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Foix-Alajouanine Syndrome

Foix Alajouanine Syndrome

Foix-Alajouanine syndrome is a rare disorder characterized by the formation of an arteriovenous fistula in the spine and subsequent venous congestion leading to myelopathy. Initial signs and symptoms are nonspecific, which is why the diagnosis is often delayed for months. Imaging studies, such as magnetic resonance imaging and computed tomography, are necessary to visualize the fistula.


Presentation

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]
Orthopnea
  • Chylothorax, Biliary colic, Acute retinal necrosis, Fissured tongue, Myocardial stunning, Sneddon's syndrome, Mallet finger, Myelophthisic anemia, Ventricular aneurysm, Vesicovaginal fistula, Kohler disease, True hermaphroditism, Intestinal atresia, Orthopnea[walmart.com]
Bronchorrhea
  • […] cord syndrome, Progressive bulbar palsy, Eosinophilic fasciitis, Klumpke paralysis, Heavy chain disease, Diffuse myelinoclastic sclerosis, Gianotti-Crosti syndrome, Acute aortic syndrome, Hughes-Stovin syndrome, Peroxisomal disorder, Digestive disease, Bronchorrhea[walmart.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]
Colic
  • Barsy syndrome, Polyphagia, Hypervolemia, Preauricular sinus and cyst, Medial medullary syndrome, Branchial cleft cyst, Coronary steal, Osteopoikilosis, Sinoatrial block, Idiopathic postprandial syndrome, Volkmann's contracture, Chylothorax, Biliary colic[walmart.com]
Biliary Colic
  • colic, Acute retinal necrosis, Fissured tongue, Myocardial stunning, Sneddon's syndrome, Mallet finger, Myelophthisic anemia, Ventricular aneurysm, Vesicovaginal fistula, Kohler disease, True hermaphroditism, Intestinal atresia, Orthopnea, Melkersson-Rosenthal[walmart.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]
  • 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]
Spastic Paraplegia
  • 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]
  • 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]
Guillain-Barré Syndrome
  • Examples of emergency symptoms of Guillain-Barré syndrome include difficulty breathing, fainting, problems swallowing, drooling and temporary cessation of breathing.[empowher.com]
  • syndrome or transverse myelitis can present as CES.[journals.sagepub.com]
Spastic Paraplegia
  • 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]
  • 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]

Workup

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]

Treatment

  • 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 clinical and radiological features of spinal vascular malformations are reviewed because prompt treatment may halt or even reverse their neurological manifestations.[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]

Prognosis

  • The prognosis is poor if treatment is not administered before neurologic deterioration occurs.[thehealthscience.com]
  • PMID: 15954517 Prognosis Iovtchev I, Hiller N, Ofran Y, Schwartz I, Cohen J, Rubin SA, Meiner Z Spine J 2015 Jun 1;15(6):e39-44. Epub 2013 Oct 2 doi: 10.1016/j.spinee.2013.08.029.[ncbi.nlm.nih.gov]

Etiology

  • 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]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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.[ncbi.nlm.nih.gov]
  • 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]
  • Objective Present a case of Foix-Alajouanine syndrome that presented as acute cauda equina syndrome and discuss the pathophysiology and management.[pubfacts.com]
  • The typical pathophysiology of the disease consists of venous congestion in the spinal cord, leading to progressive ischemia and infarction. Thrombosis is also often an accompaniment in this lesion.[journals.sagepub.com]

Prevention

  • 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) Comments Related Papers[read.qxmd.com]
  • This particular book is a collaboration focused on Neurosurgery.More info: Neurosurgery (or Neurological Surgery) is the medical specialty concerned with the prevention, diagnosis, treatment and rehabilitation of disorders that affect the entire nervous[uae.souq.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 1) .[operativeneurosurgery.com]

References

Article

  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.

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Last updated: 2019-06-28 10:10