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A 13-year-old Malay boy presented with a 3-week history of weakness and numbness of the upper and lower limbs associated with severe neck pain aggravated by movements. The weakness progressively worsened and one week prior to admission he became bedridden. One month earlier he had had fever and night sweats associated with poor appetite and weight loss. There was urinary retention and poor bowel opening. There was no family or past history of tuberculosis. Physical examination revealed an ill-looking boy with a temperature of 40°C. Mental state and other vital signs were stable. There were multiple cervical lymphadenopathies, hepatomegaly (liver span 16 cm) and an ill-defined mass in the umbilical region. There were signs of meningeal irritation. He had a spastic tetraparesis (muscle power grade 2/5 on the MRC scale for the upper limbs, 3/5 for the lower limbs) with exaggerated deep tendon reflexes and bilateral extensor plantar responses. There was loss of sensation to all sensory modalities up to the fifth cervical (C5) dermatome. Examination of the cranial nerves was normal. Investigations showed haemoglobin 10.7 g/dl, total white blood cell count 7.9 × 109/l (normal differential count), platelet count 631 × 109/l and erythrocyte sedimentation rate 52 mm in the first hour. Microscopic examination of the peripheral blood films was normal. Apart from an elevated serum alkaline phosphate of 328 IU/l, blood biochemical parameters were normal. Blood cultures were negative. The chest radiograph was normal. Magnetic resonance imaging (MRI) of the cervical spine are shown in figures 1 and2.
- What do the MR images show?
- What are the possible diagnoses?
- What further investigations are required to confirm the diagnosis?
Sagittal T1-weighted MRI scans of the neck (figure 1) show extradural compression of the cervical cord from the level of the second (C2) to sixth (C6) cervical vertebrae. The axial images show that the dura at these levels is markedly thickened (particularly at the left posterolateral aspect of the cord) and enhances after gadolinium injection (figure 2). The C4, C5 and C6 vertebral bodies show low signal intensity on the sagittal MRI (figure 1A) that enhances with gadolinium (figure 1B); patchy enhancement is also evident within the cord. The discs appear intact and there was no vertebral collapse. The MRI findings confirm involvement of the vertebral bodies, dural and spinal cord extending from the C2 to C6 vertebrae level.
The possible diagnoses for the neurological lesions include tuberculosis or lymphoma. The radiological findings are not compatible with an abscess or haematoma. The patient's unexplained fever, night sweats and weight loss are consistent with the constitutional symptoms of tuberculosis or non-Hodgkin's lymphomas (NHL). In developing countries tuberculosis of the central nervous system (CNS) is the most common type of subacute CNS infection and is a disease of younger age group, usually childhood. The meninges, brain, or spinal cord may all be affected, either individually or in various combinations. The spine is involved in less than 1% of patients with only 10% affecting the cervical segments and there is often destruction of the intervening disc spaces. Spinal cord and meningeal involvement are relatively common neurological complications of NHL with spinal cord segments C5 to T8 being most commonly affected.
The main investigations will include a detailed work-up for tuberculosis and lymphoma. The Mantoux test was non-reactive and sputum and urine examinations were negative for acid-fast bacilli. Cerebrospinal fluid analysis was unremarkable. CT of the abdomen showed numerous hypodense lesions in the liver, pancreas and kidneys. The bowel wall appeared thickened and there were masses in the para-aortic region (figure 3). The spleen was not enlarged and was free of lesion. CT of the brain and thorax were normal. Biopsy for histological diagnosis is essential in this case. The histology of the lesions in the liver, para-aortic masses and the bone marrow aspirate (figure 4) was consistent with Burkitt's lymphoma and negative for tuberculosis. HIV antibodies were negative. Serology, culture and molecular studies to detect concomitant tuberculous infection were negative.
the absence of pancytopaenia or leucoerythroblastic change does not exclude significant bone marrow infiltration
bone marrow examination remains an important diagnostic tool when the diagnosis of lymphoma is clinically suspected
NHL patients with CNS disease at presentation often have marrow involvement and typically have aggressive histology
NHL in children often grows rapidly, therefore metastatic disease at the time of first presentation is common
in NHL, the spleen may be clinically and radiologically normal in the presence of widespread disease
hospitalisation more than a week after the development of tetraparesis with sphincter dysfunction invariably results in permanent neurological sequelae
rapidly evolving tetraparesis or paraparesis represents a neurological emergency; early treatment is critical to restore full neurological function
CNS lymphoma can be primary or secondary. Secondary CNS involvement has been reported in 5–12% of patients with NHL and in up to 30% of those with high-grade NHL.1 The histological type of NHL is the most significant risk factor for secondary CNS involvement. The highest incidence is observed in patients with lymphoblastic or sporadic Burkitt's lymphoma.2 Other factors that may increase the risk of CNS involvement are the presence of an underlying immune deficiency, age below 40 years, and other extranodal sites (especially bone marrow, bone or testis).2 Lymphoma can cause neurologic symptoms in various ways. It can infiltrate the meninges or brain parenchyma and metastasize to bone or infiltrate the epidural space via the intervertebral foramina to cause neurologic dysfunction by compressing adjacent CNS structures. Direct CNS invasion by lymphoma is associated with short survival despite intensive therapy.3 Neurologic dysfunction may develop as a result of infections that occur secondary to immunosuppression, or paraneoplastic syndromes.
Burkitt's lymphoma is an aggressive, rapidly dividing B-cell lymphoma most commonly seen in children. The staging evaluation should be considered a medical emergency, and the patient should receive chemotherapy within 48 to 72 hours after establishing the diagnosis. In the majority of patients, the peripheral blood shows no abnormality. Even when the bone marrow is heavily infiltrated, circulating lymphoma cells are present in less than half of the patients. Among patients with Burkitt's lymphoma,4 CNS disease at the time of diagnosis is associated with the greatest risk of treatment failure. The clinical condition at the onset of treatment is the most important prognostic indicator. The likelihood that decompressive surgery and radiation for spinal metastasis will restore neurological function is minimized if the patients had total weakness of more than 12 hours duration, sphincter loss of more than 24 hours duration, major sensory loss, and uncontrolled metastatic disease elsewhere.5
Our patient underwent a laminectomy and received intensive chemotherapy with no improvement in the neurological status. Two weeks after initiation of chemotherapy, he died of neutropenic sepsis.
Stage IV Burkitt's lymphoma.
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