Rapidly progressive dementia and Parkinsonism as the first symptoms of dural arteriovenous fistula. The Sapienza University experience and comprehensive literature review concerning the clinical course of 102 patients

Dementia is a chronic loss of neurocognitive function that is progressive and irreversible. Dural arteriovenous fistulas (DAVFs) are acquired lesions that account for 10–15% of intracranial vascular malformations that could present with a rapid decline in neurocognitive function with or without Parkinson-like symptoms and evolve in a rapidly progressive dementia (RPD). Often the DAVFs are not even included in the differential hypotheses of this type of dementia and are not present in any type of diagnostic algorithm for evaluating RPD. We performed a systematic review of the international literature and adding the cases coming from our institutional experience and we have collected all the reported cases of DAVFs that debut with ROD identifying the most frequent forms in terms of location and type, reporting the neurological characteristics and the outcome of each patient. The exact pathogenesis for developing dementia in patients with DAVFs remains largely unknown. The imaging changes and pathologic findings support the hypothesis that the clinical course results from the delivery of excessive volumes of blood flow into a venous system with outflow obstruction and venous congestion. The large variety of clinical manifestations of DAVFs depends on its location but this is not exactly valid for the onset of dementia. It supposed that the highly variable clinical manifestation of DAVFs has been convincingly related to the pattern of venous drainage more than location. Neurologists and clinicians generally are familiar with the differential diagnoses of slowly progressive neurodegenerative dementias, but the diagnosis of RPD entails a different diagnostic approach. Due to their curable nature, the diagnosis of DAVFs must be suspected when facing a RPD picture, even more so if it is associated with characteristic abnormalities of the hemispheric white matter. •Patients affected by DAVFs can develop an RPD, mimicking prion disease encephalopathy.•DAVFs are not included in the differential hypotheses of rapid onset dementia.•with a intracranial CT scan, a DAVF is often not visible.•The onset of dementia in DAVF is related to venous drainage patterns more than the location.•Cognitive improvement occurred in most patients, treated surgically or through an endovascular procedure.