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[Neurological catastrophes due to medical errors]. / Catástrofes neurológicas por desconocimiento y retraso diagnóstico.

In 1999, the American Institute of Medicine reported an enormous rate of medical errors, representing the fifth cause of death. In Spain, there is no reliable information on the number and nature of medical adverse events, but the situation is probably similar to that described in the USA in 1999, if not higher. Diagnostic errors account for more than half of neurological adverse events and these errors can be catastrophic if the natural progression of the neurological disorder causes severe sequels or even death when the patient is left untreated. To improve patient safety, research must be undertaken to determine how these errors are produced and to develop strategies to prevent inappropriate conduct. Among many other elements, it is important to create teamwork, improve neurological knowledge among general practitioners and residents, to design clinical practice guidelines aimed at patient safety, and to promote policies that reward the absence of errors. In general, medical errors are neither exclusively due to lack of experience nor to insufficient medical knowledge, but rather to faulty organization of medical care. Therefore, it is preferable to monitor healthcare organization rather than to blame the individual supposedly responsible for the error.

[Apolipoprotein E4 in dementia with Lewy bodies]. / Apolipoproteína e4 en la demencia con cuerpos de Lewy.

INTRODUCTION: There is a strong association between the e4 allele of apolipoprotein E (APOE) and Alzheimer's disease (AD). This converts this allele into a risk factor for the development of AD. The association between APOE4 and dementia with Lewy bodies (DLB) is under discussion. In DLB, the presence of APOE4 has been related with a greater amount of senile plaques and neurofibrillary tangles. METHOD: This is a case-control study in which the APOE genotype was determined using the modified PCR technique of Wenham in 306 patients with diagnosis of probably AD, NINCDS-ADRDA criteria, 58 cases of probably DLB, McKeith et al. consensus criteria (1996), all of them with SPECT with pathological 123I-FP-CIT and 80 normal controls (NC) having similar age and gender distribution. RESULTS: The frequency of alleles was: DLB group epsilon4: 16%; epsilon3: 75%; epsilon2: 9%; AD: epsilon4: 32%; epsilon3: 67%; epsilon2: 1%; and in the normal control group: epsilon4: 12%; epsilon3: 83%; epsilon2: 5%. The percentage of alleles in both genders was similar in the three groups. CONCLUSIONS: APOE4 percentage in DLB group (16%) was lower than in AD group (32%), and similar to the control group (12%). Considering that the presence of morphopathological Alzheimer type alterations in DBL, essentially neurofibrillary tangles, is inversely correlated with the presence of Parkinsonian signs, this group may represent pure forms of the disease, although the lack of neuropathological demonstration does not make it possible to confirm this hypothesis.

[Urinary symptoms and urodynamic findings in Parkinson's disease]. / Sintomatología miccional y alteraciones urodinámicas en la enfermedad de Parkinson.

INTRODUCTION: Urinary symptoms in Parkinson's disease (PD) are minor but disabling. We have analyzed correlation of urinary symptoms with motor symptoms, duration and severity of PD and urodynamic abnormalities observed. Response to treatment with tolterodine was also assessed. METHODS: PD male patients with a score in IPSS questionnaire over 7 and female patients with a score in IU-4 scale over 5 were included in the study. Intensity of neurological symptoms (UPDRS score), seriousness of PD (Hohen-Yahr stage), urodynamic parameters, and urinary sediment were analyzed in each patient. Abdominal ultrasonography and rectal examination were performed in males to exclude obstructive prostatic pathology. Patients without evidence of urinary flow obstruction were treated with tolterodine. RESULTS: Three out of the 19 patients were excluded because of abnormal urinary sediment and the rest (n = 16) were included. Urinary symptoms correlated with rigidity severity (p < 0.01) and years of evolution of PD (p < 0.01). Rigidity (p < 0.01) was the neurological sign with the highest UPDRS motor scale score. Overactive bladder was present in 13 cases (81.2%) and 4 of them had urinary flow obstruction. Clinical improvement in nine patients treated with tolterodine was mild (33%). CONCLUSIONS: Urinary symptoms correlate with rigidity severity and with years of evolution of PD. The use of both the urinary questionnaire and urodynamic study allow us to identify the type of bladder dysfunction and select the patients who would benefit the most from anticholinergics. Tolterodine reduced miccional urgency and frequency in PD, but was ineffective on urinary incontinence.

[Advanced dementia in Alzheimer's disease and memantine]. / Demencia avanzada de la enfermedad de Alzheimer y memantina.

INTRODUCTION: Alzheimer's disease (AD) causes dementia with a progressive course. Until now, research has been aimed mainly at studying its early, pre-dementia and mild or mild to moderate dementia phases in an attempt to find treatments with which to cure it or at least halt its progression. It is true that the latest cognitive therapies are effective up to a point, but the fact is that many patients reach the state of advanced AD, which gives rise to a number of cognitive and behavioural disorders that entail all kinds of problems on a personal, familial and community health level. AIMS: This study reviews the progress of AD, the length of its stages, the speed at which the disease courses and patients' survival. Advanced AD is defined, its clinical features and the functional disability it causes are described, and we analyse the tests and scales that must be used to measure how the process is progressing and the effectiveness of the distinct forms of treatment used in the later phase of AD, which are different to those employed in the early stages. All this allows us to analyse the results of clinical trials carried out with memantine, an NMDA (N-methyl D-aspartate) receptor antagonist. We review its pharmacological characteristics and its use in everyday practice. Lastly, we refer to the classical symptomatic treatments that are usually employed to control the frequent and intense behavioural disorders produced in the advanced phase of AD. CONCLUSIONS: The later stage of AD requires our attention because many patients reach and remain for a long time in this phase, which leads to considerable personal and social disorders. Several scales and tests have been adapted to these later phases of AD, enabling the clinician to evaluate the patient, monitor the progress of the disease and determine the effectiveness of different treatments. Memantine, the most recent drug approved for use with this disease, has proved to be effective in the treatment of patients with advanced AD. This pharmaceutical has been added to the list of well-known classical medicines, such as neuroleptic drugs, antidepressants, anxiolytic agents and others, which can be used to diminish the behavioural disorders in these patients and improve their quality of life, as well as that of their caregivers.

Prophylactic treatment of episodic cluster headache with intravenous bolus of methylprednisolone.

We evaluated the efficacy of intravenous boluses of methylprednisolone followed by prednisone as a prophylactic treatment for episodic cluster headache. Fourteen male patients (mean age, 42.54 years) with episodic cluster headache were treated with 250-mg boluses of methylprednisolone on 3 consecutive days, followed by prednisone (90 mg/day orally) with gradual tapering in four weeks. Headache parameters of the active phases treated with methylprednisolone were compared with those of previous active phases in the same patients treated with other prophylactic medications. The primary efficacy criterion was decrease in the frequency of attacks during the first month of treatment. The statistical differences were calculated using Wilcoxon's test. The attacks were significantly less frequent in the active phases treated with methylprednisolone boluses than those treated with other medications ( p<0.05). This treatment seems to be more effective than the usual prophylactic treatments for episodic cluster headache.

Transient topographical disorientation.

Transient topographical disorientation (TTD) is a short-lasting inability to find one's way in a familiar environment, while the patient remains conscious and is able to recall what happened. We report the study of 10 patients with episodes of TTD, studied on the days following the last episode. The episodes of TTD could be separated into two types: the patients either reported difficulties in spatial orientation with preserved abilities to recognize landmarks and objects, or the difficulties appeared with the recognition of landmarks. Tests exploring spatial orientation, as well as higher visuoperceptive capacities were altered in most of the patients and brain SPECT showed hypoperfusion of the right hemisphere in all patients, which could also be demonstrated 2 years later in some cases. Altogether, our findings suggest that TTD is frequently associated with a more persistent right hemisphere dysfunction of unknown cause. This chronic alteration could represent either a sequel of the acute episode or a preexisting right hemisphere deficit, which inclined the acute insult to be manifested as TTD.

[Alzheimer's disease and women]. / Enfermedad de Alzheimer y mujer.

INTRODUCTION AND DEVELOPMENT: This work reviews the relation between Alzheimer s disease (AD) and women, a very interesting issue both for its socio economic, and etiopathogenic and therapeutic aspects. Much of the prevalent research conducted in this field shows that a higher proportion of suffers from this disease are women, and in the work on incidence there is at least a tendency toward the same conclusion, especially at a very advanced age. In fact, the risk of suffering from AD is greater among women and most of the patients we attend are females, which is to a large extent associated with the fact that women live longer. However, it is possible that there are other biological factors involved and for this reason the action of estrogens on the brain and the consequences of women s being deprived of them during menopause is of special interest. CONCLUSIONS: Different studies have shown that the administration of hormone replacement therapy (HRT) lowers the risk of suffering from this disease, although design defects make it necessary to wait for the conclusions from other research work currently being conducted. There are also data that supports the idea that HRT can be beneficial in AD if it is administered in suitable doses. Obviously gender can influence or modulate other risk factors (RF). Genetic factors are not easily modified and for this reason research is currently aimed at factors in which a strong environmental component is involved. Another very controversial possible RF is lack of schooling, but some data support the notion that its influence can be especially harmful among females. This is a very important hypothesis because women make up the greater part of the illiterate population in Spain. Finally, women are also prevalent among caregivers and, therefore, suffer AD from both angles: they must care and be cared for. The reaction to this situation seems to be gender specific, which means that women in particular suffer the consequences of the lack of reciprocity brought about by AD something that does not happen in other equally devastating chronic processes, but which affect the physical sphere.

Statuslike SUNCT in two young women.

OBJECTIVE: To describe the statuslike pattern of SUNCT (short-lasting unilateral neuralgiform pain with conjunctival injection and tearing) in two young women. BACKGROUND: SUNCT syndrome is a rare condition characterized by a short-lasting periocular pain associated with marked autonomic symptoms. Twenty-five cases have been reported in the literature with a high preponderance of males and a mean age of 51 years. The frequency of episodes shows a wide variability, not just among individuals but also in the same patient, and a statuslike pattern of almost continuous attacks has been described. METHODS: We report the cases of two young women (aged 26 and 23 years) with typical SUNCT features who suffered bouts of up to 60 paroxysms of pain per hour. Paraclinical investigations showed no abnormalities. CONCLUSIONS: Although unusual, paroxysms in SUNCT may overlap into a clinical status. A strong relationship with hormonal changes was noted in one patient. In both cases, the pain was refractory to treatment with indomethacin, carbamazepine, and hypnotics, and only intravenous methylprednisolone with oral carbamazepine may have been partially effective in one case.

[Posterior cortical atrophy]. / Atrofia cortical posterior.

INTRODUCTION: Posterior cortical atrophy (PCA) is a dementing syndrome characterized by an early alteration of higher visual functions. Patients usually develop a perceptive visual agnosia related to Balint syndrome. DEVELOPMENT: Verification showed Alzheimer s disease (AD) in most PCA observations (13/14 cases), and it can be concluded that there is a posterior variant of AD with prominent visual symptomatology. However, most PCA cases have not been verified and the nature of the disorder remains unknown in these observations. An early and severe unilateral occipital horn dilatation was found in one out of every six cases with non verified PCA. To the best of our knowledge, this image has not been described in AD and these observations could be of different nature. On the other hand, some non verified PCA cases have occasionally manifested as an associative visual agnosia. The nature of this type of associative disorder is unknown, but it could be similar to the nature of semantic dementia, a non Alzheimer s syndrome related to unspecific lesions of temporal lobes.

[Psychotic symptoms and Alzheimer's disease]. / Síntomas psicóticos y enfermedad de Alzheimer.

BACKGROUND: Psychotic symptoms appear during the course of Alzheimer's disease, but their frequency and intensity vary according to different studies and their nature remains unsettled. OBJECTIVES: To study the frequency and intensity of psychotic symptoms in two transversal series of patients with Alzheimer's disease and analyze its relationship with the duration of the disease and severity of cognitive impairment. PATIENTS AND METHODS: This study has been carried out in patients suffering from probable Alzheimer's disease (NINDS-ADRDA criteria). The stage of the disease was determined according to FAST, and the intensity of cognitive impairment in Mini Mental State Examination was classified as mild, moderate or severe. Frequency and intensity of psychotic symptoms (delusions, hallucinations and misidentifications) were determined by means of semistructured interviews (BEHAVE-AD 78 patients and CUSPAD 69 patients). The results obtained in these three groups of patients were compared through ANOVA variance analysis and mean contrast. Variance and covariance analysis were done to determine the relationship between psychotic symptoms and other variables (degree of cognitive impairment, length of evolution and stage of the disease). For this purpose, the patients with Alzheimer's disease but without psychotic symptoms were considered as control and compared to patients with psychotic symptoms. RESULTS: Nearly half the patients had psychotic symptoms. Delusions appeared earlier and were more frequent than hallucinations and misinterpretations. The more severe was the cognitive impairment, the more frequent and intense were psychotic symptoms, but the difference was significant only in cases with severe cognitive impairment. Hallucinations appeared mainly in patients with advanced dementia and were related firstly with the intensity of functional and cognitive impairment and secondly with the duration of the disease. CONCLUSIONS: Mild psychotic symptoms, especially delusions, appear early during the course of Alzheimer's disease. The frequency and intensity of these symptoms increase in parallel with the functional and cognitive impairments caused by the disease. Hallucinations, which appear mainly when the dementia is severe, can be considered as an evolutive marker of the process. Psychotic symptoms differ from those occurring in other disorders, either neurologic or psychiatric in nature.

[Verbal fluency and Alzheimer's disease]. / Fluencia verbal y enfermedad de Alzheimer.

UNLABELLED: BASIC: The loss of verbal fluency in Alzheimer's disease (AD) has been related to the cognitive and functional impairment caused by the disorder. GOALS: To determine the value of the different types of verbal fluency examination for the detection of AD. MATERIAL AND METHODS: We have compared the results obtained in the study of verbal fluency in 74 patients with probable AD (NINCDS-ARDRA criteria) to a control group of 64 persons of similar age, gender and educational level. Patients were divided in three categories according to the intensity of dementia: very mild (MMSE > 23), mild (MMSE 18-23) and moderate (MMSE 10-17). Semantic and literal verbal fluencies were studied by means of the "set-test", including in the analysis the results in every of four categories and total, the time consumed per category and the sum of all, the number of animal's names and words given in a minute. We also determined the number of animals in every fifteen seconds up to one minute in 66 controls y 97 patients. Results in the different groups were compared using the ANOVA variance and the contrast of means for alpha < or = 0.05. CONCLUSIONS: The time consumed in saying the names has a great value in detecting AD, with the same or even better significance than the number of words. It is feasible to shorten the examination and use only one category of names. Our results suggest that counting the names of animals given in the first fifteen seconds does not lower the value of the exam and can also suppress a "roof effect" of the test. A short version of semantic verbal fluency examination could be of potential use in general practice where time consuming exams are difficult to implement.

[Spanish study of quality of life in migraine (II). Profile of medication consumption and subjective efficacy]. / Estudio español de calidad de vida en la migraña (II). Patrón de consumo de fármacos y eficacia subjetiva.

OBJECTIVES: The response to the different antimigraine medications is variable. In this study we have analysed the profile of prescription of these antimigraine medications, both preventive and symptomatic, by a group of spanish neurologists and examined the subjective efficacy of these compounds. PATIENTS AND METHODS: Neurologists from 7 hospitals in different spanish regions interviewed 305 patients (at least 40 per hospital) who met migraine diagnostic criteria. They used an ad hoc questionnaire in which the antimigraine medications, both symptomatic and preventive, taken by the patients, as well as their subjective response were registered. Patients with transformed migraine or tension-type headache more than 2 days per week were excluded. RESULTS: Analgesics, non-steroidal anti-inflammatory drugs, ergotics and sumatriptan had been taken by 99, 69, 54 and 40% of the 305 interviewed patients, respectively. A subjective good response was refered to by 9% of patients who had taken analgesics, 23% of patients who had taken non-steroidal anti-inflammatory drugs, 39% of those who had taken ergotics and 63% of patients with sumatriptan. The current symptomatic treatment was: analgesics 34% of cases, non-steroidal anti-inflamatory drugs 26%, ergotics 13% and sumatriptan 63%. Regarding preventive treatments, 108 patients (35%) had been treated with calcium-antagonists, 87 (29%) with beta-blockers, 55 (18%) with amitriptyline and only 7 (2.2%) with valproic acid. The percentages of good responses to these drugs were: 55% for beta-blockers, 42% for calcium-antagonists and 31% for amitriptyline. CONCLUSIONS: Our data confirm that analgesics are not efficacious in the majority of migraine patients and that the advent of sumatriptan has clearly improved the quality of migrane symptomatic treatment, even though about one-third of migraine patients do not respond to this drug. This study confirm that calcium-antagonists are the antimigraine preventive treatment most frequently prescribed in our country, even though their subjective efficacy is lower than that of beta-blockers.

[Spanish study of quality of life in migraine (I). Profile of the patient with migraine attending neurology clinics]. / Estudio español de calidad de vida en migraña (I). Perfil del paciente con migraña que acude a consultas de neurología.

BACKGROUND: Migraine is the main reason for neurological consultation. OBJECTIVES: To analyse the profile of the patient with migraine attending the Neurological Services of our country. PATIENTS AND METHODS: Neurologists from 7 hospitals in different spanish regions interviewed 305 patients (at least 40 per hospital) who met migraine diagnostic criteria. They used an ad hoc questionnaire in which detailed demographic and migraine clinical data were included. Patients with transformed migraine or tension-type headache more than two days per week were excluded. RESULTS: The majority (82%), were women, with no other diseases, with an average social (88%) and cultural (41%) level. The mean age at consultation was 38 +/- 11 years, while the mean duration of migraine history was 18 +/- 13 years; 78% met criteria of migraine without aura, 15% of migraine with aura and the remaining (8%) both migraine with and without aura criteria. Main subjective precipitating factors were: stress (80%), foods (68%), drugs (34%), alcohol (20%) and menstruation (8%). Migraine pain was referred to as mild by 4% of cases, as moderate by 59% and as severe in the remaining 37%. The usual duration of migraine attacks ranged from 12 to 24 h in 35% of cases, from 24 to 48 h in 25%, from 4 to 12 h in 23% and was longer than 48 h in the remaining 17%. More than half (53%) had more than 3 attacks per month. The pain was unilateral in 70% of cases, and more than half had vomiting (57%) and sono and/or photophobia (97%). CONCLUSIONS: The typical profile of the migraine patient attending the Neurology Services in Spain is that of a woman aged from 20-50, with a long personal history of migraine, otherwise healthy and with an average socioeconomic and cultural level. Our data confirm that migraine attacks are incapacitating in a relevant number of these patients.

[Epilepsy and migraine]. / Epilepsia y jaqueca.

Migraine and epilepsy are two clearly different syndromes. All they have in common is that both cause paroxystic neurological phenomena. However, the frequency of epilepsy in patients with migraine, and migraine in those with epilepsy seems to be higher than one would expect. This suggests there may be comorbidity in both conditions. Analysis of this comorbidity is very important since it may give clues as to the physio-pathology and aetiology of certain disorders. This paper analyses the existence of migraine-epilepsy comorbidity and the levels at which it occurs. This coexistence may be due to the episode of one, for example the aura of a migrainous attack, triggering off the other condition, that is an epileptic crisis. So, it may be that the 'migrainous illness' causes the 'epileptic illness' or the other way around. Their coexistence may be due to a risk factor which is common to both, since it has caused a cerebral lesion which is the cause of both disorders. Finally, a risk factor may have a direct effect, without requiring the intervention of an intermediate cerebral lesion to cause both migraine and epilepsy. This last possibility is particularly attractive to explain the comorbidity of migraine with an aura and genetically determined epilepsy.

[Usefulness of SPECT in the study of Alzheimer's disease]. / Utilidad del SPECT en el estudio de la enfermedad de Alzheimer.

Single-photon emission photometry (SPECT) images of the brains of 60 patients with Alzheimer's disease (AD) were compared with the brain scans of 16 healthy controls of the same sex and similar ages. SPECT was repeated one year later in 16 of the AD patients. Perfusion indices for frontal, parietal, temporal and occipital regions of patients and controls were compared (Student's tests). Flow alterations were located and symmetry examined. The correlation between perfusion index for each cortical area and MMSE score was assessed (Pearson's r). Perfusion alterations were present in all except one AD patient, and in 65% of AD patients such alterations were in the parietotemporal cortex. Severity of cognitive impairment in AD patients was related to perfusion changes in the left posterior temporal region. Forty-four AD patients showed asymmetrical distribution of tracer and in 73% perfusion changes were predominantly in the left hemisphere. Altered brain blood flow is common in AD and onset is early. The parietotemporal location of defective perfusion is of great diagnostic value. Asymmetry is common and the left hemisphere is most often affected.