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]
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
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.
Diagnosisedit
Clinically, the patient may present with neurological symptoms such as numbness, weakness, loss of reflexes, or even sudden or progressive paralysis.[5] 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.[6]
Treatmentedit
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.[7] In either case, physical therapy will be an important part of the recovery process in helping the patient regain strength and coordination.
^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.
^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): 432–436. doi:10.4103/0972-2327.116931. PMC3788302. PMID 24101838.
^Palmer, Cheryl Ann. "Foix-Alajouanine Syndrome". Medscape.
^Koenigsberg, Robert (12 July 2022). "Arteriovenous Malformation Brain Imaging". Medscape.
^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.
foix, alajouanine, syndrome, also, called, subacute, ascending, necrotizing, myelitis, disease, caused, arteriovenous, malformation, spinal, cord, particular, most, cases, involve, dural, arteriovenous, malformations, that, present, lower, thoracic, lumbar, sp. 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 Theophile Alajouanine who first described the condition in 1926 3 4 Foix Alajouanine syndromeOther namesFamilial osteosclerosis with abnormalities of the nervous system and meningesT2 weighted MRI showing an arteriovenous malformation indicated by the cursorSpecialtyNeurology Contents 1 Signs and symptoms 2 Diagnosis 3 Treatment 4 See also 5 References 6 External linksSigns and symptoms editThe 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 Diagnosis editClinically the patient may present with neurological symptoms such as numbness weakness loss of reflexes or even sudden or progressive paralysis 5 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 6 Treatment editSurgical 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 7 In either case physical therapy will be an important part of the recovery process in helping the patient regain strength and coordination See also editVascular myelopathyReferences edit Foix Alajouanine syndrome Orphanet 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 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 3 432 436 doi 10 4103 0972 2327 116931 PMC 3788302 PMID 24101838 Foix Alajouanine disease www whonamedit com Retrieved 2022 04 12 Palmer Cheryl Ann Foix Alajouanine Syndrome Medscape Koenigsberg Robert 12 July 2022 Arteriovenous Malformation Brain Imaging Medscape 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 External links editsynd 1526 at Who Named It synd 1755 at Who Named It Retrieved from https en wikipedia org w index php title Foix Alajouanine syndrome amp oldid 1195829282, wikipedia, wiki, book, books, library,
