Formation of a spinal dural arteriovenous fistula (SDAVF) is the clinical hallmark of Foix-Alajouanine syndrome [1] [2]. Two types are recognized - ventral, in which shunting between the anterior spinal artery and a draining vein occurs; and dorsal, described when arteriovenous communication is established at the level of a dural root sleeve [3]. Over time, venous congestion of the spinal cord and thrombosis (but without hemorrhage) ensues, resulting in myelopathy and the appearance of non-specific symptoms - gait disturbances, ranging from mild to severe enough to impair walking, paresthesias and sensory deficits (mostly involving distal lower extremities in an asymmetrical fashion), leg weakness, and micturition problems (urinary hesitancy and retention) [1] [2] [3] [4]. Additionally, anal sphincter disturbance, cramping, erectile dysfunction and severe leg pain, are encountered in the majority patients in whom the diagnosis is delayed, and a mean delay of 15 months was observed in some reports [1] [4] [5]. The absence of symptoms related to the upper limbs is one of the main distinguishing features of Foix-Alajouanine syndrome since fistulas very rarely develop in the cervical spine. In fact, the mid-thoracic and lumbar spine are most frequent sites where SDAVFs are diagnosed [1] [5]. A slowly progressive clinical course over months or years is typical for Foix-Alajouanine syndrome [3], but acute episodes of deterioration were encountered after prolonged standing or exercise [1].

• Progressive Dementia

  We examined the prevalence and features of DAVFs in patients with progressive dementia or encephalopathy. Read More Download full-text PDF Source Unusual intramedullary vascular lesion: report of two cases. [pubfacts.com]

• Difficulty Walking

  I started having difficulty walking and incontinence. I went to my Neurologist who ordered a lot of test to rule out MS, ALS, NOS, etc. My neurologist started me on IV steroid treatment, thinking it may be MS. [avmsurvivors.org]

• Recurrent Chest Pain

  Review of systems was significant only for shortness of breath, and recurrent chest pain was relieved with nitroglycerin. He was a retired administrator currently living in New Jersey, who did not smoke, drink, or use drugs. [go.gale.com]

• Fecal Incontinence

  Other symptoms are fecal incontinence, and/or urinary incontinence. Treatment : How to Treat "foix-alajouanine syndrome"? Surgical procedures can treat this condition & post procedure medications. Otherwise, corticosteroids may be advised. [signssymptoms.org]

  Other symptoms are fecal incontinence, and/or urinary incontinence. Causes This rare disease is caused by blood vessel malformations. The blood vessels affected are responsible for supplying the spinal cord. [medigest.uk]

  Progressive paraplegia (manifested as increasing weakness and numbness or tingling in the lower extremities, frequent falls), urinary and fecal incontinence, and nonradiating lower back pain. [accessanesthesiology.mhmedical.com]

• Chest Pain

  CASE PRESENTATION: A 63-year-old woman with h/o epilepsy and hypertension presented with leftsided hemiparesis, left-sided pain, and burning substernal chest pain. Vitals on admission were stable. [kundoc.com]

  Review of systems was significant only for shortness of breath, and recurrent chest pain was relieved with nitroglycerin. He was a retired administrator currently living in New Jersey, who did not smoke, drink, or use drugs. [go.gale.com]

• Leg Pain

  Additionally, anal sphincter disturbance, cramping, erectile dysfunction and severe leg pain, are encountered in the majority patients in whom the diagnosis is delayed, and a mean delay of 15 months was observed in some reports. [symptoma.com]

  Case Report A 54-year-old male presented with gradually progressive history of walking difficulty, weakness, and decreased sensation in lower limbs, diffuse gluteal, and leg pain and bowel and bladder incontinence of 3 years duration. [ncbi.nlm.nih.gov]

• Flaccid Paralysis

  Synonym(s): subacute necrotizing myelitis Foix-Alajouanine syndrome - thrombophlebitis of spinal veins resulting in a subacute ascending painful flaccid paralysis from necrotic myelitis. [medical-dictionary.thefreedictionary.com]

  paralysis, often ascending, loss of sphincter control, and progressive sensory loss. [medicine.academic.ru]

  Prognosis Foix-Alajouanine syndrome is a subacute disorder that gradually evolves over 1-5 years. [1] Affected patients initially are spastic but eventually develop flaccid paralysis of the limbs and may become wheelchair bound. [thehealthscience.com]

  Alajouanine in two men who had experienced several years of progressive flaccid paralysis (1,2). [path.upmc.edu]

• Guillain-Barré Syndrome

  They are presumed to be acquired anomalies, creating a direct communication between the radicular artery and the draining vein.Case Report: We describe a patient with rapidly progressive paraplegia who was misdiagnosed and treated as Guillain-Barré Syndrome [amjcaserep.com]

  Examples of emergency symptoms of Guillain-Barré syndrome include difficulty breathing, fainting, problems swallowing, drooling and temporary cessation of breathing. [empowher.com]

  Everything immediately went down hill and he thought it maybe Guillain-Barré syndrome and I was admitted to the hospital for possible plasmapheresis. [avmsurvivors.org]

  syndrome or transverse myelitis can present as CES. [journals.sagepub.com]

• Spastic Paraplegia

  Initially, patients have a spastic paraplegia which progresses to flaccidity, loss of sphincter control and ascending sensory level. It is thought to be due to venous hypertension. Promoted articles (advertising) [radiopaedia.org]

  Foix-Alajouanine syndrome a necrotizing myelopathy characterized by necrosis of the gray matter of the spinal cord, thickening of the walls of the spinal vessels, and abnormal spinal fluid; symptoms include subacute spastic paraplegia of the lower extremities [medicine.academic.ru]

  paraplegia 35 Sporadic cerebellar degeneration Sporadic cerebellar degeneration (disorder) ICD 10 CM 2017, (Clinical Modification) for coding of death certificates and mortality data. [averbis.com]

• Dysmetria

  […] ophthalmoplegia One and a half syndrome Midbrain (CN 3, 4) Weber's syndrome ventral peduncle, PCA Benedikt syndrome ventral tegmentum, PCA Parinaud's syndrome dorsal, tumor Nothnagel's syndrome Claude's syndrome Other Alternating hemiplegia Cerebellum lateral (Dysmetria [en.wikipedia.org]

• Dystonia

  […] syndrome ventral tegmentum, PCA Parinaud's syndrome dorsal, tumor Nothnagel's syndrome Claude's syndrome Other Alternating hemiplegia Cerebellum lateral (Dysmetria Dysdiadochokinesia Intention tremor) medial (Cerebellar ataxia) Basal ganglia Chorea Dystonia [en.wikipedia.org]

The diagnosis of Foix-Alajouanine syndrome and SDAVF may be difficult to attain, especially in early stages of the disease. For this reason, workup must start with a detailed patient history that will identify the onset of symptoms and their features, followed by a rigorous physical examination. Physicians must conduct a meticulous neurological exam that will identify all deficits, as initial diagnosis of disorders involving the spinal cord can be made if the exam is done properly. Imaging studies, however, are necessary to confirm the presence of a fistula. Magnetic resonance imaging (MRI) of the spine can reveal T2 enhancement in the lower part of the spinal cord (conus medullaris), the presence of serpentine vessels involving the spinal roots and end-on vessels on the sagittal images, grossly abnormal perimedullary vascular flow and spinal cord edema [2] [3] [4]. Because the procedure often provides sufficient clues that support the initial diagnosis, it should be performed first, followed by more specialized techniques - digital subtraction angiography (DSA), spinal computed tomography angiography (CTA) and spinal magnetic resonance angiography (MRA) [3]. DSA is considered to be the gold standard, as it provides the most detailed view of the arterial and venous vessels [3]. But because a very high radiation dose is required, CTA and MRA are more frequently performed [3].

• Spinal Cord Necrosis

  The patients eventually succumbed to sepsis; at autopsy there was spinal cord necrosis associated with enlarged, tortuous, hyalinized veins on the surface of the cord and numerous small fibrotic blood vessels within cord parenchyma. [path.upmc.edu]

1. Jellema K, Canta LR, Tijssen CC, van Rooij WJ, Koudstaal PJ, J van Gijn J. Spinal dural arteriovenous fistulas: clinical features in 80 patients. J Neurol Neurosurg Psychiatry 2003;74:1438–1440.
2. Menon KV, Sorour TMM, Raniga SB. Foix-Alajouanine Syndrome Presenting as Acute Cauda Equina Syndrome: A Case Report. Global Spine J. 2014;4(4):269-272.
3. Sood D, Mistry KA, Khatri GD, et al. Congestive Myelopathy due to Intradural Spinal AVM Supplied by Artery of Adamkiewicz: Case Report with Brief Literature Review and Analysis of the Foix-Alajouanine Syndrome Definition. Pol J Radiol. 2015;80:337-343.
4. Muralidharan R, Saladino A, Lanzino G, Atkinson JL, Rabinstein AA. The clinical and radiological presentation of spinal dural arteriovenous fistula. Spine (Phila Pa 1976). 2011;36(25):E1641-1647.
5. Bordignon KC, Montú MB, Ramina R, Arruda WO. Foix-Alajouanine syndrome: case report. Arq Neuropsiquiatr. 2005;63(2B):527-529.