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Foix-Alajouanine Syndrome Clinical Presentation

  • Author: Cheryl Ann Palmer, MD; Chief Editor: Helmi L Lutsep, MD  more...
 
Updated: Jun 08, 2016
 

History

Patients present with increasing unilateral and/or bilateral weakness, dysesthesias, and numbness or tingling in the lower extremities, which may be symmetrical or asymmetrical.[6] Early problems with bowel, bladder, and sexual function are common.[7]

After brief exertion, symptoms begin as a heavy feeling in the legs that generally improves with rest. The symptoms gradually worsen over months, and the patient may have difficulty standing for long periods. Frequent falls can be a problem. Urinary and fecal incontinence eventually occur.

Complaints of nonradiating lower back pain in the lumbosacral or coccygeal regions are common. This may initially be interpreted as sciatica. Weakness or numbness eventually can progress to the upper extremities.

In most patients, Foix-Alajouanine syndrome follows a protracted course over a few years before a diagnosis is made.[5] In a minority of patients, however, an acute onset of symptoms is reported.

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Physical Examination

A physical examination can reveal the following:

  • Neurologic examination - Reveals an alert patient with normal mentation
  • Mental status, speech, language, and cranial nerve function - Generally are normal
  • Unsteadiness of gait - Common; may be halting in nature but on a narrow base
  • Spastic or flaccid paraparesis and a sensory level below the lesion
  • Deep tendon reflexes - May be normal or increased
  • Bilateral Babinski signs - May be present, as may clonus; upper motor neuron and lower motor neuron signs may be seen simultaneously [5]
  • Vibration and joint position senses - Usually are preserved
  • Rectal sphincter tone - Frequently is diminished
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Contributor Information and Disclosures
Author

Cheryl Ann Palmer, MD Professor of Pathology, Director of Pathology Residency Program, Director of Neuropathology, ARUP Laboratories, University of Utah School of Medicine

Cheryl Ann Palmer, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuropathologists, Society for Neuro-Oncology, International Society of Neuropathology

Disclosure: Nothing to disclose.

Coauthor(s)

Meghan J Driscoll, MD Resident Physician, Department of Pathology, University of Utah School of Medicine

Meghan J Driscoll, MD is a member of the following medical societies: College of American Pathologists, Academy of Clinical Laboratory Physicians and Scientists, Society for Pediatric Pathology, Wyoming Public Health Association

Disclosure: Nothing to disclose.

Chief Editor

Helmi L Lutsep, MD Professor and Vice Chair, Department of Neurology, Oregon Health and Science University School of Medicine; Associate Director, OHSU Stroke Center

Helmi L Lutsep, MD is a member of the following medical societies: American Academy of Neurology, American Stroke Association

Disclosure: Medscape Neurology Editorial Advisory Board for: Stroke Adjudication Committee, CREST2.

Acknowledgements

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: BMS/Sanofi Honoraria Speaking and teaching

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

Richard M Zweifler, MD Chief of Neurology, Sentara Healthcare, Norfolk, VA; Professor of Neurology, Eastern Virginia Medical School, 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.

References
  1. Krishnan P, Banerjee TK, Saha M. Congestive myelopathy (Foix-Alajouanine Syndrome) due to intradural arteriovenous fistula of the filum terminale fed by anterior spinal artery: Case report and review of literature. Ann Indian Acad Neurol. 2013 Jul. 16(3):432-6. [Medline]. [Full Text].

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

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

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

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

  6. Iovtchev I, Hiller N, Ofran Y, Schwartz I, Cohen J, Rubin SA, et al. Late diagnosis of spinal dural arteriovenous fistulas resulting in severe lower-extremity weakness: a case series. Spine J. 2013 Oct 2. [Medline].

  7. Sivakumar W, Zada G, Yashar P, Giannotta SL, Teitelbaum G, Larsen DW. Endovascular management of spinal dural arteriovenous fistulas. A review. Neurosurg Focus. 2009 May. 26(5):E15. [Medline].

  8. Andres RH, Barth A, Guzman R, et al. Endovascular and surgical treatment of spinal dural arteriovenous fistulas. Neuroradiology. 2008 Oct. 50(10):869-76. [Medline].

  9. Zhao LB, Shim JH, Lee DG, Suh DC. Two microcatheter technique for embolization of arteriovenous fistula with liquid embolic agent. Neurointervention. 2014 Feb. 9(1):32-8. [Medline].

  10. Hessler C, Regelsberger J, Grzyska U, Illies T, Zeumer H, Westphal M. Therapeutic clues in spinal dural arteriovenous fistulas - a 30 year experience of 156 cases. Cen Eur Neurosurg. 2010 Feb. 71(1):8-12. [Medline].

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

  12. Thorn, A. Spinale durale arteriovenöse Fisteln. Der Radiologe. November 2001. 41:955-960.

 
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Gross photograph of the dorsal surface of the spinal cord showing dilated and tortuous vessels.
Photomicrograph of the cervical spinal cord region showing a thickened subarachnoid vein with a thrombotic occlusion (hematoxylin and eosin stain).
Photograph of the cervical spinal cord illustrating dilated, abundant subarachnoid veins (hematoxylin and eosin stain).
Photomicrograph of the cervical spinal cord region demonstrating several dilated, hyalinized intraparenchymal vessels (hematoxylin and eosin stain).
Photomicrograph of the cervical spinal cord depicting ischemic necrosis of the parenchyma (hematoxylin and eosin stain).
 
 
 
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