Search

Introduction: Sleep and circadian rhythm disturbances are common in patients with neurodegenerative diseases such as Huntington's disease (HD). The aim of this study was to evaluate variability in circadian blood pressure (BP) to determine the association between abnormal circadian BP and sleep quality in patients with HD., Methods: Cross-sectional, multicenter study of 38 HD mutation carriers (23 premanifest and 15 early stage patients) who were compared to 38 age- and sex-matched controls. BP was evaluated by ambulatory blood pressure monitoring (ABPM). Based on the percentage decrease in nocturnal BP, subjects were classified as either dippers (≥10%) or non-dippers (<10%). Sleep quality and daytime sleepiness were measured, respectively, using the Pittsburgh Sleep Quality Index (PSQI) and the Epworth Daytime Sleepiness Scale (ESS) and the scores on these indices were correlated with the ABPM findings., Results: Sixty-three percent HD mutation carriers were non-dippers (86.7% of the symptomatic and 47.8% of the premanifest patients) versus 23.7% of controls (p = 0.001). In the HD group, sleep quality was significantly more impaired (PSQI>5) (p = 0.016) with more excessive daytime sleepiness (ESS>9) (p = 0.001) than in the control group. Nocturnal non-dipping was associated with worse sleep quality in patients (p = 0.011) but not in controls., Conclusion: These results show that patients with HD present early disturbances in the circadian rhythm of BP and that this altered nocturnal BP is associated with poor sleep quality. These findings suggest the potential role of subtle hypothalamic dysfunction in this population., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Background: Non-ergoline dopamine agonists (DA) are effective treatments for Parkinson's disease (PD). This review presents the pharmacology, evidence of efficacy and safety profile of pramipexole, ropinirole, and rotigotine, and practical recommendations are given regarding their use in clinical practice., Results: Extended-release formulations of pramipexole and ropinirole and transdermal continuous delivery rotigotine patches are currently available; these may contribute to stabilising of plasma levels. In early PD, the three drugs significantly improve disability scales, delay time to dyskinesia and allow a later introduction of levodopa. In late PD they reduced total 'off'-time, improved Unified Parkinson's Disease Rating Scale (UPDRS) in both 'on' and 'off' state and allowed a reduction in total levodopa dosage. A significant improvement in quality of life scales has also been demonstrated. Extended-release formulations have proved to be non-inferior to the immediate release formulations and are better tolerated (ropinirole). Despite a generally good safety profile, serious adverse events, such as impulse control disorder and sleep attacks, need to be routinely monitored. Although combination therapy has not been addressed in scientific literature, certain combinations, such as apomorphine and another DA, may be helpful. Switching from one DA to another is feasible and safe, although in the first days an overlap of dopaminergic side effects may occur. When treatment with DA is stopped abruptly, dopamine withdrawal syndrome may present. Suspending any DA, especially pramipexole, has been linked to onset of apathy, which may be severe., Conclusions: New non-ergotine DAs are a valuable option for the treatment of both early and late PD. Despite their good safety profile, serious adverse effects may appear; these effects may have a pathoplastic effect on the course of PD and need to be monitored., (Copyright © 2011 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.)

The aims of this study were to assess the peripapillary retinal nerve fiber layer (RNFL) thickness in patients with Parkinson's disease (PD), to determine its correlation with disease severity, and to define a simple biomarker for predicting clinical severity. One hundred two eyes from 52 patients affected by PD were compared with 97 eyes from 50 age-comparable controls. In all patients, peripapillary RNFL thickness was measured by optical coherence tomography (OCT). We used the Unified Parkinson's Disease Rating Scale (UPDRS) total score and measured responses in the on medication state. Eyes from patients with PD had a statistically significant decrease in average peripapillary RNFL thickness compared with control eyes (P < 0.001). This reduction was observed in every quadrant (inferior, superior, nasal [P < 0.001], and temporal [P = 0.017]) in patients with PD. Furthermore, a strong inverse correlation was found between the PD severity measured according to the UPDRS score and the average peripapillary RNFL thickness (r = -0.615; P < 0.001) and PD duration (r = -0.303; P = 0.002). From these results, we defined a regression equation that predicts the UPDRS score from the above-mentioned variables: UPDRS = 81.6 + 29.6 * log PD duration (years) - 0.6 * RFNL thickness (μm). We observed that, as the evolution and severity of PD progress, the peripapillary RNFL layer thickness, as evaluated by OCT, gradually diminishes. These results suggest that the average peripapillary RNFL thickness measured by OCT might be useful as a biomarker to detect the early onset and progression of PD., (Copyright © 2013 Movement Disorder Society.)

Introduction: Currently used antiparkinsonian drugs neither stop nor slow-down the progressive nature of the disease. The final phase of PD is characterized by the presence of symptoms and signs resistant to dopaminergic agents, such as depression, dementia, freezing and falls. Therefore, it is urgent to develop therapies able to positively modify this outcome. Despite neuroprotection is a research priority in PD, no effective strategies have been found so far., Method: A key informants study was conducted. A group of experts in PD fulfilled a questionnaire of 10 questions to explore the most important topics related to neuroprotection. Afterwards a consensus about the current situation of neuroprotection in PD was established and future directions of development were suggested., Results: Most of the answers emphasized the need of new concepts, the limitations of animal models and the difficulties in the difficulties in demonstrating a neuroprotective effects in humans owing to a lack of biomarkers. Some of the experts believe that we are already exerting a disease modifying effect., Conclusions: The concept of neuroprotection should be widened. Animal models should be improved. A reliable biomarker to start neuroprotective therapies long before the appearance of motor symptoms and to evaluate the neuroprotective effect of any therapy should be urgently developed.

Prompted by the lack of cross-cultural comparative data, and because a better understanding in the different clinical presentations of psychogenic movement disorders (PMDs) is relevant to neurological assessment and interventions, we compared the phenomenology, anatomical distribution, and functional impairment of PMDs in the United States and Spain. Consecutive patients diagnosed with PMD by a movement disorder specialist from one US site and from eight Spanish university centers were included in the study. The two groups were similar in their movement types, anatomical distribution, and functional impairment. PMDs were more prevalent in women than in men and were most common in upper and lower extremities. Gait and speech dysfunctions were distributed similarly in both countries. We found action tremor to be the most frequent PMD in both countries., (Copyright (c) 2005 Movement Disorder Society.)

Introduction: Approximately 20% of all ischemic strokes are due to cardioembolism and occur more frequently in the younger patients. Our objective was to determine the clinical characteristics and course of cardioembolic infarcts (ICCE) comparing them with infarcts due to other aetiologies (ICNCE)., Patients and Methods: We made a prospective study of 354 patients admitted to hospital over a period of one year, after excluding transient ischaemia and parenchymatous/subarachnoid hemorrhage. Two groups were established: ICCE (29.4%) and ICNCE (70.6%), comparing age, sex, risk factors and course of the illness. Subsequently a study lasing two years was done to assess the recurrence rate., Results: The ICCE patients were older (75.89 compared with 72.58, p = 0.004), often know the exact time of onset of their symptoms (p = 0.015) and usually are admitted to hospital during the first six hours of their illness (p = 0.01). There was a significantly higher incidence of ischemic cardiopathy or auricular fibrillation (p = 0.0052); p = 0.005); more complications arose (p = 0.000); stay in hospital was longer (13.62 as compared to 11.8 days; p = 0.035), there was a lower weekly BI score (p = 0.0023) and higher mortality (p = 0.000). In the subgroup of 70 patients evaluated two years later 11 cases recurred, with no difference observed between the anticoagulant and anti-aggregant groups., Conclusion: The ICCE occurs in older patients, they develop worse neurological defects, have a worse short-term prognosis, develop more complications and have significantly greater mortality.

Introduction: Acute transverse myelitis is an inflammatory disorder. The pathogenesis is unclear, but the probable mechanism involves an autoimmune phenomenon. Possible causes included multiple sclerosis and parainfectious and postvaccinal events. Myelitis has rarely been reported secondary to vaccinations including hepatitis B. We present a case of acute myelitis, which seems secondary to the administration of the hepatitis B vaccine., Clinical Case: A 15-years-old female presented with progressive numbness of the right arm and leg, with right leg weakness. Symptom began one week after receiving the first dose of the hepatitis B vaccine. Spinal cord magnetic resonance (MR) revealed a diffuse increased signal extending from C6 to D2. Cerebral MR and cerebrospinal fluid were normal. She was treated with high doses of methylprednisolone with a complete recovery of neurological functional. Repeat medullar cord MR was normal. There was no relapse during a four years follow up., Conclusions: Potential causal relationship between vaccination against hepatitis B and multiple sclerosis was brought to the attention and to public debate. However, no conclusive association could be made between vaccination and demyelination. In the clinical setting, the distinction between a first episode of multiple sclerosis or postvaccinal myelitis depends upon subsequent course.

Introduction: Transient global amnesia (TGA) is a clinical condition in which the etiopathogenesis is still not clear. The most generally accepted theory is of a vascular origin, although epilepsy or migraine have also been considered to possibly be the cause., Objective: To make a retrospective review of the risk factors, etiopathogenesis and clinical characteristics of 58 patients with TGA., Patients and Methods: The variables recorded were compared with those from two control groups: one of healthy individuals and one of patients with transient ischemic attacks (TIA)., Results: The average age of the patients with TGA was 66.01 years. The risk factors included: arterial hypertension (58.62%), dyslipemia (15.51%) and migraine (8.62%). In three patients the EEG showed weak bilateral frontotemporal interference. Cerebral CT scans were done in all cases and were found to be pathological in 23, with lacunar multinfarct the commonest abnormality. With regard to patients with TIA, these were older (66.01 vs 72.94), smoked more (1.72% vs 12.06%) and a previous stroke was more frequent (8.62% vs 31.03%). We did not find differences regarding dyslipemia, atrial fibrillation, arterial hypertension or cardiac ischemia. However, the prevalence of vascular risk factors was significantly higher in patients with TGA as compared to healthy controls., Conclusion: We suggest that the etiopathogenesis of TGA is probably a transient ischemic phenomenon triggered (or not) following an attack of migraine.

Objective: To determine the main etiological mechanisms of symptomatic epilepsy and its frequency according to age., Patients and Methods: We made a retrospective analysis of 208 patients admitted during a period of four and a half years, studying the variables: age, sex and type of seizures: simple partial, secondarily generalized partial, complex partial, tonic-clonic, generalized tonic, and also EEG and neuroimaging., Results: The main etiological mechanisms found were: vascular (31.25%), alcoholic (12.01%), intracranial disorders (9.61%), traumatic (5.28%), degenerative (5.28%), infectious (2.88%) and cryptogenic (33.65%). In the last group there was an outstandingly large proportion of patients with silent infarcts. When considering vascular epilepsy, those seizures occurring during the acute phase of the stroke (24/65) are differentiated from those of late onset (41/65). In the latter there was a marked predominance of ischemic etiology (48.78% corresponded to extensive infarcts in the territory of the middle cerebral artery; 36.58% were associated with partial infarcts) probably because of the greater frequency of ischemic stroke as compared with hemorrhagic stroke. After the acute phase, the latency was of 10.68 +/- 0.43 months and the most frequent seizures were tonic-clonic (48.78%)., Conclusion: In persons under 30 years of age, etiology is multifactorial; between 30 and 50 years of age alcoholic epilepsy (39.53%) and traumatic epilepsy (11.62%) predominate; over the age of 50 years the cause was vascular in 43.5%. In the latter age group there was a high proportion of patients with heraldic seizures.

Defects of the mitochondrial respiratory chain are associated with a great variety of clinical disorders. In addition to the well recognized syndromes, a significant number of patients present non-specific encephalopathic disorders. In consequence these types of mitochondrial disorder are very difficult to diagnose on clinical features alone. In this paper, we describe the clinical and the magnetic resonance findings of 2 patients with a neurological syndrome indistinguishable from multiple sclerosis (Poser). Muscle biochemistry revealed defect of complex IV of the respiratory chain. We think that these patients have a mitochondrial encephalomyopathy. We suggest that in patients presenting as atypical multiple sclerosis it could be necessary investigating mitocondrial encephalopathy.

Introduction: Hypophyseal hyperplasia is an uncommon disorder in which the gland increase in size due to excessive proliferation of strings of normal cells, which usually secrete prolactin. Different aetiologies may cause this disorder of the hypophyseal gland. However, in a small number of cases the hyperplasia is not due to any of these aetiologies and is therefore known as idiopathic hypophyseal hyperplasia. There are few references in the literature to idiopathic hypophyseal hyperplasia with hyperprolactinaemia. Usually diagnosis is reached after treatment for a hypophyseal adenoma, since the clinical features are similar., Clinical Cases: We present three cases seen in our department, in which hormone and endocrine studies were done to exclude known causes of hyperplasia, together with CT and MR scans. We analyzed the behaviour of hypophyseal hyperplasia by using imaging techniques, and the differential aspects with regard to hypophyseal adenomas. Firm diagnosis is only made on anatomopathological study of the hypophysis. However, we consider that sound knowledge of the characteristics of this condition may help to establish the correct diagnosis and thus avoid unnecessary surgery., Conclusions: We review the information published in the literature on this subject, emphasizing the importance of differential diagnosis by means of imaging techniques.

Introduction: Semi-structured questionnaires, based on clinical criteria are used in the diagnosis of headache, but none has been used to study the two commonest types of chronic headache. The first step, before clinical application, is to carry out a study of the questionnaire's validity and predictive value., Objective: The objectives of our study were to evaluate the validity and determine the effectiveness of the ZZA questionnaire in the differential diagnosis between transformed migraine (TM) and chronic tension headache (CTH), in a specialist Neurology Clinic., Material and Methods: The ZZA questionnaire, elaborated by one of the investigators, was made up of 20 questions aimed at TM, CTH or the chronicity of the headache. An observer, who did not know the diagnosis, gave the questionnaire to patients who had previously been diagnosed by the head of the Headache Clinic as having TM or CTH. The process of validation of the questionnaire consisted in showing that it was reliable, with valid contents and valid criteria., Results: On analysis of reliability, the items ZZA1, ZZA15, ZZA16 and ZZA18 gave the highest indices. The Cochran Q test showed lack of uniformity of replies. Only ZZA1, ZZA15 and ZZA18 were predictive items showing MT. None of the questions to show CTH were predictive. After final adjustment the definitive model was made up of ZZA1, ZZA15 and ZZA18., Conclusions: The proposed questionnaire was not found to be useful for differential diagnosis between TM and CTH. Only 3 of the 20 questions were accepted as having some degree of validity and effectiveness. Two of these 3 questions, based on clinical impressions of the author, might well be included in the diagnostic criteria of TM.

Objective: To study the relationship between the hormone response to stress seen after ACV (CVA) and the type, size and site of the lesion., Material and Methods: We made a prospective study of the relationship between stress hormones and the radiological characteristics of the lesion in 82 patients admitted to hospital for non AIT ACV (CVA). We assessed the 24 hour urine catecholamines (total catecholamines, adrenaline, noradrenaline, vanillylmandelic acid, metanephrines and dopamine) and the 24 hour urine cortisol, collected on the second and third day after admission respectively. The type, size and site of the lesions were studies on CT scans done between 3 and 7 days after admission., Results: We studied 82 patients, 43 men and 39 women with an average age of 71.7. In 7 patients the lesion was parenchymatous haemorrhage; in 75 it was an infarct, which was small (< 6 cm3) in 30.5%, moderate sized (6-60 cm3) in 38.6% and large in 30.6%. In the cases of infarct, only the cortisol was significantly different in the three groups (average (DE) standard deviation, respectively: 80.6 (50), 114 (124) and 246 (207); p = 0.0014). This relationship persisted when the cortisol level was compared with the volume in cm3 (p = 0.0028). The cortical infarcts had significantly higher levels of cortisol than the more deeply situated infarcts (83.2 (55) as compared to 174 (184); p = 0.0321), but the latter were smaller and no difference was seen when size was taken into account. All findings were similar in haemorrhages and infarcts of equal size., Conclusions: There was no relationship between the catecholamines and the type or size of the lesion. In our series, the site of the lesion did not appear to have any effect on the characteristics or intensity of the hormone response.

Introduction: Epileptic crises are uncommon in patients with multiple sclerosis. However, epilepsy is commoner in these patients than in the general population. An epileptic crisis as the presenting feature of multiple sclerosis is even rarer. The lesions involved in the pathogenesis of these crises are plaques of demyelinization which affect the cortical or subcortical areas. Other factors, some of which are still not clearly understood, such as the fibre, electrolytic changes, size of the plaque, reactive gliosis and the enzyme (Na(+)-K+)ATPase, seem also to play a part in the production mechanism. Magnetic resonance is a very sensitive technique used in the detection of demyelinating lesions during the acute phase. The sensitivity is further increased by the use of gadolinium., Clinical Cases and Conclusions: We present two cases of multiple sclerosis which presented as epileptic crises. In one there were generalized tonic-clonic crises and in the other partial sensitive crises. We mention the EEG findings, CSF analysis and neuroimaging diagnostic techniques.

We present a study of 165 patients with fits with normal brain computerized tomography (CT) scan or else who showed no evidence as to the etiology of such attacks. We analyzed the magnetic resonance (MR) results obtained. In 36.6% of cases MR was pathological, the most frequent finding in our series being cerebral atrophy (12.8%). We comment on the most important pathology groups, highlighting the contribution MR made in our patients.

Objective: This study wants to identify factors or components latent besides health indicators from Spanish regions, and its graphic output., Design: Observational study., Setting: Twenty eight indicators from regions were used: mortality, morbidity, communicable and no-communicable diseases, diet, dwelling and sanitary resources. Measurement was made between 1980-1988., Interventions: Principal component analysis has been applied to the indicators, reducing data dimension., Measurement and Main Results: Eight factors have been extracted, which explain 90% of the original information. This analysis, as can be seen from communnalities, represents correctly the set of original variables. The factors with more easily interpretation were: shortage sanitary resources, develop diet, mortality, chronic diseases and accidental., Conclusions: Only reduction data dimension could be justify the use of principal component analysis. Behind the agrupation of variables is mostly the socioeconomic background.

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.

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.

Background: In this study we introduce a new view of hospital infection, to apply time series techniques to it. Our objective is to complement hospital infection's epidemiological surveillance by means of obtaining alert and alarm thresholds that make easy to the epidemiologist the decision of intervention, in case they are exceeded., Methods: We have used the classic time series analysis described by Rumeau-Rouquette, and ARIMA (Autoregresive Integrated Moving Average) models developed by Box and Jenkins. The study focus on three hospital units: one intensive care, one long term care and one surgical unit. The nosocomial infection intervals have been calculated with a 68% (1SD) and 95% (2SD) confidence levels., Results: We detect an ascending general trend in the last two units, without the detection of seasonal variations. Two ARIMA (1, 0, 0) models we obtained for surgery and long term care, discarding other better adjusted models, more complex and difficult to obtain, but with no real advantage in prediction power. Confidence intervals were calculated with both methods. We did not find general trend and seasonal variations for intensive care unit. No model was considered valid, because of its high random component. The nosocomial infection intervals have been calculated with mean +/- 1SD and mean +/- 2SD., Conclusions: We think that more precise knowledge of hospital infection, with a high random component in our study, can be in addition useful to assign priority to human and material resources.

Two cases of migraine complicated with ischemic cerebral infarct are reported. Both met the established criteria for the diagnosis: definite history of classical or common migraine, development of an ischemic infarct during the crisis, duration of deficit lasting more than 24 hours and absence of other causes of cerebrovascular disease. Several laboratory examinations were carried out: routine blood tests, coagulation study, immunological study, serological tests for syphilis, ECG, echocardiogram, cerebral CT, and nuclear magnetic resonance imaging and angiography. The most remarkable finding in these two patients were multiple cerebral infarcts in different vascular territories. This fact is exceptionally reported in the literature. The clinical outcome of the patients was characterized by the virtually complete reversal of neurological deficit.

The concept of meteoropathy implicates the atmospheric properties as determining or at least provoking causes of different disease conditions. Thus, we know that changes in temperature, atmospheric pressure and humidity rate are associated with a higher incidence of stroke presentation. Considered as a whole, these three atmospheric factors have been associated with the several clinical diagnoses of stroke. A higher presentation rate of stroke has been found when the three following facts concurred: a reduction of 10 degrees (C) in the temperature of the preceding day, an increase of the atmospheric humidity rate from 20% to 60% on the day of stroke, and an increase in atmospheric pressure of 6 mlbs from the preceding day (s = 0.0000). In addition, a higher rate of presentation of stroke was found when the difference in atmospheric pressure was 16 mlbs between the day of stroke and the preceding one (s = 0.049). Also, when the atmosphere humidity rate increased from 20% to 60%, the difference in mean temperature between the day of stroke and the preceding one was 3 degrees (C), and the atmospheric pressure increased in 6 mlbs, the incidence of stroke was higher (s = 0.007). We compare these findings with those from other authors.

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.