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From Chest Pain to Hemiparesis - A Case Study on Foix Alajouanine Syndrome FREE TO VIEW

Thankam Nair, MD; Randy Luu, MD; Karthik Reddy, MD; Venkat Banda, MD; Amer Awad, MD; Tonya Jagneaux, MD
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Baton Rouge General Medical Center Affiliated With Tulane University School of Medicine, Baton Rouge, LA

Chest. 2015;148(4_MeetingAbstracts):274A. doi:10.1378/chest.2267496
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SESSION TITLE: Critical Care Student/Resident Case Report Posters II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Venous congestive myelopathy (VCM) due to spinal arteriovenous fistulas (SDAF) was first described as necrotizing myelopathy by Foix and Alajouanine in 1926. One of the theories is that high arterial pressure within the dura is transmitted to the spinal venous plexus via the intradural venous system, compromising perfusion and leading to infarction of the spinal cord. Cervical cord involvement is rare.

CASE PRESENTATION: A 63-year-old woman with h/o epilepsy and hypertension presented with left-sided hemiparesis, left-sided pain, and burning substernal chest pain. Vitals on admission were stable. Initial stroke workup including head CT was negative. Persistent weakness led to MRI of the cervicothoracic spine, which showed extensive cervical and upper thoracic myelitis, and MRI of the thoracic spine showed extensive myelitis to T7, suspicious for necrotizing myelitis. Lumbar puncture was performed with normal results. The patient was started on pulse dose steroids for suspected neuromyelitis optica. Anti-NMO antibodies and a comprehensive autoimmune workup were negative. She continued to deteriorate and was transferred to the ICU with septic shock and bowel obstruction. In spite of maximal medical efforts the patient died. Autopsy showed extensive destructive lesions primarily involving the cervical upper/thoracic spinal cord with levels confined to the gray matter, and acute and subacute ischemia, with a more acute variable hemorrhagic component. Findings of pre-existing AV malformation in the region of the most severe change implicate the malformation described as Foix-Alajouanine Syndrome.

DISCUSSION: The exact etiology of Foix-Alajouanine Syndrome is unknown, however the onset in middle age suggests that this condition may be acquired. Spinal digital subtraction angiography is the gold standard for diagnosis. The choice of treatment is either endovascular embolization or surgical ligation of the fistula. Recent literature shows that the diagnostic features of SDAVF include a progressive course and acute worsening even with corticosteroids. Studies done in 38 patients by Lee et al. postulate that steroids may reduce retrograde perfusion from the venous side in patients with long-standing venous hypertension of the spinal cord, likely because they decrease the previously increased capillary permeability of the blood spinal cord barrier. In their experience, a history of symptom aggravation may suggest a hidden spinal AVM associated with VCM. Our patient had a similar course in her disease progression.

CONCLUSIONS: Foix-Alajouanine Syndrome is often misdiagnosed as polyneuropathy, sciatica, or polyradiculopathy, and is often under-reported. A high index of suspicion is required to diagnose this disease as early surgical intervention is shown to be beneficial.

Reference #1: Legacy and current understanding of the often-misunderstood Foix-Alajouanine syndrome.Ferrell AS et al

DISCLOSURE: The following authors have nothing to disclose: Thankam Nair, Randy Luu, Karthik Reddy, Venkat Banda, Amer Awad, Tonya Jagneaux

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