eMedicine Specialties > Neurology > Neuro-vascular Diseases

Foix-Alajouanine Syndrome: Differential Diagnoses & Workup

Author: Cheryl Ann Palmer, MD, Professor, Departments of Pathology and Neurology, University of Alabama at Birmingham School of Medicine; Consulting Staff, Departments of Pathology and Neurology, University of Alabama at Birmingham Hospital; Consulting Staff, Departments of Pathology and Neurology, Veteran Affairs Medical Center; Consulting Staff, Department of Pathology, Children's Hospital of Alabama
Contributor Information and Disclosures

Updated: Dec 10, 2008

Differential Diagnoses

Amyotrophic Lateral Sclerosis
Spinal Cord Infarction
Ankylosing Spondylitis
Spinal Epidural Abscess
Multiple Sclerosis
Syringomyelia
Polyradiculopathy
Vitamin B-12 Associated Neurological Diseases
Spinal Cord Hemorrhage

Other Problems to Be Considered

Lumbosacral disk syndromes
Cervical disk syndromes
Lumbosacral spondylosis
Primary or metastatic neoplastic disease
Spinal arachnoiditis
Spinal artery thrombosis
Spinal injury
Hereditary spastic paraplegias

Workup

Laboratory Studies

  • Obtain serum vitamin B-12 levels to exclude subacute combined degeneration caused by vitamin B-12 deficiency. B-12 levels should be normal in Foix-Alajouanine syndrome.
  • Consider testing for infections caused by human T-cell leukemia virus type 1 (HTLV1) or human immunodeficiency virus (HIV), as both can produce myelopathy.

Imaging Studies

  • CT or MRI studies
    • CT or MRI studies may be normal during the early stages of Foix-Alajouanine syndrome.
    • With disease progression, T1-weighted MRI images reveal swelling of the cord and decreased signal intensity peripherally within the affected spinal cord segments.
    • On T2-weighted images, the spinal cord lesions are hyperintense in central locations.
    • Contrast administration often produces serpentine areas of enhancement, and reveals the presence of enlarged tortuous vessels in the subarachnoid space with associated "flow void" phenomena.
  • Myelographic studies are not required but may be useful.
    • Irregular filling defects frequently are observed with myelography.
    • Conventional CT myelography also may be useful.
  • MR angiograms initially more correctly predict the site and extent of the fistula prior to the more invasive catheter angiography. They generally show flow in serpentine perimedullary vessels.
  • Catheter spinal angiography remains the criterion standard for the diagnosis of Foix-Alajouanine syndrome. It may demonstrate specific arterial feeders and draining dorsal veins.

Other Tests

  • Neurophysiologic studies such as somatosensory evoked potentials may be useful in evaluating this condition. They may reveal a conduction block in the large fiber sensory system rostral to the lesion either at or below the sensory level.
  • Electromyography and nerve conduction studies can exclude a peripheral nerve lesion or motor neuron disease. Thus, they also can assist in the localization of the lesion to the spinal cord.

Histologic Findings

Histologic findings include redundancy of veins within the cord and subarachnoid space. The dilated vessels have enormously thickened, hyalinized walls composed of abundant collagen and smooth muscle cells. Vascular thrombosis may be present. The gliotic spinal cord parenchyma beneath the dilated veins may show coagulative necrosis with exudation, fibrosis of the nerve roots, and ascending degeneration of the dorsal columns. Proliferation of intramedullary blood vessels frequently is observed and may be accompanied by fibrinoid degeneration of the vessel walls (see Media files 2-4). Hemosiderin deposition may be present, predominantly perivascular, and is indicative of previous bleeding.3

More on Foix-Alajouanine Syndrome

Overview: Foix-Alajouanine Syndrome
Differential Diagnoses & Workup: Foix-Alajouanine Syndrome
Treatment & Medication: Foix-Alajouanine Syndrome
Follow-up: Foix-Alajouanine Syndrome
Multimedia: Foix-Alajouanine Syndrome
References
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References

  1. Foix CH, Alajouanine T. La myelite necrotique subaigue. Rev Neurol. 1926;46:1-42.

  2. Krause F. Chirurgie des Gehirns und Ruckenmarks nach eigenen Erfarungen. Berlin: Urban & Schwarzenberg; 1911.

  3. Rodriguez FJ, Crum BA, Krauss WE, Scheithauer BW, Giannini C. Venous congestive myelopathy: a mimic of neoplasia. Mod Pathol. May 2005;18(5):710-8. [Medline].

  4. Mishra R, Kaw R. Foix-Alajouanine syndrome: an uncommon cause of myelopathy from an anatomic variant circulation. South Med J. May 2005;98(5):567-9. [Medline].

  5. Jellema K, Tijssen CC, van Gijn J. Spinal dural arteriovenous fistulas: a congestive myelopathy that initially mimics a peripheral nerve disorder. Brain. Dec 2006;129:3150-64. [Medline].

  6. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 12-1992. A 64-year-old woman with the abrupt onset of paraparesis after 10 months of increasing episodic leg weakness. N Engl J Med. Mar 19 1992;326(12):816-24. [Medline].

  7. Criscuolo GR, Oldfield EH, Doppman JL. Reversible acute and subacute myelopathy in patients with dural arteriovenous fistulas. Foix-Alajouanine syndrome reconsidered. J Neurosurg. Mar 1989;70(3):354-9. [Medline].

  8. Graham DI, Lantos PL, eds. Foix-Alajouanine syndrome. In: Greenfield's Neuropathology. 6th ed. York, NY: Oxford Univ Press; 1997:1101-1104.

  9. Kneisley LW, Dominguez MR, Bignami A, Rossier AB. Paraplegia following surgery in Foix and Alajouanine syndrome. (Arteriovenous malformation of the spinal cord). Paraplegia. Feb 1980;18(1):33-41. [Medline].

  10. Koeppen AH, Barron KD, Cox JF. Foix-Alajouanine syndrome. Acta Neuropathol (Berl). 1974;29(3):187-97. [Medline].

  11. Minami S, Sagoh T, Nishimura K, et al. Spinal arteriovenous malformation: MR imaging. Radiology. Oct 1988;169(1):109-15. [Medline].

  12. Schmidbauer M, Lassmann J, Pilz P, et al. Subacute diencephalic angioencephalopathy: an entity similar to angiodysgenetic necrotizing encephalopathy and Foix-Alajouanine disease. J Neurol. Aug 1992;239(7):379-81. [Medline].

  13. Welsh CT, Palmer CA, Townsend JJ. Radiologic pathologic correlation of spinal dural arteriovenous fistula (Foix-Alajouanine syndrome). Int J Neurorad. 1998;4 (1):51-55.

  14. Wrobel CJ, Oldfield EH, Di Chiro G, et al. Myelopathy due to intracranial dural arteriovenous fistulas draining intrathecally into spinal medullary veins. Report of three cases. J Neurosurg. Dec 1988;69(6):934-9. [Medline].

  15. Zweifler RM. Management of acute stroke. South Med J. Apr 2003;96(4):380-5. [Medline].

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Further Reading

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Keywords

angiodysgenetic necrotizing myelopathy, spinal dural arteriovenous fistula, subacute necrotizing myelopathy, venous congestive myelopathy, spinal cord necrosis, dural arteriovenous fistula, AV malformation of the spinal cord, spinal cord malformation, laminectomy

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Contributor Information and Disclosures

Author

Cheryl Ann Palmer, MD, Professor, Departments of Pathology and Neurology, University of Alabama at Birmingham School of Medicine; Consulting Staff, Departments of Pathology and Neurology, University of Alabama at Birmingham Hospital; Consulting Staff, Departments of Pathology and Neurology, Veteran Affairs Medical Center; Consulting Staff, Department of Pathology, Children's Hospital of Alabama
Cheryl Ann Palmer, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuropathologists, Medical Association of the State of Alabama, Society for Neuro-Oncology, and Southern Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Richard M Zweifler, MD, Chief of Neurology, Sentara Healthcare, Norfolk, VA
Richard M Zweifler, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, American Medical Association, American Stroke Association, Royal Society of Medicine, and Stroke Council of the American Heart Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Howard S Kirshner, MD, Professor of Neurology, Psychiatry and Hearing and Speech Sciences, Vice Chairman, Department of Neurology, Vanderbilt University School of Medicine; Director, Vanderbilt Stroke Center; Program Director, Stroke Service, Vanderbilt Stallworth Rehabilitation Hospital; Consulting Staff, Department of Neurology, Nashville Veterans Affairs Medical Center
Howard S Kirshner, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Heart Association, American Medical Association, American Neurological Association, American Society of Neurorehabilitation, National Stroke Association, Phi Beta Kappa, and Tennessee Medical Association
Disclosure: Boehringer Ingelheim Honoraria Speaking and teaching; BMS/Sanofi Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; Novartis Consulting fee Review panel membership

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Helmi L Lutsep, MD, Professor, Department of Neurology, Oregon Health and Science University; Associate Director, Oregon Stroke Center
Helmi L Lutsep, MD is a member of the following medical societies: American Academy of Neurology and American Stroke Association
Disclosure: Co-Axia Consulting fee Review panel membership; Talecris Consulting fee Review panel membership; AGA Medical Consulting fee Review panel membership; Boehringer Ingelheim Honoraria Speaking and teaching; Boston Scientific Honoraria Speaking and teaching; Concentric Medical None Review panel membership; Northstar Neuroscience  Review panel membership; ev3 Consulting fee Review panel membership

 
 
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