study we compared 131 patients diagnosed with TGA between 1993 and 2004 with 262 patients who were diagnosed as having TIA over the same period. RESULTS: Mean age was 65.94 years in TGA versus 71.11 years in the case of TIA. There was a higher rate of arterial hypertension among the patients with TGA and diabetes mellitus was more frequent among those with TIA (p<0.05 in both cases). Emboligenic heart disease was scarce among patients with TGA. The number of patients with a history of ischaemic heart disease and a history and the development of cerebrovascular diseases was greater among those with TIA than in cases of TGA (p<0.05). The TGA recurrence rate was 12%. The percentage of pathological findings in the CAT brain scan was higher in patients with TIA (p<0.05). There are no significant differences between patients with TGA and TIA as far as treatment on hospital discharge is concerned. CONCLUSIONS: TGA does not seem to be a symptom of an arteriosclerotic pathology nor does it appear to offer a higher risk of heart or cerebrovascular disease and, therefore, antiaggregating therapy would not be indicated in such cases.

[Differential diagnosis of intracranial calcifications]. / Diagnostico diferencial de las calcificaciones intracraneales.

INTRODUCTION: Intracranial calcifications are a frequent chance finding in many neuroimaging tests. We report two clinical cases with intracranial calcifications as a common feature, but which had very different aetiologies and presenting symptoms. CASE REPORTS: The first case involved a 51-year-old male with a single tonic-clonic convulsive seizure associated to hypocalcemia secondary to hypoparathyroidism who presented extensive brain calcifications affecting the dentate nuclei, pons, periependymal and basal ganglia, and whose convulsive attacks remitted once normal Ca2+ values were restored. The second case was a 25-year-old female patient whose convulsive seizures were yet another symptom of Fahr's syndrome, which was associated to spastic paralysis, athetosis, mental retardation and occasionally hypoplasia of the optic nerve; the calcifications were located in the grey nuclei of the cerebrum and cerebellum. CONCLUSIONS: A correct history and physical and neurological examination must be carried out, and a detailed study of Ca2+/ P+ and hormonal metabolism is also required. A CAT scan is the preferred neuroimaging technique to achieve a proper differential diagnosis and to decide on the therapeutic approach that is best suited to the pathophysiological mechanisms that lead to the formation of these calcium deposits.

[Amnesic stroke caused by hippocampal infarction]. / Ictus amnésico por infarto hipocámpo.

INTRODUCTION: The term amnesic stroke is used to describe a condition in which the dominant symptom is a relatively persistent acute amnesia of a vascular origin. It may appear in cases of lesions in the posterior cerebral artery (PCA), thalamic arteries and anterior choroidal artery, and clinical reports describing this condition are scarce. CASE REPORT: We describe the case of a 77-year-old male with vascular risk factors who presented sudden onset mnemonic deficit that affected basic activities of daily living, and apathy. Examination of the patient revealed an anterograde episodic amnesia with an inability to retain new information and short-term memory was also clearly affected, although access to old memories remained relatively intact. There was no other sensory-motor focus. A CAT scan showed a right-side sylvian infarction and hypodense areas in both hippocampuses. The neuropsychological evaluation revealed global amnesia: CME (in Spanish, MEC) 17/30; working memory with CME 7/27; SVF (animals): 7; the 7-minute test (visual/verbal memory: free recall 2/16, facilitated 9/16); clock test: 3/9. Later progression was good, with recovery of short-term memory and the capacity to retain concepts, and the patient was again able to perform the activities he previously carried out. CONCLUSIONS: There are three amnesic stroke syndromes with different semiological characteristics, depending on the vascular territory, that is, the PCA, thalamic arteries and anterior choroidal artery. In this case, the most striking points are the more intense disorders affecting short-term memory and retention, with relative conservation of long-term memory, and the location of the stroke in the hippocampus. Its good outcome allows it to be distinguished from vascular dementia.

[Clinical, electrophysiological and immunological evaluation of the response to treatment with intravenous immunoglobulins in several immune-mediated neuropathies]. / Valoración clínica, electrofisiológica e inmunológica de la respuesta al tratamiento con inmunoglobulinas intravenosas en distinta neuropatías inmunomediadas.

INTRODUCTION AND AIMS: Chronic immune-mediated neuropathies are characterised by their being predominantly demyelinating, by being associated to specific antibodies and by their response to immunotherapy. We evaluated the clinical, electrophysiological and immunological responses following treatment with intravenous immunoglobulins (IVIg) in different clinical forms. CASE REPORTS: We report on three patients with: 1. Multifocal motor neuropathy, 2. Multifocal sensory-motor neuropathy, and 3. Hypertrophic brachial plexopathy, who were evaluated before and 14 days after treatment with IVIg by means of clinical scales (MRC, Rankin), electrophysiological studies (ENG-EMG), and antibody (antiganglioside) determination. The three patients showed clinical improvement (> 20% MRC, > 1 Rankin) between the 4th and 7th day after the infusion, and this was maintained for 4-16 weeks. The ENG in cases 1 and 2 revealed conduction block (CB) in some nerves, lowered amplitude in others, and signs of demyelination. Following treatment, the CB disappeared in some nerves, while in others there was an improvement in the distal amplitude (distal block), but with no correlative improvement in the proximal amplitude, revealing new CBs that had previously gone undetected. The sum of post-treatment amplitudes improved in cases 1 and 2. In case 3, we were unable to demonstrate the existence of CBs, although we believe that they did exist proximally, and we found indirect signs of CB in EMG. The three cases had increased antiganglioside IgM antibodies levels, which did not undergo any significant variations. CONCLUSIONS: A 'consistent' clinical improvement was observed following treatment with IVIg. There was no correlation between the electrophysiological response (although apparent) and the degree of clinical response. CBs exist at different levels in the same nerve, which can be revealed after treatment. Following therapy, antiganglioside antibody levels remain high.

[The supermarket test: preliminary normative data in our milieu]. / Test del supermercado: datos normativos preliminares en nuestro medio.

INTRODUCTION: Semantic verbal fluency (SVF) tests are often used in basic neuropsychological evaluation. They are not time consuming and are easy to apply, but the normative data have been validated mainly for the Anglo-Saxon population, which can lead us to make mistakes in classifying normality. AIMS: To evaluate the category 'things you can buy at a supermarket' as a task for exploring SVF applied to a Castilian-speaking population of Spaniards with the aim of conducting a pilot normative test in our milieu. SUBJECTS AND METHODS: The 'things you can buy at a supermarket' task was applied to a sample of 139 healthy subjects without cognitive impairment, whose mother tongue is Spanish. The subjects were subclassified according to their level of schooling in years, age groups and sex. RESULTS: Total mean production (1 minute) = 20.1 +/- 8. No differences were seen in the comparative analysis according to sexes. By age: < 50 years = 33 +/- 6; 50-59 = 24.6 +/- 6; 60-69 = 16.5 +/- 5; 70-79 = 15.5 +/- 6; > 79 years = 13.5 +/- 6. By years of schooling: < 10 years = 19 +/- 6; > 10 years = 29 +/- 5. CONCLUSIONS: Mean output of words is 20 in one minute, with a percentile distribution where the deficit criterion (p10) would be in an output below 10 words. Overall, greater output is observed in the first half minute. There are a number of socio-demographic factors, such as age and mean number of years of schooling, that have been proved statistically to exert an influence on semantic capacity in this test. No differences were observed according to sex. We present the mean results, as well as the overall percentile distribution and results according to age and schooling, because we think they can serve as preliminary normative data in our milieu.

[Stroke as the first manifestation of a concealed pancreatic neoplasia]. / Ictus como primera manifestación de una neoplasia pancreática oculta.

INTRODUCTION: Stroke is very seldom seen as the first manifestation of a neoplasia. The mechanisms that are most frequently involved in its pathogenesis are Trousseau's syndrome, haematological disorders such as disseminated intravascular coagulation or thrombocytosis, and the release of mucin by the tumour. Secondarily, non bacterial thrombotic endocarditis, septic or neoplastic cell embolism, venous sinus thrombosis and atherosclerosis secondary to radiotherapy have been reported. CASE REPORTS: Case 1: a 27-year-old male who presented a stroke in the right thalamus with a torpid progression and digestive complications; neuroimaging studies revealed unknown neoplasia of the head of the pancreas. Case 2: a 53-year-old male with repeated stroke (left sylvian and right occipitotemporal) and progressive neurological deterioration secondary to disseminated neoplastic disease, which had its origins in the body and tail of the pancreas. In both cases all the diagnostic explorations carried out in an attempt to determine the aetiopathogenesis of the stroke were negative. The post mortem study in the second patient revealed the existence of a non bacterial thrombotic endocarditis, which was taken as being one of the mechanisms involved. CONCLUSIONS: The pathogenic mechanisms of cerebral ischemia associated to neoplasia are discussed and the primary and secondary forms are differentiated. We highlight the idea that when dealing with a stroke with an unknown aetiology and a torpid progression, the neurologist must consider the existence of a concealed neoplasia with stroke as its first manifestation.

[Migraine with pleocytosis: a case of atypical progression]. / Migraña con pleocitosis: un caso de evolucion atipica.

AIMS: Migraine with cerebrospinal fluid pleocytosis is a clinical pattern consisting in intermittent headaches accompanied by episodes of transient neurological deficit and lymphocytic pleocytosis, which last for varying lengths of time and have a spontaneous resolution. We describe the case of migraine with pleocytosis (MP) with atypical progression that responded well to corticoids. CASE REPORT: A 27 year old male with no familial or personal history of migraine who began with bouts of intense headaches that were preceded by neurological deficit (dysphasia and hemiparesis of the right hand side) lasting varying amounts of time (including hours). The serial study of cerebrospinal fluid (CSF) revealed a notable degree of lymphocytosis (400/mm3) with increased intracranial pressure (ICP) (> 30 mmHg). The other explorations that were performed (microbiological study of CSF and blood, humoral and cellular immunity, MR angiography and thyroid hormones, among others) were all normal. The neurological exploration carried out in the following days revealed a mild paresis of the right lateral rectus muscle, which was attributed to the intracranial hypertension and which clearly disabled the patient. As a consequence of the recurrence of the seizures and the long lasting increase in ICP, therapy was begun with 1 mg/kg/day of prednisone, at a descending rate; clinical remission and normalisation of the CSF was achieved within a period of two months. DISCUSSION: We describe this case of MP because of how atypical the progression was (paresis of the right abducent nerve secondary to the increase in ICP) and the possibility of associating corticoids in cases in which the benignity of the entity is in doubt.

[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.

[Pharmacological treatment of Korsakoff's psychosis: a review of the literature and experience in two cases]. / Tratamiento farmacológico de la psicosis de Korsakoff: revisión de la bibliografía y experiencia en dos casos.

INTRODUCTION: Korsakoff s psychosis (KP) is a relatively frequent pathological condition in our community that has been infradiagnosed. The most common cause is chronic alcohol consumption, although it can be brought about by other aetiologies accompanied by vitamin deficiencies. The lack of thiamine entails an alteration in the synthesis of neurotransmitters, which provides the neurochemical foundation for the specific cognitive impairment that defines the syndrome. AIMS: To evaluate the application of pharmacological treatments, in accordance with the neurochemical disorders described in the literature, and report our experience in two cases treated with anticholinesterases. CASE REPORTS: 1) Female aged 47, with a history of addiction to alcohol. Following Wernicke s encephalopathy, which improved with parenteral thiamine, she presented a memory disorder compatible with KP. After two months treatment with donepezil, a cognitive improvement was observed in the neuropsychological tests. 2) Male aged 77, who presented KP a month after being diagnosed and treated for a post encephalitic vasculitis caused be varicella zoster virus. His cognitive and functional condition improved after 3 months treatment with donepezil. DISCUSSION AND CONCLUSIONS: There are not enough studies in the literature with representative samples that consider the effects of thiamine or of other forms of treatment on cognitive impairment in KP. Noradrenaline, serotonin, glutamate and acetylcholine have been proposed in the pathogeny of the syndrome. Based on experiences gained in cholinergic disorders, two cases responded to treatment with donepezil. Effective treatment must be based on a combination of aetiological and pharmacological treatment, and cognitive rehabilitation.

[Tuberculous meningioma en plaque without extracerebral tuberculosis. A case report]. / Tuberculoma meníngeo en placa sin tuberculosis extracerebral. A propósito de un caso.

INTRODUCTION: Tuberculosis continues to be an intensly extended disease in under developed countries in immunodepressed and immunocompetent persons. In the central nervous system tuberculous lesions are the most frequent cause of space occupying lesions. CLINICAL CASE: We report the case of a young, immunocompetent woman from Guinea, currently living in Spain. Following a seizure, neuro imaging showed there to be a right frontal space occupying lesion. The characteristics of the MR images obtained were fundamental to the determination of the meningeal site of the lesion en plaque and to suggest the aetiology as being an infectious inflammatory process, although a meningioma could not be ruled out. The absence of signs of extracerebral disease, the localization and distribution of the lesion and lack of short term response to medical treatment, which covered the different diagnostic possibilities, made it necessary to do a diagnostic meningeal biopsy. CONCLUSIONS: MR imaging is a great help in the diagnosis of tuberculous lesions of the central nervous system. The differential diagnosis between tuberculous pachymeningitis and meningioma, both in the form of plaques, is extremely difficult without obtaining a biopsy specimen. The finding of epithelioid granulomas with caseous necrosis and/or acid alcohol fast bacilli in the biopsy specimen is diagnostic of tuberculosis. A single meningeal lesion situated in the cortex but with no associated extracerebral disease causes considerable difficulty in diagnosis. Tuberculosis must always be remembered in view of the increasing number of immigrants from under developed countries.

[Neuropsychological changes in epilepsy]. / Alteraciones neuropsicológicas en epilepsia.

OBJECTIVE: The neuropsychological assessment of the epileptic patient is a very important aspect of diagnosis and treatment. It may be used to contribute to localization of the hemisphere involved in the seizures, differentiate situations of anxiety or depression or when planning treatment for rehabilitation. We review the different aspects of neuropsychological changes in patients with epilepsy. DEVELOPMENT: Firstly we review the different tests used in the neuropsychological assessment of epilepsy. Dodrill's neuropsychological battery of tests, in which the patients score less than the controls, is the most commonly used. We then evaluate and study the so-called 'transient cognitive disorder'. We also study memory problems in epilepsy. There may be episodes of seizures with amnesic features ('amnesic epileptic seizures'). Finally, the possibility of neuropsychological dysfunction secondary to antiepileptic drugs should always be considered. CONCLUSIONS: Epileptic patients have lower scores than persons taken as controls for the results of various neuropsychological tests, although there is less difference between the two groups when the patient group is made up of persons with a normal intelligence quotient. Transient cognitive involvement is common in epileptics and may cause underachievement at school or psychological problems. Memory disorders, particularly subjective, are common in epileptics, although neuropsychological tests other than those generally used may be necessary to evaluate this. It is possible that such memory disorders, if occurring as seizures, may be due to amnesic partial crises, which should always be differentiated from the diagnosis of transient global amnesia. Almost all antiepileptic drugs can cause negative neuropsychological effects, especially the benzodiazepines and barbiturates.

[Peripheral facial paralysis: etiology, diagnosis and treatment]. / Parálisis facial periférica: etiología, diagnóstico y tratamiento.

OBJECTIVE: Peripheral facial paralysis is one of the commonest mononeuropathies. The frequency of idiopathic peripheral paralysis or Bell's palsy varies between 62% and 93% of all cases, with an incidence of between 14 and 25 cases per 100,000 inhabitants per year. However, in spite of its relative frequency in the population, there is still no definite unanimously agreed strategy currently used by neurologists. Although its course is relatively benign, up to 16% of the patients show moderate or severe sequelae according to follow-up data in 1,011 untreated patients. In this article we review the aetiology, diagnostic methods and therapeutical strategies at the present time. DEVELOPMENT: The topographic diagnosis of the lesion is based on the symptoms associated with the paralysis. The neurophysiological studies (trigeminofacial reflection and electroneurogram), usually done after the first week are prognoses that permit differentiation of the degree of the lesion (neuropraxis, axonotmesis or neurotmesis). In spite of recent trials with combined therapy (acyclovir and steroids) the most generally accepted treatment at present is still prednisose at a dose of 1 mg/kg/day. CONCLUSIONS: Peripheral facial paralysis is a common condition, usually with no apparent cause and an eminently clinical diagnosis. The electrophysiological studies determine the prognosis. Currently the standard treatment is still oral prednisone.