[Cerebral amyloid angiopathy (congophilic angiopathy): a rare cause of massive cerebral hemorrhage. Report of an "age-related" sporadic case].
Cerebral amyloidosis is a form of organ-limited amyloidosis, which doesn't involve any organ other than brain and which comprises several subtypes, including "congophilic angiopathy" (CA), "senile plaques" (SP), "neurofibrillary degeneration" (ND), "stellate amyloid cores" of spongiform encephalopathies. It is found in 5 to around 20% of human population in people aged 60 to 90 years, the increasing being strictly related to ageing. Usually it is associated to SP and occasionally to ND, being distinguished into familial and non-familial (age-related) variants. It affects intracortical and leptomeningeal variously sized vessels of the brain and is a leading pathogenetic factor in determining a rare but possibly even recurrent form of a massive intraparenchymal cerebral hemorrhage, constituting a 0.2 per cent of brain vascular accidents of any origin and a 5-10 per cent if only primary non traumatic brain hemorrhages are considered. A case of non-familial CA in a previously non-demented nor hypertensive female patient aged 65 years is reported on, who was admitted due to an almost abrupt onset of neurologic symptoms mainly dominated by a sudden loss of consciousness together with a left sensory-motor deficiency syndrome. The patient who had been operated on of unilateral mastectomy eight years earlier due to an invasive ductal carcinoma of the breast was found affected by a devastating brain hemorrhage in the right temporo-occipital lobes with subsequent deflection of the brainstem axis toward the opposite side detected by means of CT/MRI and angiographic investigations.(ABSTRACT TRUNCATED AT 250 WORDS)
Bisceglia
M
M
Servizio di Anatomia Patologica, IRCCS-Ospedale Casa Sollievo della Sofferenza, S. Giovanni Rotondo, FG.
Castelvetere
M
M
Dimitri
L
L
Monte
V
V
D'Angelo
V
V
D000367:Age Factors; D000368:Aged; D016657:Cerebral Amyloid Angiopathy / Q000150:complications* / Q000473:pathology; D002543:Cerebral Hemorrhage / Q000209:etiology*; D005260:Female; D006801:Humans
Angiopatia cerebrale amiloidea (angiopatia congofila): una rara causa di emorragia cerebrale massiva. Osservazione di un caso sporadico "age-related".