Myelopathy due to intracranial dural arteriovenous fistula: a potential diagnostic pitfall

Kim, Nam Hee; Cho, Keun Tae; Seo, Hyung Suk

doi:10.3171/2010.5.jns10128

Cited by 22 publications

(14 citation statements)

References 7 publications

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“…8) Although type V dural AVFs are rare, they are rapidly progressive in 25% of the cases. 9) It was extremely rare that the present case only manifested the sensory disorders of dysesthesia and hypalgesia. Therefore, an atypical dural AVF could lead to a delayed or incorrect diagnosis.…”

Section: Discussionmentioning

confidence: 68%

Dural Arteriovenous Fistula of the Transverse and Sigmoid Sinus Manifesting Ascending Dysesthesia: Case Report and Literature Review

Kamio

Hiramatsu

Yamashita

et al. 2015

NMC Case Report Journal

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Cases involving intracranial dural arteriovenous fistulas (AVFs) with spinal perimedullary venous drainage exhibit variable presentations, which results in delayed diagnoses. We describe a case of a 66-year-old female with a transverse-sigmoid sinus dural AVF with spinal perimedullary venous drainage who developed dysesthesia and hypalgesia that ascended from the peripheral lower extremities. Sixty cases of intracranial dural AVFs resulting in myelopathy have been reported, and an absence of brainstem signs significantly correlated with a delay in diagnosis (positive group: 3.4 months vs. negative group: 9.6 months, P < 0.05). Intracranial dural AVFs with brainstem signs should be diagnosed without delay because the myelopathy and bulbar symptoms could progress aggressively without alternative drainage routes besides the perimedullary veins. We emphasize that intracranial dural AVFs should be considered as a differential diagnosis in case presenting with symptoms, such as atypical dysesthesia and hypalgesia ascending from the toes, without brainstem signs. Moreover, we should perform cerebral angiography as early as possible because dural AVFs with slow-flow venous drainage can produce false negatives on magnetic resonance angiography.

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Section: Discussionmentioning

confidence: 68%

Dural Arteriovenous Fistula of the Transverse and Sigmoid Sinus Manifesting Ascending Dysesthesia: Case Report and Literature Review

Kamio

Hiramatsu

Yamashita

et al. 2015

NMC Case Report Journal

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“…This exceptionally rare type of DAVF can present with a wide array of symptoms due to the variable extent of venous congestive myelopathy. 10,11,17,19,24 The onset can either be sudden or gradual, making a solely clinical diagnosis impossible. 18,26,31 Therefore, clinicians confronted with such a lesion are frequently misdirected to a tentative diagnosis of brainstem and spinal cord infarction, Guillain-Barré syndrome, demyelination and inflammation, or-as in our case-a neoplasm.…”

Section: Discussionmentioning

confidence: 99%

Unilateral contrast-enhancing pontomedullary lesion due to an intracranial dural arteriovenous fistula with perimedullary spinal venous drainage: the exception that proves the rule

Roelz

Velthoven²,

Reinacher

et al. 2015

JNS

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Dural arteriovenous fistulas (DAVFs) are abnormal communications between dural arteries and dural venous sinuses, meningeal veins, or cortical veins. They account for 10%-15% of intracranial arteriovenous malformations.12,33 Grading of DAVF is based on the venous drainage pattern according to the classification schemes of Borden et al. and Cognard et al. 4,8 Venous cortical or perimedullary drainage and dilation are the hallmarks of high-grade DAVF and strongly correlate with increased risk of intracranial hemorrhage and nonhemorrhagic neurological deficit. The exact etiology of DAVFs is unknown. They are predominantly idiopathic, though they may also be congenital lesions or found in association with dural venous sinus thrombosis, trauma, previous craniotomy, and tumors. While spinal DAVF is an established cause of vascular myelopathy, intracranial DAVFs with perimedullary spinal venous drainage (Cognard Type V) are rare disorders and often represent a diagnostic and therapeutic challenge. They have been mainly described in case reports or small series that reported a typical clinical and radiological presentation of progressive myelopathy, central cord edema, and engorged perimedullary veins.Here, we report a case of an intracranial DAVF with exclusive perimedullary spinal venous drainage that presented initially with a very unusual MRI appearance suggestive of a right lateral brainstem neoplasm. After delayed diagnosis, the DAVF was completely occluded by transarterial embolization and the patient's symptoms gradually improved.abbreviatioNs DAVF = dural arteriovenous fistula; DSA = digital subtraction angiography; MRA = MR angiography. A large spectrum of possible diagnoses must be taken into consideration when a contrast-enhancing lesion of the pontomedullary region is found on MRI. Among these diagnoses are neoplastic, inflammatory, and infectious, as well as vascular pathologies. The authors report a rare case of an intracranial dural arteriovenous fistula (DAVF) with perimedullary spinal venous drainage (Cognard Type V) that initially presented as a unilateral contrast-enhancing pontomedullary lesion mimicking a brainstem neoplasm in a 76-year-old man. Following occlusion of the DAVF by transarterial embolization that resulted in clinical and radiological improvement, the fistula recurred 10 months later and was finally cured by a combined endovascular and surgical approach that resulted in complete occlusion. Clinical symptoms and MRI findings gradually improved following this treatment. A literature review on the MRI findings of Cognard Type V DAVF was performed. Centrally located medullary or pontomedullary edema represents the typical imaging finding, while unilateral edema as seen in the authors' patient is exceptionally rare. The hallmark imaging finding suggestive of DAVF consisting of perimedullary engorged vessels may not always be present or may only be very subtly visible. Therefore, the authors suggest performing contrast-enhanced MR angiography or even digital subtraction angiography in the ...

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“…Diagnosis of the dAVF can be confirmed by clinical impression and high-resolution imaging including computed tomography and MRI, and its type can be classified with cerebral angiography 4) . Congestion and swelling of the blood vessels of the spinal cord are useful markers for the initial diagnosis 4 7) . In our case, serpentine vascular signal void and diffuse edema of cervical spinal cord appeared on T2-weighted MRI.…”

Section: Discussionmentioning

confidence: 99%

“…After the negative finding of the spinal angiography, we decided immediately to perform brain CTA as a screening test for brain lesion, and we could make an early diagnosis of intracranial dAVF with spinal cord lesion. Early diagnosis and early management are important for the favorable prognosis of dAVF especially in the case of high-risk types, Borden type III and Cognard type V 4 7) .…”

Section: Discussionmentioning

confidence: 99%

Cervical Myelopathy Caused by Intracranial Dural Arteriovenous Fistula

Kim

Nam

et al. 2016

Korean J Spine

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Intracranial dural arteriovenous fistula (dAVF) usually results in various problems in the brain. But it can be presented as a myelopathy, which may make early diagnosis and management to be difficult. We recently experienced a case of cervical myelopathy caused by intracranial dAVF. A 60-year-old man presented with a 3-year history of gait disturbance due to a progressive weakness of both legs. Neurological examination revealed spastic paraparesis (grade IV) and Babinski sign on both sides. Magnetic resonance imaging showed serpentine vascular signal voids at C2-T1 on T2-weighted image with increased signal intensity and swelling of spinal cord at C1-C4. We performed a brain computed tomography angiography and found intracranial dAVF with multiple arteriovenous shunts. Venous drainages were noted at tentorial veins and cervical perimedullary veins. After Onyx embolization, the patient showed gradual improvement in motor power and gait disturbance. The venous drainage pattern is a well-known prognostic factor of dAVF. In our case, the intracranial dAVF drained to spinal perimedullary vein, which seemed to result in the ischemic myelopathy. Although it is rare condition, it sometimes can cause serious complications. Therefore, we should keep in mind the possibility of intracranial dAVF when a patient presents myelopathy.

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Myelopathy due to intracranial dural arteriovenous fistula: a potential diagnostic pitfall

Cited by 22 publications

References 7 publications

Dural Arteriovenous Fistula of the Transverse and Sigmoid Sinus Manifesting Ascending Dysesthesia: Case Report and Literature Review

Dural Arteriovenous Fistula of the Transverse and Sigmoid Sinus Manifesting Ascending Dysesthesia: Case Report and Literature Review

Unilateral contrast-enhancing pontomedullary lesion due to an intracranial dural arteriovenous fistula with perimedullary spinal venous drainage: the exception that proves the rule

Cervical Myelopathy Caused by Intracranial Dural Arteriovenous Fistula

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