Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0752347 (Dementia with Lewy bodies)
 1,653 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Posterior cortical atrophy is a striking clinical syndrome in which a dementing illness begins with visual symptoms. Initially, the problem may seem to be loss of elementary vision, but over time the patient develops features of visual agnosia, topographical difficulty, optic ataxia, simultanagnosia, ocular apraxia (Balint's syndrome), alexia, acalculia, right-left confusion, and agraphia (Gerstmann's syndrome), and later a more generalized dementia. Occasional patients have visual hallucinations and signs of Parkinson's disease or Lewy body dementia. A number of different neuropathologic disorders are associated with posterior cortical atrophy.
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PMID:Posterior cortical atrophy: a brief review. 1707 82

Posterior cortical atrophy belongs to the progressive focal atrophy group of neurodegenerative diseases. It is characterized by specific clinical deficits in visual perception, which can affect either visuo-spatial features or identity of objects. The posterior cortical atrophy, initially described by Pick in 1902, is a rare and not well known syndrome. While it is easy to differentiate posterior cortical atrophy from Alzheimer's disease, some cognitive deficits are common with those of Lewy body disease and corticobasal degeneration, which results in diagnostic mistakes. This paper proposes a synthetic review of the posterior cortical atrophy illustrated by a clinical case.
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PMID:[Posterior cortical atrophy]. 1972 May 80

Dementia with Lewy bodies (DLB) and Posterior Cortical Atrophy (PCA), the visual variant of Alzheimer's disease, are neurodegenerative diseases that present with progressive deterioration in visual perception. However, little is known about the precise nature underlying the complex visual deficits associated with both conditions. The present study compared DLB, PCA, and healthy control participants, in four visual tasks designed to measure the efficiency of the visual system at different levels of processing. In ascending order of complexity there were tasks of visual acuity, line orientation, contour integration, and rotated object comparison. DLB patients did not differ from controls in low level visual functions of visual acuity and line orientation, suggesting that early visual processing areas including V1 were relatively preserved, consistent with pathology evidence (Yamamoto et al., 2006). However, higher level visual functions of contour integration, mediated by extrastriatal areas, and the most complex task of object rotation, relying on processing within inferior temporal (IT), parietal, and frontal cortices, were impaired in DLB. In contrast, PCA patients were impaired in all tasks, consistent with evidence of widespread pathology within occipital and parietal areas in PCA. The latter suggests that both lower and higher level visual impairments contribute to the complex visual symptoms associated with PCA.
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PMID:Visual impairments in dementia with Lewy bodies and posterior cortical atrophy. 2006 45

Posterior cortical atrophy (PCA) is a rare neurodegenerative disorder that has cerebral atrophy in the parietal, occipital, or occipitotemporal cortices and is characterized by visuospatial and visuoperceptual impairments. The most cases are pathologically compatible with Alzheimer's disease (AD). We describe a case of PCA in which a combination of imaging methods, in conjunction with symptoms and neurological and neuropsychological examinations, led to its being diagnosed and to AD being identified as its probable cause. Treatment with donepezil for 6 months mildly improved alexia symptoms, but other symptoms remained unchanged. A 59-year-old Japanese woman with progressive alexia, visual deficit, and mild memory loss was referred to our neurologic clinic for the evaluation of right homonymous hemianopsia. Our neurological examination showed alexia, constructional apraxia, mild disorientation, short-term memory loss, and right homonymous hemianopsia. These findings resulted in a score of 23 (of 30) points on the Mini-Mental State Examination. Occipital atrophy was identified, with magnetic resonance imaging (MRI) showing left-side dominance. The MRI data were quantified with voxel-based morphometry, and PCA was diagnosed on the basis of these findings. Single photon emission computed tomography with (123)I-N-isopropyl-p-iodoamphetamine showed hypoperfusion in the corresponding voxel-based morphometry occipital lobes. Additionally, the finding of hypoperfusion in the posterior associate cortex, posterior cingulate gyrus, and precuneus was consistent with AD. Therefore, the PCA was considered to be a result of AD. We considered Lewy body dementia as a differential diagnosis because of the presence of hypoperfusion in the occipital lobes. However, the patient did not meet the criteria for Lewy body dementia during the course of the disease. We therefore consider including PCA in the differential diagnoses to be important for patients with visual deficit, cognitive impairment, and cerebral atrophy in the parietal, occipital, or occipitotemporal cortices. A combination of imaging methods, including MRI and single photon emission computed tomography, may help identify probable causes of PCA.
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PMID:Homonymous Hemianopsia Associated with Probable Alzheimer's Disease. 2718 Jul 94