Introduction: In recent years Alzheimer’s disease research has focused on white matter pathology and microvascular involvement. Case report: A 79-year-old Italian woman presented with rapidly-progressing cognitive impairment, leading to akinetic mutism in ~ 3 weeks and to death in 1 month. Laboratory assays and cerebrospinal fluid (CSF) examination were unremarkable, except from CSF τ-protein which was increased. EEG showed widespread abnormalities with occasional periodic synchronous discharges, prevailing on the right hemisphere. A brain MRI performed at the beginning of the rapid deterioration only showed moderate leukoaraiosis. A new brain MRI performed 2 weeks after the first showed widespread white matter damage, which was ascribed to subacute demyelinization. Post-mortem examination led to a diagnosis of AD and not confirmed other hypotheses (e.g., Creutzfeldt-Jakob disease, inflammatory processes, cerebral amyloid angiopathy with ischemic lesions). In addition, there were multifocal areas of severe demylineation in subcortical white matter of all lobes, sparing the U-fibers tracts. Conclusions: This case suggests that, at least in some instances, a rapidly aggressive course of AD could directly reflect a subacute white matter involvement, possibly linked to microvascular pathology. Further systematic studies are needed to confirm this isolated observation and to better elucidate the pathophysiological mechanisms.

Subacute Alzheimer’s disease with prominent white matter involvement. Report of a pathologically-confirmed case

ORSUCCI, DANIELE;PASQUALI, LIVIA;ALI', GRETA;MURRI, LUIGI;BONUCCELLI, UBALDO;SICILIANO, GABRIELE;
2013-01-01

Abstract

Introduction: In recent years Alzheimer’s disease research has focused on white matter pathology and microvascular involvement. Case report: A 79-year-old Italian woman presented with rapidly-progressing cognitive impairment, leading to akinetic mutism in ~ 3 weeks and to death in 1 month. Laboratory assays and cerebrospinal fluid (CSF) examination were unremarkable, except from CSF τ-protein which was increased. EEG showed widespread abnormalities with occasional periodic synchronous discharges, prevailing on the right hemisphere. A brain MRI performed at the beginning of the rapid deterioration only showed moderate leukoaraiosis. A new brain MRI performed 2 weeks after the first showed widespread white matter damage, which was ascribed to subacute demyelinization. Post-mortem examination led to a diagnosis of AD and not confirmed other hypotheses (e.g., Creutzfeldt-Jakob disease, inflammatory processes, cerebral amyloid angiopathy with ischemic lesions). In addition, there were multifocal areas of severe demylineation in subcortical white matter of all lobes, sparing the U-fibers tracts. Conclusions: This case suggests that, at least in some instances, a rapidly aggressive course of AD could directly reflect a subacute white matter involvement, possibly linked to microvascular pathology. Further systematic studies are needed to confirm this isolated observation and to better elucidate the pathophysiological mechanisms.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11568/814590
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