Intracranial dural arteriovenous fistula mistaken as cervical transverse myelitis

Abstract

We describe a man with an intracranial dural arteriovenous fistula that presented as a subacute longitudinally extensive cervical myelopathy. The uncommon location of the fistula and the absence of specific radiological signs resulted in initial misdiagnosis as longitudinally extensive transverse myelitis. Neurologists should have a high index of suspicion for dural arteriovenous fistula in older men, especially those with subacute or chronic symptoms, acellular cerebrospinal fluid and, particularly, if there is neurological deterioration soon after corticosteroid treatment. Patients need early angiography to identify this treatable cause of myelopathy.

Choroidal arteriovenous anastomoses: a hypothesis for the pathogenesis of central serous chorioretinopathy and other pachychoroid disease spectrum abnormalities

“…These AVFs are associated with tortuous vessel dilatation, venous hypertension, flow voids, haemorrhage and oedema, being strikingly reminiscent of choroidal abnormalities in pachychoroid spectrum diseases (Gandhi et al 2012;Laakso & Hernesniemi 2012;Nasr et al 2017;Spaide et al 2021). Intriguingly, after administration of corticosteroids, dural AVFs have been observed to induce (sub)acute worsening of neurological complaints (DiSano et al 2017;Nasr et al 2017;Peethambar et al 2019;Le Goueff et al 2020). This phenomenon has been described in multiple case reports of neurological patients that were initially misdiagnosed with for example an auto-immune neurological disorder, for which corticosteroids were prescribed.…”

Watch brain circulation in unexplained progressive myelopathy: a review of Cognard type V arterio-venous fistulas

“…It is important to notice that (1) mildly elevated CSF protein and absence of CSF pleocytosis (“albumino-cytological dissociation”) may occur in arteriovenous fistula and therefore should not necessarily be attributed to idiopathic transverse myelitis or Guillain-Barrè syndrome; (2) post steroid worsening should raise the suspicion about a non-inflammatory disease of the spinal cord, particularly a spinal or an intracranial fistula [ 19 ].…”

Tentorial dural arteriovenous fistula with perimedullary venous drainage–associated cervical myelopathy: illustrative case

“…1,9,[19][20][21][24][25][26] When embolization was attempted (8 of 20 cases), the procedure had to be repeated in 1 case, and in 4 others, microsurgery was the final treatment. 9,18,22,23,[27][28][29] The initial trigger of DAVFs is unknown. Nevertheless, in some cases such as ours, previous head trauma is reported.…”

“…Also, the embolization of meningeal ICA feeders is difficult; risky; and, as detailed in Table 1 , when attempted, the number of cases requiring additional treatment, predominantly microsurgery, was not negligible. 9 , 23 , 24 , 27 , 29 For this reason, given the feasibility and in order to avoid further risk or delay in a very fragile patient, we directly opted for the open procedure. Therefore, although endovascular treatment has become the leading therapy for intracranial DAVFs, tentorial DAVFs may require microsurgical discontinuation.…”