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Clin Pract Cases Emerg Med 2022 Feb;6(1):13-16

Harbor-UCLA Medical Center, Department of Emergency Medicine, Torrance, California.

Introduction: There is a limited list of emergent spinal cord pathology that must be considered in patients with focal neurological deficits in the emergency department. Identification of these conditions requires a detailed history and neurological exam and may also require advanced testing and imaging.

Case Report: Here we present the case of a patient with a rare arteriovenous malformation of the spinal cord vessels causing congestive myelopathy (Foix-Alajouanine syndrome) that presented as a clinical mimic of spinal cord compression. Read More

Radiol Case Rep 2021 Aug 12;16(8):2187-2191. Epub 2021 Jun 12.

Department of Radiology, Tehran University of Medical Sciences (TUMS), Tehran, Iran.

Foix-Alajouanine syndrome is a rare progressive form of spinal AVM predominantly affecting the lower thoracic and/or lumbosacral regions. This study aims to describe the imaging findings of spinal AVM causing Foix-Alajouanine syndrome and to review the literature. We present a 48-year-old man with progressive back pain, leg weakness, and gait imbalance without urinary retention. Read More

Br J Neurosurg 2021 Apr 27:1-4. Epub 2021 Apr 27.

Department of Neurosurgery, Royal Sussex County Hospital, Brighton, UK.

Background: Spinal dural arteriovenous fistulas (SDAVF) are rare with an incidence of 5-10/million annually. They can be difficult to diagnose causing a delay in treatment with significant morbidity. We describe the first case in the literature of a symptomatic mirror lumbar SDAVF which may go unnoticed due to its unique vascular anatomy. Read More

Eur J Case Rep Intern Med 2020 10;7(12):002063. Epub 2020 Nov 10.

Department of Neurology, Henry Ford Health System, Detroit, Michigan, USA.

Foix-Alajouanine syndrome is an arteriovenous malformation causing subacute congestive myelopathy that can lead to progressive paraplegia. It typically affects the lower thoracic and lumbosacral levels. Arteriovenous fistula (AVF) leads to increased venous pressure, decreasing the arteriovenous pressure gradient and leading to a decrease in spinal cord perfusion, oedema and necrosis. Read More

Rev Assoc Med Bras (1992) 2017 Jul;63(7):564-565

Division of Neurosurgery, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brazil.

Subacute necrotizing myelopathy (SNM) or Foix-Alajouanine syndrome is a rare disease characterized by progressive neurological dysfunction caused by a spinal dural arteriovenous fistula (AVF). Radiological diagnosis is usually suspected when there is intramedullary nonspecific enhancement and perimedullary flow voids. Ring-enhancement is rarely reported in the scope of AVF, which poses a diagnostic challenge and raises the suspicion of a spinal cord tumor. Read More

World Neurosurg 2017 Dec 29;108:998.e7-998.e16. Epub 2017 Sep 29.

Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota, USA. Electronic address:

Objectives: Spinal dural arteriovenous fistulas (DAVFs) have diverse presentations. Magnetic resonance imaging (MRI) reveals spinal cord swelling in only 45% to 74% of cases. We present an unusual case of a 57-year old man with a craniocervical junction DAVF in which the edema appeared in the thoracolumbar region, skipping the cervical spinal cord. Read More

World Neurosurg 2017 Oct 30;106:206-210. Epub 2017 Jun 30.

Department of Neurosurgery, Ronald Reagan UCLA Medical Center, Los Angeles, California, USA; Department of Radiation Oncology, Ronald Reagan UCLA Medical Center, Los Angeles, California, USA; Department of Head and Neck Surgery, Ronald Reagan UCLA Medical Center, Los Angeles, California, USA; Jonsson Comprehensive Cancer Center, Ronald Reagan UCLA Medical Center, Los Angeles, California, USA; Department of Neurosurgery, Harbor-UCLA Medical Center, Torrance, California, USA; Los Angeles Biomedical Research Institute, Harbor-UCLA Medical Center, Torrance, California, USA. Electronic address:

Objective: To assess the use of functional scores in the evaluation of patients with dural arteriovenous fistula and Foix-Alajouanine syndrome.

Methods: We systematically surveyed the literature to identify relevant patients. Aminoff-Logue Scale (ALS) and modified Rankin Scale (mRS) scores were ascertained and combined to form a novel functional score, the Aminoff-Rankin Composite (ARC) score. Read More

World Neurosurg 2017 Jan 26;97:750.e1-750.e3. Epub 2016 Oct 26.

Division of Neurosurgery, Department of Clinical Neurological Sciences, London Health Sciences Centre, University Hospital, London, Ontario, Canada.

Background: Foix-Alajouanine syndrome is defined as acute neurologic deterioration in the setting of a spinal dural arteriovenous fistula.

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 neurologic function despite preoperative paraplegia.

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 venous thrombosis, resulting in necrotic myelopathy. Read More

No Shinkei Geka 2015 Aug;43(8):733-8

Department of Neurosurgery, Kashiwa Hospital, Jikei University School of Medicine.

Foix-Alajouanine syndrome (FAS), also known as congestive myelopathy due to spinal vascular malformations, presents with paraplegia, sensory disturbance of lower limbs, and dysfunction of the bladder and rectum. Although FAS is characterized by a subacute onset of neurological symptoms that may wax and wane over a few years, the progression mechanism remains unclear. We report a case of FAS due to an angiographically occult arteriovenous fistula (AVF) that was diagnosed by an open surgical biopsy and intraoperative indocyanine green (ICG) angiography. Read More

Pol J Radiol 2015 1;80:337-43. Epub 2015 Jul 1.

Department of Physiology, Baroda Medical College, Vadodara, India.

Background: Spinal arteriovenous malformations (AVMs) can lead to development of congestive myelopathy (Foix-Alajouanine syndrome). Spinal AVMs are rare and so is this syndrome. Diagnosis is often missed due to its rarity and confusing definitions of the Foix-Alajouanine syndrome. Read More

Zh Nevrol Psikhiatr Im S S Korsakova 2014 ;114(9):88-92

The article contains the description of Foix-Alajouanine syndrome (FAS) from literature. Three our own cases of FAS, which developed in two men and one woman, are presented. An analysis of FAS clinical picture revealed a step-like progression of the disease with a possibility of short-term fluctuation of some focal neurological signs. Read More

Global Spine J 2014 Dec 22;4(4):269-72. Epub 2014 May 22.

Department of Radiology, Khoula Hospital, Mina al Fahal, Muscat, Sultanate of Oman.

Study Design Case report. Objective Present a case of Foix-Alajouanine syndrome that presented as acute cauda equina syndrome and discuss the pathophysiology and management. Methods An adult male patient developed sudden onset of back pain and leg pain with weakness of the lower limbs and bladder/bowel dysfunction typical of cauda equina syndrome. Read More

Spine J 2015 Jun 2;15(6):e39-44. Epub 2013 Oct 2.

Department of Physical Medicine and Rehabilitation, PO Box 24035, Hadassah Hebrew-University Medical Center, Jerusalem, Israel. Electronic address:

Background Context: Spinal dural arteriovenous fistula (SDAVF) is a slow-flow extramedullary vascular lesion affecting primarily the lower thoracic and lumbar spine. The clinical sequela of these vascular changes is progressive myelopathy and severe lower-extremity weakness. Although surgical or embolic treatment of SDAVFs has improved significantly in the last years, the ambiguity of the symptoms may complicate and delay the diagnosis. Read More

Ann Indian Acad Neurol 2013 Jul;16(3):432-6

Department of Neurosurgery, National Neurosciences Centre, Peerless Hospital Complex, II Floor, 360 Panchasayar, Kolkata, West Bengal, India.

Spinal arteriovenous fistulas are rare entities. They often present with congestive myelopathy but are infrequently diagnosed as the cause of the patients' symptoms. Only one such case has been described previously in Indian literature. Read More

Brain Behav 2013 May 17;3(3):207-10. Epub 2013 Feb 17.

Department of Neurosurgery, School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York Buffalo, New York.

We present what is to our knowledge the first reported case of thoracic disc herniation leading to venous congestive myelopathy (VCM), which was clinically and radiographically suggestive of Foix-Alajouanine syndrome (angiodysgenetic necrotizing myelopathy). In addition, we review current concepts in evaluating the etiology of VCM and discuss indications for surgery. Read More

Surg Neurol Int 2012 21;3:87. Epub 2012 Aug 21.

Department of Neurosurgery, Institute of Clinical Medicine, University of Tsukuba, Ibaraki, Japan.

Background: Venous congestive myelopathy is a progressive myelopathy that is generally caused by a spinal dural arteriovenous fistula.

Case Description: We report a patient with histologically confirmed venous congestive myelopathy without concurrent vascular malformations in radiological and intraoperative findings.

Conclusions: The definitive underlying etiology of this congestive myelopathy was unclear. Read More

Coll Antropol 2011 Jan;35 Suppl 1:319-25

University of Zagreb, Zagreb University Hospital Center, Department of Neurology, Zagreb, Croatia.

Spinal arteriovenous malformations (AVM) have been devided into dural (Type I), intramedullary glomus (Type II), juvenile (Type III), and perimedullary direct arteriovenous fistulae (Type IV). AVMs are usually associated with subacute myelopathy in what has been known as Foix-Alajouanine syndrome. We presented two patients with two intradural spinal arteriovenous malformations associated in what we call Foix-Alajouanine syndrome. Read More

J Neurosurg 2009 Nov;111(5):900-1

J Neurosurg 2009 Nov;111(5):902-6

Department of Radiology, University of Alabama at Birmingham, Birmingham, Alabama 35294-3412, USA.

Foix-Alajouanine syndrome has become a well-known entity since its initial report in 1926. The traditional understanding of this clinical syndrome is as a progressive spinal cord venous thrombosis related to a spinal vascular lesion, resulting in necrotic myelopathy. However, spinal venous thrombosis is extremely rare and not a feature of any common spinal vascular syndrome. Read More

Eur J Neurol 2007 Aug;14(8):e4-5

Neuropathology 2007 Jun;27(3):284-9

Department of Neurology and Geriatrics, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu 501-1194, Japan.

We report a rapidly progressive myelopathy in a 74-year-old Japanese man who was admitted to our hospital with a 4-month history of progressive gait disturbance and died of pneumonia followed by respiratory failure on the 22nd day of admission. During the course of his illness, magnetic resonance imaging (MRI) revealed intramedullary lesions with edematous swelling from the medulla oblongata to the spinal cord at the level of the fourth vertebra. After administration of contrast medium, the ventral portion of the lesion was mildly and irregularly enhanced and a dilated vessel was recognized along the ventral surface of the upper cervical cord. Read More

Arq Neuropsiquiatr 2005 Jun 25;63(2B):527-9. Epub 2005 Jul 25.

Instituto de Neurologia de Curitiba, Curitiba, PR, Brazil.

In a 52-year-old woman, spinal arteriovenous malformation (AVM) has been associated with what has been known as Foix-Alajouanine syndrome. The pathophysiology of the AV fistula is probably related to increased venous pressure from the AVM plus thrombotic process. The most common initial symptoms are sensory disturbance, pain and leg weakness. Read More

South Med J 2005 May;98(5):567-9

Department of Internal Medicine and Section of Hospital and Peri-operative Medicine, Cleveland Clinic Foundation, Cleveland, OH, USA.

Foix-Alajouanine syndrome is a rare cause of myelopathy caused by dural arteriovenous malformation of the spinal cord, mostly lower thoracic and lumbar. Patients are usually over 50 years of age and can present with acute lower extremity dysesthesias or intermittent sciatica. Progression to paraplegia may be slow. Read More

No To Shinkei 1998 Dec;50(12):1133-41

Department of Neurology, School of Medicine, Fukushima Medical University, Japan.

A 63-year-old man, whose father died of malignant lymphoma, developed subacutely cauda equina/conus medullaris syndrome progressed over 3 months. Initial radicular pain, ascending motor and sensory paralysis without sacral sparing, vesicorectal dysfunction were similar with signs of spinal dural arteriovenous fistula. However, mild inflammatory signs, raised serum LDH, predominantly of LDH 3, lymphocytic pleocytosis and elevated beta 2 microglobulin in CSF suggested neurolymphomatosis. Read More

AJNR Am J Neuroradiol 1998 Aug;19(7):1267-73

Department of Radiology, Hospital of the University of Pennsylvania, Philadelphia 19104, USA.

Purpose: Dural arteriovenous fistulas (DAVFs) are acquired arteriovenous shunts located within the dura. The highly variable natural history and symptomatology of DAVFs range from subjective bruit to intracranial hemorrhage and are related to the lesion's pattern of venous drainage and its effect on the drainage of adjacent brain. We examined the prevalence and features of DAVFs in patients with progressive dementia or encephalopathy. Read More

Nervenarzt 1998 Apr;69(4):279-86

Abteilung für Neuroradiologie, Universitäts-Krankenhaus Eppendorf, Hamburg.

Congestive myelopathy, formerly referred to as varicosis spinalis or Foix-Alajouanine syndrome, is caused by a spinal dural arteriovenous fistula (SDAVF). So far, the blood supply from the meningeal arteries draining through the fistula into the medullary venous system can only be verified by spinal angiography. Patients predominantly male and over the age of 60 are afflicted. Read More

Neurosurgery 1997 Jun;40(6):1295-301

Department of Neurological Surgery, Neurological Institute of New York, Columbia-Presbyterian Medical Center, New York, New York, USA.

Objective And Importance: Spinal arteriovenous malformations have been divided by location into dural (Type I), intramedullary glomus (Type II), juvenile (Type III), and perimedullary direct arteriovenous fistulae (Type IV). We report two cases of an unusual intramedullary proliferation of hyalinized capillaries that do not fit into any of these categories.

Clinical Presentation: A 27-year-old woman and a 62-year-old man presented with subacute progressive caudal myelopathy. Read More

Clin Radiol 1993 Feb;47(2):134-6

Radcliffe Infirmary, Oxford.

We describe a patient with a classical presentation of a spinal dural arteriovenous malformation which probably underwent spontaneous thrombosis. This is known as the Foix-Alajouanine syndrome. The diagnosis was not made in this patient until after cord infarction had occurred. Read More

Acta Med Okayama 1991 Dec;45(6):451-6

Department of Neuropsychiatry, Okayama University Medical School, Japan.

An autopsy case of spinal arteriovenous malformation (AVM) was reported. The patient was a 75-year-old male and his initial neurologic symptoms were paraplegia, paresthesia below the umbilical level and urination difficulty. Subsequently night delirium and parkinsonism also appeared. Read More

Arkh Patol 1991 ;53(5):55-9

One observation of a rare disease--angiodysgenetic necrotizing myelopathy--in a 50-year-old patient is described. The diagnosis was established at necropsy and did not confirm suspected spinal cord tumor and myelitis. The disease is angiodysplasia with resulting necrotizing myelopathy. Read More