Clinical Outcomes of Patients with Delayed Diagnosis of Spinal Dural Arteriovenous Fistulas

W. Brinjikji; D.M. Nasr; J.M. Morris; A.A. Rabinstein; G. Lanzino

doi:10.3174/ajnr.A4504

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Fig 1.
A 57-year-old woman with a 3-month history of bilateral lower extremity tingling and progressive lower extremity weakness. A and B, T2-weighted lumbar spine MR images demonstrate high T2 signal in the conus with multiple flow voids in the intradural space. C, T2-weighted MR image of the thoracic spine demonstrates high T2 signal in the lower thoracic cord to the conus. The patient was diagnosed with neuromyelitis optica and received no spinal-vasculature imaging before referral to our institution. Two rounds of IV methylprednisolone (Solu-Medrol) therapy resulted in worsening of symptoms, and rituximab therapy was of no benefit. D, Spinal angiography demonstrates the spinal dural AVF with an arterial feeder from the L3 radiculomeningeal artery.
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Fig 2.
A 68-year-old man with a 3-month history of saddle anesthesia, constipation, difficulty voiding, and numbness in the lower extremities. T2-weighted images of the lumbar and thoracic spine demonstrate high T2 signal in the lower thoracic cord and conus (A and B). Due to clinical suspicion of SDAVF, an angiogram was obtained before referral to our center. C, The angiogram clearly demonstrates the fistula arising from the L2 radiculomeningeal artery; however, it was interpreted as a negative finding. Before the diagnosis was made, the patient underwent an extensive imaging and clinical evaluation, including a panel negative for paraneoplastic syndrome, PET/CT, and lumbar puncture. Two rounds of IV Solu-Medrol therapy resulted in worsening of symptoms. The patient also underwent a T10–T11 laminectomy and 2 spinal cord biopsies. D, Repeat spinal angiography re-demonstrates the fistula.

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Table 1:

Patient characteristics and additional procedures

	No. (%)
No.	53
Mean (SD) age	65.0 (10.8)
No. (%) male	43 (81.1)
Mean delay in diagnosis (mo)	9.2 ± 11.1
Symptoms at presentation
Bilateral motor symptoms	48 (90.6)
Sensory symptoms	20 (37.7)
Bowel or bladder symptoms	13 (24.5)
Focal unilateral motor deficit	3 (5.7)
Initial working diagnosis
Spinal stenosis	13 (24.5)
Myelopathy NOS	10 (18.9)
Transverse myelitis	9 (17.0)
Ischemic myelopathy	4 (7.6)
Peripheral neuropathy	3 (5.7)
Myopathy	2 (3.8)
NMO	2 (3.8)
CIDP	2 (3.8)
Other	8 (15.1)
Additional interventions
Systemic steroids	18 (34.0)
IVIG	5 (9.4)
Surgery	6 (11.3)
Biopsy	2 (3.8)
Plasma exchange	4 (7.6)
Rituximab	2 (3.8)
No. of additional spine MRIs or CTs until diagnosis
1	10 (18.9)
2	8 (15.1)
3	12 (22.6)
4	12 (22.6)
≥5	11 (20.8)

Note:—NOS indicates not otherwise specified; NMO, neuromyelitis optica; CIDP, chronic inflammatory demyelinating polyneuropathy.

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Table 2:

Imaging characteristics

Imaging Findings	No. (%)
High T2 cord signal (including conus)	46 (95.8)
Increased conus signal	44 (91.7)
Prominent intradural vessel on CT myelography or MRI	51 (96.2)
Cord enhancement	38 (79.2)
High T2 signal and flow void	44 (91.7)

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Table 3:

Clinical outcomes

	Presenting (No.) (%)	At Diagnosis (No.) (%)	90 Days after Treatment (No.) (%)
mRS
0	0 (0.0)	0 (0.0)	2 (3.9)
1	42 (79.2)	2 (3.8)	8 (15.7)
2	8 (15.1)	12 (22.6)	12 (23.5)
3	3 (5.7)	16 (30.2)	16 (31.4)
4	0 (0.0)	21 (39.6)	10 (19.6)
5	0 (0.0)	2 (3.8)	2 (3.9)
6	0 (0.0)	0 (0.0)	1 (2.0)
Aminoff motor score
0 (No deficit)	1 (1.9)	1 (1.9)	2 (4.0)
1 (Hyposthenia)	34 (64.2)	9 (17.0)	6 (12.0)
2 (Reduced tolerance)	6 (11.3)	11 (20.8)	5 (10.0)
3 (Need for cane)	12 (22.6)	15 (28.3)	14 (28.0)
4 (Need for crutches or walker)	0 (0)	15 (28.3)	11 (22.0)
5 (Patient in wheelchair)	0 (0)	17 (32.1)	12 (24.0)
Bowel or bladder symptoms
Yes	13 (24.5)	27 (50.9)	23 (45.1)
No	40 (75.5)	26 (49.1)	28 (54.9)
Sensory symptoms
Yes	20 (37.8)	28 (52.8)	20 (39.2)
No	33 (62.2)	25 (47.2)	31 (60.8)