Transverse myelitis is an inflammation of the spinal cord. It is a neurological condition in which the inflammation of the spinal cord leads to damage of the nerve fibers causing them to lose their myelin sheath and decrease in their ability to conduct electricity within the nervous system.
Presentation
There are 4 classic features of transverse myelitis,
The presentation can either be acute when it develops within days or subacute when it develops within weeks. Symptoms include lower back pain, sudden paresthesia in the legs which may described by patient as burning, tingling, pricking, or tickling sensations. There is also sensory loss in the lower limbs and paraparesis (partial paralysis of the lower limbs) which may progress to paraplegia (total paralysis of the lower limbs). Sensory defect will also affect the autonomic nervous system causing symptoms like urinary and bowel incontinence. There will be history of muscle spasms, headache, fever, general feeling of unwell and discomfort, and loss of appetite. If the lesion is higher up the spinal cord, respiratory problems may be experienced [6].
Entire Body System
- Weakness
Unilateral posterior column loss 5. pyramidal weakness 6. contralateral spinothalamic loss 18. [slideshare.net]
Physical exam is notable for bilateral lower extremity sensory deficits and motor weakness. Both bulbocavernous and anal wink reflex are absent. MRI of the brain and spine are performed. There is no intracranial pathology. [medbullets.com]
Case Report A 24 year old male smoker presented with sudden onset of weakness and loss of sensation over both upper limbs for 2 days. The weakness was symmetric, involving distal and proximal muscles and had not progressed since its onset. [casereports.in]
Abstract Guillain-Barré syndrome is a rapidly progressive symmetrical muscle weakness associated with acute inflammatory disease. [ncbi.nlm.nih.gov]
The involvement of motor and sensory control pathways frequently produce altered sensation, weakness and sometimes urinary or bowel dysfunction. [hopkinsmedicine.org]
- Disability
Pain was found to be correlated strongly with quality of life in both groups but only correlated with disability in the AQP4-Ab +ve group. [ncbi.nlm.nih.gov]
Twelve (52%) patients recovered with minimal disability (Expanded Disability Status Scale (EDSS) ≤2.5) and three (13%) remained wheelchair dependent. [idibgi.org]
Individuals with severe cases of TM may qualify for Social Security disability. In order to meet Social Security’s definition of disability, the disabling condition must have lasted or be expected to last at least one year. [disabilitysecrets.com]
All disability in TM can be attributed to one lesion in the spinal cord, whereas in MS, disability relates to the number and location of many lesions. [brainandlife.org]
- Fatigue
Konsekvenser i dagligt liv kognitiv nedsättning och fatigue efter transitorisk ischemisk attack Engelsk titel Daily life consequences,cognitive impairment and fatigue after transient ischemic attack Populärvetenskaplig sammanfattning av projektet Objectives [researchweb.org]
We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: August 4, 2019 [patientslikeme.com]
The patient reported a reduction in pain, dyspnea, and fatigue accompanied by an improvement in the quality of sleep and mood. [ncbi.nlm.nih.gov]
[…] and progress rapidly, with muscle weakness on both sides of the body, (if the upper part of the spinal cord is affected, both arms and legs are affected- if the thoracic part is affected, both legs only will be affected), back pain, limb spasticity, fatigue [nicklauschildrens.org]
Respiratoric
- Rhinorrhea
She stated that her symptoms initially started with rhinorrhea and chills a week ago. [ncbi.nlm.nih.gov]
Ten days previously he had received the first dose of the hepatitis B vaccine and presented mild upper airway respiratory symptoms such as rhinorrhea and cough but no fever. His previous medical history and family history were unremarkable. [scielo.br]
Gastrointestinal
- Fecal Incontinence
Key points Acute widespread neurologic deterioration, particularly involving urinary and fecal incontinence and para- or tetraparesis in a patient with systemic lupus erythematosus should arouse suspicion for longitudinally extensive transverse myelitis [cmaj.ca]
Deficits may progress over several more days to a complete transverse sensorimotor myelopathy, causing paraplegia, loss of sensation below the lesion, urinary retention, and fecal incontinence. [merckmanuals.com]
Skin
- Erythema
[详细] 持久性隆起性红斑 持久性隆起性红斑(erythema;elevatum;diutinum)是一种少见的皮肤病,其特点是肢体伸侧发生的持久性... [详细] [jbk.99.com.cn]
Clinical manifestations initially may show the classic erythema chronicum migrans with subsequent spread to other organ systems. [karger.com]
Examination should attempt to detect the presence of uveitis or retinitis, decreased lacrimation or salivation, skin rash (malar, livedo reticularis, erythema nodosum), oral or genital ulcers, adenopathy, pleuritic or pericardial friction rub, or organomegaly [bioscience.org]
- Formication
On the fifth postoperative day, the patient developed signs of paralytic ileus, associated with formication and progressive paresis in the lower extremities. He had fever and complained of severe abdominal and lumbar pain. [signavitae.com]
Musculoskeletal
- Muscle Weakness
Abstract Guillain-Barré syndrome is a rapidly progressive symmetrical muscle weakness associated with acute inflammatory disease. [ncbi.nlm.nih.gov]
Muscle weakness in the legs can progress to a complete loss of movement (paralysis) very quickly. [myelitis.org.uk]
About one third continue to have some muscle weakness and urinary problems (urgency or loss of bladder control). About one third recover very little. [msdmanuals.com]
They may include pain, muscle weakness, abnormal sensations, and bowel/bladder dysfunction. Imaging, blood tests and a lumbar puncture will confirm the diagnosis. [luriechildrens.org]
This damage disrupts the messages the spinal cord nerves send throughout the body, which can result in pain, sensory problems, muscle weakness, and loss of bladder/bowel control. Transverse myelitis can affect patients of any age, gender or race. [chop.edu]
- Neck Pain
Otherwise, Rabinovici says, "Neck pain is not going to be any more serious than a pain in the neck." In some instances, transverse myelitis will lead to another autoimmune disease, like multiple sclerosis or lupus, according to Balabanov. [self.com]
A 7-year-old girl presented with complaints of neck pain, spout-style vomiting, cough, shortness of breath, and acute paraparesis with sensory and sphincter disturbance. The patient was intubated because of increased respiratory distress. [ncbi.nlm.nih.gov]
These are possible symptoms: Back or neck pain Weakness in arms or legs Abnormal feelings in the legs, such as burning, tingling, or pricking Loss of bladder or bowel control Heightened sensitivity to touch Where in the body these symptoms occur depends [stlouischildrens.org]
Common symptoms are back or neck pain, weakness or sensation changes in the arms or legs, or loss of bladder or bowel control. Treatment with steroids or other medicines doesn't cure transverse myelitis. But it might relieve symptoms. [urmc.rochester.edu]
- Numbness of the Feet
A bandlike tightness around the chest or abdomen, weakness, tingling, numbness of the feet and legs, and difficulty voiding develop over hours to a few days. [merckmanuals.com]
- Muscular Atrophy
Doug was the global lead for a series of programs in Alzheimer’s disease, Amyotrophic Lateral Sclerosis (ALS) and Spinal Muscular Atrophy (SMA). Doug led the team that developed the drug Spinraza, now approved for SMA. [myelitis.org]
Psychiatrical
- Suggestibility
Furthermore, these cases suggest a potential role for approaching how we classify subtypes of transverse myelitis and acute disseminated encephalomyelitis. [ncbi.nlm.nih.gov]
In the setting of a characteristic brain MRI, the acute partial variant suggests high future risk of developing multiple sclerosis (MS). [bestpractice.bmj.com]
History may suggest a drug as a cause. [merckmanuals.com]
Urogenital
- Incontinence
Symptoms of TM include sudden onset of lower back pain, muscle weakness, or abnormal sensations in the toes and feet which can rapidly progress to more severe symptoms, including paralysis, urinary incontinence, increased urinary urge, urinary incontinence [cureus.com]
We describe a middle-aged man who presented with dengue fever and in whom weakness of the bilateral lower limbs and urinary incontinence developed on the third day of fever. [ncbi.nlm.nih.gov]
Clinical manifestations include motor weakness, sensory loss, and incontinence. (Adams et al., Principles of Neurology, 6th ed, pp1242-6) Svenska synonymer Inga svenska synonymer finns. [mesh.kib.ki.se]
- Urinary Retention
Keywords : acute urinary retention; acute transverse myelitis; upper motor neuron lesion. [scielo.mec.pt]
A 35 year-old male Caucasian recently returned from the tropics presented to our hospital with urinary retention and acute paraparesis. After extensive diagnostic workup he was diagnosed with acute HIV infection presenting as transverse myelitis. [ncbi.nlm.nih.gov]
- Urinary Urgency
An otherwise healthy 28-year-old woman presents with progressive weakness for the past 3 days, numbness below her breasts, and urinary urgency. Neck flexion triggers an electrical sensation that radiates to the coccyx. [nejm.org]
Another one-third show only fair recovery and are left with significant deficits, such as spastic gait, sensory dysfunction, and prominent urinary urgency or incontinence. [sharecare.com]
About one third of patients recover, one third retain some weakness and urinary urgency, and one third are bedbound and incontinent. Multiple sclerosis eventually develops in about 10 to 20% of the patients in whom the cause is initially unknown. [merckmanuals.com]
Neurologic
- Myelopathy
We discuss the diagnostic clues and pitfalls of the not uncommon clinical scenario of a presumed "myelopathy with normal MRI." Finally, we suggest an algorithm for the diagnosis and management of acute myelopathies. [ncbi.nlm.nih.gov]
4) delayed radiation myelopathy, 5) spinal cord infarct, and 6) idiopathic myelopathy. [bioscience.org]
Since that time, the syndrome of progressive paralysis due to spinal cord inflammation has been known as transverse myelopathy or transverse myelitis. What is the difference between ‘myelopathy’ and ‘myelitis?’ [myelitis.org]
There is insufficient evidence to determine whether clinical features of the myelopathy are associated with myelitis vs other myelopathies. Laboratory features. [neurology.org]
- Tingling
Most have some numbness, tingling, or a burning sensation in their back, belly, arms, or legs. Almost all lose some bladder control. How much of your body is affected depends on which part of your spinal cord has the problem. [webmd.com]
There are four classic symptoms of TM, including: Weakness in the arms and/or legs Sensory symptoms such as numbness or tingling Pain and discomfort Bladder dysfunction and/or bowel motility problems. [kennedykrieger.org]
There are four classic symptoms of transverse myelitis: weakness in the arms/legs sensory symptoms such as numbness or tingling pain and discomfort bladder dysfunction and/or bowel motility problems The distribution of those symptoms may be symmetric [hopkinsmedicine.org]
Symptoms include lower back pain, sudden paresthesia in the legs which may described by patient as burning, tingling, pricking, or tickling sensations. [symptoma.com]
Transverse myelitis can cause low back pain, spinal cord dysfunction, muscle spasms, a general feeling of discomfort, headache, loss of appetite, and numbness or tingling in the legs. Almost all patients develop leg weakness. [medicinenet.com]
- Paresthesia
C3 Yes No MS F, 26 Pain, paresthesia, weakness, urinary retention Medulla-C5 No Yes ADEM F, 33 Paresthesia, weakness C3–C6 No No ADEM F, 36 Paresthesia, paraparesis, urinary incontinence C1–C4 No Yes NMO M, 54 Pain, stiffness, paresthesia, paraparesis [hindawi.com]
Soon after, the patient presented to the emergency department with symptoms of bilateral paresthesia of the lower limbs which ascended to the pelvis. After a thorough work up and imaging via MRI a diagnosis of transverse myelitis was made. [ncbi.nlm.nih.gov]
We report a case of a 13-year-old girl affected by transverse myelitis who rapidly developed leg paresthesia and weakness, difficulty in deambulation, sensor and sphincter deficits. [medicoebambino.com]
About one-third of people affected with TM experience good or full recovery from their symptoms; they regain the ability to walk normally, and experience minimal urinary or bowel effects and paresthesias. [sharecare.com]
- Headache
She had a low-grade fever and headache without other clinical neurological abnormalities while at our hospital. Cerebrospinal fluid (CSF) showed mononuclear pleocytosis. Other laboratory tests indicated no apparent abnormalities. [ncbi.nlm.nih.gov]
The most seen neurological complication of Neuro-Behçet syndrome is headache. In Behçet\'s syndrome if the neurological symptoms are not completely occur, to diagnose neuro-Behçet may be very difficult. [atif.sobiad.com]
Transverse myelitis can cause low back pain, spinal cord dysfunction, muscle spasms, a general feeling of discomfort, headache, loss of appetite, and numbness or tingling in the legs. Almost all patients develop leg weakness. [medicinenet.com]
- Seizure
G40.0 Localization-related (focal) (partial) idiopathic epilepsy and epileptic syndromes with seizures of localized onset G40.00 Localization-related (focal) (partial) idiopathic epilepsy and epileptic syndromes with seizures of localized onset, not [icd10data.com]
A 19-year-old female developed generalized tonic seizures lasting several minutes. She had a low-grade fever and headache without other clinical neurological abnormalities while at our hospital. [ncbi.nlm.nih.gov]
[…] polyradiculoneuritis, Guillain Barre Syndrome), Neuritis (including optical neuritis), Myelitis (including transverse Myelitis), Encephalitis, Demyelinating disease of the central nervous system, Exacerbation of multiple sclerosis, Multiple sclerosis, Seizure [mymemory.translated.net]
Workup
- Radiological tests are important in diagnosis to rule out other causes of the experienced symptoms or an underlying cause of this condition. An MRI is done to examine structural damage to the brain or spinal cord, like spinal cord compression which could also produce similar symptoms [7]. The brain MRI may detecting underlying causes like multiple sclerosis. A contrast CT scan is done when an MRI is not available [8].
- Various blood tests are also done to rule out other underlying conditions like HIV, vitamin B12 deficiency, and systemic lupus erythematosus.
- A lumbar puncture will reveal increased white blood cells in case of an infection and can be used to look for disease markers.
- If no specific cause can be gotten from these tests, the patients is said to have idiopathic transverse myelitis [9].
Treatment
- The treatment for this condition is designed to manage symptoms and reduce spinal cord inflammation as there are no definitive treatments. Intravenous steroids are used soon after diagnosis to reduce inflammation and improve neurological recovery. Steroids that might be used are methylprednisolone and dexamethasone.
- Plasma exchange therapy is instituted in those with moderate to severe disease and in those who don’t respond to steroid therapy.
- Other immunomodulatory treatment like intravenous cyclophosphamide can be used in patients that do not respond to the above treatments.
- The most critical part of management involves maintaining the physiological functioning of the patient.
Prognosis
Recovery from this condition may begin from anytime between 2 to 12 weeks and lasts for up to 2 years. If there is no improvement within the 6 months, then complete recovery is unlikely. About one-third of patients experience full recovery, the other one-third experience partial recovery while the last one-third experience no recovery at all, and will depend on others for basic daily functions. Aggressive treatment and physical therapy will help improve outcomes. Research has shown that if symptoms start rapidly, prognosis will most likely be bad [5].
Etiology
There are several causes of transverse myelitis. Known causes are optic neuritis and multiple sclerosis. It could also be caused by aortic dissection which extends to the spinal arteries as well as viruses like cytomegalovirus, herpes simplex, HIV, Epstein-Barr virus, rabies, enteroviruses and human T-cell leukemia virus. Bacterial infections like tuberculosis, syphilis and Lyme borreliosis have also been implicated along with conditions like paraneoplastic syndrome, and vascular causes like thrombosis of spinal artery, spinal arteriovenous malformations and vasculitis caused by heroin use [2].
Epidemiology
It is a fairly rare condition with the incidence thought to be up to 8 new cases per million per year. It has a bimodal peak age incidence occurring mostly between 10 – 19 years and 30 – 39 years, although it may occur at any age. About 25% of transverse myelitis patients are children and it has no familial association. It has no gender predilection. Most cases are monophasic, occurring just once, but there is recurrence in a minute number of patients and this is mostly due to a predisposing underlying illness [3].
Pathophysiology
- Multiple sclerosis is an autoimmune disease that occurs when T-cells are activated against unknown antigen which produces a cascade of inflammation that leads to demyelination and also axonal loss.
- Optic neuritis on the other hand involves the B-cells. These B-cells produce a substance, anti-aquaporin-4 antibodies which activate the complement system and cause the nerves to lose their myelin sheaths.
- Most idiopathic transverse myelitis are para-infections which has led to the belief that the immune response against the offending organism causes an autoimmune attack on myelin and some other antigens in the spinal cord. Mechanisms of injury could be molecular mimicry, where T-cells or antibodies found on these infectious agents cross-react with those in the central nervous system. Another mechanism could be a super-antigen response by the offending organism [4].
Prevention
There is no known way to prevent the primary attack. Recurrent attacks can be prevented with the use of immunosuppressants [10].
Summary
The origin of the name of this disorder is derived from the Greek word myelos which means spinal cord and the suffix –itis which means inflammation. Transverse implies that it spans the thickness of the cord. Nerve fibers are responsible for conducting electrical impulses and the myelinated fibers conduct these impulses better. In transverse myelitis, these myelin sheaths are destroyed [1].
Patient Information
Definition: Transverse myelitis is a disease of the spinal cord in which the nerves lose their ability to conduct electrical impulses leading to loss of many important daily nervous functions.
Cause: It can be caused by one of several autoimmune diseases. It can also be due to viral and bacterial infections. Some disorders of the arteries have also been known to cause it and it may also be brought about by heroin use.
Symptoms: Symptoms include pain of the legs as well as tingling, numbing and tickling sensations. There is also weakness of the leg muscles which may graduate to full paralysis. There will be loss of bladder and bowel function and in some patients, there will be respiratory difficulty.
Diagnosis: This is done with imaging techniques like MRI and CT scans which will show lesions of the brain and/or spinal cord. Blood tests will also be done to check for underlying diseases and part of the cerebrospinal fluid will be checked.
Treatment: This condition has no known cure but steroids are used to manage the inflammatory process. Plasma exchange therapy is used in patients who don't respond to steroids. Cyclophosphamide can be used in severe cases that don’t respond to any of the above.
References
- Altrocchi PH. Acute Transverse Myelopathy. Arch Neurol 1963; 9:111.
- Borchers AT, Gershwin ME. Transverse myelitis. Autoimmun Rev 2012; 11:231.
- Paine RS, Byers RK. Transverse myelopathy in childhood. AMA Am J Dis Child 1953; 85:151.
- Torabi AM, Patel RK, Wolfe GI, et al. Transverse myelitis in systemic sclerosis. Arch Neurol 2004; 61:126.
- Christensen PB, Wermuth L, Hinge HH, Bømers K. Clinical course and long-term prognosis of acute transverse myelopathy. Acta Neurol Scand 1990; 81:431.
- Oh DH, Jun JB, Kim HT, et al. Transverse myelitis in a patient with long-standing ankylosing spondylitis. Clin Exp Rheumatol 2001; 19:195.
- Bakshi R, Kinkel PR, Mechtler LL, et al. Magnetic resonance imaging findings in 22 cases of myelitis: comparison between patients with and without multiple sclerosis. Eur J Neurol 1998; 5:35.
- Wolf VL, Lupo PJ, Lotze TE. Pediatric acute transverse myelitis overview and differential diagnosis. J Child Neurol 2012; 27:1426.
- Bruna J, Martínez-Yélamos S, Martínez-Yélamos A, et al. Idiopathic acute transverse myelitis: a clinical study and prognostic markers in 45 cases. Mult Scler 2006; 12:169.
- Tippett DS, Fishman PS, Panitch HS. Relapsing transverse myelitis. Neurology 1991; 41:703.