Rates of verbal fluency decline in the longitudinal assessment of Alzheimer's disease.
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Alzheimer Disease↗
Biomedical subjects
S Santos
Publications and source records attributed to S Santos.
[Neurological alterations related to Crohn's disease].
INTRODUCTION: The incidence of alterations of the central and/or peripheral nervous system in a patient with Crohn s disease (EC) is 33.2%. Casual association may occur in 19.3% of the cases. The disorder of the nervous system may precede the diagnosis of EC. DEVELOPMENT: We review the main neurological complications of EC. The predominant cerebral vascular pathology is arterial although cases of venous thrombosis have been reported. One of the mechanisms involved is the state of hypercoagulability secondary to thrombocytosis and increase in the factors V, V111 and fibrinogen. In other cases there was confirmation of the presence of antiphospholipid antibodies and lupus anticoagulant, and therefore of an autoimmune mechanisms being involved. Other neurological features include peripheral neuropathy (axonal, demyelinating and autonomic), myopathies, pseudotumor cerebri, papilloedema, psychiatric disorders (anxiety, phobias, depression) and association with syndromes such as multiple sclerosis, Cogan s syndrome, Melkersson Rosenthal syndrome, connective tissue disorders and vasculitis. CONCLUSION: As well as hypercoagulability being one of the pathogenic mechanisms of cerebral ischaemia, there is alteration of humoral and cellular immunity in patients with EC. This justifies this and other neurological manifestations, and explains its association with other immunity disorders.
Antiphospholipid Syndrome↗
[Working memory and complex activities of everyday life in the initial stages of Alzheimer's disease].
INTRODUCTION: Evaluation of the working memory (WM) permits analysis of the degree of anterograde amnesia and fixation deficit present in patients with Alzheimer s disease (AD). Complex activities of everyday life (AEL) are also affected early. There is a close relation between the degree of cognitive deterioration and loss of functional competence in AEL. OBJECTIVE: To analyze the degree of memory loss and performance in carrying out complex AEL in patients with AD and mild cognitive disorder. PATIENTS AND METHODS: Evaluation of functional disorder, using Pfeffer s functional activity questionnaire (FAQ), and cognitive evaluation, using the WM examination protocol in a group of 36 patients. RESULTS: In the total MT (4.2+/-5.7) there were significant differences between patients with age associated memory impairment (AAMI) (11+/-7) and possible EA (3.2+/-4.6) and between AAMI and probable AD (2.8+/-4.8). The functional evaluation of the AEL (FAQ) gave a mean of 8.5+/-7.1. There were significant differences between probable AD (11+/-8) and possible EA (6+/-4) with the group AAMI 1+/-1.8. There was significant correlation between FAQ and total WM ( 0.70, p= 0.0001). It was found that patients with high FAQ scores had lower total WM scores. CONCLUSION: FAQ and WM have high correlation and are useful instruments for the evaluation of patients with cognitive deterioration at the initial stages.
Activities of Daily Living↗
[Clinical diagnostic of brain death and transcranial Doppler, looking for middle cerebral arteries and intracranial vertebral arteries. Agreement with scintigraphic techniques].
INTRODUCTION: The Real Ordinance 2070/1999 meant an important modification in the legislation, when including transcranial Doppler (TCD) in explorations to confirm the clinical diagnosis of brain death (BD). Habitually for their employment in the diagnosis of BD, we look for blood flow signal from the middle cerebral arteries (MCA) and the basilar artery (BA). OBJECTIVES: To check the effectiveness of the TCD like test of BD, looking for both middle cerebral arteries (MCA) and both intracranial vertebral arteries (VA), instead of the BA, and taking as Gold Standard cerebral scintigraphic techniques. PATIENTS AND METHODS: . We present 25 patients diagnosed clinically with BD; on these TCD was carried out to confirm BD. Later on we proceeded to carry out cerebral scintigraphic techniques in all these cases. As statistical tool the test of c2 is used with confidence interval of 95%. RESULTS: In 24 of the 25 cases, the TCD was effective in confirming the diagnosis of BD. In the remaining patient, a false positive result was obtained, since the TCD didn't reveal flow in the infratentorial compartment, as contrary to the cerebral scintigraphic techniques which showed the presence of residual flow at this level; this residual flow disappeared in 36 hours. This patient was hemodynamically unstable during TCD exploration. CONCLUSIONS: In our results the TCD obtains a reliability of 100% when confirming the absence of blood flow in the supratentorial compartment; nevertheless the false positive result obtained at the infratentorial level, warns us to be cautious in accepting the flow from the VA as a test of absence of flow at the infratentorial compartment, especially in those patients with hemodynamic instability.
Adolescent↗
[Pseudotumor cerebri: analysis of our cases and review of the literature].
INTRODUCTION: Pseudotumor cerebri (PC) is a complex syndrome characterized by increased intracranial pressure in the absence of any space occupying lesion, usually self limiting, but often relapsing. Diagnosis is based on a record of intracranial pressure of over a limit of 250 mmH2O. Morbidity is basically due to possible loss of vision associated with atrophy of the optic nerve. When the aetiology is unknown this clinical condition is known as idiopathic intracranial hypertension. PATIENTS AND METHODS: We describe the principal characteristics of a series of patients who were admitted to our department with the diagnosis of PC and review the relevant literature. RESULTS: Altogether 14 patients (12 women and 2 men) were admitted with the diagnosis of PC. In 12 patients the disorder presented with headache, with or without associated symptoms of disorders of vision. The main visual symptoms were transient darkening of vision, scotomas, photopsias and reduction in visual acuity. The headache was holocranial, continuous and well tolerated or alternatively uni or bilateral, pulsatile, moderate, with or without photophobia, and with or without nausea and vomiting. Bilateral papilloedema was seen in all patients. In one case there was also bilateral facial palsy and neck rigidity. In four cases visual field measurement were abnormal (inferoneasal scotoma, bilateral concentric reduction). Progress was poor in only one case. CONCLUSIONS: In spite of being theoretically benign, and there being many forms of treatment, PC may cause considerable morbidity of vision. Therefore, early diagnosis and close follow up attention is essential.
Acetazolamide↗
[Pure lower motoneurone syndrome in a series of six patients].
INTRODUCTION: Lower motoneurone syndromes (SMNI) have been defined by their clinical pattern, anomalies in nerve conduction and the presence of antiglycolipid antibodies. The possible response to immunotherapy is another characteristic which makes these syndromes interesting. PATIENTS AND METHODS: We studied the clinical and electrophysiological data, clinical progress and response to treatment of a series of six patients, with a pure lower motoneurone syndrome, with a follow up period of between 5 and 13 years. CONCLUSIONS: The clinical phenotype of our patients was: male, middle aged, the clinical topography showed only lower motoneurone involvement, predominantly or exclusively limited to the upper limbs, asymmetrical, distal rather than proximal, with a chronic course progressing initially but later becoming stable. From the electrophysiological point of view some of our cases were of SMNI. In these the conduction block was not permanent although there were signs of axon loss and multifocal demyelination. Response to treatment with intravenous immunoglobulins was seen in patients with anti GMI antibodies of IgM type and recent signs of clinical deterioration, whether there were conduction blocks present or not. In the cases which responded to this treatment there was tolerance and dependence following repeated transfusions of IV IgG.
Adult↗
[Cerebral gliomatosis. A report of three cases and review of the literature].
INTRODUCTION: Cerebral gliomatosis is an infiltrating, non destructive process of glial type, with a diffuse growth pattern and great clinical variation in the initial symptoms. Confirmation of the diagnosis requires histological study of the lesion showing infiltration by well differentiated mature astrocytes (atypical forms are rare). We report three cases diagnosed by our department over the past five years. CASE REPORTS: Case1. A 64 year old man presented with olfactory crises and right homonymous hemianopsia. On cerebral MR there was an extensive left occipital cortico subcortical lesion infiltrating the genum of the corpus callosum, with irregular uptake of contrast material. CASE REPORT 2: A 68 year old woman was admitted complaining of paresia of her right arm which had worsened over the past two months. On MR there was an extensive left paraventricular subcortical lesion, involving the anterior temporal and capsulo lenticular regions. Case 3. A 63 year old woman complained of staggering gait and headache. On examination she had a right facial palsy and intention tremor of her right arm. All the patients died. In all cases stereotactic biopsy confirmed the diagnosis of cerebral gliomatosis. CONCLUSIONS: Cerebral gliomatosis is an infiltrating glial disorder with a poor prognosis. The clinical diagnosis is suggested on cerebral MR studies and confirmed on histological studies.
Aged↗
[Opercular syndrome of vascular aetiology].
INTRODUCTION: Facio linguo masticatory paralysis resulting from bilateral lesion of the anterior opercular region, known today as Foix Chavany Marie syndrome, appears very frequently in adult patients as a consequence of generally ischemic vascular lesions, which directly affect the Rolandic opercula or the subcortical area surrounding them. However, forms that are secondary to infections, neoplasias and even unilateral lesions have been reported. CASE REPORT: Female patient, aged 70, with unknown auricular fibrillation, which started suddenly with right faciobrachial paresis secondary to a left striated ischemic infarction. Approximately 24 hours after beginning intravenous heparinization, the patient presents anarthria, facial hypomimia without asymmetries, dysphagia above all for liquids and bilateral lingual paresis. Extraoccular movements, blinking and corneal reflex were normal. Understanding of spoken and written language was maintained and there were no apraxias or agnosias. Neurological exploration was compatible with an opercular syndrome (OS). Urgent brain CT revealed the existence of a right cortiico subcortical temporal haemorrhage, in addition to the prior contralateral ischemia. The association of both lesions, ischemic and haemorrhagic, mirrored, justified all the symptomatology. DISCUSSION: Anterior OS is characterised by a bilateral voluntary central paresis of the muscles innervated by the 5th, 7th, 9th, 10th and 12th cranial pairs with preservation of emotional or automatic mobility. Underlying mechanisms are also discussed.
Aged↗
["Household items" : an alternative task to "animals" in the assessment of semantic verbal fluency: a validation study].
INTRODUCTION: The semantic verbal fluency (SVF) is usually evaluated with the task animals in 1 minute . This is a test widely applied in the screening of cognitive impairment. In our best knowledge there are not other alternatives categories for this task validated in our environment (Spanish as mother tongue, Spain). OBJECTIVE: To validate the category households items as an alternative parallel task to animals in the assessment of SVF. PATIENTS AND METHODS: Prospective assessment of two categories (animals and household items) in two groups: normal controls and patients with Alzheimer s disease (AD). RESULTS CONTROL GROUP: n= 22 (13 M, 9 W); age: 73 5 years, education: 12 5 years; MMSE MEC 35: 30 4; Animals : 15 4. Percentile distribution 10th: 11; 50th: 15; 90th: 20; household items : 17 4. Percentile distribution: 10th: 12; 50th: 17; 90th: 22. AD group: n= 24 (9 M, 15 W); age: 74 5 years; education: 11 5 years; MMSE MEC 35: 17 8; Animals : 6 3. Percentile distribution: 10th: 2; 50th: 6; 90th: 9; household items : 6 4. Percentile distribution: 10th: 1; 50th: 6; 90th: 11. It is observed a positive correlation between the two categories (Spearman s Rho: 0.83, p< 0.0001). CONCLUSIONS: Both SVF categories had a similar distribution and a high correlation. This findings demonstrated the validity of the household items as an alternative and parallel form to animals in the assessment of SVF in patients with cognitive impairment or dementia.
Aged↗
[Gabapentin in the treatment of tremor].
PATIENTS AND METHODS: We evaluated the efficacy of gabapentin added to basal tremor treatment by means of a 16 month non randomized open study conducted at our Unit on treated and monitored patients. Efficacy was evaluated by means of the following scales: Tremor Scale (TS), Global Disability Examiner Scale (GDE) and Global Disability Patient Scale (GDP). For the GDE and GDP scales we constructed a dichotomic result evaluation variable (improvement vs. non improvement) and adjusted a logistic regression model (independent variables: age, gender, tremor duration and number of antitremor drugs associated with gabapentin). Two multiregression models were adjusted for the TS (12 month score result variable). Model 1: TS (items 1 14) and model 2: TS (items 15 21). INDEPENDENT VARIABLES: age, gender, tremor duration, initial test score and number of antitremor drugs associated with gabapentin. We studied 63 patients aged 59.4 years (SD, 16 years): 34 essential tremor, 16 Parkinson s disease tremor, 10 multiple sclerosis tremor, 4 writing tremor and 3 orthostatic tremor. RESULTS: At 12 months gabapentin improved the clinical results. The largest decrease (absolute terms) was observed in multiple sclerosis tremor, and in percentage terms the largest decrease was in orthostatic tremor. Logistic regression showed that masculine gender and a shorter tremor duration predicted a better result. Multiregression showed association between end and basal score, on the one hand, and a better result in males and shorter tremor duration, on the other
Acetates↗
[Visual neglect secondary to left frontal haemorrhage].
INTRODUCTION: The term visual neglect refers to a lack of attention to visual stimuli coming from the contralateral hemifield. The patient does not seek objects in the abnormal field and often only sees half of a paragraph when reading. Although the right inferior parietal lobe is the most frequently damaged region, the same symptomatology can be due to injury to the frontal lobe, the basal ganglia and the thalamus. CASE REPORT: Female aged 71 with a mitral prosthesis and atrial fibrillation in treatment with dicumarols, who presented a left frontal parenchymatous haemorrhage. The exploration revealed right visual hemineglect without ipsilateral homonymous hemianopsia, associated to a right hemiparesis. DISCUSSION: Even when visual neglect has been described in lesions affecting the parietal lobe, especially on the right hand side, the same symptomatology can be due to injury to the basal ganglia, the thalamus and the frontal lobe. In this last case, it seems that the basis for the pathogenesis lies in the damage done to the underlying white matter, which would disconnect the posterior parietal cortex from the prefrontal cortex.
Aged↗
[Progressive myoclonic cerebellar ataxia as a manifestation of Creutzfeldt-Jakob disease].
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.
Cerebellar Ataxia↗
[Evaluation of patients who do not visit neurology units].
AIMS: Our aim was to study the relation between non-attendance at neurology units and the delay that exists, as well as the demographic factors that may come to bear on non-attendance. PATIENTS AND METHODS: Data on neurology outpatients over the period between November and December 2002 were collected prospectively. Both patients who were referred for evaluation and those on their first programmed visit were included in the study. RESULTS: The total number of subjects was 1,035, with an average age of 55.31 +/- 19.84 (14-96). Of these, 605 were females and 430 were males, with no significant differences in the age of the two groups. 40% of the cases came from rural areas. 237 (22.9%) did not attend the visit. We found a statistically significant relation between age and non-attendance (t=2.67; p=0.008, Student's t). There was also a relation between not going to the visit and the delay that existed (t=5.84; p=0.000, Student's t). Patients who were referred from the Casualty department missed their visit more often those who were sent by their GP (Pi2=2.4; p=0.07, chi2). The date on which they received the appointment was also related to non-attendance (t=2.55; p=0.011, Student's t). CONCLUSIONS: Non-attendance was associated, above all, with the delay, with youth, being sent from casualty and with the length of time since they received notice of the visit.
Adolescent↗
[Stroke as the first manifestation of a concealed pancreatic neoplasia].
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.
Adult↗
[Migraine with pleocytosis: a case of atypical progression].
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.
Abducens Nerve Diseases↗
[Paraproteinemic neuropathies: foundations for a specific therapy related to patients' quality of life].
INTRODUCTION: Monoclonal gammopathy (MG) is reported in 1% of subjects above 50 years of age and in 3% of those above the age of 70. Of all patients with MG, 3% present polyneuropathy (PNP). The abbreviation MGUS (monoclonal gammopathy of undetermined significance) is used to describe a benign proliferation of M component (monoclonal) according to Kyle's criteria, with a potential to be malignant that is indicated by clinical, biochemical and haematological parameters. Aims. The aim of this study was to evaluate the effect of the clinical exacerbation of MGUS associated neuropathy, by referring to the existing literature and to our own experience in order to set out a number of proposals for treatment based on the deterioration of the patient's quality of life. CASE REPORTS: Case 1: a 53 year old female with predominantly sensory sensory motor demyelinating polyradiculoneuropathy, associated to IgG lambda MGUS. Case 2: a 65 year old female with demyelinating sensory motor polyradiculoneuropathy associated to IgM kappa MGUS. In both cases there was a rapidly progressing neurological deterioration and no other data exist to suggest malign lymphoproliferation. CONCLUSIONS: In addition to the treatments established for MG linked PNP, which are based on the modulation of the immune response, it has also been proved that when malign proliferation of plasmacytes exists specific treatment of the gammopathy can significantly stabilise or improve the neuropathic symptoms. We propose gammopathy specific haematological treatment in patients who still meet Kyle's criteria for MGUS with a torpid neurological course and scarce response to therapy with immunomodulators, prior to an important deterioration in the quality of life. We also suggest establishing this rapidly progressing clinical course as a criterion that indicates the process of becoming malign as put forward by Eurelings et al.
Adrenal Cortex Hormones↗
[The supermarket test: preliminary normative data in our milieu].
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.
Age Factors↗
[Quetiapine and ziprasidone in the treatment of the psychotic disorders in Parkinson's disease].
INTRODUCTION: As neurologists we often find it necessary to use antipsychotics in our clinical practice. They are not only used in the treatment of the behavioural disorders accompanying dementias, but also in the psychotic symptoms that frequently appear or complicate the clinical progress of our patients suffering from Parkinson. PATIENTS AND METHODS: Taking into account the numerous side effects of the typical antipsychotic drugs, we began a prospective study on the use of a new antipsychotic agent, quetiapine, in the treatment of 134 patients with Parkinson's disease who presented signs or symptoms of parkinsonian psychosis at some time during the course of their clinical progression; results were analysed at three and six months. At the same time, we review the efficacy of another antipsychotic agent, ziprasidone, which from the chemical point of view fulfils a profile that suits the needs of our patients. At the time of writing this paper we have recorded 43 cases of patients with Parkinson's disease and concomitant parkinsonian psychoses, which responded adequately to treatment without any kind of side effects. RESULTS: The administration of quetiapine in doses of 25-50 mg/day brought about an important improvement in the control over the signs of parkinsonian psychoses in our patients. The same occurred with the administration of 20-40 mg/day of ziprasidone. Both drugs were found to be extremely well tolerated and of great clinical value in the treatment of this clinical entity. CONCLUSIONS: Both quetiapine and ziprasidone are drugs that can be of great value in the treatment of parkinsonian psychoses. In addition to their high degree of clinical effectiveness, they also have a broad safety profile and no side effects.
Aged↗