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.
Entire Body System
- Sepsis
Causes of ASAS include aortic disease, thoracolumbar surgery, sepsis, hypotension, and thromboembolic disorders. Case reports of 2 patients. [ncbi.nlm.nih.gov]
Causes of ASAS include aortic disease, thoracolumbar surgery, sepsis, hypotension, and thromboembolic disorders. Methods: Case reports of 2 patients. [tandfonline.com]
Finally, died of sepsis complications and multiorgan failure. DISCUSSION Paraplegia following heart and aorta interventions and surgery is an uncommon complication. [scielo.br]
The prognosis of CCS is poor, with a 5-year mortality rate of 55% and the majority of mortality being associated with malnutrition, hypoalbuminemia, repetitive infection, sepsis, heart failure and GI bleeding ( 8 ). [spandidos-publications.com]
Recombinant Thrombomodulin Used to Successfully Treat Cronkhite-Canada Syndrome with Disseminated Intravascular Coagulation due to Sepsis in a Compromised Patient. Intern Med. 2018 Nov 1. 57 (21):3079-3085. [Medline]. [emedicine.medscape.com]
- 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]
- Hypersomnia
[…] prescribed off-label, using diagnostic Read code lists, compiled by two clinical academics, a GP and a psychiatrist.18 These non-SMI conditions comprised depression, anxiety disorders, sleep disorders (insomnia, non-specific sleep disorders, apnoea, hypersomnia [bmjopen.bmj.com]
Gastrointestinal
- Vomiting
A diligent search was made for any medullary involvement in view of giddiness and vomiting but repeated brain imaging did not reveal any diffusion restriction. [jneuro.com]
Gastrointestinal problems in Cronkhite–Canada syndrome include: Nausea and vomiting, loss of appetite, and weight loss, often with cachexia (physical wasting and malnutrition) Upper abdominal pain, watery diarrhoea, and melaena (bleeding) Swelling and [dermnetnz.org]
These people may also have abnormally low levels of protein in the blood (protein-losing enteropathy), causing a feeling of general ill health (cachexia), malnutrition, nausea and vomiting. [rarediseases.org]
They can be used to reduce the required dosage of steroids, however, they can also present with the following side effects: High blood pressure Nausea and vomiting Body parts tingling or becoming numb Reducing your appetite A rash on your skin Your hair [essilor.co.uk]
Cardiovascular
- Hypertension
The literature supports the view that hypotension is more detrimental to spinal cord perfusion, and short episodes of hypertension are generally well tolerated especially in hypertensive patients [ 3 Gottesman M.H. Saraya I. Tenti F. [annalsthoracicsurgery.org]
Ten days prior to her death, a 52-year-old hypertensive female developed severe chest pain and lower extremity weakness. A diagnosis of aortic dissection resulting in an anterior spinal artery syndrome was made. [ncbi.nlm.nih.gov]
Musculoskeletal
- 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]
Infarction is suspected when severe back pain and characteristic deficits develop suddenly. Diagnosis of spinal cord infarction is by MRI. [merckmanuals.com]
Psychiatrical
- Fear
This is the most feared, though rare complication of bronchial artery embolization done in massive hemoptysis.[6] Anatomy[edit] The anterior portion of the spinal cord is supplied by the anterior spinal artery. [en.wikipedia.org]
People around you can’t fully understand the pain, fear, and distress of this condition. Just know you are not alone, and there are even support groups and organizations you can join for free. [webmd.com]
- Euphoria
Drugs that are addictive produce a feeling of euphoria, a strong desire to continue using the drug, and a need to increase the amount used to achieve the same effect. Antipsychotics do not have these effects. [camh.ca]
Neurologic
- Myelopathy
The presentation is usually with an acute and painful myelopathy with impaired bladder and bowel control. [ncbi.nlm.nih.gov]
@article{3a364de4fe59438f9a76527b4dd2966b, title = "Anterior spinal artery syndrome presenting as cervical myelopathy in a patient with subclavian steal syndrome", author = "Payam Mohassel and Robb Wesselingh and Zinozy Katz and McArthur, {Justin Charles [jhu.pure.elsevier.com]
Spinal stenosis of cervical spine w myelopathy Spondylosis of cervical (neck) joint with myelopathy Spondylosis of cervical joint W myelopathy Spondylosis of cervical joint with myelopathy Stenosis of cervical (neck) spine with myelopathy Stenosis of [icd9data.com]
- Stroke
Anterior spinal artery syndrome (ASAS) is often a devastating spinal stroke occurring when the anterior spinal artery or one of its supplying anterior medullary arteries are occluded. [ncbi.nlm.nih.gov]
Conversely, if spinal stroke is approximately 1.2% of strokes, an overall annual incidence of 12 in 100,000 can be estimated. International International incidences are also unclear. [emedicine.com]
Stroke unit care has been shown to be beneficial regardless of the stroke etiology following acute ischemic and hemorrhagic stroke ( 30 ). [journal.frontiersin.org]
One of the treatments to acute ischemic stroke is providing thrombolysis. As tested and validated in numerous studies for ischemic events in the brain, until today no validated study in ischemic spinal stroke using thrombolysis has been completed. [clinicaltrials.gov]
Nov 19, 2019 · Vertebrobasilar Occlusion and Vertebral Artery Syndrome is also known as vertebrobasilar stroke or vertebrobasilar insufficiency. [ventfreen.cmamso.site]
- Irritability
SYMPTOMS 5. 4 5 3 Due to reflex between sensory fibres of 5th nerve (which are irritated) and motor fibres of 7th nerve, supplying the orbicularis oculi muscle occurs as a result of lacrimatory reflex mediated by 5th nerve (afferent) and secretomotor [slideshare.net]
Here are the common treatment options for each type of uveitis: Treatment for anterior uveitis, or iritis, includes dark glasses, eye drops to dilate the pupil and reduce pain, and steroid eye drops to reduce inflammation or irritation. [healthline.com]
Risperdal (risperidone) is used to treat schizophrenia, bipolar disorder, and irritability associated with autism. While less sedating than some of the other atypical antipsychotics, Risperdal tends to have more extrapyramidal side effects. [verywellmind.com]
Other common side effects include irritability, depression, emotional lethargy and decreased concentration. [madinamerica.com]
Episcleritis is characterized by irritative symptoms, similar to a viral or allergic conjunctivitis. [cancertherapyadvisor.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]
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]
The acute stages are characterized by flaccidity and loss of deep tendon reflexes; spasticity and hyperreflexia develop over ensuing days and weeks. [oatext.com]
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]
- Paresis
A young man was hospitalised with sudden onset paresis of the lower extremities, and spinal cord ischaemia was detected. [tidsskriftet.no]
Brown-Séquard syndrome (hemisection syndrome) Hemisection of the cord Trauma (e.g., penetrating injury) Spinal cord compression Ipsilateral Loss of proprioception, vibration, and tactile discrimination below the level of the lesion Segmental flaccid paresis [amboss.com]
Motor recovery in the following groups of patients was noted: (1) Partial loss of motor function and pain sensation--70.4 per cent (19/27); (2) Complete motor loss but partial loss of pain--83.3 per cent (5/6); (3) Paresis but pain sensation absent--66.7 [ncbi.nlm.nih.gov]
Motor recovery in the following groups of patients was noted: (1) Partial loss of motor function and pain sensation—70.4 per cent (19/27); (2) Complete motor loss but partial loss of pain—83.3 per cent (5/6); (3) Paresis but pain sensation absent—66.7 [doi.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]
Diagnosis[edit] MRI Treatment[edit] Treatment is determined based on the primary cause of anterior cord syndrome. When the diagnosis of anterior cord syndrome is determined, the prognosis is unfortunate. [en.wikipedia.org]
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. [casereports.bmj.com]
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]
10–15% functional recovery; if no recovery is evident and progressive after 24 hours, prognosis is poor Vibration and proprioception are typically spared because of an intact dorsal column! [amboss.com]
Treatment and prognosis Prognosis of anterior cord syndrome is worst among all other spinal cord injury syndromes 5. It is associated with high mortality and poor functional outcome in terms of poor recovery of motor power and coordination. [radiopaedia.org]
Etiology
Spinal cord infarction is a well-described, but rare, etiology of myelopathy, especially in children. [ncbi.nlm.nih.gov]
A spine MRI is the diagnostic modality of choice to determine the etiology, level, and extent of the lesion. Treatment depends on the underlying etiology. [amboss.com]
Temporal artery biopsy is indicated only if confirmation of giant cell arteritis as the underlying etiology of ASAS is suspected. [symptoma.com]
Background: Spinal cord infarction is a well-described, but rare, etiology of myelopathy, especially in children. [tandfonline.com]
Epidemiology
References:[11][13][14][15][16][17][18] Posterior cord syndrome Definition: injury of the posterior spinal cord affecting the posterior column (fine touch, vibration, pressure, and proprioception) Epidemiology: very rare Etiology: occlusion of the posterior [amboss.com]
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]
Summary Epidemiology To date, there have been more than 500 cases reported worldwide. Individuals of European and Asian descent are most often affected, with most case reports emerging from Japan. A slight male predominance has been reported. [orpha.net]
Hardoon et al17 determined that the prevalence of SMI in THIN is similar to that of epidemiological studies. [bmjopen.bmj.com]
Pathophysiology
The clinical features, treatment and proposed pathophysiology for this condition are presented. [ncbi.nlm.nih.gov]
Anterior Uveitis <ul><li>Prevalence </li></ul><ul><li>Symptoms </li></ul><ul><li>Diagnostic techniques and signs </li></ul><ul><li>Pathophysiology </li></ul><ul><li>Associated conditions </li></ul><ul><li>Management </li></ul> 3. [slideshare.net]
[…] carcinomas documented in 15% - 25% of cases ( J Clin Pathol. 2014;67:891 ) Sites Polyps affect entire gastrointestinal tract, except the esophagus Also associated with ectodermal changes including alopecia, nail atrophy and cutaneous hyperpigmentation Pathophysiology [pathologyoutlines.com]
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]
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]
Data Do Not Support Routine Use for Prevention In the first of two reviews, Esther S. [medscape.com]
References
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- Faivre A, Bonnel S, Leyral G, et al. Essential thrombocythemia presenting as spinal cord infarction.Presse Med. 2009 Apr 22.
- 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