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].

• Asymptomatic

  This means that most individuals afflicted with this neuropathologic entity may be completely asymptomatic despite living with a “sword” hanging over their heads. [burtonreport.com]

  The term disease has both a count sense and a noncount sense, by contrast, an infection that is asymptomatic during its incubation period, but expected to produce symptoms later, is usually considered a disease. [wikivisually.com]

  Although dedicated spinal imaging studies were not routinely obtained on these patients, asymptomatic spinal AVMs are extremely uncommon (∼1%). 28 Discussion Diagnostic inaccuracy is common in overgrowth disorders, 29 particularly when vascular anomalies [ajnr.org]

  Whole body gallium scanning can disclose asymptomatic foci of systemic disease. Cranial MRI may show multiple white matter lesions and/or, in about a third of patients, meningeal enhancement. [jnnp.bmj.com]

• Anemia

  Coronary steal, Osteopoikilosis, Sinoatrial block, Idiopathic postprandial syndrome, Volkmann's contracture, Chylothorax, Biliary colic, Acute retinal necrosis, Fissured tongue, Myocardial stunning, Sneddon's syndrome, Mallet finger, Myelophthisic anemia [walmart.com]

• Pulmonary Valve Stenosis

  valve stenosis, Polymorphous light eruption, Marburg multiple sclerosis, Guyon's canal syndrome, Schmorl's nodes, Chorioamnionitis, Anti-NMDA receptor encephalitis, Perforated ulcer, Seckel syndrome, Esophagitis, Situs ambiguus, Posterior cerebral artery [walmart.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. [accesspediatrics.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]

• Increased Appetite

  Neurological features of Whipple's disease (in approximate order of frequency) Cognitive changes, dementia and/or psychiatric disease Supranuclear gaze palsy Pyramidal signs Hypothalamic features: somnolence, polydipsia, increased appetite, hypogonadism [jnnp.bmj.com]

• Hypertension

  View Frank Gaillard's current disclosures Foix-Alajouanine syndrome refers to subacute, progressive myelopathy due to venous hypertension from a spinal dural arteriovenous fistula. [radiopaedia.org]

  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]

  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]

  – Venous hypertension and progressive myelopahty due to type I dural AVM. DDs: Cord Demyelination. Cord Infarction. Tumor. Cord Contusion / Trauma. [neuroradiologycases.com]

  This type of drainage was then thought to prevent the venous hypertension in the spinal cord. [jnnp.bmj.com]

• 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]

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

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

• 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]

  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 syndrome or transverse [ncbi.nlm.nih.gov]

• Somnolence

  […] mesothelioma, Dysdiadochokinesia, Familial hypocalciuric hypercalcemia, Hereditary neuropathy with liability to pressure palsy, Distal muscular dystrophy, Cervicitis, Cartilage-hair hypoplasia, Paraphrenia, Lipodermatosclerosis, Renovascular hypertension, Somnolence [walmart.com]

  Neurological features of Whipple's disease (in approximate order of frequency) Cognitive changes, dementia and/or psychiatric disease Supranuclear gaze palsy Pyramidal signs Hypothalamic features: somnolence, polydipsia, increased appetite, hypogonadism [jnnp.bmj.com]

• Spastic Paraplegia

  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]

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]

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

Management and treatment Endovascular treatment is possible in around two thirds of cases. In the remaining cases, surgical disconnection of the emissary vein from the shunt zone leads to resolution of the fistula. [orpha.net]

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

Early recognition and surgical intervention can result in a good prognosis. VIEW THE ENTIRE ARTICLE HTML PDF References Foix C, Alajouanine T. La myélite nécrotique subaigue. Rev Neurol (Paris) 1926;2:1–42. Jellema K, Tijssen CC, van Gijn J. [ejcrim.com]

Prognosis - Foix Chavany Marie syndrome Not supplied. Treatment - Foix Chavany Marie syndrome Not supplied. Resources - Foix Chavany Marie syndrome Not supplied. [checkorphan.org]

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]

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

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 pathophysiology of AFS is still unclear but it is hypothesized that AFS is a progressive spinal cord venous thrombosis related to a spinal vascular lesions, resulting in necrotic myelopathy. [jns-journal.com]

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

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]

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

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]

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.