Rapidly Progressive Cognitive Impairment, Ataxia, and Myoclonus: An Unusual Presentation of a Dural Arteriovenous Fistula

Geraldes, Ruth; Albuquerque, Luı́sa; Ferro, José M.; Sousa, Rita; Sequeira, Paulo; Campos, Jorge

doi:10.1016/j.jstrokecerebrovasdis.2011.01.002

Cited by 26 publications

(31 citation statements)

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“…A delay in diagnosis and/or treatment of the offending dAVF was specifically noted in 4 of the 19 cases (21%)-3 from the published literature 21,53,73 and 1 from the present series (Case 3). In 3 of these 4 cases (including our Case 3), precise time data were provided; 21,53 the average delay from presentation to angiographic diagnosis was 23 days.…”

Section: Delays In Diagnosis/treatmentmentioning

confidence: 57%

“…These symptoms are accompanied by a more focused profile of hyperintensities on FLAIR and T2-weighted imaging primarily involving the bilateral thalami. [21][22][23]32,34,48,49,53,62,65,73,[76][77][78]86,88 dural avF-induced thalamic dementia: presentation, imaging, treatment, and outcome Dural AVF -induced syndrome occurs almost exclusively in men in their 5th-7th decades of life, typically without prior history of neurological trauma. The most common symptoms of this syndrome are deficits in attention, memory, executive functioning, and disorientation.…”

Section: Discussionmentioning

confidence: 99%

“…In the context of dAVFs, the hallmarks of the symptom complex-deficits in executive function, memory, learning, attention, disinhibition, and confabulation-correspond best to the anteromedial and central thalamic regions. 12,20,21,38,43,51,56,59,62,65,74,85 Sensory and motor symptoms typically ascribed to posterolateral and inferior thalamic distributions tend to be uncommon or absent in patients with dAVFs. The term thalamic dementia syndrome has also been applied to patients having similar symptoms related to other pathological conditions affecting the thalami, including deep venous system thrombosis, bilateral diencephalic tumors, viral and prion diseases, osmotic myelinolysis, and toxic insults (see Table 4).…”

Section: Thalamic Dementia Syndrome: Differential Diagnosis and Workupmentioning

confidence: 99%

“…All were individual case reports, and several lacked adequate clinical, radiographic, and/or treatment specifics to permit detailed assessment as to manner of presentation, underlying hemodynamic pathophysiology, and long-term outcome. 18,[21][22][23]32,49,53,62,65,73,[76][77][78]86,88 None provided a comprehensive literature review.Here, we report 4 cases of dAVF with CVD involving the vein of Galen that presented with a rapidly progressive thalamic dementia syndrome. The specific pattern of presentation, diagnostic imaging findings, method of treatment, and long-term patient outcome as well as a critical review of the literature are provided.…”

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confidence: 99%

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

Holekamp

Mollman

Murphy

et al. 2016

JNS

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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.http://thejns.org/doi/abs/10.3171/2015.5.JNS15473Key words dural arteriovenous fistula; cortical venous hypertension; thalamic edema; nonhemorrhagic neurological deficit; thalamic dementia; vascular disorders ©AANS, 2016 davF-induced thalamic dementiaBorden-Shucart classification system-based not only on angiographic appearance but also mode of presentationto permit more accurate risk stratification and assist with clinical decision making for the type and timing of dAVF treatment (Table 1). 68,91One relatively underappreciated type of NHND is progressive dementia resulting from dAVF-induced cortical venous hypertension, though correct diagnosis and treatment is increasing, thanks to improved imaging techniques.18,79 Dural AVF-induced progressive dementia can be differentiated as either cortical or thalamic in origin-2 categories that have relatively distinct patterns of clinical symptomatology 18,54,62,80,85 and highly specific venous outflow patterns.18,80 Of the two, dAVFinduced thalamic dementia is less frequent, with only 15 published cases reported in the literature to date. All were individual case reports, and several lacked adequate clinical, radiographic, and/or treatment specifics to permit detailed assessment as to manner of presentation, underlying hemodynamic pathophysiology, and long-term outcome. 18,[21][22][23]...

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Section: Delays In Diagnosis/treatmentmentioning

confidence: 57%

Section: Discussionmentioning

confidence: 99%

Section: Thalamic Dementia Syndrome: Differential Diagnosis and Workupmentioning

confidence: 99%

mentioning

confidence: 99%

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

Holekamp

Mollman

Murphy

et al. 2016

JNS

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“…Seizures could be attributed to global venous congestion (8). Hemorrhage in the basal ganglia has been reported to cause seizure-like tonic-clonic movements (5). It appears that the seizures seen in our patient were more from venous congestion than from hemorrhage.…”

Section: Dural Arteriovenous Fistula As a Treatable Dementiamentioning

confidence: 52%

Dural Arteriovenous Fistula as a Treatable Dementia

Enofe

Thacker

Shamim

2017

Baylor University Medical Center Proceedings

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Reversible Parkinsonism and Rapidly Progressive Dementia Due to Dural Arteriovenous Fistula: Case Series and Literature Review

Lai

Heran

Stoessl

et al. 2017

Movement Disord Clin Pract

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Background: Dural arteriovenous fistula (dAVF) rarely presents with a syndrome of reversible parkinsonism and rapidly progressive dementia, which has been described in 19 patients to date. However, its presenting features, pathophysiology, and response to treatment have not been reviewed. Methods: We report the clinical course and treatment of two novel patients with this syndrome. Results: Despite severe presentations, both patients recovered fully after fistula obliteration. Conclusions: Atypical parkinsonism with early cognitive changes and other focal neurological signs, along with features such as pulsatile tinnitus or worsening headache, should raise the possibility of dAVF. When treated, this syndrome is nearly always reversible, highlighting the importance of early diagnosis.Dural arteriovenous fistulae (dAVF) are pathologic shunts between dural-based arteries and dural venous sinuses, meningeal veins, or cortical veins. Common presenting symptoms include pulsatile tinnitus and mastoid bruit in transverse-sigmoid fistulae, and proptosis, visual deterioration, chemosis, ophthalmoplegia, and orbital bruit in cavernous sinus fistulae. 1

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Rapidly Progressive Cognitive Impairment, Ataxia, and Myoclonus: An Unusual Presentation of a Dural Arteriovenous Fistula

Cited by 26 publications

References 6 publications

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

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

Dural Arteriovenous Fistula as a Treatable Dementia

Reversible Parkinsonism and Rapidly Progressive Dementia Due to Dural Arteriovenous Fistula: Case Series and Literature Review

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