Transverse Myelitis Association
Volume 5 Issue 1
December 2002

Page 11
Gevallestudie: Transvers Miëlitis
Alet Uys’ Case Study as Published by Geneeskunde/The Medicine Journal, South Africa


Alet Uys has TM and is a member of the TMA.  She is a 13 year old living in Pretoria South Africa.  Alet has conducted research and wrote a case study which has been published by MedPharm’s journal, The Medical Journal.  Douw Greeff, MD, the Managing Editor of Geneeskunde/The Medicine Journal, has granted us permission to publish Alet’s paper about TM.  According to Dr. Greeff, Alet is the youngest person to ever publish in The Medical Journal. 

Alet’s research and paper were exhibited at the Gauteng Provincial Exposition.  She received a gold medal for her work and received an invitation to participate in the National Science Exposition.  She also received a certificate of achievement from The Centre for Schools of Quality AFRICA for excellence in Medical Research.  The Centre has trustees in the United States, Turkey, China and South Africa.  Alet has received the AVENTIS First Prize, for outstanding achievement in medical science.

No less than seven doctors in various branches of medicine visited her stand during the Gauteng Provincial Exposition.  The doctors asked her many questions about her research and paper.  She dealt with the learned men in a very calm and confident manner, as she was so sure of her facts.
 
The National Exposition, held in October 2002, is a Youth Science Competition and the most prestigious competition of its kind in Africa.  No less than 400 schools participated in the Provincial Exposition; whilst 450 schools participated with 700 projects at the National Exposition.  Alet received the gold medal at the National Exposition for the best project in the category, “primary school medical sciences.”  Alet will have the opportunity to exhibit her project in Moscow next year.

Alet’s ultimate goal is to go to medical school and to become a neurologist.
 
Alet Uys's article has been published in Afrikaans in the 2002 July issue of Geneeskunde/The Medicine Journal: Uys A. Transverse Myelitis. Case Study. (Afr) Geneeskunde 2002;43(July)(6):47-50  (ISSN 0016-643X) The article is also available on the Geneeskunde/The Medicine Journal website www.medpharm.co.za.

The following article is a direct translation of the Afrikaans article as published.

I am a 13 year old, grade seven pupil and currently headgirl at Lynnwood Primary school (an Afrikaans school in Pretoria, South Africa). My experience with Transverse myelitis at the age of 11 motivated me to do research on this topic for our school’s grade 6 and 7 science expo.


My goal with this research is to improve awareness about Transverse myelitis, especially among medical students and qualified doctors, as the disease is very rare, information in medical literature does not always provide all the answers and the disease can only be cured if it is diagnosed and treated very early.

Two thirds of all Transverse myelitis sufferers do not recover completely; I am very fortunate and grateful to be one of the patients in the world who recovered completely from this disease because of early diagnosis.  My research is a way through which I also want to reflect my appreciation for regaining my health.

My work is dedicated to dr Joe Terblanche (Neurologist at Unitas hospital, Centurion, South Africa) who helped me and motivated me to progress, step by step, to where I am today.

My experience with Transverse myelitis started on 2 October 2000. I felt pain from deep within my upper legs and the sensation of a tight band around my waist. The constant and terrible pain kept me awake at night and I felt very sick. 

Tuesday October 03: I went to see my family doctor. He drew some blood and suggested that my condition was caused by growing pains. When we phoned him the following day for the results, we were told that the blood got ‘lost’ and again the doctor said that my condition was only caused by growing pains.

Thursday October 05: My parents decided to take me to an orthopedic surgeon who confirmed that my condition was probably due to ‘growing pains’ and he prescribed Voltaren gel, which he believed would help to relieve the pain. I was also told to go for an isotope scan the following day in order to confirm the diagnosis.

Friday October 06: The Voltaren gel did not relieve my pain. I went for the isotope scan and the results indicated that everything was fine.   My parents and I found it hard to believe that growing pains could be so constant and bad, causing me to wake up time and again during the night.

Sunday evening October 08:  I fell out of bed when I tried to get up to go to the bathroom. My parents phoned the orthopedic surgeon at his home and informed him about the weakening condition of my legs.  We were told to visit his rooms the following day; when he realized that my problem was not of an orthopedic nature, but neurological, I was immediately referred to a neurologist for a complete neurological examination.

Monday morning October 09: I was finally admitted to hospital with the diagnosis of acute Transverse myelitis and accompanying minor bladder complaints.

Transverse myelitis
is a very rare neurological disease that affects one out of a million people each year. The patient’s autoimmune system mistakes the myelin sheath of the spinal cord for intruders and attack the sheaths, causing inflammation of the spinal cord.

Autoimmune diseases are the result of antibodies or T cells that attack molecules, cells, or tissues of the organism that produce them; the following process occurs:

When a germ enters a person’s bloodstream, white blood cells are activated to arrest (via phagocytosis) and transport the germ to the closest lymph node.  The lymph node then produces T cells and antibodies to attack and destroy the germ.  The antibodies attack the person’s tissues by mistake, because certain proteins of the person’s tissues resemble the protein covers of the germ.

In the case of Transverse myelitis, the antibodies attack the nerve tissues located in the person’s spinal cord.  The person’s own antibodies become a greater danger than the original germ itself, because the antibodies do not only attack and destroy the original germ, but also the person’s own tissues (spinal cord nerves).  Messages between central and peripheral nerves are interrupted because of myelin damage caused by the antibodies and the person becomes sick. The condition may deteriorate and progress to paralysis if the disease is not treated promptly, because the antibodies will damage the nerve cells permanently and the person will lose complete function of the bodily areas supplied by these nerves e.g., loss of function of legs and bladder.

Speedy and timely administration of high intravenous doses of cortisone decrease inflammation and swelling of the spinal cord by suppressing the patient’s immune response, providing the possibility of less structural tissue damage incurred to nerve cells and a greater chance for recovery.

Autoimmune disease in itself is not rare; Multiple sclerosis, rheumatoid arthritis, SLE, rheumatic fever and nephritis in children are all examples of autoimmune diseases. Transverse myelitis is however a rare way in which autoimmune disease presents. In this instance the antibodies specifically attack the spinal cord tissues.

The myelin sheaths of the nerves become damaged or destroyed at the point of inflammation. This prevents communication between peripheral and central nerves, causing various degrees of motoric and sensoric dysfunction.

Characteristically, the clinical picture presents on both sides of the body, below the spinal cord area affected by the disease. This occurs because the disease process involves the complete width of the spinal cord. 

Transverse myelitis may be acute or sub-acute. During an acute attack, the disease develops over a period of a few hours or days. The clinical picture of a sub-acute attack may develop over a period of 1 to 2 weeks.

Early symptoms may include sudden low back pain, loss of muscle power, paresthesia in the toes and feet (within the first few hours up to several weeks), pain in the legs and sensoric loss with partial or total motoric paralysis. Loss of sphincter function may also occur (loss of bladder control is common).

Other symptoms and signs include muscle spasms, poor appetite, headache, fever, a general feeling of discomfort and hyperesthesia during changing weather conditions.  Some patients may also experience respiratory problems, depending on the level of spinal cord damage.

Most people only experience one episode of Transverse myelitis in a lifetime.

Children and adults, both male and female, may present with Transverse myelitis. At present, there is no proof of any hereditary predisposition. All races and families are therefore vulnerable. The highest incidence is found amongst age groups 10 to 19 and 30 to 39 years.

The four classic characteristics of Transverse myelitis are
  • Varying degrees of motoric loss in arms and legs
  • Pain (the most prominent symptom), especially in the lower back, as well as sharp shooting pains in the leg
  • Sensoric changes
  • Loss of bladder control
Most patients experience various degrees of weakness in their legs. Patients may stumble or drag one foot when they try to walk. They may also experience a sensation where one or both legs feel heavier than normal.  Total motoric paralysis may follow as the disease progresses, confining the patient to a wheelchair.

Although some patients recover completely, the majority suffer permanent damage to their spinal cords, causing considerable morbidity in their daily lives.

The extent of pathological dysfunction to areas of the body relates directly to the level of the segment affected in the spinal cord. 

The results of my tests indicated that I had an absolute motoric level at T10 and a strength grading of 1/5 in my left leg and 2/5 in my right leg.  Other tests included a MRI scan and later that week a Lumbar puncture. Both investigations were indicative of problems.

The diagnosis for Transverse myelitis is reached by obtaining a complete medical history and performing a complete neurological examination.

Special investigations that are valuable in assisting to make the diagnosis include, MRI (magnetic resonance imaging) scan of the spinal cord; Myelography (contrast medium is injected into the subarachnoid space and X-rays are then obtained); Hematological (blood) tests for HIV status and vitamin B12 levels; CSF (cerebrospinal fluid) analysis to exclude high protein levels and a high white cell count.

Spinal cord injuries caused by trauma, tumors or abscesses and shortages of vitamin B12 need to be excluded as possible causes for the symptoms.

Idiopathic Transverse myelitis is diagnosed when no specific cause can be identified.

I was started on treatment with Soli-Medrol, 500mg per day for 5 days, and my condition improved dramatically.

The treatment focus is mainly geared towards providing the patient with symptomatic relief (the degree of neurological dysfunction may have an effect on the severity of symptoms experienced by the patient); Treatment includes
  • Corticosteroid therapy, which is the primary form of treatment during the first weeks of the disease. It decreases inflammation and swelling of the spinal cord and it also suppresses the patient’s immune response, but it does not alter the course of the disease
  • Relief of pain, on an ‘as needed’ basis
  • Bed rest during the initial phase of the disease
  • The monitoring of vital signs, especially in patients with respiratory problems
  • Expectant observation
  • A multi-disciplinary approach by a team consisting of a neurologist, specialist physician, hematologist, microbiologist and nursing staff
  • Physiotherapy, which plays a very important role during the recovery phase, should focus on strengthening of muscles and improvement of coordination and general movement. 
After my treatment, I received physiotherapy for about 2 months. I had to learn to walk again and had to do exercises to develop and regain my balance and strength. The sensation in my body and limbs also started to return and I made very good progress.

Sunday, October 15: The cortisone treatment was stopped and that morning, the same pain of the previous week returned. I was re-admitted to hospital at about 7am and treated with a three-day course of cortisone.

At this stage the doctor decided to start treatment for Bilharzia (a chronic condition that I was diagnosed with during all the special investigations that occurred) for which I received Biltricide, 1200mg once that evening and 600mg the following morning.


October 19: I felt healthy in general and was discharged from hospital. I continued treatment with Prednisone 60mg per day for four days, which was reduced by 5mg every day thereafter, Slow K 1 to 2 times per day, Calcium Sandoz 1 per day and Losec.


The neurologist explained that my autoimmune system over-reacted against another type of infection or virus, and attacked my own spinal cord. No other causes could be found. Because of all this I could not attend the last quarter of school or write my exams.


At present there is little known about the possible etiological factors that may cause Transverse myelitits to occur. The condition mostly results after viral infections, followed by an abnormal immune reaction or it may also occur as a result of insufficient blood supply to certain segments of the spinal cord. Infective agents that may play a role include chicken-pox (Varicella), shingles (Herpes simplex), flu virus, rubella (German measles) Hepatitis A and rubeola (Measles). Other causes may include bacterial infections of the skin, middle ear and respiratory tract.

It may also occur as a complication of vaccination against e.g., rabies or chicken-pox.

When Transverse myelitis follows an infection, the secondary immune response causes more damage to the spinal cord than the primary viral or bacterial infection. The secondary immune response (autoimmune reaction) is considered to be the main cause of the symptoms, resulting in the clinical picture of Transverse myelitis.

Recovery from Transverse myelitis starts within 2 to 12 weeks after the onset of the disease. It may however prevail for up to two years. It is very unlikely that a patient will recover if there is no improvement within the first 3 to 6 months. One third of patients with Transverse myelitis recover completely or partially i.e., they can walk again and are able to maintain bladder control. Another third show moderate recovery with permanent areas of sensoric deficit on the skin, have problems with bladder control and present with spastic movements. The last third do not recover and remain paralyzed and confined to a wheelchair.

Early diagnosis and treatment with corticosteroids mean less damage to the myelin, an improved prognosis and a better chance of recovery.

It is my wish that more people in the medical profession (especially family doctors) will in future be able to recognize Transverse myelitis in its early stages so that the patient can be referred to a neurologist as soon as possible for immediate treatment.

I would like to thank

Dr Joe Terblanche (Neurologist)
Dr  Izelle Smuts (Pediatric Neurologist)
Dr Andre Nel (Prometheus Healthcare)
Prof. Suzanne Delport (Pediatrician)
Dr Douw Greeff (Editor : Medical Science)
Mr At Meyer (Menlopark Science Academy)
Me Saartjie Roos (Science teacher, Lynnwood Primary School)

Alet Uys (13)
Headgirl 2002, Lynnwood Primary School


Web links :

Google.com  TRANSVERSE MYELITIS FACT SHEET

www.paralysis.org

www.spinalvictory.org

www.themiamiproject.org

www.myelitis.org

www.naric.com

Areas of research include :
  • To determine the role of the immune system in the destruction of myelin
  • The development of strategies to facilitate the regeneration and repair of damaged myelin
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