Cognitive Decline and Hypersomnolence: Thalamic Manifestations of a Tentorial Dural Arteriovenous Fistula (dAVF)
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Cognitive Decline and Hypersomnolence: Thalamic Manifestations of a Tentorial Dural Arteriovenous Fistula (dAVF) Neha Morparia • Gary Miller • Alejandro Rabinstein Giuseppe Lanzino • Neeraj Kumar
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Published online: 31 July 2012 Ó Springer Science+Business Media, LLC 2012
Abstract Background Intracranial dural arteriovenous fistulas (dAVFs) often present with pulsatile tinnitus, orbital congestion, and headache. Occasionally, they present with focal neurologic deficits, a dementia-like syndrome, hemorrhage, or ischemic infarction. Methods This study is based on the case of a 71-year-old gentleman who presented with 6 months of progressive forgetfulness, inattention, and hypersomnolence. Four weeks prior to presentation, he developed symptoms of leftsided pain, numbness, and worsening weakness. Neurologic examination demonstrated hypersomnolence, a score of 30/38 on the Kokmen Short Test of Mental Status, and left hemiparesis. MRI brain revealed bilateral thalamic T2 hyperintensities with associated enhancement. MR venogram (MRV) showed a vascular malformation in the posterior fossa and occlusion of the straight sinus. Conventional cerebral angiogram confirmed a tentorial dAVF. The dAVF was definitively treated with transarterial embolization, followed by clip ligation of the arterialized draining vein. Twelve weeks later, there was clinical resolution of left hemiparesis and improvement in cognitive status. MRI revealed complete resolution of the thalamic hyperintensities. MRV demonstrated recanalization of the straight sinus. Results Intracranial dAVFs are uncommon but potentially life-threatening acquired vascular malformations. N. Morparia (&) A. Rabinstein N. Kumar Department of Neurology, Mayo Clinic, Rochester, MN, USA e-mail: [email protected] G. Miller Department of Radiology, Mayo Clinic, Rochester, MN, USA G. Lanzino Department of Neurosurgery, Mayo Clinic, Rochester, MN, USA
The initiating factor is venous hypertension, causing retrograde flow, venous congestion, ischemia, and sometimes infarction. The spectrum of clinical manifestations in dAVFs reflects the degree of venous congestion present. If retrograde venous flow is surgically obliterated, then venous hypertension may be reversible. Bilateral thalamic venous congestion can present as a thalamic dementia. Conclusion We conclude that intracranial dAVFs with thalamic venous congestion should be considered in the diagnostic differential for patients who present with subacute cognitive decline and T2 hyperintense thalamic signal change. Keywords Dural arteriovenous fistula Venous hypertension Bithalamic hyperintensity Thalamic dementia
Background Intracranial dural arteriovenous fistulas (dAVFs) constitute 10–15 % of intracranial vascular malformations [1]. Many dAVFs may remain clinically silent or involute spontaneously, suggesting that their incidence may be underestimated [2]. The concept of acquired dAVFs was first introduced in adults in 1951 [3]. The diagnosis of dAVFs has improved due to advances in angiograp
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