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Determinar el valor a-adido de los parámetros semicuantitativos en el análisis visual y estudiar los patrones del depósito cerebral de 18F-Florbetaben. Análisis retrospectivo de pacientes con deterioro cognitivo leve o demencia de origen incierto procedentes de un estudio multicentrico. Los PET con 18F-Florbetaben fueron interpretados de forma visual por dos observadores independientes, analizando las regiones "diana" con la finalidad de calcular el acuerdo interobservador. Se realizó análisis semicuantitativo de todas las regiones corticales con respecto a tres regiones de referencia para obtener índices de captación (SUVRs). Se analizó la capacidad de los SUVRs para predecir el resultado de la interpretación visual, la posibilidad de depósito preferencial del radiotrazador en algunas regiones "diana" así como las diferencias interhemisféricas. Se evaluaron 135 pacientes. En la valoración visual, 72 estudios se clasificaron como positivos. El acuerdo interobservador fue excelente. Todos los SUVRs fueron significativamente superiores en pacientes con PET positivos con respecto a los negativos. Las regiones corticales correspondientes al área prefrontal y al cingulado posterior mostraron la mejor correlación con la evaluación visual, seguidas por la valoración integrada cortical. Usando análisis de ROC, los SUVRs obtenidos en las mismas regiones "diana" mostraron la mejor capacidad diagnóstica. La información obtenida de las regiones "diana" parece ser de ayuda en la clasificación visual, basado en un depósito preferencial de amiloide, lo que permitiría el "machine learning". El depósito de amiloide, aunque difuso en todas las regiones corticales, parece no ser uniforme ni simétrico. To assess the added value of semiquantitative parameters on the visual assessment and to study the patterns of 18F-Florbetaben brain deposition. Retrospective analysis of multicenter study performed in patients with mild cognitive impairment or dementia of uncertain origin. 18F-Florbetaben PET scans were visually interpreted by two experienced observers, analyzing target regions in order to calculate the interobserver agreement. Semiquantification of all cortical regions with respect to three reference regions was performed to obtain standardized uptake value ratios (SUVRs). The ability of SUVRs to predict the visual evaluation, the possibility of preferential radiotracer deposition in some target regions and interhemisphere differenceswere analyzed. 135 patients were evaluated. In the visual assessment, 72 were classified as positive. Interobserver agreement was excellent. All SUVRs were significantly higher in positive PET scans than in negative ones. Prefrontal area and posterior cingulate were the cortical regions with the best correlations with the visual evaluation, followed by the composite region. Using ROC analysis, the SUVRs obtained in same target locations showed the best diagnostic performance. The derived information from target regions seems to help the visual classification, based on a preferential amyloid deposit, allowing machine learning. The amyloid deposit, although diffuse in all cortical regions, seems not to be uniform and symmetric. [ABSTRACT FROM AUTHOR]

Introduction: Myasthenia gravis (MG) and Alzheimer's disease (AD) are two of the most important diseases where the dysregulation of acetylcholine activity plays a crucial role. In the first, this dysregulation happens at the level of the neu-romuscular junction and in the second, in the central nervous system (CNS)., Aim: To analyze the possible relationship between these two pathologies, analyzing the prevalence and the odds ratio of AD within patients previously diagnosed with MG. We will compare these data with respect to the prevalence of AD in the general population., Patients and Methods: We examined the data obtained by the electronic medical records of patients in the health care system of Castilla La Mancha using the Natural Language Process provided by a clinical platform of artificial intelligence known as the Savana Manager?., Results: We identified 970,503 patients over the age of 60 years, of which 1,028 were diagnosed with MG. The proportion of the patients diagnosed with AD within this group (4.28%) was greater than the rest of the population (2.82%) (p = 0,0047) with an odds ratio of 1.54 (confidence interval at 95% 1.13-2.08; p = 0.0051) without finding significant differences in the bivariate analysis for the rest of the most important actual known risk factors for AD., Conclusion: Our results suggest that there might be an increase in the prevalence of AD in patients previously diagnosed with MG.

Purpose: To assess the correlation between profile and severity deterioration in the neuropsychological assessment and the most affected regions in amyloid PET semiquantification. The influence of vascular risk and other potential confounding factors was also evaluated., Methods: A retrospective, observational, and multicenter study including all patients referred for amyloid PET in daily practice was conducted. Patients underwent neuropsychological assessment, and cognitive decline severity and domain(s) affected were recorded. The patients were grouped according to cognitive impairment (CI) profile and severity: (A) no CI, single-domain amnestic CI, multiple-domain amnestic CI, and nonamnestic CI; and (B) mild CI, moderate and severe dementia. An adapted Framingham Stroke Risk Profile was calculated for each individual. Depression and parkinsonism were also recorded. Standardized quantitative analysis software was used to obtain standardized uptake value ratio (SUVR) values from PET/CT images. The corresponding associations were assessed with the most appropriate statistical tests., Results: One hundred twenty-nine patients were included (62 men, 67 women; 64.67 ± 7.47 years old). Significant differences in global and regional amyloid load were exclusively found in women between non-CI and moderate dementia ( P = 0.006, for total-cerebellum SUVR). Posterior and anterior cingulates and prefrontal cortex best represented CI severity ( P = 0.003, 0.006, and 0.006, respectively). No relationship between the CI profile and the regional amyloid load was shown. A significantly high positive correlation was found between age and vascular risk and between these variables and amyloid load in nearly all regions, especially in women with moderate dementia., Conclusion: Semiquantitative analysis of amyloid PET by SUVR values revealed a significant correlation between amyloid burden and CI severity, although only in women., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Aim: To assess the added value of semiquantitative parameters on the visual assessment and to study the patterns of 18 F-Florbetaben brain deposition., Materials and Methods: Retrospective analysis of multicenter study performed in patients with mild cognitive impairment or dementia of uncertain origin. 18 F-Florbetaben PET scans were visually interpreted by two experienced observers, analyzing target regions in order to calculate the interobserver agreement. Semiquantification of all cortical regions with respect to three reference regions was performed to obtain standardized uptake value ratios (SUVRs). The ability of SUVRs to predict the visual evaluation, the possibility of preferential radiotracer deposition in some target regions and interhemisphere differences were analyzed., Results: 135 patients were evaluated. In the visual assessment, 72 were classified as positive. Interobserver agreement was excellent. All SUVRs were significantly higher in positive PET scans than in negative ones. Prefrontal area and posterior cingulate were the cortical regions with the best correlations with the visual evaluation, followed by the composite region. Using ROC analysis, the SUVRs obtained in same target locations showed the best diagnostic performance., Conclusions: The derived information from target regions seems to help the visual classification, based on a preferential amyloid β deposit, allowing machine learning. The amyloid β deposit, although diffuse in all cortical regions, seems not to be uniform and symmetric., (Copyright © 2021 Sociedad Española de Medicina Nuclear e Imagen Molecular. Published by Elsevier España, S.L.U. All rights reserved.)

Introduction: In recent years there has been an increase in the number of endovascular examinations in the study of both cardiovascular and cerebrovascular diseases. One very infrequent complication is neurotoxicity due to contrast, and it must be suspected within the context of a neurological deficit following such examinations in order to be able to diagnose it., Case Report: A 61-year-old male who presented an epileptic attack and later deficit in the left hemisphere following diagnostic arteriography. After ruling out the possibility of an urgent vascular pathology, he was submitted to a magnetic resonance brain scan with the suspicion of this condition. The clinical signs and symptoms were limited to 72 hours with cortisone therapy., Conclusions: Contrast-induced encephalopathy covers a wide range of clinical features which, in the vast majority of cases, are self-limiting to 24-72 hours, and within the context of neuronal damage following contrast infusion. Its prognosis is excellent, and diagnostic suspicion is crucial in its treatment.

Introduction: Pharmaceutical preparations with the capacity to block dopamine receptors at the brain level quite often cause secondary movement disorders, but are not the only cause. These movement disorders can be due to a poor or difficult capacity to carry out movements similar to the type observed in idiopathic Parkinson's disease (parkinsonism secondary to drugs) or also because of excess movement with acute or chronic dystonic or choreic clinical pictures. Why the same drugs cause apparently opposing effects is not altogether clear, although it is supposed that individual factors related to the pharmacokinetics, pharmacodynamics and sensitivity of different types of sub-receptors play a crucial role., Development: We describe the most frequent groups of symptoms, divided into two parts depending on whether the disorder is hyper- or hypokinetic, and the drugs that are most commonly responsible for those clinical pictures are also discussed. The most frequent disorders caused by excessive movement are acute dyskinesia, akathisia and tardive dyskinesia. The disorder caused by scarcity of movement is a parkinsonian picture that it may be impossible to distinguish from idiopathic Parkinson's disease., Conclusions: An awareness of the possible existence of these disorders is essential for the clinical suspicion and diagnosis. On most occasions, withdrawing the causing drug reverts the symptoms without the need for complicated or costly diagnostic studies.

Introduction: Gliomatosis cerebri is a neoplastic disorder characterized by diffuse infiltration of glial cells with relative conservation of the underlying structures. Seizures, headache and behavior disorders are generally the initial manifestations., Clinical Case: A 38 year-old male who had complex partial seizures and behavior disorder of three months' evolution. The brain magnetic resonance imaging showed hyperintense lesions in T2 suggestive of gliomatosis cerebri, this being confirmed with the brain biopsy. Several months later, he suffered rapid clinical deterioration, observing the development of a glioblastoma multiforme over the lesion., Conclusions: In spite of its rareness, gliomatosis cerebri should be taken into account in the differential diagnoses of diffuse infiltrative lesions of the white matter. Rapid evolution clinical deterioration and the appearance of focal lesions that capture contrast should make us suspect a transformation to lesions of greater malignancy.

Introduction: Type 1 glutaric aciduria is a genetic disease that produces a deficiency in the glutaryl CoA dehydrogenase enzyme. This deficiency entails an elevation of glutaric acid and 3-OH glutaric acid. Dystonia is the predominant symptom. The symptoms appear after an asymptomatic period during the first months of life. It may present chronically and insidiously or acutely as metabolic encephalopathy during a banal infectious condition or vaccination. Treatment with Carnitine, protein restriction and early approach of the hypermetabolism conditions has been shown to be effective in decreasing the encephalopathy episodes in the first years of life., Case Report: A 16 year old woman with macrocrania and picture of insidious onset of left hemidystonia from 6 months of age. Early treatment was begun as she had a sister suffering from type 1 glutaric aciduria. She has a clinical picture with less seriousness than her sister even though both have the same genotype., Conclusions: This disease must be considered when there is dystonic cerebral palsy and paternal consanguinity. Early onset of treatment could entail a decrease in the symptoms even in cases of insidious initiation. This would suggest therapeutic maintenance even after having passed the age in which acute encephalopathies occur.

Introduction: Chronic progressive external ophthalmoplegia (CPEO) is a common mitochondrial disease. The different conditions in this group of diseases overlap clinically, enzymatically and genetically. There is no effective treatment. Ptosis improves with corrective surgery involving tarsorrhaphy as a palliative measure., Case Reports: Code numbers were examined in a retrospective study conducted in order to search for patients with ptosis or ophthalmoplegia who had either visited or been admitted to the neurology department over the last 10 years. Data concerning these patients' clinical features and results of complementary tests were collected. Six patients with CPEO were identified, five of whom were females. Ages ranged from 44 to 72 years. All the patients had ptosis, although 50% were asymmetric. Half of them reported mild dysphagia while swallowing liquids. Levels of creatine phosphokinase and acetylcholine antireceptor antibodies were normal. Half the patients showed increased jitter and a muscle biopsy revealed that five of them had ragged red fibres. The most frequent enzyme deficit was complex I and IV deficiency. There were no familial forms; the most common genetic anomaly was single deletion in the mitochondrial deoxyribonucleic acid., Conclusions: In cases of ptosis and ophthalmoplegia that do not respond to anticholinesterases, knowledge of this condition makes it possible to avoid the use of immunosuppressant drugs, which have important side effects.

Introduction: Congenital afibrinogenemia is a very rare autosomal recessive disorder of the coagulation. The lack of fibrinogen makes the blood inclotting and patients suffer from bleeding at a minimal trauma. It is usually noticed within the first few days of life because of the umbilical cord bleeding. Intracerebral hematoma is infrequent., Case Description: 46-year-old woman who suffers from congenital afibrinogenemia and has five episodes of intracerebral bleeding, all of them in the posterior circulation. As a complication, she also presents an epilepticus status and the therapy with concentrated fibrinogen caused pulmonary thromboembolism., Conclusions: After analysing the cases described in literature, there are evidences that the majority of the intracerebral hematoma are located in the posterior circulation. A few patients developed major thomboemolisms following infusion with concentrated fibrinogen and there are only isolated descriptions of beneficial prophylactic effects. So, it is would necessary studies more extensive research to achieve effectiveness in the prevention of the bleeding.

Introduction: Tolosa-Hunt syndrome is caused by an unspecific inflammation in the cavernous sinus or superior orbital fissure. Recurrence occurs in 40% of cases. Magnetic resonance (MR) of the head and orbital phlebography can reveal characteristic data, although diagnosis is reached by exclusion according to International Headache Society criteria. Corticoids are the preferred treatment, since their use gives rise to the disappearance of the clinical condition in a few days., Case Report: We describe the cases of three patients with recurrent Tolosa-Hunt syndrome and MR images of the head with normal contrast. Case 1: a 52-year-old male with paralysis of the oculomotor nerve with recurrence on the contralateral side. Case 2: a 42-year-old female with recurrent paralysis of the abducent nerve. Case 3: a 34-year-old male with involvement of the oculomotor nerve, optic nerve and the first branch of the trigeminal nerve with recurrence of the ophthalmoplegia. In all cases the clinical symptoms disappeared with the use of corticoid therapy., Conclusions: The oculomotor disorder is produced by nerve infiltration and not by compression. The time for recurrence varies largely, and the clinical condition in the recurrence is usually milder owing to the fact that diagnosis is reached and treatment is started earlier. The absence of alterations in the MR of the head does not rule out the existence of Tolosa-Hunt syndrome, even in cases of recurrence, and these disorders therefore should not be included in the diagnostic criteria.

Introduction: Horner syndrome (HS) is produced by damage to the oculosympathetic pathway and gives rise to palpebral ptosis, enophthalmos and myosis. Anhidrosis also occurs in cases in which the lesion is located before the separation of the vasomotor and sudomotor fibres at the start of the internal carotid artery. Nasopharyngeal cancer may damage the cranial nerves, mainly in the cavernous sinus, and very few cases of HS due to infiltration of the tumour into the parapharyngeal space have been reported., Case Reports: Case 1: a 67-year-old male with a 10-day history of HS on the left side without anhidrosis. The aetiological study revealed a nasopharyngeal lymphoepithelioma; the tumour became smaller and HS disappeared following treatment with chemotherapy. Case 2: a 48-year-old male with a 1-week history of HS without anhidrosis, and a 2-month history of deafness and pain in the right ear. The aetiological study showed a nasopharyngeal lymphoepithelioma and serous otitis. The tumour became smaller and HS disappeared following treatment with chemotherapy., Conclusions: It is important to carry out studies of the nasopharynx in patients with this syndrome to allow early diagnosis and treatment of a region that is not readily available for direct examination. The existence of an associated pathology in the ear or the paranasal sinuses strongly suggests that its origins lie in the nasopharynx.

Introduction: Parotiditis is a viral infection that produces a non-suppurative inflammation of the parotid glands, although it may affect other salivary glands, the testicles, meninges and the pancreas. Clinical meningitis appears in 1-10% of cases, normally between the 4th and the 10th day after infection of the parotid glands. In this study, we analyse the clinical and analytic characteristics of the patients admitted to our Neurology Department over the last 12 years., Case Reports: A retrospective study was conducted by analysing the hospital discharge reports. The eligibility criterion was the existence of an acute meningeal syndrome linked to the presence of positive IgM titres for the parotiditis virus. In all, 13 cases were found, with a mean age of 16.5 years, and most of them were males (92.3%). Parotiditis preceded meningitis in all our patients with an average of 8.3 days, while orchiepididymitis, when it occurred, appeared at the same time or after meningitis. Analysis of the cerebrospinal fluid showed a lymphocyte profile with glycorrhachia above 40%, except in two cases that showed a non-persistent neutrophilic profile. There was a transient 30.7% reduction in prothrombin time in the patients. No sequelae were observed., Conclusions: Vaccination campaigns have drastically reduced the number of cases of meningitis due to this germ since the second half of the 90s. This virus must be borne in mind in cases of meningitis in young males who come from underdeveloped countries.

Bruns' syndrome is characterized by sudden and strong headache, accompanied by vomiting, acuphenos and vertigo, triggered by abrupt movement of the head and can produce deep coma and death. This can be due to neurocysticercosis by cyst in the fourth ventricle, which contains the larval stage of Taenia solium. In the last years, new cases of this parasitosis are being seen in Spain because of the immigration from endemic areas, but the clinical forms of this infestation are not varied. We present a 44 year old man Ecuadorian who has clinical symptoms consistent with Bruns' syndrome secondary to cyst in the fourth ventricle that progressed badly despite undergoing surgery with ventriculoperitoneal shunt and treatment with albendazol. Finally, he was operated with the excision of the lesion by means of suboccipital craniectomy. We conclude that this syndrome must rule out a cystic or non-cystic, intraventricular lesion and that the appropriate manipulation of the cysticerotic ventricular cysts carries a more favourable prognosis.

Introduction: The implementation of an on-call neurology service at our hospital prompted us to analyse the health care it offers., Patients and Methods: We conducted a retrospective descriptive study by reviewing the status report of the on-call services performed between 15 April 2002 and 14 April 2003. The data collected included the age, sex, day of the week, month, requesting service, destination and pathology grouped under different codes to facilitate their later analysis., Results: A total of 1910 patients were attended with a mean age of 53 and with no differences between sexes. The service that most often requested evaluation was casualty with over 80% of cases. The most frequently involved pathologies were headaches (19.7%), followed by ischemic strokes (16.1%) and epilepsy (13.4%). In 12.3% of cases no neurological pathology was observed. The percentage of direct hospital discharges was 29.16%, while that of admissions to neurology was lower (23.56%). Patients requiring further follow-up visits to Neurology were remitted normally (12.56%), compared to those who were in need of preferential treatment (4.45%)., Conclusions: The need for a duty neurologist has provided our hospital with a better evaluation of neurological pathologies and a more rational use of complementary tests. Many of the patients who were evaluated were given a direct hospital discharge. This has led to a drop in the number of admissions to Neurology by almost 30% and a reduction in the mean length of time spent in hospital.

Introduction: Transient global amnesia is a syndrome mainly characterised by an anterograde memory deficit, which disappears in less than 24 hours without leaving any kind of sequelae. Its aetiopathogenesis is still unclear. The ischemic theory became important after hypoperfusion was observed in the mesial region of the temporal lobe using different imaging techniques, although recently the use of diffusion-weighted magnetic resonance imaging by other authors has not revealed these alterations in the cerebral blood flow., Case Report: We report on a transcranial Doppler study carried out in a female patient during the acute phase of the amnesia, with no evidence of hemodynamic alterations or significant asymmetries. Repeating the test after clinical recovery offered values that were similar to those of the previous study., Conclusions: It is posited that the basis of this process would not be related to ischemia but instead to a mechanism enabling spreading neurogenic depression that is similar to that which takes place during a migraine attack. Treatment with antiplatelet drugs would not, therefore, be recommended but calcium antagonists or beta blockers would be useful in preventing the clinical picture from being repeated.

Background: To assess the prevalence of dementia and its subtypes in a population with a low level of education, and to determine whether there is any association between occupation, education and dementia., Subjects and Method: Cross-section study of the population, with a sample of 3,214 individuals representative of the population of Toledo, Spain, aged 65 years and older. The Mini-Mental State Examination (MMSE) was used in the first phase of the study to detect those individuals with cognitive deterioration. The second phase consisted of a semi-structured clinical interview and neuropsychological evaluation. Dementia and sub-types of dementia were determined using well-established diagnostic criteria. The process was applied using an algorithm to optimise diagnostic agreement., Results: The overall prevalence of dementia, Alzheimer's disease and vascular dementia amounted to 7.6, 4.6 and 1.8%, respectively. A direct relationship was detected between these three entities and age. The prevalence of dementia was greater in women, illiterate persons and occupations which require less skill. Once adjustment had been made for other socio-demographic variables, only age was associated with the presence of dementia., Conclusions: The overall prevalence of dementia, Alzheimer's type dementia and vascular dementia increases with age. No clear association was found between education, occupation and dementia.

We present a 28-year-old woman with encephalomyelitis and concomitant central nervous system infection by Epstein-Barr virus. Progression took place in 2 phases, with myelitis at the beginning and encephalitis developing later. Despite great functional impairment, recovery was good and the patient was left suffering only from urinary retention that was presumably caused by spinal lesions. The etiology of the case is unusual, particularly in adults.