Dural arteriovenous fistula-induced thalamic dementia: report of 4 cases

Author:

Holekamp Terrence F.1,Mollman Matthew E.1,Murphy Rory K. J.1,Kolar Grant R.2,Kramer Neha M.3,Derdeyn Colin P.143,Moran Christopher J.14,Perrin Richard J.5,Rich Keith M.14,Lanzino Giuseppe6,Zipfel Gregory J.13

Affiliation:

1. Departments of Neurological Surgery,

2. Immunology,

3. Neurology, and

4. Department of Radiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine in St. Louis, Missouri; and

5. Pathology, Washington University School of Medicine in St. Louis;

6. Department of Neurological Surgery, Mayo Clinic, Rochester, Minnesota

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.

Publisher

Journal of Neurosurgery Publishing Group (JNSPG)

Subject

Genetics,Animal Science and Zoology

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