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Anterior Spinal Artery Syndrome

Anterior spinal artery syndrome or Beck's syndrome or anterior cord syndrome is a rare neurovascular condition characterized by sudden ischemia with damage to the anterior 2/3rd of the spinal cord. The anterior spinal artery of Adamkiewicz which supplies this region of the spinal cord is susceptible to occlusion in the mid-lumbar region as the radicular artery supplying it is an end artery with no collateral circulation.


Presentation

Anterior spinal artery syndrome (ASAS) is a very rare condition which occurs following infarction of the anterior two-third of the spinal cord supplied by the anterior spinal artery. The cause of the infarction can be either iatrogenic or secondary to diseases. Common etiologies include mediastinal surgeries [1] [2], diabetes with atherosclerosis [3], diseases of the aorta [4], hematological disorders (sickle cell, polycythemia), cervical spine injury or spondylosis [5] [6] [7], infections (tuberculosis, N.meningitidis) [8] [9] [10], drugs (cocaine) [11], vasculitis, and idiopathic.

The common clinical presentation of ASAS is sudden onset, severe, pain along the spinal nerve roots radiating to the lower limbs with quadriparesis due to corticospinal tract involvement. The myelopathy can be associated with impaired bladder, bowel and sexual function depending upon the level at which the spinal cord is affected. Pain, as well as, temperature sensation are lost below the level of the infarction as the lateral spinothalamic tract is affected while the posterior column vibration and position sense are preserved. Orthostatic hypotension may be present due to autonomic dysfunction. Occasionally the spinal cord gray matter may be involved, preferentially with the preservation of sensory, bladder and bowel functions.

Lower Extremity Pain
  • The patient started complaining of spasmodic lower extremity pain and general weakness. She was unable to flex her knees upon request.[anesthesiology.pubs.asahq.org]
Wound Infection
  • The post-operative course was complicated by a temporary anterior spinal artery syndrome despite normal intraoperative somatosensory evoked potentials (SSEP) and by a wound infection requiring removal of the implant.[ncbi.nlm.nih.gov]
Sepsis
  • Causes of ASAS include aortic disease, thoracolumbar surgery, sepsis, hypotension, and thromboembolic disorders. Case reports of 2 patients.[ncbi.nlm.nih.gov]
Edema of Lower Extremity
  • A 51-year-old woman with a history of breast cancer developed pulmonary edema and lower extremity paraplegia with preservation of proprioception as the initial manifestation of abdominal aortic thrombosis.[ncbi.nlm.nih.gov]
Chest Pain Radiating to the Back
  • This was preceded by a two weeks' history of severe, sharp, lancinating, tearing left parasternal chest pain radiating to the back.[ncbi.nlm.nih.gov]
Protein S Deficiency
  • Individuals with protein S deficiency have an 8-fold increased risk of thrombosis.[ncbi.nlm.nih.gov]
  • The laboratory results showed a hereditary protein S deficiency.[ncbi.nlm.nih.gov]
Ulcer
  • We present a patient with systemic lupus erythematosus who developed an anterior spinal artery syndrome (ASAS) in association with livedo reticularis, leg ulcerations and thrombocytopenia.[ncbi.nlm.nih.gov]
Back Pain
  • Abstract Reported here is a 37-yr-old professional diving instructor who had developed complaints of back pain and weakness in the lower extremities after diving.[ncbi.nlm.nih.gov]
  • Back pain was present in the lumbar region radiating to buttocks and legs in a bilateral radicular pattern. Femoral pulses were initially palpable.[ncbi.nlm.nih.gov]
  • METHODS: A 63-year-old woman suffered from persistent severe back pain that radiated to both sides of the chest wall 1 week before medical consultation.[ncbi.nlm.nih.gov]
  • A 36-year-old woman developed sudden onset of back pain followed by evolving paraparesis and sensory loss consistent with anterior spinal artery distribution ischemia.[ncbi.nlm.nih.gov]
  • The following illustrates such a case: A 40 year old theatre nurse who recently had a long-haul flight woke up at 2 am complaining of back pain, chest pain and bilateral leg weakness. She was then brought to the emergency department.[oatext.com]
Hyperreflexia
  • Detrusor hyperreflexia was noted in 8 patients, a normal bladder in 1 and detrusor areflexia in 1. External urethral sphincter electromyography revealed detrusor-sphincter dyssynergia in 4 patients and normal findings in 6.[ncbi.nlm.nih.gov]
  • The acute stages are characterized by flaccidity and loss of deep tendon reflexes; spasticity and hyperreflexia develop over ensuing days and weeks.[oatext.com]
  • At first the lower limb paralysis was flaccid and areflexic but after several weeks spasticity, hyperreflexia, and Babinski signs appeared. After an initial period of incontinence bowel and bladder control was regained.[lksom.temple.edu]
  • Initially areflexia is present due to spinal shock but, hyperreflexia and spasticity appear later The most common form is anterior spinal artery syndrome.[sci-recovery.org]
Flaccid Paralysis of the Lower Extremity
  • Postoperative neurologic examination disclosed flaccid paralysis of the lower extremities and sphincter incontinence.[ncbi.nlm.nih.gov]
  • Anterior spinal artery syndrome manifests as flaccid paralysis of the lower extremities and bowel and bladder dysfunction with sparing of proprioception and sensation, due to the selective ischemia to the anterior portion of the cord.[openanesthesia.org]

Workup

ASAS should be suspected in any adult or child presenting with acute onset painful quadriparesis with preservation of posterior column sensations. History may indicate the etiology but a thorough physical and neurological examination are vital for diagnosis of the condition as well a to detect the level and extent of the neurological deficits. Routine laboratory tests such as complete blood count with differential, serum blood glucose, erythrocyte sedimentation rate (ESR), antinuclear antibody (ANA) levels, complement assay, nuclear antibody assays, serum lipids, serum electrolytes, and serology for syphilis should be ordered. An infectious etiology is indicated by leukocytosis while inflammatory markers may be elevated in infections as well as vasculitis. Besides diabetes, it is important to exclude coagulation disorders with tests like activated partial thromboplastin time, antiphospholipid antibody titer, protein C and protein S levels and platelet count [12] [13]. Cerebrospinal fluid analysis (CSF) is performed to look for infectious and autoimmune conditions while blood and CSF polymerase chain reaction (PCR) may be required to exclude viral etiologies.

However, the diagnosis of ASAS can only be confirmed with a magnetic resonance (MRI) scan of the spinal cord. This can detect all the causative lesions within or outside the spinal cord [14] [15] [16] [17] [18] [19]. Ideally, it should be performed at the earliest to avoid complications such as renal failure from developing [1]. A concomitant brain MRI may be useful in identifying lesions of multiple sclerosis, sarcoid, and other infections. A computed tomography (CT) scan and plain radiography do not have a significant role in the diagnosis of ASAS. If MRI is not available then CT myelography can help to detect tumors. Spinal angiography (arteriography) may be performed to identify an arteriovenous malformation

Other supportive tests in ASAS include electromyography (EMG) and nerve conduction velocity (NCV) tests to document neurological deficits and denervation changes. They also help to differentiate ASAS from polyneuropathy.

Temporal artery biopsy is indicated only if confirmation of giant cell arteritis as the underlying etiology of ASAS is suspected.

Treatment
  • The treatment has generally been supportive. We believe thrombolysis should be considered in the acute phase of this condition, and present a case with ASAS who experienced partial recovery after treatment given 4.5 h after symptom onset.[ncbi.nlm.nih.gov]
  • The patient recovered from the first episode of ASAS under corticosteroid treatment but remained paralytic after a second episode. Repeated magnetic resonance imaging of the spinal cord failed to show altered signal intensity.[ncbi.nlm.nih.gov]
  • Prevention and early treatment of specific complications, especially chronic pain, is of vital importance in young patients. This article describes our experiences diagnosing and treating a 16-year-old boy with anterior spinal artery syndrome.[ncbi.nlm.nih.gov]
  • The left vocal fold gradually regained mobility, permitting decannulation 3 months after treatment. This complicated vocal fold immobility was found to be due to adhesion and partial paralysis combined.[ncbi.nlm.nih.gov]
  • The clinical features, treatment and proposed pathophysiology for this condition are presented.[ncbi.nlm.nih.gov]

Prognosis
  • In our opinion, CMAP could be seen a marker of prognosis for ASAS patients, and absent CMAP might forecast the bad prognosis.[ncbi.nlm.nih.gov]
  • The prognosis is variable, in spite of considerable initial impairment restitution in certain cases can be very good. Proposals for investigations are given.[ncbi.nlm.nih.gov]
  • When the diagnosis of anterior cord syndrome is determined, the prognosis is unfortunate. The mortality rate is approximately 20%, with 50% of individuals living with anterior cord syndrome having very little or no changes in symptoms.[en.wikipedia.org]
  • WHAT IS THE PROGNOSIS FOR THOSE WITH ANTERIOR MYELOPATHY? Prognosis depends on individual factors such as the cause of the condition, presence of other medical complications, age, severity of the condition, etc.[medfriendly.com]
  • The prognosis is variable, in spite of considerable initial impairment restitution in certain cases can be very good. Proposals for investigations are given. 1987 S. Karger AG, Basel Article / Publication Details First-Page Preview[karger.com]

Etiology
  • Temporal artery biopsy is indicated only if confirmation of giant cell arteritis as the underlying etiology of ASAS is suspected.[symptoma.com]
  • Spinal cord infarction is a well-described, but rare, etiology of myelopathy, especially in children.[ncbi.nlm.nih.gov]
  • The possible mechanisms of spinal cord damage are discussed, and the relevant data regarding the etiology are reviewed.[ncbi.nlm.nih.gov]
  • We summarize the etiology, neurological findings and outcomes of 19 children found in the literature with anterior spinal artery syndrome.[ncbi.nlm.nih.gov]
  • The etiology is discussed with special reference to the possibility of a traumatic origin. The prognosis is variable, in spite of considerable initial impairment restitution in certain cases can be very good. Proposals for investigations are given.[ncbi.nlm.nih.gov]

Epidemiology
  • Thoracic (Lower) & Lumbar: 90% Sacral: 4% Cervical 3% Left Right: 2:1 Feeding vessels Location: Often lumbar Number: Usually 1; Occasionally 2 or 3 Fistula in dorsolateral root sleeve High venous pressure in spinal cord Reduced spinal cord perfusion Epidemiology[neuromuscular.wustl.edu]
  • However, no epidemiologic studies are available because of the relatively small number of patients affected.[emedicine.com]
  • […] lesion, and ipsilateral Babinski sign Horner syndrome in lesions above T1 Contralateral : loss of pain and temperature sensation one or two levels below lesion Spinal arteriovenous malformation Definition : congenital malformation of spinal blood vessels Epidemiology[amboss.com]
Sex distribution
Age distribution

Pathophysiology
  • The clinical features, treatment and proposed pathophysiology for this condition are presented.[ncbi.nlm.nih.gov]
  • […] and anatomical characteristics of the circulation of the anterior spinal artery in the cervical spinal cord, makes of the portion C3 to C5, the most vulnerable portion to ischemic damage, since the radicular arteries supply begins bellow C5 and the pathophysiology[ncbi.nlm.nih.gov]
  • Pathophysiological classification of human spinal cord ischemia. J Spinal Cord Med. 1997 Jan. 20(1):74-87. [Medline]. Cheng MY, Lyu RK, Chang YJ, Chen CM, Chen ST, Wai YY, et al.[emedicine.com]
  • […] above T1 Contralateral : loss of pain and temperature sensation one or two levels below lesion Spinal arteriovenous malformation Definition : congenital malformation of spinal blood vessels Epidemiology : usually presents in younger patients ( mid-20s ) Pathophysiology[amboss.com]
  • Pathophysiologic Features The source of massive hemoptysis is usually the bronchial circulation (90% of cases) rather than the pulmonary circulation (5%) (, 23 ).[pubs.rsna.org]

Prevention
  • For this reason avoidance of prolonged aortic cross clamp time, hypotension, and its associated low flow to the spinal cord, paying attention to prevent atheromatous embolization of Adamkiewicz artery and pelvic circulation can prevent this complication[ncbi.nlm.nih.gov]
  • Prevention and early treatment of specific complications, especially chronic pain, is of vital importance in young patients. This article describes our experiences diagnosing and treating a 16-year-old boy with anterior spinal artery syndrome.[ncbi.nlm.nih.gov]
  • Prevention may therefore require other neurophysiologic monitoring techniques.[ncbi.nlm.nih.gov]
  • Although there have been reported cases of spontaneous recovery, complete recovery is uncommon and awareness and prevention remains the mainstay of treatment.[ncbi.nlm.nih.gov]
  • Prevention may therefore require other neurophysiologic monitoring techniques. Full Text Link to Item Rivelli, Sarah K.[scholars.duke.edu]

References

Article

  1. Sohal AS, Sundaram M, Mallewa M, et al. Anterior spinal artery syndrome in a girl with Down Syndrome: case report and literature review. J Spinal Cord Med. 2009 Jun; 32(3): 349-353
  2. Brewer LA III, Fosburg RG, Mulder GA, Verska JJ. Spinal cord complications following surgery for coarctation of the aorta. A study of 66 cases. J Thorac Cardiovasc Surg. 1972;64(3):368–381.
  3. Satran R. Spinal cord infarction. Stroke. 1988;19(4):529–532.
  4. Sandson TA, Friedman JH. Spinal cord infarction. Report of 8 cases and review of the literature. Medicine (Baltimore). 1989;68(5):282–292.
  5. Hughes JT, Brownell B. Cervical spondylosis complicated by anterior spinal artery thrombosis. Neurology.1964;14:1073–1077.
  6. Schneider RC, Schemm GW. Vertebral artery insufficiency in acute and chronic spinal trauma, with special reference to the syndrome of acute central cervical spinal cord injury. J Neurosurg. 1961;18:348–360.
  7. Foo D, Rossier AB, Cochran TP. Complete sensory and motor recovery from anterior spinal artery syndrome after sprain of the cervical spine. A case report. Eur Neurol. 1984;23(2):119–123.
  8. Kumar R. Spinal tuberculosis: with reference to the children of northern India. Childs Nerv Syst. 2005;21(1):19–26.
  9. Liblau R, Chiras J, Orssaud C, et al. Spinal infarction in the anterior spinal territory with possible relation with bilharziasis. J Rev Neurol (Paris) 1991;147(8–9):605–608.
  10. O'Farrell R, Thornton J, Brennan P, et al. Spinal cord infarction and tetraplegia—rare complications of meningococcal meningitis. Br J Anaesthesiol. 2000;84(4):514–517.
  11. Schreiber AL, Formal CS. Spinal cord infarction secondary to cocaine use. Am J Phys Med Rehabil. 2007;86(2):158–160.
  12. Weidauer S, Nichtweiss M, Lanfermann H. Spinal cord infarction: MR imaging and clinical features in 16 cases. Neuroradiology. 2002 Oct; 44(10):851-7.
  13. Luo CB, Chang FC, Teng MM. Magnetic resonance imaging as a guide in the diagnosis and follow-up of spinal cord infarction. J Chin Med Assoc. 2003 Feb; 66(2):89-95.
  14. Weber P, Vogel T, Bitterling H, et al. Spinal cord infarction after operative stabilization of the thoracic spine in a patient with tuberculous spondylodiscitis and sickle cell trait. Spine. 2009 Apr 15; 34(8): E294-7.
  15. Joseph G, Santosh C, Marimuthu R. Spinal cord infarction due to a self-inflicted needle stick injury. Spinal Cord. 2004 Nov; 42(11):655-8.
  16. Hogan EL, Romanul FC. Spinal cord infarction occurring during insertion of aortic graft. Neurology. 1966 Jan; 16(1):67-74.
  17. Ross RT. Spinal cord infarction in disease and surgery of the aorta. Can J Neurol Sci. 1985 Nov; 12(4):289-95.
  18. Faivre A, Bonnel S, Leyral G, et al. Essential thrombocythemia presenting as spinal cord infarction.Presse Med. 2009 Apr 22.
  19. Lyders EM, Morris PP. A Case of Spinal Cord Infarction Following Lumbar Transforaminal Epidural Steroid Injection: MR Imaging and Angiographic Findings. AJNR Am J Neuroradiol. 2009 Oct;30(9):1691-3

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Last updated: 2019-07-11 20:09