Frontal lobe dementia syndrome as a first manifestation of primary angiitis of the central nervous system (PACNS)

Clinical Neurology and Neurosurgery
Marlene BönstrupTim Magnus

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.

References

Sep 17, 1999·Neurology·A AlrawiD C Musch
Oct 23, 2008·The American Journal of Surgical Pathology·Dylan V MillerCaterina Giannini
Dec 14, 2011·Current Opinion in Rheumatology·Carlo SalvaraniGene G Hunder
Mar 17, 2012·Acta Neuropathologica·Caterina GianniniRobert D Brown
May 12, 2012·Lancet·Carlo SalvaraniGene G Hunder

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