JRSM >
Volume 97, Number 3 >
Pp. 125-127
Department of Haematology and Oncology, Great Ormond Street Hospital for
Children, London WC1N 5JH, UK
1 Department of Radiology, Great Ormond Street Hospital for Children, London
WC1N 5JH, UK
Correspondence to: Dr R Antony, c/o Dr S Arun, 79 Bloomsbury Close, Western Gardens, London W5 3SF, UK E-mail: reubena{at}hotmail.com
Acute paraplegia in childhood requires immediate diagnostic evaluation and treatment. Lymphoblastic leukaemia/lymphoma is a cause easily missed if signs of bone marrow dysfunction are absent.
CASE HISTORIES
Case 1
A 4-year-old boy complained of knee pain and had increasing difficulty with
weight-bearing for five weeks. A week before admission he stopped walking
completely and the pain spread to involve most of his bones, especially his
spine and ribs. He also described numbness over his thighs and strained to
pass urine and stool. At this time an antero-posterior X-ray of his spine
showed no abnormality. Intermittent spinal traction for five days was of no
benefit, and on the day before admission an MRI of his spine was done. This
showed a paraspinal mass from T2 to T8 passing through the intervertebral
foramina into the spinal epidural space and compressing the cord
(Figure 1). The radiological
differential diagnosis was neuroblastoma or non-Hodgkin lymphoma. After
starting dexamethasone for tumour-related cord compression he was transferred
to our centre for further management. The relevant physical findings were
hepatomegaly, decreased power and sensation in the lower limbs, and bilateral
calf wasting more pronounced on the left. Though his full blood count was
normal, leukaemic blast cells were seen on a peripheral blood film. The bone
marrow contained 61% blast cells and immunophenotyping was positive for CD10,
CD19 and CD79a. Acute lymphoblastic leukaemia was diagnosed and he was started
on treatment according to the UK ALL 2003 protocol. Within a week his lower
limb function was improving.
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Case 2
A girl of 4 had experienced fourteen days of progressive lower limb
weakness following two episodes of upper respiratory tract infection. The
initial clinical diagnosis was Guillain–Barré syndrome and she
was treated with intravenous immunoglobulin. An electromyogram three days
after admission was normal and there was no response to immunoglobulin. MRI of
her brain and spine then showed a mass from T2 to T10 compressing the spinal
cord (Figure 2) and she was
started on dexamethasone. On transfer to our centre she had flaccid paralysis
of both lower limbs and absent deep tendon reflexes, but bladder and bowel
function and lower limb sensation were intact. The white cell count was not
raised and no blasts were seen on a peripheral smear. The initial diagnosis
was neuroblastoma and she was treated with carboplatin, vincristine and
etoposide. On histological examination the cellular elements of the mass were
strongly positive for CD79a, TDT and CD10 and the diagnosis was revised to pre
B cell lymphoblastic lymphoma. Bone marrow was normal. She was started on
treatment according to the MRC ALL 97 protocol and a repeat MRI 2 days later
showed almost complete disappearance of the tumour mass
(Figure 2b). After intensive
physiotherapy for eight months she was walking with support.
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COMMENT
The common causes of acute lower limb paraplegia in childhood are spinal cord tumours, epidural abscess, haematoma and transverse myelitis. Guillain–Barré syndrome can present as an ascending lower limb paralysis. Back pain in children is unusual and should always be investigated to rule out serious underlying illness. In both the children we are reporting, neurological symptoms progressed considerably before appropriate treatment was started. While a neurological examination can help determine the level of involvement of the spinal cord, imaging with MRI is necessary both for diagnosis and for planning of further investigations and treatment. Leukaemic/lymphomatous masses spread via lymphatics and through intervertebral foramina and cause cord compression before bone destruction.1 So while plain X-rays are useful in detecting bony involvement in primary or metastatic solid tumours they can be falsely reassuring in lymphoid malignancies.2 In the absence of bone marrow or peripheral blood involvement a biopsy of the spinal mass may be necessary. Examination of cerebrospinal fluid may help with the diagnosis but a lumbar puncture may not be safe and a neurosurgical opinion should first be sought. Acute lymphoid malignancies are common in childhood. Patients with acute lymphoblastic leukaemia/lymphoma usually present with infections or signs of bone marrow dysfunction such as anaemia and bruising. An initial presentation with lower limb weakness is exceedingly rare. In one series of 1412 children with acute lymphocytic leukaemia only 5 (0.35%) presented with a spinal mass.2 Early chemotherapy and physical rehabilitation offer the best chance of cure and functional recovery.
Acknowledgments
We thank the Department of Medical Illustration, Great Ormond Street Hospital for Children, for their help.
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