Patient Presentation
An 8-year-old female came to clinic for back pain and fevers. She complained of thoracic back and chest pain for two weeks.
The pain increased over the two weeks with shooting pains into her hands and feet. During one of these episodes, three days prior, she complained of severe chest pain and was taken to the local emergency room where she was diagnosed with pneumonia and pleurisy but was not begun on any medications.
She had intermittent fevers to 101º Fahrenheit. The fevers and pain improved with acetaminophen or ibuprofen, but would return when the medication wore off.
Over the previous week, she had several episodes of urinary incontinence, intermittent headache and several episodes of emesis.
The parents also noted that the pupils of her eyes seem to be different sizes in the morning which prompted the return visit.
The patient also says that she has been dizzy at times and the parents said that she seemed disoriented occasionally. She had an upper respiratory infection about 3 weeks ago but has had no trauma or other illnesses.
The past medical history was non-contributory.
The family history is positive for seizures in a cousin.
The review of systems revealed no weakness, sore throat, rhinorrhea, constipation, diarrhea, dysuria, rashes or heart palpitations. She had not been eating or drinking well.
The pertinent physical exam shows normal vital signs and the patient was not experiencing pain. Eyes showed a right pupil of 4 mm and left pupil of 2 mm in size; both were reactive to light.
Ears, nose and throat were negative.
Neck was supple with shoddy anterior cervical lymphadenopathy and no change in symptoms with motion.
Heart, lungs and abdomen were normal. No pain could be elicited on palpation or motion of the spine or chest.
Neurological examination showed cranial nerves were normal other than her anisocoria. Deep tendon reflexes were 2+/2+ throughout. She had no motor or sensory abnormalities. She had a normal Romberg test and normal gait but was somewhat hesitant to move much for fear of the pain.
Tone was normal as was cerebellar testing.
The laboratory evaluation included normal electrolytes and glucose, erythrocyte sedimentation rate of 6 mm/hr, and C-reactive protein of 0.5 mg/dl.
Her complete blood count had a hemoglobin of 15 mg/dl, hematocrit of 40%, platelets of 239 x 1000/mm2 and a white blood cell count of 8.3 x 1000/mm2 with 5204 neutrophils, no bands, and 2117 lymphocytes.
Because of the concerning neurological examination and a differential diagnosis that included diseases which required neurosurgical intervention, the radiologic evaluation of a magnetic resonance imaging study of her spinal cord and head was ordered.
The head was normal but the spine showed extensive T2 signal involving the cervical spine that was consistent with transverse myelitis.
The diagnosis of transverse myelitis was made and the patient was begun on methylprednisolone intravenously for 3 days and then discharged home on oral prednisone.
She had some improvement of her symptoms at discharge.
Mycoplasma pneumoniae titres were positive during her hospitalization and she was treated with a 5 day course of oral azithromycin.
The patient’s clinical course over the next 2 months showed her to improve with occasional episodes of mild back pain but with no radiation. The family has also noticed a few episodes of short duration anisocoria. Both of these symptoms are decreasing in frequency.
The patient was to continue tapering the prednisone and follow-up in another month.
Figure 46 – Magnetic resonance imaging including sagittal (left) and axial (right) T2-weighted images through the thoracic spine demonstrate bright signal intensity in the center of the thoracic spinal cord throughout its length. The thoracic spinal cord showed no enhancement after the administration of intravenous gadolinium contrast.
Discussion
Transverse myelitis is a segmental spinal cord disease that usually has both sensory and motor abnormalities at and below the lesion.
The onset of symptoms is hours to days and is often associated with respiratory infections or viral illnesses such as Epstein-Barr, mumps or varicella viruses.
Mycoplasma pneumoniae is also a known cause of transverse myelitis.
Differential diagnosis includes:
- Intraspinal problems
- Abscess
- Hematoma
- Intraspinal cyst
- Necrosis, idiopathic
- Neoplasm
- Syringomyelia
- Vascular occlusion or accident
- Extraspinal problems
- Arteriovenous malformation
- Epidural abscess
- Hematoma
- Neoplasm
Other flaccid paralyses that should be distinguished from transverse myelitis include poliomyelitis, Guillian-Barré and traumatic neuritis following injections.
Evaluation usually includes magnetic resonance imaging to look for a surgically-treatable condition.
Cerebrospinal fluid testing may be necessary but should be done only after consultation with neurology and neurosurgery because of the risk of further damage from a mass lesion.
If performed, it may be normal or show mild inflammatory changes.
Nerve conduction velocities and electromyograms usually are normal.
Treatable causes of transverse myelitis should be given specific appropriate treatment.
Treatment for idiopathic transverse myelitis is usually steroid medications but sometimes intravenous immunoglobulin is used. Both are used for their immunomodulatory effects.
Outcome for children is better than adults with often complete recovery.
Learning Point
The level of the spinal lesion determines the clinical symptoms of transverse myelitis.
Above the lesion – no abnormalities
At the level of the lesion
- Paraesthesias that are usually painful. They begin at the back and circumferentially radiate.
- Cranial nerves are often not involved unless the lesion is cervical.
Below the level of the lesion
- Motor abnormalities – flaccid paralysis is common, but this depends on the extent of the lesion
- Sensory abnormalities – loss of pain but depends on the extent of the lesion. Posterior columns are usually spared.
- Autonomic abnormalities – loss of temperature control
- Reflex loss
Questions for Further Discussion
1. What clinical features distinguish poliomyelitis and Guillian-Baré from transverse myelitis?
2. What are the treatment options for recurrent transverse myelitis?
Related Cases
- Disease
- Transverse Myelitis
Spinal Cord Diseases
Spinal Diseases
- Symptom/Presentation
- Age
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Information prescriptions for patients can be found at MedlinePlus for this topic: Spinal Cord Diseases.
To view current news articles on this topic check Google News.
Rudolph CD, et.al. Rudolph’s Pediatrics. 21st edit. McGraw-Hill, New York, NY. 2003:1061, 2315.
Lucchinetti CF, Pittock SJ. Inflammatory tranverse myelitis: evolving concepts. Current Opinion in Neurology. 2006;19:362-368.
ACGME Competencies Highlighted by Case
1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
5. Patients and their families are counseled and educated.
7. All medical and invasive procedures considered essential for the area of practice are competency performed.
8. Health care services aimed at preventing health problems or maintaining health are provided.
9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.
13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
14. Knowledge of study designs and statistical methods to appraisal clinical studies and other information on diagnostic and therapeutic effectiveness is applied.
23. Differing types of medical practice and delivery systems including methods of controlling health care costs and allocating resources are known.
24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.
25. Quality patient care and assisting patients in dealing with system complexities is advocated.
Author
Donna M. D’Alessandro, MD
Associate Professor of Pediatrics, Children’s Hospital of Iowa
Date
February 19, 2007
Pediatric Education 