Makindo Medical Notes.com

Foix-Alajouanine syndrome is an extreme form of spinal dural AVF that affects a minority of patients. These patients present with rapidly progressive myelopathy due to venous thrombosis from spinal venous stasis

About

• Can cause acute, subacute or chronic myelopathy
• Often like AV fistulas with high-pressure flow into the venous system

Aetiology

• Type 1 Dural AVMS 85% : Most common and cause venous hypertension and bleeding. Venous hypertension increases the size of the lesion and puts pressure on the cord as well as reducing blood flow. Corticospinal and posterior columns most affected. Management is spinal angiography and obliteration. Middle-aged men.
• Type 2 : Intradural AVMs or glomus AVM:Can bleed at arterial like pressure into the subarachnoid space. Seen in younger patients. progressive and fluctuating myelopathy interrupted by periods of acute neurologic deterioration secondary to haemorrhage within the AVM
• Type 3 Spinal AVM: Juvenile AVM: high flow and high pressure, bruit audible. Present as Type 2
• Type 4 Spinal AVM: Peri medullary Arteriovenous Fistula: rare

Clinical

• Painful radiculopathy may be present with Type 1
• Spinal cord compression symptoms
• Gradual onset of weakness and sensory symptoms
• Bladder and bowel disturbance
• Sudden haemorrhage with pain and weakness
• Intradural lesions cause subarachnoid haemorrhage and excruciating back pain referred to as coup de poignard.

Investigations

• MRI/MRA brain and cord to look for AVM
• Arteriography is the standard modality for visualizing arteriovenous malformations

Management

• Surgical removal can be considered
• Stereotactic radiosurgery to scar and cause the lesion to clot off
• Interventional neuroradiology/endovascular neurosurgery to insert some form of occlusive device