title: Dural arteriovenous fistula-induced thalamic dementia: report of 4 cases.
abstract: Nonhemorrhagic neurological deficits are underrecognized symptoms of intracranial dural arteriovenous fistulas (dAVFs) having cortical venous drainage. These symptoms are the consequence of cortical venous hypertension and portend a clinical course with increased risk of neurological morbidity and mortality. One rarely documented and easily misinterpreted type of nonhemorrhagic neurological deficit is progressive dementia, which can result from venous hypertension in the cortex or in bilateral thalami. The latter, which is due to dAVF drainage into the deep venous system, is the less common of these 2 dementia syndromes. Herein, the authors report 4 cases of dAVF with venous drainage into the vein of Galen causing bithalamic edema and rapidly progressive dementia. Two patients were treated successfully with endovascular embolization, and the other 2 patients were treated successfully with endovascular embolization followed by surgery. The radiographic abnormalities and presenting symptoms rapidly resolved after dAVF obliteration in all 4 cases. Detailed descriptions of these 4 cases are presented along with a critical review of 15 previously reported cases. In our analysis of these 19 published cases, the following were emphasized: 1) the clinical and radiographic differences between dAVF-induced thalamic versus cortical dementia syndromes; 2) the differential diagnosis and necessary radiographic workup for patients presenting with a rapidly progressive thalamic dementia syndrome; 3) the frequency at which delays in diagnosis occurred and potentially dangerous and avoidable diagnostic procedures were used; and 4) the rapidity and completeness of symptom resolution following dAVF treatment.
authors.affiliation: Departments of 1 Neurological Surgery.
authors.forename: Terrence F
authors.initials: TF
authors.lastname: Holekamp
authors.affiliation: Departments of 1 Neurological Surgery.
authors.forename: Matthew E
authors.initials: ME
authors.lastname: Mollman
authors.affiliation: Departments of 1 Neurological Surgery.
authors.forename: Rory K J
authors.initials: RK
authors.lastname: Murphy
authors.affiliation: Immunology.
authors.forename: Grant R
authors.initials: GR
authors.lastname: Kolar
authors.affiliation: Neurology, and.
authors.forename: Neha M
authors.initials: NM
authors.lastname: Kramer
authors.affiliation: Departments of 1 Neurological Surgery.|Neurology, and.|Department of Radiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine in St. Louis, Missouri; and.
authors.forename: Colin P
authors.initials: CP
authors.lastname: Derdeyn
authors.affiliation: Departments of 1 Neurological Surgery.|Department of Radiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine in St. Louis, Missouri; and.
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Moran
authors.affiliation: Pathology, Washington University School of Medicine in St. Louis;
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Perrin
authors.affiliation: Departments of 1 Neurological Surgery.|Department of Radiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine in St. Louis, Missouri; and.
authors.forename: Keith M
authors.initials: KM
authors.lastname: Rich
authors.affiliation: Department of Neurological Surgery, Mayo Clinic, Rochester, Minnesota.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Lanzino
authors.affiliation: Departments of 1 Neurological Surgery.|Neurology, and.
authors.forename: Gregory J
authors.initials: GJ
authors.lastname: Zipfel
mesh_terms: D000368:Aged; D020785:Central Nervous System Vascular Malformations / Q000150:complications* / Q000000981:diagnostic imaging* / Q000473:pathology / Q000628:therapy; D002533:Cerebral Angiography; D057210:Delayed Diagnosis; D003704:Dementia / Q000000981:diagnostic imaging* / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D003937:Diagnosis, Differential; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D013788:Thalamus / Q000000981:diagnostic imaging* / Q000473:pathology / Q000601:surgery; D014057:Tomography, X-Ray Computed
keywords: CVD = cortical venous drainage; ICH = intracranial hemorrhage; NHND = nonhemorrhagic neurological deficit; cortical venous hypertension; dAVF = dural arteriovenous fistula; dural arteriovenous fistula; nonhemorrhagic neurological deficit; thalamic dementia; thalamic edema; vascular disorders