Foix-Alajouanine Syndrome Mimicking Longitudinally Extensive Transverse Myelitis
Rathnam AS, Zaman I, Memon AB. Foix-Alajouanine Syndrome Mimicking Longitudinally Extensive Transverse Myelitis. Neurology 2020; 94(15):3.
Objective: To describe an interesting case of Foix-Alajouanine Syndrome presenting as a longitudinally extensive transverse myelitis (LETM).
Background: Foix-Alajouanine Syndrome is caused by a spinal dural arteriovenous malformation and presents as paraparesis and progressive walking impairment. It most commonly involves the thoracolumbar region and affects elderly men. Though treatable, it is a cause of progressive myelopathy often misdiagnosed or missed.
Case Discussion: A 77-year-old man with a history of coronary artery disease presented with a one-year history of progressive lower extremity paresthesia, weakness, gait instability, and recurrent falls. He had no lumbar or lower extremity pain. He had no bladder incontinence. His symptoms were attributed to lumbar spinal stenosis and he underwent L3–L4 lumbar decompression at an outside hospital with no improvement. MRI of the lumbar spine without contrast done at the outside facility was reviewed and it showed an LETM from T8 through the tip of the conus medullaris. MRI of the entire spine was repeated with gadolinium. MRI lumbar spine with gadolinium showed flow voids at the dorsal aspect of T8–T9 consistent with a Type I spinal dural AV fistula. This was confirmed by the spinal angiogram. The patient had a negative MRI brain and cervical spine imaging. The spinal fluid analysis was unremarkable. Aquaporin-4 antibody and anti-MOG antibody tests were negative. He underwent a laminectomy with microsurgical obliteration of the AV fistula and regained 50% of his lower extremity strength within 48 hours of his surgery and continues to improve with physical therapy.
Conclusions: Foix-Alajouanine Syndrome should be considered in the differential diagnosis of LETM. It is a reversible cause of progressive myelopathy and needs a careful review of imaging, laboratory data, and clinical findings.