Clinico-radiologic profile of a dorsal variant of posterior cortical atrophy in a 55- year old female

@#Posterior Cortical Atrophy is a group of neurodegenerative disorders characterized by early, prominent and progressive impairment of visuospatial and visuoperceptual functions in the context of relatively preserved memory and insight in the early phases. Initial visual symptoms are vague, compelling patients to seek ophthalmologic consult. They present with simultagnosia and spatial disorientation, which are often missed by routine ophthalmologic and neurologic exams, causing delay in diagnosis. As the disease progresses, Posterior Cortical Atrophy ultimately leads to a more diffuse pattern of cognitive dysfunction. The underlying pathology is believed to be Alzheimer’s Disease and a greater level of amyloid plaques is correlated with earlier clinical symptoms of Posterior Cortical Atrophy. The clinical features of reported cases are heterogenous, leading to a classification of different variants and underlying pathologies. We report the serial clinical, cognitive and imaging data of a variant of Posterior Cortical Atrophy primarily affecting the dorsal stream.

A patient with posterior cortical atrophy due to Alzheimer's disease / Paciente com atrofia cortical posterior devida à doença de Alzheimer

We describe a 68-year-old right-handed male with 16 years of education. He worked as a bank manager until his retirement in 1999. Ha had a past history of alcohol abuse for 19 years with no other comorbidities or family history of dementia. Four years before the diagnosis, the patient had consulted with some ophthalmologists for visual difficulties such as reading and driving at night. He had been involved in two car accidents - neither was related to drink driving. He also presented several other minor problems driving, which might have been the first true symptoms of his illness.

Descrevemos um homem destro de 68 anos com 16 anos de escolaridade. Ele trabalhou como gerente de banco até sua aposentadoria em 1999. Ha um histórico de abuso de álcool há 19 anos, sem outras comorbidades ou histórico familiar de demência. Quatro anos antes do diagnóstico, o paciente consultou alguns oftalmologistas em virtude de dificuldades visuais, como ler e dirigir à noite. Ele esteve envolvido em dois acidentes de carro - nenhum deles estava relacionado a dirigir alcoolizado. Ele também apresentou vários outros pequenos problemas de condução, que poderiam ter sido os primeiros sintomas verdadeiros de sua doença.

Posterior Type of Alzheimer's Dementia Presenting with Homonymous Hemianopsia

BACKGROUND: Alzheimer's disease is a chronic neurodegenerative condition, mostly affecting the medial temporal lobe and associated neocortical structures. In this report, we present a rare clinical manifestation of this disease. CASE REPORT: A 61-year-old female with word finding difficulty and memory disturbances was diagnosed with Alzheimer's disease. Two years later, she complained of right homonymous hemianopia without optic ataxia, ocular apraxia, and simultagnosia. No findings other than parenchymal disease were apparent in magnetic resonance imaging and laboratory tests. CONCLUSIONS: In this case, in a patient initially diagnosed with Alzheimer's dementia with progressive disease, we found only homonymous hemianopia, without signs of Balint's syndrome or Gerstmann's syndrome. After careful investigation showing that Alzheimer's dementia with visual symptom was not associated with parenchymal disease, we concluded a case of atypical variant of Alzheimer's disease.

Progress in posterior cortical atrophy research / 复旦学报（医学版）

Posterior cortical atrophy (PCA) is a kind of neurodegenerative dementia,which is characterized by the progressive decline of visuoperceptual,visuospatial,reading and writing ability and praxic skills.Neuroimaging usually shows atrophy or metabolic decrease in posterior brain regions.The most common neuropathologic changes of PCA are amyloid plaques deposition and neurofibrillary tangles in posterior cortex.In general,PCA is considered as a variant form of Alzheimer's disease.

One glasses too many: A case report of Benson’s syndrome.

We report a case of Benson’s Syndrome, a form of occipital Alzheimer’s disease, with posterior cortical atrophy on magnetic resonance imaging, in a 62‑year‑old male, who presented with visual problems, ascribed to the eyes, and had even undergone cataract/intraocular lens surgery in the right eye; and change of glasses 21 times over the past 2 years, with no apparent benefit. This case is of interest both on account of its rarity, and to highlight its features since the diagnosis may be missed in an ophthalmological setting where such patient may go for first consult.

Posterior Cortical Atrophy with Acute Onset and Rapid Progressive Visual Symptoms: A Case Report

BACKGROUND: Posterior cortical atrophy (PCA) is characterized by slowly progressive early onset dementia with cortical visual dysfunction and disproportionate atrophy of the posterior cortex. CASE REPORT: A 55-year-old right-handed woman developed visuo-spatial impairments that progressed rapidly into cortical blindness over the following 3 months. Neuro-psychological evaluation revealed Gerstmann syndrome and severe constructional impairments with all components of Balint syndrome. However, her memory, insight, and judgment were preserved. Her brain MRI was normal. However, 18F fluorodeoxyglucose positron emission tomography revealed a marked hypometabolism in the bilateral parieto-occipital region. CONCLUSIONS: Although rapid progression of visuo-spatial dysfunction without memory impairment occurred, we considered PCA as well.

Atrofia cortical posterior / Posterior cortical atrophy. Report of five cases

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.

A case of Posterior Cortical Atrophy Presenting with Features of Atypical Dementia

Posterior cortical atrophy(PCA) is a presenile dementia that presents primarily with signs and symptoms of cortical visual dysfunction, while memory is relatively preserved until the late stage of the disease. We report a patient with PCA, confirmed by brain magnetic resonance imaging (MRI) and F18-fluorodeoxyglucose positron emission tomography(FDG PET). A 58-year-old right-handed woman presented initially with visual dimness and difficulty finding things around her. She had partial Balint's syndrome, partial Gerstmann syndrome, and idiomotor apraxia. She also had a mild memory disturbance, but preserved insight of her disease. Neuropsychological evaluation showed decreased parietal and left temporal functions bilaterally. Brain MRI and F18-FDG PET revealed typical bilateral occipitoparietal atrophy and hypometabolism, which were slightly worse on the right side. Cholinesterase inhibitor administration for 6 months improved the memory impairment slightly, but not the cortical visual dysfunction. This is a typical case of PCA, confirmed by neurologic signs and imaging findings.

Two Cases of Posterior Cortical Atrophy

Posterior cortical atrophy (PCA) is a subgroup of focal cortical atrophy with progressive degenerative dementia that begins with higher visual dysfunction. We present two patients with symptoms suggestive of PCA. They have mild memory impairment early in the course of disease and intact primary motor and sensory modalities. Parieto-occipital atrophy was evident on brain MRI in one patient and the other was suspicious. We think that these findings are consistent with posterior cortical atrophy which is variant of Alzheimer's disease.

A Case of Alzheimer's Disease Manifesting as Posterior Cortical Atrophy

A subgroup of patients with progressive degenerative dementia that begins with higher visual dysfunction has been referred to as posterior cortical atrophy (PCA). A 55-year-old woman presented with progressive visual disturbance for 4 years, which was followed by geographical disorientation, impairment of writing and calculation, and memory distur-bance. Neuropsychological deficits were characterized by Balint syndrome, visuospatial dysfunction, prosopagnosia, Gerstmann syndrome and apraxia. Brain MRI showed mild diffuse atrophy. F18-FDG-PET disclosed bilateral occipi-totemporoparietal hypometabolism, more pronounced on the right. Biopsy from right temporal lobe revealed neu-ropathological findings consistent with Alzheimer's disease.

A Case of Alzheimer's Disease Manifesting as Posterior Cortical Atrophy

A subgroup of patients with progressive degenerative dementia that begins with higher visual dysfunction has been referred to as posterior cortical atrophy (PCA). A 55-year-old woman presented with progressive visual disturbance for 4 years, which was followed by geographical disorientation, impairment of writing and calculation, and memory distur-bance. Neuropsychological deficits were characterized by Balint syndrome, visuospatial dysfunction, prosopagnosia, Gerstmann syndrome and apraxia. Brain MRI showed mild diffuse atrophy. F18-FDG-PET disclosed bilateral occipi-totemporoparietal hypometabolism, more pronounced on the right. Biopsy from right temporal lobe revealed neu-ropathological findings consistent with Alzheimer's disease.