ABSTRACT
Resumen Introducción: La degeneración lobar frontotemporal (DLFT) constituye un término que abarca distintas entidades clínicas de carácter neurodegenerativo con compromiso en el lóbulo frontal y temporal, siendo la variante conductual el fenotipo más frecuente. Esta se caracteriza por compromiso conductual y cognitivo, con predominio de la disfunción ejecutiva y relativa preservación de la memoria episódica. Pese a ello, se ha demostrado que la variante conductual de la DLFT puede presentarse con alteraciones significativas en la memoria episódica, similares a las experimentadas en la demencia por enfermedad de Alzheimer (EA), diferenciándose las DFT en las con y sin amnesia. Caso clínico: Mujer de 62 años, quien consulta con su hija por dificultades de memoria de aproximadamente dos años de evolución, de inicio insidioso y curso progresivo, las cuales orientaban a una posible EA. Sin embargo, en la evaluación neuropsicológica se encontró una disfunción ejecutiva con preservación de la memoria episódica, mientras que el estudio imagenológico mostró una atrofia cortical de predominio frontal; resultados que sugieren una probable demencia frontotemporal variante conductual. Discusión: Se analiza el caso y se aborda la información disponible respecto a los posibles mecanismos que justifican las quejas de memoria en esta población, además, de la descripción de subtipos "amnésicos de DFT".
Introduction: Frontotemporal lobar degeneration (FTLD) is a term that encompasses different neurodegenerative clinical entities that compromise the frontal and temporal lobes, being the behavioral variant the most frequent presentation. This is characterized by behavioral and cognitive impairment, with greater affection in executive functions and an apparent preservation of episodic memory. In spite of it, studies have shown the behavioral variant of FTLD can occur with significant impairment of episodic memory, in a similar way to those experienced in Alzheimer's Type of Dementia, differing frontotemporal dementia with or without amnesia. Case report: 62-year-old woman, consults with her daughter due to memory difficulties of insidious onset and progressive course during approximately two years, which led to conclusions of a possible case of Alzheimer's Disease (AD). Nevertheless, the findings of the neuropsychological assessment confirm disturbances at the executive level with preservation of episodic memory, and imaging study show frontotemporal cortical atrophy of frontal predominance; results that suggest a probable behavioral variant of Frontotemporal Dementia (FTD). Dicussion: Include a review of the case and the information avaliable about possible mecanism that explain memory complaints in this population, also a description of "amnesic" subtypes of FTD. Importance of considering a possible compromise in episodic memory and a complete evaluation that includes neuropsychological tests and imaging studies is emphasized.
Subject(s)
Humans , Female , Middle Aged , Behavior , Frontotemporal Lobar Degeneration , Frontotemporal Dementia , Alzheimer Disease , MemoryABSTRACT
Posterior cortical atrophy (PCA) is a neurodegenerative syndrome, usually due to Alzheimer's disease. The first symptoms are progressive impairment of visuo spatial (Balint's and Gertsmann's syndromes) or visuo perceptive (visual agnosia, alexia) function. Episodic memory and executive function are spared until later stages. We report two males aged 51 and 55years and three females aged 50, 54 and 56 years, with posterior cortical atrophy. Ophthalmologic study was normal in all. Presenting signs and symptoms were visual ataxia, simultagnosia, agraphia, acalculia, spatial disorientation and unilateral neglect (Balint's and Gerstmann's syndromes). Apperceptive visual agnosia, aphasia, apraxia and alexia were also observed. One female had cortical blindness. Structural images were inconclusive, but PET scan and SPECT disclosed functional impairments in occipitotemporal or occipitoparietal areas.
Subject(s)
Female , Humans , Male , Middle Aged , Cerebral Cortex/pathology , Cognition Disorders/pathology , Neurodegenerative Diseases/pathology , Atrophy/pathology , Occipital Lobe/pathology , Ocular Motility Disorders/classification , Ocular Motility Disorders/pathology , Visual Perception/physiologyABSTRACT
The pharmacological interventions for Alzheimer disease should be based in its pathogenic mechanisms such as amyloidogenesis, tau hyperphosphorilation, disturbances in neurotransmission and changes in neuronal trophism. Other therapies derive from epidemiological observations, such as antioxidants and anti-inflammatory drugs, estrogens, statins and anti hypertensive drugs. Some life style interventions, such as changes in diet, exercise and brain stimulation could also be beneficial for the prevention of Alzheimer disease. Ongoing research on pathogenic mechanisms promises the discovery of more effective therapies. Healthy life style should always be recommended due to its benefit and lack of untoward effects.
Subject(s)
Animals , Humans , Alzheimer Disease/therapy , Life Style , Amyloid beta-Peptides/metabolism , Amyloidosis/drug therapy , Amyloidosis/prevention & control , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Antihypertensive Agents/therapeutic use , Diet , Disease Models, Animal , Exercise , Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic useABSTRACT
Dementia is characterized by at least two cognitive domains defects that affect daily living activities. Before it, cognitive impairment is mild (Mild Cognitive Impairment). In next years,dementia will become a public health matter, so it is very important to have proper tools that allow early diagnosis. These tools should be validated, sensitive, specific and easy to performby non specialist physician. We discuss utility and weakness of a selected group of behavior and cognitive scales.
Subject(s)
Humans , Male , Female , Aged , Aged, 80 and over , Neurocognitive Disorders/diagnosis , Cognition Disorders/diagnosis , Cognition Disorders/prevention & controlABSTRACT
Neurological manifestations, secondary to perfusion problems, vasogenic edema or small infarcts, are common in thrombotic purpura. Moreover, they are the first symptoms of the disease in 50% of patients. We report a 50 year-old woman who presented with focal intermittent neurological signs with aphasia and right hemiparesis, who then developed progressive impairment of consciousness with stupor and generalized tonic-clonic seizures. Despite the severe neurological impairment, first neuroimaging studies were normal. A second magnetic resonance imaging showed small cortical infarcts, that were visible only with the technique of diffusion weighted imaging. The standard electroencephalograms showed focal left temporal slowing and low voltage first and then diffuse slowing accordind to the clinical condition of the patients. She was treated with plasmapheresis and had a partial neurological recovery at the fifth day, but died at the twelfth day of therapy .