Clinical Neurology and Neurosurgery 108 (2006) 278–283

The differential diagnosis of acute transverse myelitis

Vesna V. Brinar a,∗ , Mario Habek a , Marko Brinar b , Branko Malojčić a , Marina Boban a
a University Department of Neurology, Zagreb School of Medicine and University Hospital Center, Kišpatićeva 12, Zagreb, Croatia
b University Department of Internal Medicine, Zagreb School of Medicine and University Hospital Center, Kišpatićeva 12, Zagreb, Croatia

Abstract

The clinical and paraclinical characteristics of acute transverse myelitis (ATM) were analyzed in 31 patients. In some patients there was
clinical evidence of complete transection, in others of only partial lesions. Magnetic resonance imaging (MRI) in the acute phase in the first
group was normal, but showed cord atrophy subsequently. It is probable that the clinical picture was due to parenchymatous neuronal lesions,
analogous to those of axonal polyneuropathy. In the patients with incomplete transverse lesions, the most common finding was demyelination.
In the patients with circumscribed demyelinating lesions, the symptoms and MRI were suggestive of clinically isolated syndromes (CIS)
predictive of multiple sclerosis (MS). Extensive demyelination was indicative of acute disseminated encephalomyelitis (ADEM) due to
hyperergic vasculopathy or various forms of chronic vasculitis. In two patients with variable clinical symptoms, a vascular malformation was
the cause of the clinical presentation, and in one patient demyelination was due to the disc compression.
© 2005 Elsevier B.V. All rights reserved.

Keywords: Transverse myelitis; Disseminated encephalomyelitis; Clinically isolated syndrome; Multiple sclerosis

1. Introduction brospinal fluid (CSF) and MRI findings in patients presenting

with acute transverse myelitis (ATM) in order to differentiate
The symptoms of complete or incomplete spinal cord between cases of idiopathic transverse myelitis from trans-
transection are occasionally due to demyelinating lesion diag- verse myelitis due to multiple sclerosis (MS), disseminated
nosed by MRI, lesions that are of special interest because of encephalomyelitis (DEM) or other conditions.
the extensive differential diagnosis [1]. A different diagnostic
problem is encountered in patients who develop a complete
transverse spinal syndrome without signs of demyelination 2. Materials and methods
or of any other kind of pathological involvement of the spinal
We reviewed the medical records of 31 patients who were
cord.
referred to our hospital with the diagnosis of ATM. All the
Current literature includes different diseases under the
patients were admitted within a few days after the onset of
same term—idiopathic transverse myelitis, which is used
symptoms.
to designate either bilateral spinal cord involvement or par-
The following information was obtained: the presence of
tial, unilateral disease. The terms myelopathy and myelitis
an antecedent febrile illness, initial symptoms and neurolog-
are often used interchangeably simply because both refer to
ical signs during the peak of the illness. Immunological and
involvement of the spinal cord by some pathological event;
viral tests, MRI, neurophysiological and CSF examinations
however, more correctly, myelopathy is generic and implies
were performed. The neurological signs were assessed as
no particular etiological factor, whereas myelitis refers to
development of sensory (sensory level, paresthesias), motor
inflammatory diseases of the spinal cord.
(paraparesis, tetraparesis) or autonomic (urine or fecal incon-
The purpose of this article is to describe the symptoms,
tinence) dysfunction attributable to spinal cord involvement.
the results of viral and immunological tests, and the cere-
Particular attention was paid to determining if the spinal
cord transection was complete or not. Serum was tested for
∗ Corresponding author. Tel.: +385 1 2388 310; fax: +385 1 2421 046. immunological markers, and CSF was tested for viruses, HIV,
E-mail address: vesna.brinar@zg.htnet.hr (V.V. Brinar). Borrelia burgdorferi, fungi and tuberculosis.

0303-8467/$ – see front matter © 2005 Elsevier B.V. All rights reserved.
doi:10.1016/j.clineuro.2005.11.008
 V.V. Brinar et al. / Clinical Neurology and Neurosurgery 108 (2006) 278–283
Fig. 1. Patient #8 (F/24) with a history of retrobulbar neuritis developed
paraparesis and urinary incontinence. MRI of the spinal cord revealed a
large demyelinating lesion extending from C2 to 6 which does not involve
the entire diameter of the spinal cord. The final diagnosis was NMO.
3. Results
Clinical data were available for 29 patients. The most fre-
quent symptoms were sensory disturbances: paresthesiae in
17 (59%) and sensory level in 15 (52%); motor symptoms:
paraparesis in 15 (52%), tetraparesis in 7 (24%), hemiparesis
in 2 (7%) and monoparesis in 2 (7%); hypereflexia in the legs
was present in 2 (7%) patients. Sphincter dysfunction was
also common: urinary incontinence in 17 (59%) and fecal
incontinence in 6 (21%) patients. Optic neuritis was present
in three patients, and it was the only symptom that argued
against the diagnosis of isolated transverse myelitis. MRI
revealed demyelinating lesions in the cervical cord in 19, in
the thoracic cord in 9 and in the lumbar cord in one patient.
The final diagnoses in our patients were as follows: six cases
of CIS, all confirmed as MS in follow-up, 11 patients with
DEM, three cases of neuromyelitis optica (NMO) (Fig. 1),
279
Fig. 2. Patient #31 (M/51), there is a small demyelinating lesion at the level
of the upper border of C6. There appears to be compression of the cord
just above the lesion by a herniated disk. The diagnosis was compressive
myelopathy.
one patient with a tumor, one with a compressive myelopa-
thy (Fig. 2), two patients had an ischemic myelopathy and in
seven the diagnosis was transverse myelopathy of unknown
origin (TM) (Table 1). Four of the 29 patients (14%) had nor-
mal spinal cord as well as brain MRI. These patients showed
the most devastating neurological deficit, characterized by a
complete spinal cord transection syndrome; follow-up MRI
after several months showed pronounced atrophy of the spinal
cord (Fig. 3).
In some cases of ATM with incomplete signs of spinal
cord transection, the lesions were either small, extending over
one to one and a half vertebral segments, or much larger,
extending over several segments (Figs. 4 and 5); sometimes
the lesions involved the entire axis of the spinal cord.
280 V.V. Brinar et al. / Clinical Neurology and Neurosurgery 108 (2006) 278–283

Table 1
CSF, neurophysiological and MRI findings of patients with symptoms suggestive of transverse myelitis
No. Patient Spinal MRI CSF cell OCB Systemic inflammatory Brain MRI VEP Final diagnosis
sex/age (demyelination) count disorders (serology)†
1 F/28 C6–7 U U N N Normal CIS
2 F/28a C2 22 OCB N N Normal CIS
3 M/25 C2–3 3 OCB N N Normal CIS
4 F/34 C3 18 OCB U N U CIS
5 F/36a* C1, C4 10 OCB N Left cerebellum U CIS
6 M/27* T10–11 49 OCB U Multiple lesions Prolonged latencies CIS
7 F/29 C2-T3 144 N N Optic nerve Prolonged latencies NMO
8 F/24# C2–5 14 OCB U Multiple lesions U NMO
9 F/36 C1–4 5 N N N Prolonged latencies NMO
10 F/25 Negative 8 N N N U TM
11 M/26 Negative 11 N ANF 1:32 N Prolonged latencies TM
12 F/54 Negative 3 N B2m 1.82 N Prolonged latenicies TM
13 F/62 Negative 7 OCB ANF1:16 N Prolonged latencies TM
14 M/52 T2 26 N N N Prolonged latencies TM
15 F/67 T2 12 N U N U TM
16 F/24a T5 5 N N N Normal TM
17 M/44* T4–6 17 OCB U 4–5 lesions Normal ADEM (neuroboreliosis?)
18 M/25a C2–3, T3–4, T8–9 122 U ACE 99 N Prolonged latencies ADEM
19 F/35 C2–C5 4 OCB N N Normal ADEM
20 M/44a C2 4 N N U ADEM
21 M/33# C2, C3–4 6 N N N Normal ADEM
22 F/25a C2 5 N AST 367 IJ/ml N Normal ADEM
23 F/34a C3–C6 U U N N U ADEM
24 M/45# Thoracic& to 8 OCB C3 2.24 (high) N Prolonged latencies ADEM
conus
25 M/26a* C2 35 N AST 317 IJ/ml, low CH50 Multiple lesions Prolonged latencies ADEM
26 F/36b Thoracic& 1 OCB N N Normal ADEM
27 F/26# C1–C5 16 N N N Normal RDEM
28 M/38 T9–12 55 N N N U Tumor (ependymoma)
29 M/77 C4–C7 5 OCB N N Normal Ischemic myelopathy
30 M/54 C2–3 7 N N N Normal Ischemic myelopathy
31 M/51# C5–6 1 N N N Normal Compressive myelopathy
F/xx: female/age, M/xx: male/age, N: negative, U: unavailable, OCB: oligoclonal bands, C: cervical spine, T: thoracic spine. ANF: antinuclear factor; B2m:
beta-2-microglobulin; ACE: angiotensin converting enzyme; AST: antistreptolysin O-test; C3: complement factor 3; CH50: complement activity.
* Patients described under Section 3.
# See figure.
& Involves entire axis of cord.

4. Comments
Patient 25 with slowly progressive paraparesis, a T9 sen-
sory level and sphincter disturbances was scheduled for
surgery because MRI of the cervical spinal cord revealed
a tumor-like lesion at the level of C2. Because brain MRI
showed multiple T2 hyperintense lesions, and he had a CSF
protein level of 55 mg%, 35 cells but no oligoclonal bands
(OCB), he was correctly diagnosed as DEM.
The patients who were diagnosed as having CIS were cat-
egorized as such only after they had a second bout. Patients
5 and 6 presented with signs of spinal cord involvement
(tetraparesis with sphincter disturbances and paraparesis with
T10 sensory level, respectively) and MRI lesions in the
spinal cord; it was only later on, when brain MRI revealed
clinically silent MS plaques and the CSF was positive for
OCBs, that they were diagnosed as having CIS (MS). The
spinal cord lesions of MS were usually multiple and did
not occupy the complete cord diameter. Brain MRI was
also very helpful, when showing dissemination of lesions in
space, pointing to the diagnosis of MS. The MRI lesions of
patients with ischemic myelopathy were discontinuous, par-
tially hyperintense and partially hypointense in T2-weighted
images.
The analysis of CSF in patients with TM and nor-
mal MRI findings revealed positive OCB in one patient
(#13), and elevation of total CSF protein in all four (range
55–67 mg%). Immunological tests were negative for sys-
temic autoimmune illnesses in all of those who were tested,
but serological tests for viruses and borreliosis revealed one
case of transverse myelitis possibly due to neuroborrelio-
sis (Table 1). The patient was treated with antibiotics but
his condition remained stationary. The final diagnosis was
DEM, based on the suspicion that the test for borreliosis
was a false positive, and his good response to corticosteroid
therapy.
 V.V. Brinar et al. / Clinical Neurology and Neurosurgery 108 (2006) 278–283
Fig. 3. Patient #24 (M/45) with paraplegia, sensory level and urinary incon-
tinence. MRI of the spinal cord revealed extensive demyelinating lesions
occupying the entire axis of the cord and extending from the lower thoracic
level to the conus. The final diagnosis was ADEM.
5. Discussion
ATM can be the clinical manifestation of various dis-
orders, have different presentations and be due to different
pathological processes; therefore, when the patient presents
with symptoms suggestive of ATM, an extensive differential
diagnosis must be considered, including different bacterial
[2,3] or viral [4–6] infections, autoimmune disorders such as
systemic lupus erytheatosus [7], mixed connective tissue dis-
ease [8], Sjögren’s syndrome [9], scleroderma [10], antiphos-
pholipid antibody syndrome [11], ankylosing spondylitis [12]
and sarcoidosis [13]. All these conditions are quite rare and
sporadic, but once they are excluded, the need still exists to
281
Fig. 4. Patient #21 (M/33) with left hemiparesis and paresthesiae of the left
side of the body. MRI of the cervical spinal cord revealed two demyelinating
lesions at C2 and at C3–4, involving the entire cord diameter. Both lesions
enhanced with gadolinium. This patient was diagnosed as having ADEM.
distinguish between recurrent demyelinating diseases such
as MS, different forms of DEM (acute, recurrent, multipha-
sic), acute and recurrent NMO [14] or with endocrinopathies
[15], which may prove to be quite difficult. Furthermore, MRI
findings in patients with ATM are often described as local
enlargement of the cord and increased signal intensity on
T2 weighted sequences, which is not sufficient to differenti-
ate this condition from intramedullary tumors, compressive
282 V.V. Brinar et al. / Clinical Neurology and Neurosurgery 108 (2006) 278–283
Fig. 5. Patient #27 (F/26) with tetraparesis and C3 sensory level. MRI of the
cervical spinal cord revealed a large demyelinating lesion in T2 weighted
images extending from C1 to 5, which enlarges the cord, and enhances after
gadolinium infusion. The final diagnosis was recurrent DEM.
spinal cord disease [16,17], hematomas and ischemic events
[18]. On the other hand, about 40% of acute transverse
myelopathies cannot be documented by MRI [19]; in fact,
this was the case with four of our patients despite the fact
that they had the symptoms of complete cord transection. If
the patient is evaluated on the basis of the current diagnostic
criteria [20], it may be possible to distinguish idiopathic TM
from disease-associated TM. This is clearly very important,
because patients with disease-associated transverse myelitis
may need to be closely monitored for recurrent episodes of
neurological recurrences and should be offered immunosup-
pressive treatment [21]. An important consideration in cases
of ATM is their progression to MS or recurrent forms of DEM.
It has been suggested that patients with multifocal lesions in
the spinal cord, demyelinating lesions in the brain and optic
nerves, OCB in the CSF and clinical or laboratory evidence
of systemic autoimmune disorder are at greater risk of recur-
rence [21,22]. It is well known that about 80% of patients
with spinal cord demyelination and asymmetrical clinical
symptoms develop MS, and such cases in our series were cat-
egorized as CIS. On the other hand, patients with large lesions
occupying the whole spinal cord, or with long lesions mostly
localized in the posterior part of the cord were regarded as
DEM, which is mostly but not always monophasic disease
[1]. Patients with concomitant demyelinating lesions of optic
nerve were diagnosed as NMO, a variant of recurrent DEM
[1].
An interesting and puzzling situation is the remarkable
number of patients with clear clinical evidence of spinal cord
symptoms, but no demyelination by MRI. Analysis of CSF
in these patients with normal spinal MRI showed positive
OCB (patient F/62) and elevated protein levels, underscoring
the importance of such studies. In this group of patients with
symmetrical clinical symptoms of transverse spinal cord syn-
drome, with normal brain and spinal cord MRIs, we suggest
that parenchymatous neuronal lesions are the sites of patho-
logical involvement, analogous to the acute axonal lesions
of axonal polyneuropathy; we believe that they are proba-
bly variants of DEM but without a vigorous inflammatory
reaction and demyelination.
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