Multiple Dural Arteriovenous Fistulas Presenting with RapidlyProgressive Dementia: Case Report and Review of the Literature
David J. Teeple, MD
Cameron G. McDougall, MD*
S. Gabe Rice, MS
Patricio F. Reyes, MD
Divisions of Neurology and *Neurological Surgery, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, Arizona
Rapidly progressive dementia is usually associated with prion disease, exposure to neurotoxins, and carcinomatous meningitis. We report a 70-year-old man with relentless dementia and multiple dural fistulas who recovered from most of his neurobehavioral deficits after undergoing a two-stage embolization. We suggest that multiple dural fistulas should be considered in the differential diagnosis of patients exhibiting rapid cognitive decline.
Key Words: Dementia, dural arteriovenous fistula
Abbreviations used: CSF, cerebrospinal fluid; CT, computed tomography; DAVFs, dural arteriovenous fistulas; EEG, electroencephalography; MR, magnetic resonance; PCR, polymerase chain reaction; PET, positron emission tomography
Dementia affects 10% of persons 65 years and older and as many as 50% of those older than 85 years.4 Dementia usually refers to medical conditions characterized clinically by a gradual decline in cognition, behavior, and activities of daily living. Rapid onset dementia is less common, and its differential diagnosis includes prion disease (Creutzfeldt-Jakob disease), neoplastic processes (lymphoma, paraneoplastic syndrome), central nervous system infections (syphilis, Herpes simplex), acute thiamine deficiency, organ failure, and toxic metabolic conditions. Careful and comprehensive medical neurobehavioral assessments coupled with a thorough history, laboratory studies, and neuroimaging (CT and MR imaging) are necessary to identify the cause. In certain cases EEG and CSF testing (smear, culture, antigen and antibody levels, PCR, 14-13-3 protein) are essential in establishing the diagnosis. We report a case of rapidly progressive dementia caused by multiple DAVFs.
A 70-year-old man with a history of hypothyroidism was transferred to our institution for evaluation of rapidly progressive dementia of an undetermined etiology. Four months earlier, the patient was first noted to have cognitive and behavioral symptoms. Before the onset of his symptoms, the patient reportedly had slept through the night in a room that had recently been fumigated. The next morning he had difficulty finding the right words, a symptom associated with agitation and diminished comprehension. He saw his primary care physician and neurologist who found mild cognitive and attentional deficits.
The patient's neurologic evaluation included brain MR imaging with and without contrast, EEG, sedimentation rate, an antinuclear antibody test, the venereal disease research laboratory test, level of angiotensin-converting enzyme, human immunodeficiency virus testing, lumbar puncture, and thyroid function studies. MR imaging showed mild cerebral atrophy and EEG showed generalized slowing. Pertinent laboratory studies revealed slightly elevated levels of CSF protein and mild hypothyroidism. No treatment was prescribed.
Figure 1. Axial T-1 weighted MR imaging sequences at different levels with gadolinium show prominent cortical vessels (arrow).
In the ensuing weeks the patient's neurobehavioral status declined rapidly. He became increasingly confused, disoriented, and nonverbal. He was unable to recognize his wife, friends, and relatives and became completely dependent on his wife for his activities of daily living. A month later the patient was prescribed steroids that mildly improved his cognitive functioning for a few days. However, the patient’s condition continued to decline, and he was transferred to our neurology service for further evaluation.
On admission the patient exhibited global language deficits, an inability to follow commands, fluctuating level of consciousness, and startle myoclonus. Brain MR imaging with and without contrast showed prominent cortical vessels (Fig. 1). Four-vessel cerebral angiography showed four DAVFs involving the transverse sinuses bilaterally, the superior sagittal sinus, and torcula (Fig. 2).
Figure 2. (A) Digital subtraction angiogram of the right common carotid artery shows the DAVF involving the occipital artery (white arrows) and transverse sinus. There is drainage into the cortical veins (black arrows) and transverse sinus. (B) Digital subtraction angiogram of the right vertebral artery shows the DAVF involving a muscular branch (black arrows) off the vertebral artery and right posterior cerebral artery (white arrows). (C) Digital subtraction angiogram of the right vertebral artery and right common carotid artery. (D) Digital subtraction angiogram confirms complete obliteration of the DAVF.
The patient underwent endovascular surgery to obliterate the DAFVs, which was performed successfully in a two-staged embolization. His postoperative course was uneventful, and his neurologic deficits gradually improved. He underwent aggressive rehabilitation for 2 weeks and was conversant and ambulatory with minimal assistance at discharge.