Acute Brainstem Dysfunction Caused by Cavernous Sinus Dural Arteriovenous Fistula

Area of Science:

• Neurology
• Vascular Neurology
• Neuroimaging

Background:

• Cavernous sinus dural arteriovenous fistulas (CS-DAVF) are abnormal connections between dura mater arteries and veins.
• Symptoms vary widely based on venous drainage patterns, with brainstem dysfunction being a rare but severe complication.

Observation:

• An 80-year-old woman presented with acute, isolated, and rapidly progressive brainstem dysfunction, including gait disturbance, confusion, dysarthria, and hemiparesis.
• Initial magnetic resonance imaging (MRI) showed pontine swelling without infarction, and magnetic resonance angiography (MRA) suggested a subtle abnormality near the cavernous sinus.
• Despite rapid deterioration to coma and worsening pontine swelling on MRI, digital subtraction angiography (DSA) confirmed a right CS-DAVF with posterior fossa venous reflux.

Findings:

• This case represents the first reported instance of CS-DAVF presenting solely with isolated and rapidly progressive brainstem dysfunction, unlike previous cases where orbital or ocular symptoms preceded neurological deficits.
• The acute presentation and lack of typical symptoms made the diagnosis challenging, initially mimicking inflammatory conditions.

Implications:

• Prompt diagnosis of CS-DAVF, even with atypical presentations, is crucial for preventing permanent neurological damage.
• Advanced neuroimaging techniques like contrast-enhanced computed tomography (CT) and MRI are essential for early detection.
• Emergent treatment is vital for patients with CS-DAVF presenting with acute neurological deterioration to improve outcomes.