April 14, 2020; 94 (15 Supplement) Monday, April 27 First published April 14, 2020, Abstract Objective: To describe an interesting case of Foix-Alajouanine Syndrome presenting as a longitudinally extensive transverse myelitis (LETM). [n.neurology.org]

The condition is named after Charles Foix and Théophile Alajouanine who first described the condition in 1926.[3][4] Signs and symptoms[edit] The patients can present with symptoms indicating spinal cord involvement such as (paralysis of arms and legs [en.wikipedia.org]

On the other hand, dural AV fistulas typically cause progressive myelopathy and only 10-15% present an acute presentation. [scielo.br]

At current presentation, he was unable to stand, even with support. 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]

• Weakness

  Early development of weakness may portend a more aggressive course. Less commonly, weakness or bowel and bladder dysfunction may be presenting symptoms. [dixion-healthcare.com]

  This pain with a radicular pattern of S1 was followed by progressive worsening of the pain and progressive leg weakness. [scielo.br]

  He had noted bilateral lower extremity weakness at the time, but this was thought to be secondary to deconditioning from the prolonged admission. [westjem.com]

  Patients have paraparesis (leg weakness), sensory loss in the legs, and loss of bowel and bladder sphincter control 1. Spinal dural arteriovenous fistula results in venous hypertension 1. [radiopaedia.org]

  Case Discussion: A 77-year-old man with a history of coronary artery disease presented with a one-year history of progressive lower extremity paresthesia, weakness, gait instability, and recurrent falls. He had no lumbar or lower extremity pain. [n.neurology.org]

• Falling

  Case Discussion: A 77-year-old man with a history of coronary artery disease presented with a one-year history of progressive lower extremity paresthesia, weakness, gait instability, and recurrent falls. He had no lumbar or lower extremity pain. [n.neurology.org]

  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]

  No satisfactory explanation of abrupt falls was ever given. [slideheaven.com]

  Classifying FCMS based solely upon lesions yields five specific subtypes of FCMS currently known to fall into the bilateral and unilateral categories: [1] [3] Bilateral anterior opercular syndrome (lesion in both the anterior or in the frontal operculum [en.wikipedia.org]

• Wheelchair Bound

  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. [2] Affected patients initially are spastic but eventually develop flaccid paralysis of the limbs and may become wheelchair bound. [emedicine.medscape.com]

• Swelling

  The diagnosis of dural AV fistulas can be made by MRI that, typically, shows swelling of medullary conus and slight central spinal enhancement. [scielo.br]

  One study showed that 40–63% of patients have the disease for one to three years, and 10–34% have it for more than three years before they are accurately diagnosed.9 The MRI of an affected patient may show swelling of the medullary conus and some central [westjem.com]

• Hyperthermia

  There are no indications that malignant hyperthermia occurs; however, avoiding trigger anesthetic agents during the acute phase of neuromuscular degeneration is recommended. [accessanesthesiology.mhmedical.com]

• Fecal Incontinence

  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]

• Choking

  Another proposed mechanism is that remittent leakage of blood from the arteriovenous malformation promotes progressive adhesive arachnoiditis resulting in iterative “chokes” of the spinal cord. [accessanesthesiology.mhmedical.com]

• Thrombosis

  However, spinal venous thrombosis is extremely rare and not a feature of any common spinal vascular syndrome. [thejns.org]

  Pathologically, they had an endomesovasculitis with vascular wall thickening but no thrombosis. [radiopaedia.org]

  Therefore, the low-flow aspect plus venous congestion predisposes to venous thrombosis as final event. [scielo.br]

  Thrombosis may occur, but venous stasis is probably the primary cause of infarction. Proliferation of intramedullary blood vessels is frequently observed and may be accompanied by fibrinoid degeneration of the vessel walls. [accessanesthesiology.mhmedical.com]

  PMID: 19344217 Case report: spontaneous thrombosis of a spinal dural AVM (Foix-Alajouanine syndrome)--magnetic resonance appearance. Renowden SA, Molyneux AJ. Renowden SA, et al. Clin Radiol. 1993 Feb;47(2):134-6. doi: 10.1016/s0009-9260(05)81190-0. [pubmed.ncbi.nlm.nih.gov]

• Back Pain

  Cauda Equina Syndrome: Defined as low back pain, bladder and bowel dysfunction, and variable lower extremity motor and sensory loss. [accessanesthesiology.mhmedical.com]

  Then, in a few days she developed a sudden spontaneous and severe low back pain with paresthesias in both legs that rapidly evolved to flaccid paraplegia, with sphincters dysfunction and complete reflex loss, resembling spinal shock. [ejinme.com]

  These findings rise our attention, about the need to keep in mind the clinical suspicion of AVM in cases of back pain and motor deficit, and an early surgical conduct in this situation. Key words: paraplegia, AV fistula, venous thrombosis. [scielo.br]

  DISCUSSION Back pain with associated bowel and/or bladder incontinence, saddle anesthesia, numbness, or weakness of the lower extremities is concerning for emergent spinal pathology. [westjem.com]

• Low Back Pain

  Then, in a few days she developed a sudden spontaneous and severe low back pain with paresthesias in both legs that rapidly evolved to flaccid paraplegia, with sphincters dysfunction and complete reflex loss, resembling spinal shock. [ejinme.com]

  Cauda Equina Syndrome: Defined as low back pain, bladder and bowel dysfunction, and variable lower extremity motor and sensory loss. [accessanesthesiology.mhmedical.com]

  After 3 months of conservative treatment (rest, analgesics), she developed a sudden spontaneous and severe low back pain with paresthesias in both legs that rapidly evolved to flaccid paraplegia, with sphincters dysfunction (urinary retention) and complete [scielo.br]

• Incontinence

  He denied any saddle anesthesia, leg numbness, bowel incontinence, weight loss, intravenous drug use, fevers, or recent trauma. [westjem.com]

  He had no bladder incontinence. His symptoms were attributed to lumbar spinal stenosis and he underwent L3–L4 lumbar decompression at an outside hospital with no improvement. [n.neurology.org]

  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]

  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]

  […] than ocular Anatomical cases Clinical cases 66 56 55 38 22 17 10 9 8 150 96 82 52 42 35 20 14 19 Dysarthria Hyperactive deep tendon reflexes, spasticity Dysphagia Babinski sign Jaw-jerk Explosive crying, exaggerated emotional responses Glabellar reflex Incontinence [slideheaven.com]

• Myelopathy

  Subacute necrotizing myelopathy: MR imaging in four pathologically proved cases. AJNR Am J Neuroradiol. 1991; 12(6):1077-83. 2 Kim RC. Necrotizing myelopathy. [scielo.br]

  Spinal dural arteriovenous fistulas: a congestive myelopathy that initially mimics a peripheral nerve disorder. Brain 2006;129:3150–3164. Nasr DM, Brinjikji W, Rabinstein AA, Lanzino G. [ejcrim.com]

  In retrospect, it seems possible that both patients had progressive myelopathy due to Type I dAVFs. [thejns.org]

  Based on this understanding of the syndrome, treatment and reversal of the progressive myelopathy was considered futile. However, recent literature shows that spinal venous thrombosis may not be responsible for the myelopathy. [westjem.com]

  The high flow in these fistulas caused intramedullary venous hypertension and the entity of congestive myelopathy. [ncbi.nlm.nih.gov]

• Stroke

  […] societies: American Academy of Neurology, American Stroke Association Disclosure: Medscape Neurology Editorial Advisory Board for: Stroke Adjudication Committee, CREST2; Physician Advisory Board for Coherex Medical; National Leader and Steering Committee [emedicine.medscape.com]

  The type of strokes associated with this syndrome include embolic and thrombotic strokes. [en.wikipedia.org]

  Autopsy diagnosis of progressive supranuclear palsy mistaken clinically for stroke. Case report, Mo, Med., 75, 227-229, 1978. [153] SCI'U.EIDEZ M.A., NAGURNEY J.T.: Progressive supranuclear ophthalmoplegia. [slideheaven.com]

• Paresthesia

  Case Discussion: A 77-year-old man with a history of coronary artery disease presented with a one-year history of progressive lower extremity paresthesia, weakness, gait instability, and recurrent falls. He had no lumbar or lower extremity pain. [n.neurology.org]

  Then, in a few days she developed a sudden spontaneous and severe low back pain with paresthesias in both legs that rapidly evolved to flaccid paraplegia, with sphincters dysfunction and complete reflex loss, resembling spinal shock. [ejinme.com]

  The symptoms of subacute myelopathy are paraparesia, paresthesias, spasticity, and urinary hesitancy. These symptoms can be enhanced or exacerbated by Valsalva-like manoeuvers. [scielo.br]

• Seizure

  This procedure can result in a complete recovery from epileptic seizures. [3] Physical therapy is also used to manage symptoms and improve quality of life. [en.wikipedia.org]

  Among other miscellaneous signs (Table X), muscular wasting with denervation potentials, seizures, and sleep changes were encountered on some occasions. [slideheaven.com]

• Flaccid Paralysis

  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. [2] Affected patients initially are spastic but eventually develop flaccid paralysis of the limbs and may become wheelchair bound. [emedicine.medscape.com]

To the best of our knowledge, the ED workup and management for this rare syndrome has not been reviewed in the literature. [westjem.com]

(See Workup.) Gross photograph of the dorsal surface of the spinal cord showing dilated and tortuous vessels. [emedicine.medscape.com]

• Fibrinoid Necrosis

  Intramedullary blood vessels frequently showed fibrinoid necrosis and there was proliferation of blood vessels within necrotic parenchyma. [link.springer.com]

Treatment of spinal cord vascular malformations by surgical excision. J. Neurosurgery, 30, 427–435. Article Google Scholar Logue, V (1976). Practical aspects of surgical treatment. In Spinal Angiomas, pp. 131–136. M. J. Aminoff (ed.). [nature.com]

Operations, if successful, are permanent modalities of treatment. [emedicine.medscape.com]

[citation needed] Treatment[edit] 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 [en.wikipedia.org]

Endovascular and surgical treatment of spinal dural arteriovenous fistulas. Neuroradiology 2008;50(10):869–876. HTML PDF Published: 2020-11-10 Issue: Vol 7 No 12 (view) Section: Articles How to cite: 1. Rathnam AS, Memon AB. [ejcrim.com]

Cited by 1 article Arteriovenous fistula of the filum terminale: diagnosis, treatment, and literature review. Fischer S, Aguilar Perez M, Bassiouni H, Hopf N, Bäzner H, Henkes H. Fischer S, et al. [pubmed.ncbi.nlm.nih.gov]

Prognosis likely depends on the severity of the spinal pathology and the timeliness of diagnosis and intervention. Patients who present with rapidly progressing neurological deficits may have a worse prognosis. Early diagnosis is critical. [emedicine.medscape.com]

Early recognition and surgical intervention can result in a good prognosis. VIEW THE ENTIRE ARTICLE HTML PDF References Foix C, Alajouanine T. La myélite nécrotique subaigue. Rev Neurol (Paris) 1926;2:1–42. Jellema K, Tijssen CC, van Gijn J. [ejcrim.com]

Factors determining prognosis are said to be duration of illness, sphincter involvement at presentation and location of fistula however, there is no consensus on any of these.[ 1 ] Before our case, only one such has been published in Indian literature [ncbi.nlm.nih.gov]

Etiology The etiology of Foix-Alajouanine syndrome is not well understood. Most patients have an AV fistula in the lower thoracic dura. [emedicine.medscape.com]

It was not until later that the etiology was proposed to be a dural arteriovenous fistula, an entity that was not known at the time of the original publication. [radiopaedia.org]

No classification has been possible in the face of ignorance of etiologic factors; pathogenesis has been equally uncertain, and the interpretation of pathologic material has produced no uniformity of opinion regarding the nature of the morbid changes. [jamanetwork.com]

The etiology of the Foix-Alajouanine syndrome remains unknown but the lesions are probably acquired. Access options Buy single article Instant access to the full article PDF. 43,34 € Tax calculation will be finalised during checkout. [link.springer.com]

(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]

If suspicion is high for spinal AVM and Foix-Alajouanine syndrome, the gold standard for diagnosis is spinal angiography, which would typically follow MRI.10,11 Given the rarity of the disease, epidemiological data are limited; however, one study reported [westjem.com]

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. [scielo.br]

The pathophysiology of spinal vascular malformations. J Neurol Sci. 1974;23(2):255-63. 9. Jellema K, Tijssen CC, van Gijn J. Spinal dural arteriovenous fistulas: a congestive myelopathy that initially mimics a peripheral nerve disorder. [westjem.com]

Pathophysiology The enlarged, abnormal veins are associated with dural AV shunts or fistulas, usually intradurally, but rarely extradurally. [1] These AV shunts are associated with reflux of arterial blood into the venous drainage of the cord. [emedicine.medscape.com]

Spinal intradural arteriovenous fistulas acquired in late adulthood: Absent spinal venous drainage in pathogenesis and pathophysiology. Report of two cases. J Neurosurg Spine. 2005; 3 :488–94. [ PubMed ] [ Google Scholar ] [ncbi.nlm.nih.gov]

*'a: clinical diagnosis d: pathophysiology e: EEG, EMG, neuroradiologic procedures, high function tests f: biochemistry and pharmacology g: nosography Symptoms o f PSP reported as appearing within the first year from the onset. [slideheaven.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. [thejns.org]

The spinal vascular lesions can cause neurologic deficits, including paralysis. [2, 3] Early recognition of the association between the vascular skin lesions and associated spinal lesions may prevent or minimize neurological sequela. [emedicine.medscape.com]

Therefore, despite of some controversies, the surgical procedure is the standard treatment, preventing clinical deterioration. Improvements are often possible to some extent. [scielo.br]