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].

• Unable to Stand

  At current presentation, he was unable to stand, even with support. Bilateral foot drop was present. Right lower limb power was grade 3/5 in hip flexors and knee extensors and 2/5 in knee flexors, ankle plantar flexors, and hip extensors. [ncbi.nlm.nih.gov]

• 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’m glad you are able to walk after surgeries. I also have difficulty with stairs if I over-exert myself the day before. [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

  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]

  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]

• 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]

  His dysesthesias and leg and gluteal pain also disappeared. A repeat MRI done at 5 months follow-up showed disappearance of flow voids as well as cord T2 hyperintensity [Figure 4]. [ncbi.nlm.nih.gov]

• Urinary Hesitancy

  The symptoms of subacute myelopathy are paraparesia, paresthesias, spasticity, and urinary hesitancy. These symptoms can be enhanced or exacerbated by Valsalva-like manoeuvers. [scielo.br]

• 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]

  Affected patients initially are spastic but eventually develop flaccid paralysis of the limbs and may become wheelchair bound. [accessanesthesiology.mhmedical.com]

  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]

• Foot Drop

  Bilateral foot drop was present. Right lower limb power was grade 3/5 in hip flexors and knee extensors and 2/5 in knee flexors, ankle plantar flexors, and hip extensors. [ncbi.nlm.nih.gov]

• Guillain-Barré Syndrome

  Foix-Alajouanine mimicking Guillain-Barre Syndrome Mitra Assadi, Benjamin M. Green, Larissa Bilaniuk, Christopher G. [amjcaserep.com]

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

  […] disk prolapse, spinal stenosis, intra- and extradural tumors of the spine, spinal infections, fractures, cystic lesions, hemorrhage and hematoma, arachnoiditis, as well as noncompressive lesions like demyelinating diseases of the cord.2,3 Occasionally Guillain-Barré [journals.sagepub.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]

• 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]

  […] amaurotic axonal idiocy|Spastic 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]

• Lower Motor Neurone Lesion

  neurone lesion (Clasp-knife response) Lower motor neurone lesion [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]

CASE DESCRIPTION: This case report on a young patient with an unusual clinical onset of Foix-Alajouanine syndrome coincidentally occurring after his outpatient clinic appointment illustrates how prompt surgical treatment can result in rapid recovery of [ncbi.nlm.nih.gov]

Operations, if successful, are permanent modes of treatment. [thehealthscience.com]

Operations, if successful, are permanent modalities of treatment. [emedicine.medscape.com]

The prognosis is poor if treatment is not administered before neurologic deterioration occurs. [thehealthscience.com]

In the last 20 years there is increasing knowledge regarding etiology and diagnosis of FCMS however there is very little, concerning assessment, prognosis and treatment of dysphagia in this population group. [ispub.com]

Etiology The etiology of Foix-Alajouanine syndrome is not well understood. Most patients have an AV fistula in the lower thoracic dura. [thehealthscience.com]

The first chapter stresses the etiology and pathophysiology of childhood stroke as the basis for evaluations and treatment. [books.google.com]

Summary Epidemiology The prevalence is unknown. The syndrome most commonly affects men over the age of 50. [orpha.net]

(See Etiology and Treatment.) [2, 5] Epidemiology Frequency United States No recent data exists describing the incidence or prevalence of patients with or treated for Foix-Alajouanine Syndrome in the United States or across other countries. [emedicine.medscape.com]

CONCLUSIONS: Venous hypertension with subsequent rapid resolution after surgical treatment is the pathophysiological mechanism underlying a dural arteriovenous fistula, in contrast to historical views suggesting that these lesions result from irreversible [ncbi.nlm.nih.gov]

The first chapter stresses the etiology and pathophysiology of childhood stroke as the basis for evaluations and treatment. [books.google.com]

We describe the different types of arteriovenous malformations and their pathophysiology. [elsevier.es]

The inclusion of thrombosis in the clinical picture of this syndrome is not only incorrect but may leave one with the impression of therapeutic futility, thus possibly preventing successful surgical or endovascular therapy. [ncbi.nlm.nih.gov]

Results: Our patient with a diagnosis of Foix-Alajuannine syndrome developed intramedullary hemorrhage while receiving anticoagulation therapy for prevention of deep vein thrombosis. [omicsonline.org]

Prevention of thrombotic, trophic and purulent complications is required. Read this article (multiple options) Related Papers [read.qxmd.com]

Early diagnosis and treatment can prevent the disease-progression. We report the case of a 40-year old man initially diagnosed of sciatica that evolved to acute paraparesia. Finally, he was diagnosed of spinal arteriovenous fistula. [elsevier.es]

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.