dementia
Encephalitis and rapidly progressive dementia due to probable Prion disease presenting with a non-convulsive status epilepticus. Case report and literature review. Encefalitis y demencia rápidamente progresiva por probable enfermedad priónica que se presenta con un estado epiléptico no convulsivo. Reporte de caso y revisión de la literatura.
Prion diseases are rare and rapidly progressive fatal neurological disorders characterized by abnormal folding of neuronal proteins.
The diagnosis is often challenging and relies on a high clinical suspicion, imagenological findings, electroencephalographic (EEG) patterns and cerebrospinal fluid (CSF) analysis.
We present a case of probable prion disease with an accelerated neurological decline and a non-convulsive status epilepticus (NCSE), which has only been described in a few cases worldwide, and seems to be associated with worse neurological outcomes and shorter survival time.
Clinical manifestations, treatment, and outcomes are shown below.
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Perfiles cognitivos-lingüísticos en personas mayores con Deterioro Cognitivo Leve, Demencia Vascular, Demencia con Cuerpos de Lewy y Enfermedad de Parkinson. Cognitive-linguistic profiles in older people with Mild Cognitive Impairment, Vascular Dementia, Dementia with Lewy Bodies and Parkinson’s Disease
Introduction: There is a wide heterogeneity of neuropsychological symptoms reported in pathological ageing. Current research has focused on patients with Alzheimer’s disease and frontotemporal dementia. However, still no detailed knowledge of the cognitive-linguistic profiles in patients whit Mild Cognitive Impairment (MCI) and other forms of dementia.
Objective: To characterize cognitive and linguistic profiles in older people with MCI, vascular dementia, Lewy body dementia, and Parkinson’s disease.
Methods: The present study followed the PRISMA guidelines and included studies from the PubMed, Scopus, and Web of Science databases, published between 2000 and 2020.
Results: 49 articles were included in this literature review for critical analysis. Older people with MCI, vascular dementia, Lewy body dementia, and Parkinson’s disease shows a significant decline in the following cognitive domains: memory, executive function, attention, and visuospatial/visuoconstructive skills. However, linguistic symptoms are also reported, especially the deficit in verbal fluency (semantic and phonological) and syntactic-grammatical and discursive skills.
Conclusion: This literature review characterized cognitive-linguistic profiles in older people with MCI, vascular dementia, Lewy body dementia, and Parkinson’s disease. These profiles could be helpful in clinical practice to improve early neuropsychological assessment processes and even determin e differential diagnoses between these clinical conditions.
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Palabras clave: Cognición,
demencia,
Deterioro Lingüístico,
Disfunción cognitiva,
evaluación neuropsicológica,
Relación entre la depresión y la demencia. Relationship between depression and dementia
The high association of depression and dementia in the elderly has motivated to investigate the type of relationship that exists between them. The objective of this narrative review was to describe the relationship between depression and dementia, for which the Medline, Science Direct, Dialnet, Redalyc databases were reviewed between 2000 and 2021, with the verbal descriptors “dementia” AND “depression” AND “relationship” AND “older adult” OUT “caregiver depression” to locate the candidate documents and then select the final sample made up of 60 published articles, which were reviewed by three judges for selection. Seven explanations of the relationship between dementia and depression were identified in which depression is considered a risk factor for dementia, a prodrome, a consequence, among others. Likewise, there was evidence that, although all the hypotheses have scientific support, there are also indications of their refutability. The types of relationship with the greatest scientific support were “depression as a risk factor” and “as a prodrome of dementia”, although the limitations in the studies prevent clarifying the relationship between these entities. Longitudinal studies that review the history of depression are suggested as a useful methodology to determine the relationship between them.
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Palabras clave: adulto mayor,
demencia,
depresión,
depresión geriátrica,
factor de riesgo,
pródromo,
Enfermedad de Creutzfeldt-Jakob. Creutzfeldt-Jakob disease
Palabras clave: (DeCS),
demencia,
difusión,
Enfermedad de Creutzfeldt-Jakob,
enfermedad priónica,
resonancia magnética,
Deterioro Cognitivo y Demencias en Adultos con Trastorno del Espectro Autista. Cognitive Impairment and Dementia in Adults with Autism Spectrum Disorder.
Autism Spectrum Disorder (ASD) has been studied mainly in children. However, currently, 1/68 adults live with this neurodevelopmental disorder. ASD in adulthood shows suggestive symptoms of cognitive impairment, quickly compromising various cognitive functions. Cognitive impairment and behavioral disorders help to develop neurodegenerative diseases such as Frontotemporal dementia (FTD) and Alzheimer’s Disease (AD) in people with ASD during adulthood, which affects activities of daily living (ADL). The objective of this systematic review is to investigate the progression of ASD to cognitive impairment and dementia in adulthood. The methodology included the qualitative analysis of studies published in the years 2000 and 2020, exclusively in english. Results reveal that adults with ASD develop early cognitive impairment and dementia according to the general population, mainly affecting cognitive functions such as memory and executive functions. In conclusion, moderate to a profound degree of intellectual disability, as well as reductions in white matter, seems to be precursors for the development of cognitive impairment and dementia in adults with ASD.
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Palabras clave: Adultez,
Demencias,
deterioro cognitivo,
Discapacidad Intelectual,
Trastorno del Espectro Autista,
Demencia Rápidamente Progresiva Como Manifestación de Recaída en Linfoma de Células Del Manto: Experiencia en Diagnóstico y Tratamiento. Rapidly Progressive Dementia As A Manifestation Of Relapse In Mantle Cell Lymphoma: Experience In Diagnosis And Treatment
Introduction: Rapidly progressive dementia is an entity that has a multiple and heterogeneous etiology. It is characterized by the alteration of two or more cognitive domains in a period of less than 1 to 2 years. The involvement of the central nervous system attributed to mantle cell lymphoma is rare with a poor prognosis and mainly debuts in the late stages of the disease as a relapse. Case Report: A 61-year-old male with a history of mantle cell lymphoma who presents a relapse of the central nervous system, given by a clinical course compatible with a rapidly progressive dementia and which is confirmed by flow cytometry studies in cerebrospinal fluid. It presents an adequate response to management with a tyrosine kinase inhibitor (Ibrutinib), resolving clinical symptoms and imaging findings. Discussion: The involvement of the central nervous system secondary to mantle cell lymphoma is a rare complication and debuts as a relapse with variable clinical manifestations that requires a timely intervention with the aim of improving patient survival. Therapy with a single agent such as Ibrutinib seems to be a good alternative in cases of refractoriness and neurological involvement.
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Palabras clave: demencia,
Imagen por Resonancia Magnética,
linfocitos B,
linfoma de células del manto,
Rendimiento Diagnóstico de Minimental Frente Al DSM-5 en Trastorno Cognitivo: Experiencia de una Cohorte en Colombia. Diagnostic Performance Of Minimental Against DSM-5 In Cognitive Disorder. Experience Of A Cohort In Colombia.
Background: Cognitive screening tools are useful in the clinical and research setting. MiniMental (MMSE) is one of the most used instruments in Colombia, the objective of this work is to determine its performance against the new criteria for the diagnosis of cognitive disorder (DSM-5). Materials and methods: Diagnostic test study, assembled in a Colombian cohort, we evaluated a consecutive sample of 200 participants older than 50 years (66.5,+/-8.86) that represented the whole spectrum of the condition of interest, the index test (MMSE) was compared with the clinical reference standard (consensus diagnosis and classification by criteria DSM-5). Results: For mild cognitive impairment (MCI), the diagnostic performance of MMSE was: Sensitivity 45.3% (95% CI 33.7 to 57.4), specificity 96.9% (95% CI 91.4 to 99.0) and 76.9% accuracy (95% CI 69.5 to 82.4) at a cut-off point of 26 and, for major cognitive disorder (MCD) was: Sensitivity 76.32% (95% CI 60.8-87.0) and specificity 97.53% (95% CI 93.8-99.0) at the cut-off point of 24. Conclusions: MMSE is a valid alternative for the diagnosis of MCD, however it has limited validity for the detection of MCI, so new tools for the purpose of screening of MCI should be considered.
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Palabras clave: demencia,
MiniMental,
MMSE,
prueba diagnóstica,
Trastorno cognitivo,
Trastorno cognitivo leve,
Tasa de Hospitalización Según Comorbilidades en Pacientes con Alzheimer en el Hospital Teodoro Maldonado Carbo. Hospitalization Rate According To Comorbidities In Alzheimer’s Patients At Hospital Teodoro Maldonado Carbo.
Introduction: According to World Health Organization (WHO) 2017 report there are 50 millions of people with dementia worldwide and 60% to 70% of cases belong to Alzheimer’s Disease. The WHO admit that dementia is a public health priority. Aims: Descriptive analysis of patients Alzheimer’s Disease in Guayaquil, Ecuador, focused hospitalization and mortality predictors. Methods: Cross-sectional study. Multivariate and univariate logistic regression analysis to indicate predictors of hospitalization by pneumonia and mortality. Results: 42.57% of the sample was men and the average age was 80.02 years. In a multivariate logistic regression analysis, the most associated comorbidity to increase the risk of pneumonia were Diabetes Mellitus combined with Arterial Hypertension (OR 5.62, 95% CI (1.17 – 26.96) p 0.031), statistically significant. Antipsychotic medication increase the pneumonia risk with a statistically significant meaning (OR 3.03, 95% CI (1.23 – 7.44) p 0.016). Conclusion: Future studies should focus on report the effect of medications and comorbidities on admissions in patients with Alzheimer. Guidelines to focus prevent common causes of hospitalization in these patients should be adapted.
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Heterogeneidad Sintomatológica. Perfiles de Pacientes Diagnosticados con Demencia Tipo Alzheimer en Antioquia (Colombia) Symptomatology Heterogeneity. Profiles Of Patients Diagnosed With Alzheimer’s Type Dementia In Antioquia (Colombia).
Objective: To describe and contrast the symptomatic variability of cases with sporadic or non-sporadic Alzheimer’s dementia (DTA + E) with the data obtained from the cases with early familial Alzheimer’s dementia caused by the E280A of the Neurobank of the Neurosciences Group of Antioquia (GNA). Materials and Method: This study was of exploratory – descriptive and correlacional type, 83 donors’ cases were taken with DTA stored in the Neurobank. These cases were divided in two groups, i) a group defined genetically like E280A; and ii) another not carrying group of the mutation (DTA+E); the scoreboards and / or characteristics neuropsychiatric, neuropsychological, neurological and neuropathological of both groups were confirmed. Results: The symptom that showed higher differences between both groups was iteration iteration (DTAF E280A with 1.2% and 18.4% for the DTA+E group). Other symptoms as depression or the time of appearance of progressive loss of memory did not show big differences among groups (DTAF E2080A=55.9%; DTA+E =53.1%) and (DTAF E2080A=55.9%; DTA+E =53.1%). The language disorders that were observed with major frequency among the donors were the loss of the language, mutism, anomia and aphasia. The sign with higher frequency in both groups was lost of sphincter control. The atrophy was with more intensity in the temporary lobes of the brains of the donors with DTA+E (83.3%). The weight of the brain and of the posterior fosse content, they have a moderate, directly proportional and highly significant relation from the statistical point of view. Conclusions: DTA +E has neuropathological differences with DTAF E280A that can be associated with the physiology hereditary from of DTAF E280A.
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Deterioro Cognitivo en Pacientes Diabéticos De 55 a 65 Años de Edad. Reporte Final de Estudio Observacional, Transversal en la Ciudad de Guayaquil. Cognitive Impairment In Diabetic Patients Between 55 And 65 Years Old. Final Report Of A Cross-Sectional, Observational Study In Guayaquil City.
Introduction: Diabetes mellitus is a frequent and systemic illness. Deleterious effects on cognition are one of its lesser known consequences. Diabetic individuals are at an increased risk for development of dementia in the future. Objective: To compare cognitive function in middle aged diabetic population with non-diabetic control group, in order to determine high risk population for developing cognitive decline or dementia in the future. Methodology: This is a cross-sectional, observational study conducted in Guayaquil. We studied 309 individuals between the ages of 55 and 65 years, of which 142 were diabetics and 167 were non-diabetic controls. A neuropsychological evaluation was performed to assess memory, attention, executive functioning and processing speed. Results: Group comparisons revealed significant differences between diabetics and non-diabetics in systolic blood pressure (p<.001), hyperlipidemia (p<.001) and cardiovascular risk (p < .001). Cognitive performance, after considering differences in scholarship, was lower in diabetic people (memory p values between .000 and .002; attention p values between .000 and .019; executive function p values between .000 and .001). Correlation between years of disease and cognitive decline was not significant (memory -.055; attention -.040; executive function .0169). Correlation between glycated hemoglobin and cognitive performance was significant for all evaluated functions (memory -.219; attention -.186; executive function -.269). Conclusion: Middle aged diabetic population has lower cognitive performance compared with non diabetics. The identification of individuals at risk for cognitive decline will contribute to the development and implementation of intervention strategies that will allow the slowing of cognitive decline in vulnerable individuals.
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Palabras clave: Alzheimer,
Cognición,
demencia,
deterioro cognitivo,
Diabetes mellitus,
pruebas neuropsicológicas,
salud cerebral,
Hidrocefalia de Presión Normal. Normal Pressure Hydrocephalus.
Normal pressure hydrocephalus is a pathology causing dementia that is reversible. Despite the time elapsed since its discovery, its pathophysiology has not been clearly established and two theories have been proposed that try to explain the process, one based on changes in cerebrospinal fluid flow and the other on cerebral blood flow. In addition, the evaluation and diagnosis process is not determined, since there is no standard method and the symptoms are similar to other pathologies of the central nervous system, leading to the diagnosis as the summation of clinical symptoms and some findings in imaging which are not always constant.
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Impacto del Aprendizaje del Inglés Sobre los Procesos Cognitivos en Adultos Mayores. Un Estudio Preliminar en Cuenca
A descriptive exploratory study, before and after a six-month english learning course for seniors is conducted to evaluate the cognitive effect of learning another language in this age group in the city of Cuenca.
Materials and Methods: 80 adults of average age 70.48 ± 4.9 years (31 men, 19 women) were included; who underwent neurological and neuropsychological evaluation at baseline, six and twelve months after the course; including tests for executive function (working memory and attention processes).
Results: Data show significant statistical association between impaired olfactory perception related to age. Neuropsychological tests show interesting data relating cognitive maintenance and even improvement in the Performance Testing Direct and inverse series (Wais III) and Symbol Digital Modality Test (SMDT) six and twelve months after initiating a learning course of English.
Conclusion: The study suggests that learning English, even without functional objective of a new language, may have protective effect on the executive functions and neuronal brain networks are claimed the first findings in patients with cognitive impairment. We suggest perform this follow up long term in time to find strongest evidence, related to international bibliography.
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Demencia y Ley. El Papel Jurídico del Neurólogo
Dementia has several legal effects under ecuadorian law. These effects are produced once dementia has been judicially established. The judge decides that a person suffers dementia based on a neurological report. When a neurologist elaborates such report, he has to take into account that legal concept of dementia resembles the medical concept of dementia.
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Deterioro Cognitivo y Riesgo de Demencia, una Realidad para el Ecuador. Estudio de Factores de Riesgo en un Grupo de Pacientes Jubilados del IESS en Cuenca en el año 2013
According to WHO in 2050, 110 million people will be affected by dementia. Ecuador will see among the diseases related to underdevelopment, those related to increased life expectancy. We conducted a cross-sectional study in a health facility IESS in Cuenca. 391 peopled signed an informed consent and answered a form to define the demographics and identify potential risk factors associated to dementia. The mental state was studied using the Minimental-test (MMSE) tool and comorbidity of depression by Yesabage screening test. Data was analyzed using SPSS 15 for Windows. The average age was 70 years, 84% were women and 97% of them live as urban residents in Cuenca. By applying the MMSE, 17.1% showed cognitive impairment with RR of 3.81 for ages 65 and older and female condition. Chronic diseases such as diabetes and arthrosis were the most frequent comorbidities with statistical association for risk; hypertension, COPD, etc., showed no statistical association. 40.9% of the sample group where possitive for depression showing serious nature in 37.5%.
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Concepto de la Demencia como Enfermedad en un Grupo Poblacional de la Ciudad de Guayaquil
Background: Dementia is a clinical condition that causes progressive cognitive impairment, and early diagnosis may have a significant impact on the quality of life. So far, no studies have been conducted to evaluate the conception of the general population about this disease.
Methods: Cross-sectional, descriptive and analytical study, which surveyed individuals between 55 and 65 years old without neurocognitive pathology about the concept of the term “dementia”. The educational level of each respondent was also collected. The responses were assigned to one of 6 possible response groups for analysis, depending on which group better matched each individual response. The percentage of each type of response was calculated, and chi-square was used to observe the relationship between level of education and the type of answers given.
Results: a total of 274 individuals were surveyed, 35.8% were male and 64.2% female. Most patients (36.5%) had only elementary education. 11.7% did not know what dementia means. 45.5% defined it as memory loss and 23% incorrectly identified it as a psychiatric illness. A very small percentage of individuals correctly defined it as a type of cognitive impairment. We found a statistically significant relationship between the level of education and the type of answers obtained (p <0.001).
Discussion: more than half of respondents gave an acceptably correct answer about the concept of dementia, but there is still a significant percentage of people who do not know its meaning, or confuse it with psychiatric illness. It is imperative to carry out health campaigns to alert people about this disease and how to delay it’s onset by lowering risk factors, and to train as well primary care physicians to provide better information to their patients in order to improve the prevention of dementia and lessen its impact on quality of life.
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Enfermedad de Lafora: Diagnóstico histopatológico de un caso por biopsia de piel.
Lafora disease constitutes one of the most common types of progressive myoclonic epilepsy in South Europe, especially in Spain. Its onset is between 10 and 18 years old, with myoclonus which can be segmentary, erratic, intentional and associated frequently with focal occipital seizures. There could also be primary or secondary generalized tonic-clonic seizures. A progressive dementia is evident as is the presence of intra citoplasmic inclusion bodies (Lafora bodies) in neurons and other glucose consuming body cells. The diagnosis can be done by identifying Lafora bodies in the sweat glands of the underarm skin. There are two genes fundamentally implicated in this disease: the EPM2A in 6q24 and the EPM2B in 6p22. We present, to our knowledge, the first documented case in Ecuador, diagnosed by histopathological material obtained by skin biopsy, and analyze its clinical, electroencephalographic and evolving characteristics. Patient’s family tree is analyzed in detail and an exhaustive literature update related with this disease is performed.
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Conversión de Deterioro Cognitivo Leve a Demencia.
Great social efforts and sanitary conditions have decreased child mortality rates, decreased birth rates and increased life expectancy. Consequently, the proportion of adults has increased. The increased incidence of neurodegenerative disease such as dementia occurs in parallel to this demographic transition. Dementia may be associated with several risk factors. However, few studies have examined the rate of cognitive and functional decline.
Objective: To determine the prevalence of cognitive decline and dementia, risk factors and report results of cognitive and functional decline in patients in the National Institute of Neurology and Neurosurgery.
Methods: We included all outpatients aged ≥ 50 years attending between 1999 and 2000. We sought to asses the Petersen criteria for the mild cognitive impairment (MCI). Patients were assessed using the Consortium to Establish a Registry for Alzheimer’s Disease, Diagnostic and Statistical Manual of Mental Disorders (4th edition), and the National Institute of Neurological and Communicative Disorders and Stroke and the Alzheimer’s Disease and Related Disorders Association criteria. We followed for up to 8 years.
Results: A total of 239 individuals were included. The conversion of MCI was of 25.6% per year and 56.4% at the eight years. The consumption of alcohol were associated with dementias (p<0.01).
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Fractura de cadera y deterioro cognitivo: un estudio seccional-cruzado.
Cognitive impairment is a non modifiable risk factor for hip fracture.
Objective: To determine if patients older than 60 years old with hip fracture presented association with cognitive impairment.
Method: Cross-sectional study.
Patients. All patients older than 60 years admitted in the traumatology service of Enrique Garcés Hospital in Quito-Ecuador.
Data recollection. It was made by: clinical history, interview with the patient that included valoration of cognitive state by minimental test and the photograph test, interview with the relatives of the patients if they where during his or her hospital stay. The variables were analyzed in percentages and the data was utilized to evaluate if there was a mayor association between cognitive impairment and hip fracture.
Results. Of the 56 patients included 83,92% presented cognitive impairment, of this patients 85,10% presented dementia and 42,55% presented severe dementia. The association between the following variables was significant: patients older than 80 years old with severe dementia OR 8 (IC 1,75 – 41,18), analphabets with severe dementia OR 5,33 (IC 1,13 – 27,97), hip fracture with severe dementia OR 6,28 (IC 1,54 – 30,24), hip fracture with falling OR 19,09 (IC 3,36 – 187,38).
Conclusions: Severe dementia presented an association with age older or equal to 80 years and analphabetism, and hip fracture presented an association with severe dementia and falling.
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Diabetes Mellitus y Cognición. Estudio Transversal.
Diabetes Mellitus Type 2 is associated with greater impairment of cognitive ability, and abnormalities in brain imaging studies compared with people without diabetes.
Objective: To determine if there is a difference in the score of cognitive function tests Addenbrooke (ACE) and Cognitive Ability Screening Instrument (CASI) among patients with type 2 diabetes and non-diabetics.
Methods: Cross sectional study of patients with a history of more than 5 years of diabetes mellitus type 2, between 45 and 64 years of Outpatient of Endocrinology Department, Hospital Teodoro Maldonado Carbo Guayaquil between September 1 and December 31, 2008, who had the cognitive assessment test: ACE and CASI.
Results: There were a total of 68 patients, of which 60.3% had diabetes mellitus, with an average age of 60 years, of this group: 53.6% were men and 46.3% were women. Hypertension was present in 39%. 14.6% were treated with oral antidiabetic agents and 73.2% with insulin. Glycated haemoglobin (Hb1Ac) was abnormal in 33.8%. With regard to the tests of cognitive assessment: the average score of ACE in diabetic patients was 88. 8 and in non-diabetic patients 90.1 and in the CASI the average was 91.5 in diabetics and 92.5 in non-diabetics.
Conclusions: The score of cognitive function tests Addenbrooke and CASI was lower in diabetics compared to non-diabetics, although there was no significant difference.
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Palabras clave: Addenbrooke,
CASI,
Cognición,
Complicaciones de la Diabetes,
demencia,
Diabetes mellitus,
Demencia y Parkinsonismo como Síntomas Iniciales en un Paciente con SIDA.
Dementia and Parkinsonism as early symptoms of AIDS are infrequent. We report the case of a male patient 62 years old, with a history of diabetes – insulin dependent and illicit drug use, who began his symptomatology with dementia and parkinsonism two months before his death.
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Progresive Dementia and Behavioral Changes: Pick’s Disease: A rare disease or an underdiagnosed disorder?
Pick’s disease is a nosological entity with frontal dementia, early cortical dementia with severe frontal lobe disturbances, absence of apraxia, and absence of gait disturbance at onset. This disorder is underdiagnosed in clinical practice. When specific criteria for the clinic, imaging and neuropathologic diagnosis including the presence of Pick bodies, are used, the diagnosis of Pick’s disease is achieved. However, taking into account that the definitive diagnosis of PD is achieved only with pathological study, in vivo diagnosis requires of the combination of neuroimaging techniques.
More than 100 years ago, Arnold Pick described several patients who presented with progressive behavioral changes (apragmatism, outbursts of rage, and later stages, mutism) and who, at autopsy, had characteristic frontal or temporal lobar atrophy [1]. Alloys Alzheimer histologically characterized the disorder when he described “argentophylic globes” in the cytoplasm of neurons and the presence of ballooned neurons and spongy cortical wasting in the absence of neurofibrillary tangles or plaques [2]. Pick’s disease is considered a relatively rare neurodegenerative disorder, affecting subjects in their 60s with the progressive development of frontal lobe type features (e.g., difficulty planning, reasoning, abnormal social behavior), language disturbances (decreased fluency followed by echolalia, mutism), later followed by memory and gait abnormalities and occasional parkinsonism [3]. In autopsy studies of progressive dementia, only about 5% are due to PD. The underlying cause is not know, but there does appear to be a hereditary component, with clear autosomal dominant transmission in some families. The disease usually progresses inexorably over 2 to 5 years to death. At the present time there is no specific treatment available [4]. We report our findings in a patient with the purpose of to attract attention about this entity with the aim to avoid unnecessary, expensive and dangerous treatment when this kind of patients received a wrong diagnosis.
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Keywords: dementia,
Pick’s disease,
