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Spinal epidural arteriovenous fistula with nerve root enhancement mimicking myeloradiculitis: a case report
Sharon Chiang1,2, Douglas B Pet3, Jason F Talbott4
1Department of Neurology and Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA. Sharon.Chiang@ucsf.edu.
Background:
Gadolinium enhancement of spinal nerve roots on magnetic resonance imaging (MRI) has rarely been reported in spinal dural arteriovenous fistula (SDAVF). Nerve root enhancement and cerebrospinal fluid (CSF) pleocytosis can be deceptive and lead to a misdiagnosis of myeloradiculitis. We report a patient who was initially diagnosed with neurosarcoid myeloradiculitis due to spinal nerve root enhancement, mildly inflammatory cerebrospinal fluid, and pulmonary granulomas, who ultimately was found to have an extensive symptomatic SDAVF.
Case Presentation:
A 52-year-old woman presented with a longitudinally extensive spinal cord lesion with associated gadolinium enhancement of the cord and cauda equina nerve roots, and mild lymphocytic pleocytosis. Pulmonary lymph node biopsy revealed non-caseating granulomas and neurosarcoid myeloradiculitis was suspected. She had rapid and profound clinical deterioration after a single dose of steroids. Further work-up with spinal angiography revealed a thoracic SDAVF, which was surgically ligated leading to clinical improvement.
Conclusions:
This case highlights an unexpected presentation of SDAVF with nerve root enhancement and concurrent pulmonary non-caseating granulomas, leading to an initial misdiagnosis with neurosarcoidosis. Nerve root enhancement has only rarely been described in cases of SDAVF; however, as this case highlights, it is an important consideration in the differential diagnosis of non-inflammatory causes of longitudinally extensive myeloradiculopathy with nerve root enhancement. This point is highly salient due to the importance of avoiding misdiagnosis of SDAVF, as interventions such as steroids or epidural injections used to treat inflammatory or infiltrative mimics may worsen symptoms in SDAVF. We review the presentation, diagnosis, and management of SDAVF as well as a proposed diagnostic approach to differentiating SDAVF from inflammatory myeloradiculitis.
Insights
Spinal dural arteriovenous fistula (SDAVF) can mimic neurosarcoidosis due to nerve root enhancement on MRI. Correct diagnosis is crucial, as treatments for inflammatory conditions can worsen SDAVF symptoms.
Area of Science:
- Neurology
- Radiology
- Neurosurgery
Background:
- Spinal dural arteriovenous fistulas (SDAVF) rarely present with gadolinium enhancement of spinal nerve roots on MRI.
- Nerve root enhancement and CSF pleocytosis can lead to misdiagnosis of myeloradiculitis.
Observation:
- A patient with a longitudinally extensive spinal cord lesion showed nerve root enhancement and mild lymphocytic pleocytosis.
- Pulmonary granulomas and suspected neurosarcoid myeloradiculitis were noted.
- The patient deteriorated rapidly after steroid treatment.
Findings:
- Spinal angiography revealed a thoracic SDAVF.
- Surgical ligation of the SDAVF led to clinical improvement.
- This case highlights SDAVF presenting with nerve root enhancement and pulmonary granulomas, mimicking neurosarcoidosis.
Implications:
- SDAVF should be considered in the differential diagnosis of longitudinally extensive myeloradiculopathy with nerve root enhancement.
- Misdiagnosis of SDAVF can lead to adverse outcomes with treatments intended for inflammatory mimics.
- Accurate differentiation between SDAVF and inflammatory myeloradiculitis is critical for appropriate management.
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