Nonspecific dementia, cortical blindness, and Congophilic angiopathy

Abstract

Postmortem examination of an elderly male with cortical blindness and features of both cortical and subcortical dementia revealed extensive neuronal dropout most marked in the occipital cortex, moderate gliosis, rare neuritic plaques, no neurofibrillary tangles, and extensive Congophilic angiopathy. The unprecedented association of Congophilic angiopathy with nonspecific dementia provides further support for the concept that Congophilic angiopathy is usually a nonspecific manifestation of neuronal degeneration and of little clinical significance except when it results in intracerebral hemorrhage.

Dementia in hereditary cerebral hemorrhage with amyloidosis‐Dutch type is associated with cerebral amyloid angiopathy but is independent of plaques and neurofibrillary tangles

“…This finding suggests that CAA in the absence of NP/NFT is sufficient to cause dementia. Thus, CAA alone may be a valid pathological substrate for dementia,13–19 and routine grading for CAA in addition to grading NFT and NP may improve clinicopathological correlations in AD and other dementing illnesses with CAA. In HCHWA‐D, the number of ALSSV is a practical tool to assess if CAA is severe enough to be significant for dementia.…”

“…Furthermore, CAA may affect cognition by inducing white matter changes 13, 31. Even if CAA results in dementia by causing brain hemorrhage/infarcts, from a clinical point of view it is important to realize that the absence of a history of stroke or focal radiological lesions in demented persons does not exclude the possibility that the dementia is purely CAA related 17, 30, 34, 44. For instance, in the present patient series 1 patient presented with dementia in the absence of strokes, transient ischemic attacks (TIAs), or focal lesions on computed tomography scan.…”

“…The contribution of CAA to dementia is less well established. Severe CAA in the near absence of Alzheimer's disease (AD) pathology (neuritic plaques [NP] and neurofibrillary tangles [NFT]) has been reported in demented and nondemented persons 13–19. A major problem to determine a relation between CAA and dementia is that most patients with CAA also have AD pathology, and that extensive CAA is generally associated with more NP/NFT 1, 10, 19–24…”

Brain Parenchymal and Microvascular Amyloid in Alzheimer's Disease

“…Reports of patients with 'non-specific' dementia and related clinical featues (e.g. cortical blindness) who, at necropsy, show primarily severe cortical CAA and relatively few SP's or NFT's (86), highlight the crucial clinicopathologic question of whether severe CAA represents a 'variant' of AD/SDAT with primarily microvascular deposition of betalA4, or is a unique nosologic entity with distinctive clinical features in addition to characteristic neuropathologic changes (7,145).…”

Das klinische Syndrom der posterioren kortikalen Atrophie

“…Im Unterschied zu den Verteilungsmustern bei der herkömmlichen DAT zeigt sich das größ-te Ausmaß histopathologischer Verän-derungen in den visuellen Assoziationsfeldern, frontale Regionen hingegen bleiben weitgehend ausgespart. Einzelne Fäl-le wurden post mortem als Morbus Pick [31], Heidenhain-Variante der Creutzfeldt-Jakob-Erkrankung [32], subkortikale Gliose [33], familiäre Variante [34] oder kongophile Angiopathie [35] diagnostiziert. Auch ein Fall von PCA nach einer langjährigen Intoxikation mit Aluminium wurde beschrieben [36].…”