LETTER TO THE EDITOR

Longitudinal myelitis in systemic lupus erythematosus:a paediatric case

Andrea Campana • Paola Sabrina Buonuomo •

Antonella Insalaco • Claudia Bracaglia • Matteo Di Capua •

Elisabetta Cortis • Alberto G. Ugazio

Accepted: 17 May 2009 / Published online: 27 July 2011

� Springer-Verlag 2011

Abstract Acute transverse myelitis (ATM) is a very rare

manifestation of the central nervous system in systemic

lupus erythematosus (SLE), especially in case of involve-

ment of continuous segments (longitudinal myelitis). We

describe a 12-year-old female with lupus correlated with

transverse myelitis with a longitudinal involvement of the

spinal cord (D2 to D10) at the onset of the disease. Despite

the administration of an early aggressive therapy, the out-

come proved to be unfavourable. After 2 years of follow-

up, the child still complains of paraplegia, sphincter

incontinency and ipo-paresthesias of both legs.

Keywords Acute transverse myelitis (ATM) � Systemic

lupus erythematosus (SLE)

Dear editor,

SLE is a multisystemic disease that can affect any organ

and system in the body. Central nervous system (CNS)

involvement constitutes the second most common cause of

death after renal failure and is also a leading cause of

morbidity in patients with SLE. ATM is a serious and rare

CNS manifestation of SLE that usually involves one to four

segments of the thoracic or cervical spinal cord [1–3].

Myelitis may present at the onset of SLE, but more

often, it occurs during the disease course. It was proposed

to name ‘‘longitudinal myelitis’’ when many and continu-

ous segments are involved. The onset is acute and char-

acterized by severe low back pain, sphincter dysfunction,

paraparesis and sensory-level paresthesias. The prognosis

is generally poor since it can generate a prolonged paralysis

or even death.

We describe the clinical and neurological features of a

paediatric patient with longitudinal involvement of nine

spinal segments (D2 to D10) on the onset of SLE. SLE has

been diagnosed in a 12-year-old Caucasian girl that

developed a rapid onset of paraplegia. Two years before,

she had been diagnosed with mild thrombocytopenia

(40,000 per mm3). Until her admission, the number of

platelet was 80,000–1,000,000 per mm3. Some days before

the admission, she developed fever, headache, arthralgias,

abdominal pain, diarrhoea and vomiting. At admission, she

presented malar rash, mild dehydration, abdominal pain

and fever (40�C). Laboratory examinations showed a white

blood cell count of 3,800 per mm3, haemoglobin 13.4 gr/dl,

58,000 per mm3 platelets, PCR 0.62 mg/dl, erythrocyte

sedimentation rate 48 mm/h, anti-dsDNA 1:320 U/ml, C3

61 mg/dl; C4 8 mg/dl and ANA 1:640. Lupus coagulant

and anticardiolipin antibody were negative. The next day,

her general conditions worsened, fever persisted, and she

developed severe low back pain and acute urinary reten-

tion; in the following hours, she presented rapidly evolving

weakness and numbness of both legs with absent knee and

ankle reflex that within a few hours evolved into a com-

plete paralysis. The cerebrospinal fluid analysis showed no

pleocytosis and normal values of protein and glucose.

Urinalysis, blood and cerebrospinal fluid culture were

unremarkable. The spinal cord MRI showed an increased

signal in correspondence with T2, typical of transverse

A. Campana � P. S. Buonuomo (&) � A. Insalaco �C. Bracaglia � E. Cortis � A. G. Ugazio

Department of Medicine, Division of Rheumatology,

Bambino Gesu Children’s Hospital, Rome, Italy

e-mail: sabrina.buonuomo@gmail.com

M. D. Capua

Department of Child Neuropsychiatry, Bambino Gesu Children’s

Hospital, Rome, Italy

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Rheumatol Int (2012) 32:2587–2588

DOI 10.1007/s00296-011-2061-1

myelitis with longitudinal involvement of the spinal cord

from D1 to D10. The somatosensory evoked potentials

after tibial nerve stimulation showed a normal spinal cord

response at T12 level but the absence of a cortical poten-

tial. This is suggestive for a lesion of the central somato-

sensory pathways above the T12 spinal level. The magnetic

transcranial stimulation of the motor cortex did not produce

any muscular response from the lower limbs; instead, the

stimulation of the lumbar roots evoked a normal motor

response. This is in agreement with a lesion of the motor

pathways along the spinal cord. She was immediately

treated with high-dose corticosteroids (methylprednisolone

1 g i.v. for 3 days repeated after 1 week) and i.v. pulse

cyclophosphamide (500 mg/m2 in 2 days and 750 mg/m2

monthly for the following 6 months). Subsequently,

oral methylprednisolone was continued at 2 mg/kg/day.

Although the treatment reduced the symptoms, the patient

still complained of sensory and strength deficit in both legs

with knee and ankle reflexes absent bilaterally. A second

MRI performed after 1 month showed a D6 segmental

atrophy with a marked signal alteration from D2 to D10,

suggesting myelomalacia. After 2 years of follow-up, the

patient has not active disease, but no neurological

improvement occurred, persisting sphincter incontinency

and paraplegia.

Discussion

ATM is a rare manifestation of SLE and generally has a

poor prognosis. It has been demonstrated that its evolution

is influenced by factors such as the rapidity of diagnosis,

the extent of involvement and a prompt treatment [4, 5]. If

the infectious aetiology is unlikely, several mechanisms

have been suggested such as vascular injuries secondary to

immune-complex-mediated vasculitis, hypercoagulability

from aPL or non-vascular injuries caused by antineuronal

antibodies and white matter degeneration. Several reports

indicate that there is a significant association between

transverse myelopathy and the presence of antiphospho-

lipid antibodies [5]. The differential diagnosis of transverse

myelopathy [6] in patients with SLE includes the follow-

ing: acute transverse myelitis, viral infection, medullar

compression due to vertebral fractures, epidural or subdural

lipomatosis, epidural and/or paraspinal abscess, compli-

cating disc space infection and atlantoaxial subluxation.

MRI is the radiological investigation of choice in the

management to detect transverse myelitis correlated with

lupus [7, 8].

The prognosis is generally poor and is related to factors

such as diagnosis rapidity, extension of the lesion and

prompt treatment. Treatment with high-dose corticoste-

roids and pulse cyclophosphamide has been adopted by

most authors in recent years [9]. Because of the rarity of

lupus transverse myelitis, no double-blind central studies

are available to compare the efficacy of pulse cyclophos-

phamide with that of high-dose i.v. corticosteroids.

Although some satisfactory responses and outcomes have

been reported, our clinical report underlines how prognosis

remains inauspicious, in spite of prompt recognition and

early aggressive therapy, attributable to the rapid evolution

and degree of spinal cord involvement.

References

1. Lehnhardt FG, Impekoven P, Rubbert A, Burghaus L, Neveling M,

Heiss WD, Jacobs MD (2004) Recurrent longitudinal myelitis as

primary manifestation of SLE. Neurology 23:63 (10):1976

2. Chen HC, Lai JH, Juan CJ, Kuo SY, Chang DH (2004)

Longitudinal myelitis as an initial manifestation of systemic lupus

erythematosus. Am J Med Sci 327(2):105–108

3. Kovacs B, Lafferty T, Brent LH, De Horatius RJ (2000)

Transverse myelopathy in systemic lupus erithematosus; an

analysis of 14 cases and review of the literature. Ann Rheum

Dis 59(2):120–124

4. Kimura KY, Seino Y, Hirayama Y, Aramaki T, Yamaguchi H,

Amano F, Takano T (2002) Systemic lupus erythematosus related

transverse myelitis presenting longitudinal involvements of spinal

cord. Int Med 41:156–160, 2000; 59:120–124

5. D’Cruz DP, Mellor-Pita S, Joven B et al (2004) Transverse

myelitis as the first manifestation of systemic lupus erythematosus

or lupus-like disease: good functional outcome and relevance of

antiphospholipid antibodies. J Rheumatol 31:280–285

6. Mok CC, Lau CS, Chan EYT, Wong RWS (1998) Acute transverse

myelopathy in systemic lupus erythematosus: clinical presentation,

treatment and outcome. J Rheumatol 25:467–473

7. Provenzale JM, Barboriak DP, Gaensler EHL, Robertson RL,

Mercer B (1994) Lupus related myelitis: serial MR findings. Am J

Neuroradiol 15:1911–1917

8. Boumpas DT, Patronas NJ, Dalakas MC, Hakim CA, Klippel JH,

Balow JE (1990) Acute transverse myelitis in systemic lupus

erythematosus: magnetic resonance imaging and review of liter-

ature. J Rheumatol 17:89–92

9. Bavile L, Lavalle C (1992) Transverse myelitis in systemic lupus

erythematosus—the effect of IV pulse methylprednisolone and

cyclophosphamide. J Rheumatol 19:370–372

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