Pearls & Oy-sters: Primary Cerebral Buerger Disease

Pearls

• Buerger disease (thromboangiitis obliterans [TAO]) typically occurs in men younger than 40–45 years who have a history of heavy tobacco use.

• The involvement of small and medium-sized vessels with normal proximal arteries and corkscrew-like collaterals in proximity to occluded arteries are angiographic features of TAO.

• Cessation of tobacco use is mandatory to stop disease progression and recurrent strokes.

Oy-sters

• Other causes of distal emboli and occlusions must be excluded for diagnosis of TAO.

• The corkscrew-like collaterals may be missed or misinterpreted on CT or magnetic resonance angiography.

• A primary cerebrovascular manifestation without peripheral vascular ischemic complications is rare but possible.

A 41-year-old man presented with acute left upper motor neuron facial palsy and dysarthria. He had a medical history of recurrent ischemic strokes at ages 26 and 35 years with residual minor right hemiparesis and coordination deficits. Previous strokes were classified as embolic stroke of undetermined source and secondary prevention with acetylsalicylic acid was initiated. The patient had no vascular risk factors except being a heavy smoker (>20 cigarettes/day) for 25 years. Besides occasional marijuana smoking, he did not have any illicit drug abuse. He had no family history of stroke, autoimmune conditions, or cancer.

Diffusion-weighted and fluid-attenuated inversion recovery MRI revealed acute infarction in the right and chronic infarction in the left middle cerebral artery territory (figure). We performed a comprehensive etiologic workup including serologic screening for coagulopathies and Fabry disease, drug screening, duplex ultrasound of extracranial and intracranial arteries, cardiac workup including Holter electrocardiogram for 72 hours, and transthoracic and transesophageal echocardiography, which were all unremarkable and particularly ruled out patent foramen ovale as potential cardioembolic stroke mechanism.

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Figure Imaging Findings of Cerebrovascular Buerger Disease

(A) Diffusion-weighted imaging of the brain. Arrows indicate acute infarction in the right middle cerebral artery territory (rostral insular and operculum). (B) Fluid-attenuated inversion recovery imaging of the brain. Arrows indicate chronic infarction in the left middle cerebral artery territory (frontal region). (C) Time-of-flight angiography of the cerebral arteries. The label indicates prominent vessels originating from the right anterior cerebral artery. (D) Maximum intensity projection images of 3D rotational angiography. The magnification indicates corkscrew-like collateral vessels in the proximity to an occluded branch of the right anterior cerebral artery. (E, F) Cerebral angiograms of the anterior and left middle cerebral arteries (E) and the posterior cerebral arteries (F). Arrowheads and magnification indicate corkscrew-like collateral vessels next to occluded vessels.

As time-of-flight (TOF) angiography showed no occlusion of proximal arteries but prominent small vessels in the right anterior cerebral artery territory (figure) suggesting arteriovenous malformation (AVM), we decided to perform digital subtraction angiography (DSA), which ruled out AVM but revealed multiple stenoses and occlusions of distal cerebral arteries surrounded by corkscrew-like collateral vessels (figure). In retrospect, these corkscrew-like collaterals originating from the right anterior cerebral artery could already be seen on the TOF images (figure).

With the suspicion of cerebral vasculitis, we performed antibody screening for systemic vasculitis including antineutrophil cytoplasmic (ANCA), antinuclear (ANA), and extractable nuclear antigens (ENA-A), cardiolipin, and anti-double stranded DNA antibodies (anti-dsDNA), with negative results. CSF analysis was unremarkable (cell count 3 MPt/L [reference <5], protein level 388 mg/L [150–450 mg/L], glucose 3.56 mmol/L [2.8–4.4 mmol/L], lactate 1.66 mmol/L [1.3–2.4 mmol/L]), including antigen tests for Lyme disease, neurosyphilis, and herpes simplex virus and varicella-zoster virus PCR. We further discussed leptomeningeal biopsy, which was declined by the patient.

Based on the absence of other stroke etiologies, DSA results, age, and smoking status, we suspected Buerger disease (TAO) as stroke etiology. Consistently, Doppler ultrasound of lower extremities showed clinically asymptomatic occlusions of crural arteries bilaterally, which were confirmed by DSA. The patient did not report any Raynaud phenomenon, extremity pain, or ischemia. Antibody testing showed G-protein receptor autoantibodies directed against α1 receptor and endothelin A receptor. Levels of erythrocyte sedimentation rate (4 mm/1 hour [reference <15 mm/1 hour]) and C-reactive protein (0.7 mg/L [<5.0 mg/L]) were within normal limits.

With the diagnosis of TAO, we strongly recommended cessation of tobacco and cannabis use. The patient received oral varenicline medication and was referred to our smoking cessation outpatient clinic and stroke follow-up program. At discharge, the patient had recovered from facial palsy and dysarthria. The patient reduced (<5 cigarettes/day) but did not quit smoking after 3 months of follow-up. During this time course, he had no further cerebral or peripheral vascular ischemic complications.