Thalamic Dementia in the Setting of Dural Arteriovenous Fistula: A Rare Neurologic Sequelae
Objective:
Dural arteriovenous fistulae (dAVF) are direct pathologic anastomoses between meningeal arterial and dural venous sinuses or cortical veins, lacking parenchymal niduses, that account for approximately 10-15% of all intracranial vascular malformations. Neurologic sequelae of dAVF include acute complications such as hemorrhage, seizure, and headache. Reversible thalamic or cortical dementia have been rarely described as a potential sequalae of dAVF. Herein, we describe a patient with thalamic dementia secondary to dAVF complicated by deep cerebral venous sinus thrombosis.
Background:
A 70-year-old male developed progressively worsening headache over months. Neuroimaging revealed venous congestion predominantly surrounding the deep grey matter areas. Two years later he developed subacute progressive memory impairment, executive dysfunction, hypersomnolence, and personality changes including apathy and impulsivity necessitating assistance with instrumental daily activities. He then suffered acute decline in level of consciousness and progressed to being comatose within 24 hours. Neuroimaging demonstrated increase T2-FLAIR signal hyperintensity involving bilateral thalami extending to the dorsal midbrain and posterior limb of the internal capsules. Cerebrospinal fluid analysis including Alzheimer’s disease biomarkers revealed no abnormalities. MR brain venogram showed straight sinus thrombosis. He was heparinized but developed intracerebral hemorrhage involving the right mesial temporal lobe and right dorsal thalamus. Digital subtraction angiography was performed and revealed tentorial dAVF which was subsequently embolized. The patient’s neurological status improved with repeated short-interval neuroimaging showing improvement in the degree of venous congestion involving bilateral thalami. Final diagnosis was suspected progressive cognitive decline secondary to venous congestion of bilateral thalami with subsequent acute decline due to straight sinus thrombosis in the setting of long-standing tentorial dAVF.
Conclusions:
Thalamic dementia is a rare, potentially reversible complication of dAVF. Early recognition and treatment can lead to improvement in cognition and functional outcomes. Failure to identify dAVF may result in serious neurological complications including cerebral venous sinus thrombosis and dementia.
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