Foix–Alajouanine syndrome: An uncommon cause of myelopathy

Abstract

Introduction: Foix–Alajouanine syndrome (FAS) is a rare cause of myelopathy caused by dural arteriovenous malformation of the spinal cord, mostly lower thoracic and lumbar. Progression to paraplegia may be slow. Spinal angiography is needed for definitive diagnosis, based on the clues provided by the symptoms. If diagnosed early, vascular embolization or neurosurgical excision may be curative. We report a new case and a review of the literature. Case report: A 59-year-old woman developed a spontaneous lumbar pain irradiating to the right entire. This pain with a radicular pattern of S1 was followed by progressive worsening of the pain and progressive leg weakness. Then, in a few days she developed a sudden spontaneous and severe low back pain with paresthesias in both legs that rapidly evolved to flaccid paraplegia, with sphincters dysfunction and complete reflex loss, resembling spinal shock. Neurological examination showed a flaccid arreflexic paraplegic patient, with sensory level loss at S1, and urinary retention. Babinski sign was present. Laboratory tests included both serological and immunological tests: herpes simplex, cytomegalovirus, VDRL, FTA-abs, hepatitis B, antinuclear antibodies, complement (C3, C4), beta 2 glycoprotein, ANCA and HTLV, were negative. A lumbar cerebrospinal fluid was performed and showed a normal formule. Lumbar spine MRI showed changes in keeping with multiple abnormal vascular structures at the upper portion of the spinal and an additional serpentine vessels involving the spinal roots. A spinal arteriography couldn’t be performed due to a major allergy to the contrast products. Unfortunately, the diagnosis of FAS was retained only after the second MRI showing a pictures of spinal necrosis, when any intervention couldn’t be efficient. Conclusions: In conclusion, spinal arteriovenous malformation has been associated with what has been known as Foix–Alajouaine syndrome. The clinical picture is a subacute progressive myelopathy. The FAS clinical high suspicion must led us to an early surgical conduct to patient benefit. Arteriography and MRI are sensitive diagnostics tools to vascular alterations, but can be inconclusive at some point. So, although this syndrome is rare, it must be known by clinicians to offer much more chances of recovery to such patients.