Foix–Alajouanine syndrome

Foix–Alajouanine syndrome
Other names: Familial osteosclerosis with abnormalities of the nervous system and meninges
T2 weighted MRI showing an arteriovenous malformation indicated by the cursor

Foix–Alajouanine syndrome, also called subacute ascending necrotizing myelitis,[1] is a disease caused by an arteriovenous malformation of the spinal cord.[2] In particular, most cases involve dural arteriovenous malformations that present in the lower thoracic or lumbar spinal cord. The condition is named after Charles Foix and Théophile Alajouanine who first described the condition in 1926.[3][4]

Signs and symptoms

The patients can present with symptoms indicating spinal cord involvement such as (paralysis of arms and legs, numbness and loss of sensation and sphincter dysfunction), and pathological examination reveals disseminated nerve cell death in the spinal cord.

Cause

The etiology of this syndrome finds a dural arteriovenous shunt of medullary emissary vein and is linked to reflux into the venous drainage of cord, causing chronic medullary ischemia.[5]

Diagnosis

a-c)Diffuse fusiform enlargement of spinal cord to level of conus medullaris together with ring-like enhancement at D12-L1 d) frontal view revealed enlarged vessels on the left side of the spinal canal

Clinically, the patient may present with neurological symptoms such as numbness, weakness, loss of reflexes, or even sudden or progressive paralysis.[6] The affected portion of the body will correlate to where the lesion lies within the spinal cord. The disease typically has an insidious onset, but symptoms may manifest suddenly. A thorough physical exam may lead a physician toward targeted imaging, with MRI being the most appropriate imaging modality for initial diagnosis. A spinal MRA will serve as a superior imaging technique to visualize the extent of the arteriovenous malformation within the cord and may be especially useful if surgical treatment is attempted.[7]

Treatment

Surgical treatment may be attempted with endovascular embolization or ligation of the arteriovenous malformation within the spinal cord.Corticosteroids may be used acutely to help slow the progression of symptoms or they may be used chronically in a poor surgical candidate.[8] In either case, physical therapy will be an important part of the recovery process in helping the patient regain strength and coordination.

See also

References

  1. ↑ "Foix-Alajouanine syndrome". Orphanet. Archived from the original on 2023-08-18. Retrieved 2023-05-10.
  2. ↑ Mishra R, Kaw R (May 2005). "Foix–Alajouanine syndrome: an uncommon cause of myelopathy from an anatomic variant circulation". South. Med. J. 98 (5): 567–9. doi:10.1097/01.SMJ.0000154409.48800.43. PMID 15954517. S2CID 41113400.
  3. ↑ Krishnan, Prasad; Banerjee, TapasKumar; Saha, Manash (2013). "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". Annals of Indian Academy of Neurology. 16 (3): 432. doi:10.4103/0972-2327.116931.
  4. ↑ "Foix-Alajouanine disease". www.whonamedit.com. Archived from the original on 2022-04-12. Retrieved 2022-04-12.
  5. ↑ RESERVED, INSERM US14-- ALL RIGHTS. "Orphanet: Foix Alajouanine syndrome". www.orpha.net. Archived from the original on 5 June 2022. Retrieved 14 August 2023.
  6. ↑ Palmer, Cheryl Ann. "Foix-Alajouanine Syndrome". Medscape. Archived from the original on 2022-02-20. Retrieved 2023-05-10.
  7. ↑ Koenigsberg, Robert (12 July 2022). "Arteriovenous Malformation Brain Imaging". Medscape. Archived from the original on 10 May 2023. Retrieved 10 May 2023 – via Medscape.
  8. ↑ D’haeseleer, Miguel (1 June 2014). "Systemic use of corticosteroids in neurological disorders". Journal of Translational Internal Medicine. 2 (2): 70–72. doi:10.4103/2224-4018.135603.
Classification
External resources


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