Thought I'd take a crack at this JC thing and see if I get any biters. Let's discuss transverse myelitis - a diagnosis that can be made in the ED. Early treatment with corticosteriods may save patients from a lifetime of paralysis!
See article below:

Hammerstedt HS, Edlow JA, Cusick S. Related Articles, Links
Emergency department presentations of transverse myelitis: two case reports.
Ann Emerg Med. 2005 Sep;46(3):256-9.
PMID: 16126136 [PubMed - indexed for MEDLINE]



Emergency Department Presentations of Transverse Myelitis: Two Case Reports


Transverse myelitis, a diagnosis that may be made in the emergency department (ED) by emergency physicians, can be difficult to diagnose because of its variable signs and symptoms and its poorly understood pathogenesis. In this article, we recount 2 cases of transverse myelitis to demonstrate its presentation, diagnosis, and management in the ED.


Introduction

Transverse myelitis is an acute or subacute inflammatory disorder of the spinal cord. Though quite variable, as demonstrated in the cases included, transverse myelitis often presents with focal neck or back pain, followed by dermatomal paresthesias, sensory loss, paraplegic symmetric motor weakness, sphincter disturbance, and urinary retention. Depending on which portion of the cord is involved, motor, sensory, and autonomic symptoms may predominate.1 These symptoms can evolve over hours or several days and quite often can have atypical presentations.

There are many possible causes of transverse myelitis, and often the individual cause can be obscure. Postinfectious transverse myelitis follows a recent infection or vaccination. Although Epstein Barr2 and cytomegalic3 viruses are most common, practically all human viruses have been associated with transverse myelitis, including human T-cell lymphotrophic viruses.4 Mycoplasma5 and 6 is the only known bacterial trigger. Some acute infections such as schistosomiasis and Lyme disease can directly cause transverse myelitis. Systemic diseases such as multiple sclerosis,7 systemic lupus erythematosus,8 or cancer9 can be the cause. Cord ischemia from aortic dissection is another diagnostic consideration. If no cause is found, transverse myelitis is said to be idiopathic.

We present 2 cases of transverse myelitis in patients who presented to the emergency department (ED) to familiarize emergency physicians with this condition.

Case 1

A 20-year-old man with no significant past medical history presented to the ED with lower extremity weakness. One week before, he had developed sore throat and fatigue without fever, headache, neck stiffness, visual changes, joint pain, or diarrhea. During the ensuing week, he was evaluated twice by other physicians; according to the patient, test results for streptococcal pharyngitis and mononucleosis had been negative, although further information was unavailable. On the evening before his final presentation, the patient noticed difficulty walking, particularly while climbing stairs. He awoke approximately 4 to 5 hours later with burning dysesthesias in both legs. On attempting to get out of bed, he fell to the floor, unable to support his weight. The patient was then transported to the ED, at which time he was unable to move his legs or urinate.

On physical examination, he was afebrile (vital signs of 98.4° F, 132/84 mm Hg, 90 bpm, 19 breaths/min, 100%RA) with a supple neck. His cardiovascular examination result was normal, and he had good distal pulses. His back was unremarkable, with no tenderness or skin findings. His mental status, cranial nerves, and upper extremities were normal. However, both lower extremities were paralyzed, atonic, and areflexic. Sensory testing revealed loss of light touch, temperature, and pinprick below T-8. Rectal sphincter tone was decreased.

Normal respiratory mechanics (negative inspiratory force and vital capacity) were documented before spinal cord magnetic resonance imaging (MRI). MRI showed abnormal enhancement and swelling of the thoracic cord (see Figure).


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Figure. In this sagittal T2-weighted sequence of the MRI, there are multiple areas of increased signal intensity and cord expansion consistent with an inflammatory or demyelinating process.



A lumbar puncture revealed a normal opening pressure (170 mm H2O), pleocytosis (WBC count 247 per cubic millimeter, with 39% polymorphonuclear cells and 51% lymphocytes), a normal glucose level (45 mg/dL), and an elevated cerebrospinal fluid total protein level (279 mg/dL). The cerebrospinal fluid did not show oligoclonal banding. Blood testing for Borrelia burgdorferi, HIV (antibody levels), herpes simplex (polymerase chain reaction), bacteria and fungi (blood cultures), and antinuclear antibody titers were all negative.

We administered intravenous steroids, as well as empiric ceftriaxone and ganciclovir, and admitted him to the ICU. He was discharged on hospital day 4 to a neurologic rehabilitation center with persistent paraplegia and a T-6 sensory level. He continued to receive high-dose oral steroids for this acute transverse myelitis. At a 4-month follow-up evaluation, a patchy sensory loss of the bilateral lower extremities remained, and the patient exhibited a spastic paraparesis; however, he is able to ambulate with assistance.

Case 2

A 38-year-old female marathon runner presented in August with 2 weeks of gradual onset, progressively worsening, sharp, midthoracic, diffuse, right paraspinal back pain. She also reported “tight” right upper abdominal muscles and low-grade fevers but denied pleuritic chest pain, bowel or bladder incontinence, nausea, vomiting, or leg weakness. One month previously, she recalled a painless, nonpruritic right midscapular rash; a physician had prescribed cephalexin and obtained antibody titres to Borrelia burgdorferi (which were negative according to the patient).

On examination, the patient had moderate to severe back pain and was lying flat in bed. Vital signs were 98.6° F, 58 bpm, 142/89 mmHg, 12 breaths/min, 99%RA. There was no midline spine tenderness. Straight leg testing results were negative, and a range of motion of the back was painful but within normal limits. Full neurologic examination results were normal except for a 1-cm band of decreased sensation to light touch along the right T5-6 front distribution just barely crossing the midline. There was a 4-cm irregularly shaped flat purple blanching rash without vesicles in the right subscapular region (the site of her original rash). The patient's pain was relieved with morphine and diazepam. An ECG demonstrated sinus bradycardia without evidence of ischemia or right-sided heart strain. Plain radiographs of the chest and spine and a computed tomographic examination with angiography of the chest were normal. However, because of her pain severity and her small band of decreased abdominal sensation, we obtained thoracic and lumbar spine MRIs with the suspicion of cord myelitis or epidural abscess from possible Lyme or zoster. Both MRI results were normal. After this negative and thorough evaluation, we diagnosed her with musculoskeletal strain and discharged her from the ED with ibuprofen and diazepam prescriptions.

One week later, she returned with excruciating thoracic and lower back pain that kept her awake at night. She was unable to walk comfortably despite compliance with the prescribed medications. She described her worsening band of abdominal “tightness” as “silicone or Novocain wrapped around me.” Her examination was unchanged except for an expanded area of decreased sensation to pinprick, light touch, and temperature, now from T4 to T12 on both front and back, although more prominent on the front and on the right. Also, she had decreased vibratory sense of bilateral toes, but position, gait, and strength were preserved. Lumbar puncture revealed clear cerebrospinal fluid, with 541 leukocytes per cubic millimeter (90% lymphocytes), normal glucose levels (51 mg/dL), and elevated cerebrospinal fluid protein (171 mg/dL). Her erythrocyte sedimentation rate was normal (5 mm/hr). Repeated thoracic and lumbar MRI results showed patchy longitudinal enhancement of the cord from T4 to T8 levels. She was admitted to the neurology service with a diagnosis of transverse myelitis. During her admission, further testing revealed the etiology of her transverse myelitis to be Lyme disease. Her cerebrospinal fluid was positive for immunoglobulin M and immunoglobulin G antibody to B burgdorferi, and her serum Lyme antibody titer was positive by enzyme-linked immunosorbent assay (but negative by Western blot). She was treated with intravenous ceftriaxone during admission and as an outpatient for a planned course of 3 weeks.

Discussion

Transverse myelitis is a diagnosis that may be made in the ED, although varied presentations are common. Some patients may have a classic presentation (patient 1) with rapidly evolving back pain, most often in the thoracic area, and lower-extremity weakness. Others may present with abrupt segmental back or radicular pain, followed by ascending paresthesias and weakness, similar to that with Guillain-Barré syndrome. Urinary and fecal retention or incontinence is common, as is ataxia and leg weakness. As in patient 2, it is also possible for transverse myelitis to present as a slowly progressive back pain with prominent sensory deficit and no other associated symptoms. Symptoms may evolve over hours, days, or weeks.

Transverse myelitis should be included in the differential diagnosis of any patient with back pain and weakness. The variable presentation of transverse myelitis may mimic other emergencies, which should be pursued appropriately by the clinician on an individual case basis. In rapidly progressive cases, one must consider causes such as aortic dissection and other vascular pathology that may cause cord infarction. When symptoms evolve over days or weeks, it is important to exclude cord compression from tumor, abscess, hematoma, or central disc herniation. Patients with slow onset symptoms may have to be admitted to a neurology service for multiple specialist consultations to rule out mimics such as multiple sclerosis, Guillain-Barré syndrome, tick paralysis, botulism, or poliomyelitis because these may be difficult to exclude in the ED.

MRI best establishes the diagnosis of transverse myelitis by demonstrating high-intensity signals on T2-weighted images extending longitudinally along the cord.10 The number of segments involved may be as few as one, and occasionally, the entire cord is involved.11 It is possible to differentiate between multiple sclerosis and transverse myelitis by MRI, as transverse myelitis usually affects the central cord and multiple sclerosis affects the peripheral cord and involves brain abnormalities.11 Also, as in patient 2, magnetic resonance abnormalities may lag behind symptoms. Other tests that may be helpful to elucidate the cause of the transverse myelitis include basic chemistries and CBC counts, antinuclear antibody, erythrocyte sedimentation rate, rapid plasmid reagin, Lyme titers, titers for viruses such as West Nile, polio, hepatitis, Epstein Barr virus, cytomegalovirus, HIV, and mycoplasma antibody and bacterial cultures. Lumbar puncture most commonly demonstrates lymphocytosis and elevated protein, but checking the opening pressure, glucose, immunoglobins, and protein electrophoresis is warranted to narrow the differential diagnosis. Cultures and polymerase chain reaction sometimes make a specific identification. When the diagnosis of transverse myelitis is strongly suspected in the ED, an admission to neurologic specialists for completion of the evaluation is necessary to specify the cause of the transverse myelitis and obtain further MRI imaging, serial examinations, and culture results.

The most common therapy for transverse myelitis is supportive. Immunoglobin infusion and corticosteroids have been mainstay therapy in the past; however, one recent study has questioned their utility. Some authors advocate that corticosteroids may only be helpful if acute swelling of the cord is suspected,12 whereas others strongly argue that early steroid treatment speeds recovery time and morbidity.1 Because of the dichotomy of academic opinion, until more clinical trials allow for a consensus for standard of steroids in the treatment of transverse myelitis, the addition of steroids should be used at the clinical discretion of the clinician while taking into account the risk-benefit analysis of each patient. When a treatable infection is diagnosed, in particular schistosomiasis13 or Lyme disease,14 disease-specific antimicrobials may be useful. Consider Lyme disease in the appropriate setting,14 keeping in mind that patients presenting with erythema migrans will often be seronegative early in the course and that cephalexin is not an appropriate antibiotic choice. A therapeutic alternative for severe transverse myelitis may be plasma exchange therapy to remove presumptive factors that may contribute to autoimmune-mediated inflammation,15 and in cases of HTLV16 or mycoplasma,17 associated transverse myelitis. Recovery depends on the amount of cord necrosis; independent ambulation occurs in 50% of pediatric and 35% of adult cases, and improvement may continue beyond 6 months.18 and 19

Transverse myelitis is an often obscure disorder that may be diagnosed in the ED in certain clinical scenarios. The emergency physician should include transverse myelitis into the differential diagnosis of back pain, especially when associated with neurological deficits, and obtain an early MRI, both to rule out compression and rule in transverse myelitis. Although the presentation of transverse myelitis, as demonstrated above, can be variable, early diagnosis and treatment can minimize its accompanying morbidity and mortality, the cornerstone of the emergency medicine practice.

24get,

A nice review article. I disagree with the commentor who said this will be a consultant driven workup. It is urgent to rule out cord compression or treat it if present. I've often noticed that residents and even graduates are pretty weak in this uncommon emergency. Things that I would like EPs to know:

1. Bilateral acute nontraumatic weakness with a level will likely be either a cord disease or ascending dyemyelinating paralysis (Guillan-Barre-Landry).

2. GBL will usually present with no reflexes. Cord disease may have "spinal shock" early, but will have hyperreflexia later. This is because GBL is a peripheral neuropathy but cord disease will be a upper motor neuron lesion. BTW "spinal shock" refers to a temporary hyporeflexia seen after cord transection or disease. "neurogenic shock" refers to the hypotension with bradycardia seen with upper cord lesions.

3. Cord disease is more likely to have a clear dermatomal sensory level.

4. MRI is the diagnostic study of choice. X-rays and CT may show lesions that are contiguous from bone, but are unlikely to visualize those from hematogenous spread.

5. LP IS CONTRAINDICATED UNTIL CORD COMPRESSION HAS BEEN RULED OUT BY MRI.

6. The most common compressive lesions are tumors and spinal epidural abscess. Steroids usually indicated. Tumor is treated with emergent XRT with or without surgery. Abscess is treated with surgical drainage. Neurosurgical consultation now indicated for either.

7. A normal or near normal MRI leads to GBL or transverse myelitis. LP can now be accomplished safely. Neurology consult now indicated.

Cheers, BKN

And an LP with a normal or low protein virtually excludes Guillan-Barré.

24get,

A nice review article. I disagree with the commentor who said this will be a consultant driven workup. It is urgent to rule out cord compression or treat it if present. I've often noticed that residents and even graduates are pretty weak in this uncommon emergency. Things that I would like EPs to know:

1. Bilateral acute nontraumatic weakness with a level will likely be either a cord disease or ascending dyemyelinating paralysis (Guillan-Barre-Landry).

2. GBL will usually present with no reflexes. Cord disease may have "spinal shock" early, but will have hyperreflexia later. This is because GBL is a peripheral neuropathy but cord disease will be a upper motor neuron lesion. BTW "spinal shock" refers to a temporary hyporeflexia seen after cord transection or disease. "neurogenic shock" refers to the hypotension with bradycardia seen with upper cord lesions.

3. Cord disease is more likely to have a clear dermatomal sensory level.

4. MRI is the diagnostic study of choice. X-rays and CT may show lesions that are contiguous from bone, but are unlikely to visualize those from hematogenous spread.

5. LP IS CONTRAINDICATED UNTIL CORD COMPRESSION HAS BEEN RULED OUT BY MRI.

6. The most common compressive lesions are tumors and spinal epidural abscess. Steroids usually indicated. Tumor is treated with emergent XRT with or without surgery. Abscess is treated with surgical drainage. Neurosurgical consultation now indicated for either.

7. A normal or near normal MRI leads to GBL or transverse myelitis. LP can now be accomplished safely. Neurology consult now indicated.

Cheers, BKN


BKN, you are my hero :love:

I humbly agree. Especially in the case that the fast and furious steroid treatment of transverse myelitis holds up, the idea should be to r/o compression/ICP, then tap. Since GB and TM can both respond to steroids, why not start them and then consult neuro?

Thanks a million for posting!!!! :o

And an LP with a normal or low protein virtually excludes Guillan-Barré.


See, we are developing an algorithim, here!
Thanks for posting!!!!!!!!!!!!!! :clap:

Egad. I was not discounting the importance of assessing neurological function/level or the presence or absence of cord compression. My point was that by the time we have determined whether the patient has a neurological or neurosurgical problem, I'll probably be on the phone. Perhaps that is over consulting or life in an academic center and my tune will change when I finish. Certainly if I were in a position to start steroids for suspected Transverse Myelitis I would.

Egad. I was not discounting the importance of assessing neurological function/level or the presence or absence of cord compression. My point was that by the time we have determined whether the patient has a neurological or neurosurgical problem, I'll probably be on the phone. Perhaps that is over consulting or life in an academic center and my tune will change when I finish. Certainly if I were in a position to start steroids for suspected Transverse Myelitis I would.

And I was not trying to be snarky. It's just that we had a similar case a couple of days ago, and as usual the juniors weren't up to speed. :mad: I certainly agree that anyone with these syndromes is going to be consulted. But it would be nice if we consulted the right service with more or less the right diagnosis and initial therapy started. Particularly if we're gonna transfer a good distance.:)

See, we are developing an algorithim, here!
Thanks for posting!!!!!!!!!!!!!! :clap:

Thanks for rejuvenating the journal board! Nice to hear some folks views too.

Thanks for rejuvenating the journal board! Nice to hear some folks views too.

Hurray, hopefully we will keep this up, eh? NinerNiner suggested LLSA articles, so the next one I post will be:

Thielman NM, Guerrant RL. Acute infectious diarrhea. N Engl J Med. Jan 2004;350(1):38-46.

from the 2006 LLSA reading list. I will include a summary and maybe some corollary stuff. If anyone wants to be involved, PM me.