title: Frontal lobe dementia syndrome as a first manifestation of primary angiitis of the central nervous system (PACNS).
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.
authors.affiliation: Department of Neurology, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246 Hamburg, Germany. Electronic address: m.boenstrup@uke.de.
authors.forename: Marlene
authors.initials: M
authors.lastname: Bönstrup
authors.affiliation: Department of Psychiatry, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246 Hamburg, Germany. Electronic address: k.ott@uke.de.
authors.forename: Katja
authors.initials: K
authors.lastname: Ott
authors.affiliation: Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246 Hamburg, Germany. Electronic address: m.glatzel@uke.de.
authors.forename: Markus
authors.initials: M
authors.lastname: Glatzel
authors.affiliation: Department of Neurology, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246 Hamburg, Germany. Electronic address: t.magnus@uke.de.
authors.forename: Tim
authors.initials: T
authors.lastname: Magnus
mesh_terms: D003937:Diagnosis, Differential; D057180:Frontotemporal Dementia / Q000175:diagnosis* / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D013577:Syndrome; D020293:Vasculitis, Central Nervous System / Q000150:complications* / Q000175:diagnosis*
keywords: Cerebral vasculitis; Frontal dysexecutive syndrome; Primary angiitis of the central nervous system