Foix–Alajouanine syndrome

T2 weighted spinal MRI showing AV malformation

Foix–Alajouanine syndrome is a rare neurological disorder characterized by a progressive myelopathy due to a spinal dural arteriovenous fistula (SDAVF). This condition is named after the French neurologists Charles Foix and Théophile Alajouanine, who first described it in the 1920s.

Pathophysiology

Foix–Alajouanine syndrome is caused by an abnormal connection between the arteries and veins in the dura mater of the spinal cord. This arteriovenous malformation (AVM) leads to venous hypertension, which in turn causes congestion and hypoxia of the spinal cord. The resulting ischemia can lead to progressive neurological deficits.

Clinical Presentation

Patients with Foix–Alajouanine syndrome typically present with a combination of motor, sensory, and autonomic dysfunction. Common symptoms include:

• Progressive weakness, often starting in the lower extremities
• Sensory disturbances, such as numbness or tingling
• Bladder and bowel dysfunction
• Pain in the back or legs

Diagnosis

The diagnosis of Foix–Alajouanine syndrome is often challenging and requires a high index of suspicion. Diagnostic tools include:

• Magnetic resonance imaging (MRI) of the spine, which may show spinal cord edema and flow voids indicative of an AVM
• Spinal angiography, which is the gold standard for identifying and localizing the SDAVF

Treatment

The primary treatment for Foix–Alajouanine syndrome is the surgical obliteration of the arteriovenous fistula. Endovascular techniques, such as embolization, may also be used in some cases. Early intervention is crucial to prevent irreversible spinal cord damage.

Prognosis

The prognosis for patients with Foix–Alajouanine syndrome varies depending on the timing of diagnosis and treatment. Early surgical intervention can lead to significant improvement in symptoms and prevent further neurological decline. However, delayed diagnosis and treatment may result in permanent neurological deficits.

Related Pages

• Arteriovenous malformation
• Spinal cord
• Neurology
• Magnetic resonance imaging
• Spinal angiography