Scandinavian Journal of Rheumatology

ISSN: 0300-9742 (Print) 1502-7732 (Online) Journal homepage: http://www.tandfonline.com/loi/irhe20

Spastic quadriparesis: an unusual early

manifestation of systemic lupus erythematosus

A. Gupta, S. Singh, P. Singh, J. Ahluwalia, S. Rath & R.W. Minz

To cite this article: A. Gupta, S. Singh, P. Singh, J. Ahluwalia, S. Rath & R.W. Minz (2003) Spastic
quadriparesis: an unusual early manifestation of systemic lupus erythematosus, Scandinavian
Journal of Rheumatology, 32:3, 189-190, DOI: 10.1080/03009740310002579

To link to this article: http://dx.doi.org/10.1080/03009740310002579

Published online: 12 Jul 2009.

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 Scand J Rheumatol 2003;32:189–190

CASE REPORT

Spastic quadriparesis: an unusual early manifestation of systemic

lupus erythematosus

A. Gupta1, S. Singh1, P. Singh2, J. Ahluwalia3, S. Rath1, and R.W. Minz4

Departments of 1Pediatrics, 2Radiodiagnosis, 3Hematology, and 4Immunopathology Postgraduate Institute of Medical Education and
Research, Chandigarh, India

Systemic lupus erythematosus (SLE) is an uncommon immunological disorder with multisystemic involvement. Neurological and
neuropsychiatric manifestations in this disease are multifactorial and can involve any part of this system. We describe one patient presenting
with spastic quadriparesis as an early clinical manifestation of SLE. This disease should be kept in mind in such a setting, especially if
abnormalities in hemogram and urine analysis are seen. Early diagnosis and aggressive therapy may improve the neurological outcome.

Key words: SLE, childhood, spastic quadriparesis

Downloaded by [University of Cincinnati Libraries] at 12:09 30 May 2016

Systemic lupus erythematosus (SLE) is an uncom-
mon immunological disorder with multisystemic
involvement (1). Neurological and neuropsychiatric
manifestations in this disease have been described in
18 – 70% cases and usually occur late in the course of
the illness (2). These manifestations may be asso-
ciated with disease activity, superimposed infections,
toxins or metabolic derangements (3), however the
exact pathogenesis still remains unknown. Though
almost all parts of the neurological system can be
involved in this disease (4), acute spastic quadri-
paresis has been rarely reported in the pediatric
literature (5 – 7). We report one such case.
Case report
A ten year old girl presented to us with a history of
fever and weight loss for last two months and
weakness of all four limbs for one month. She had
been bedridden for the last fifteen days. There was
no history of trauma, seizures, headache, altered
sensorium or bladder and bowel complaints. At
admission, she was found to be grossly emaciated
(weight for height 54% of expected, height for age
96% of expected). She was euthermic with pulse rate
of 90/min, blood pressure of 100/70 mmHg and
respiratory rate of 16/min. She had mild pallor and
generalized adenopathy. Neurological examination
revealed spastic quadriparesis with brisk deep tendon
reflexes and upgoing plantars. There was no sensory
Surjit Singh, Advanced Pediatric Center, Postgraduate Institute of
Medical Education and Research, Chandigarh, India – 160012.
E-mail: surjitsingh@sify.com
Received 10 December 2002
Accepted 24 February 2003
or bladder /bowel involvement. She had normal
sensorium and there were no cranial nerve palsies or
meningeal signs. The other systems were unremarkable.
Investigations revealed presence of microcytic,
hypochromic anemia with a hemoglobin level of
7.8 gm/dL and thrombocytopenia (platelet count
87000/cu.mm.). Total and differential leucocyte
counts and liver and renal function tests were
normal. Cerebrospinal fluid (CSF) examination
showed clear acellular fluid with no malignant cells
and normal sugar and protein content. Bacterial,
mycobacterial and fungal cultures of CSF were
noncontributory. Adenine deaminase (ADA) levels
in CSF were normal. Mantoux test (1TU) was
nonreactive and chest radiograph was normal. Fine
needle aspiration cytology from the right axillary
lymph node showed reactive hyperplasia. Serological
tests for syphilis and human immunodeficiency virus
(HIV) infection were normal.
During the hospital stay, she developed a faint
malar rash, painless oral ulcers, emotional lability
and retention of urine. This symptom-complex led to
a clinical suspicion of SLE and she was investigated
accordingly. Antinuclear factor was 4z with a
peripheral and diffuse pattern. Anti-dsDNA titres
were 220.25 IU/ml (normal v55 IU/ml). C3 level
was 52.3 mg/dL (normal 50 – 120 mg/dL). T2
weighted images of magnetic resonance imaging
(MRI) of brain and spine showed multiple foci of
increased signal intensity in bilateral corona radiata
and centrum semiovale. Her antiphospholipid anti-
body workup revealed a positive lupus anticoagu-
lant, IgM anticardiolipin antibody titres of 12 MPL
units (normalv7 MPL units) and IgG anticardio-
lipin antibody titres of 10.10 GPL units (nor-
malv20 GPL units).

# 2003 Taylor & Francis on license from Scandinavian Rheumatology Research Foundation 189
 A. Gupta et al.
With a provisional diagnosis of neuropsychiatric
SLE, she was started on intravenous pulse dexa-
methasone at a dose of 5 mg/kg/day for five days
followed by oral prednisone at a dose of 2 mg/kg/
day and azathioprine at a dose of 2 mg/kg/day. She
showed a remarkable recovery in her neurological
symptoms within two months. She has been followed
up for two and a half years after her initial
presentation and her neurological examination is
completely normal. She is now on a maintenance
dose of prednisolone (5 mg/day) and azathioprine
(37.5 mg/day).
Discussion
Since the first description of spastic quadriplegia in
SLE by Hardie et al (8) in 1985, a number of adult
Downloaded by [University of Cincinnati Libraries] at 12:09 30 May 2016
SLE patients with spastic quadriparesis due to
transverse myelitis have been reported (9 – 12).
However, we could come across only three reports
of spastic quadriparesis in children with SLE (5 – 7).
Spastic quadriparesis being the first and sole
manifestation of SLE at presentation was seen in
only one of the previously reported cases (7). If
manifestations of other organ system involvement
are absent, as was seen in our case and in a
previously reported case by Iqbal et al (7), making a
clinical diagnosis of SLE can be very difficult.
Abnormalities of hemogram and urine examination
may give a clue to the diagnosis, as these are
reported to be positive in as high as 85% patients
presenting with atypical manifestations (7). Anemia
and thrombocytopenia were present in our patient at
admission, but the diagnosis was suspected only
when she developed oral ulcers and malar rash.
Pathologically, quadriparesis has been usually
linked to transverse myelitis and hence presents with
sensory as well as prominent bladder/bowel involve-
ment in most of the patients (6, 8 – 10). Our child is
different from the previously reported cases because
she had pure motor spastic quadriparesis and there
was no evidence of transverse myelitis on imaging.
Spastic quadriparesis in our child seems to be related
to the involvement of upper motor neuron fibres in
corona radiata, which was demonstrated in MRI as
increased signal intensity in the same regions. This
seems to be an unusual aspect of our patient.
190
Therapy involves use of immunosuppressive
agents besides supportive care (6, 10, 12). Choice
of immunosuppressants is largely empirical and
steroids are used most frequently. Most patients
with transverse myelitis show significant but variable
recovery (10, 12). Our patient showed a complete
recovery with therapy and is now completely
asymptomatic.
In summary, spastic quadriparesis can be the
only presentation of childhood SLE. Early diagnosis
and aggressive treatment can result in prompt
recovery.
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