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- W2230136067 abstract "This case presents a clinical course of a frontal lobe dysexecutive syndrome with dementia caused by a primary angiitis of the central nervous system (PACNS) of exclusively very small vessels. An isolated frontal lobe dementia syndrome as a primary manifestation of PACNS highlights the diverse clinical manifestations of the disease. The patient presented with a progressive cognitive decline with loss of memory, disinhibited behavior, inappropriate affect and frontal release signs. The diagnostic workup essentially revealed a lymphocytic pleocytosis in the cerebrospinal fluid and a generalized cortical atrophy without any vascular abnormalities. To grasp a diagnosis for this enigmatic clinical picture of a frontal lobe syndrome with signs of inflammation we targeted a tissue-based diagnosis. A brain biopsy gave the decisive hint towards a microvasculitis. Although the histopathologic picture showed peculiarities, a destruction of the vascular bed of very small vessels by lymphocytic infiltration was evident. Our case illustrates an uncommon clinical picture of a PACNS and points to shortcomings of the current histopathologic criteria if only very small vessels are involved." @default.
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- W2230136067 date "2016-02-01" @default.
- W2230136067 modified "2023-10-06" @default.
- W2230136067 title "Frontal lobe dementia syndrome as a first manifestation of primary angiitis of the central nervous system (PACNS)" @default.
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- W2230136067 doi "https://doi.org/10.1016/j.clineuro.2015.12.016" @default.
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