Indian Pediatrics 1998;35:1015-1017 

Spinal Neuroblastoma Masquer- ading as Acute Flaccid Paralysis

Riyaz A.
Vinayan K.P.

From the Departments of Pediatrics Institute of Maternal and Child Health, Medical College, Calicut, Kerala, India.

Reprint requests: Dr. Riyaz A, Assistant Professor and Pediatric Gastroenterologist, Department of Pediatrics, Institute of Maternal and Child Health, Medical College,
Calicut 8, India.
Manuscript Received: December 17, 1997; Initial review completed: January 19, 1998; Revision Accepted: May 1, 1998.

 

Spinal cord compression is a rare complication of malignant tumors of childhood. The most common causes are bone and soft tissue sarcoma, lymphoma, leukemia and neuroblastoma(1). They produce an acute block in the CSF pathway, owing to the rapid growth within the confined space. Such children usually present with flaccid paraplegia, urinary retention and patulous anus. Here we are reporting a rare case of intraspinal neuroblastoma, which presented as acute flaccid paralysis, unfortunately following pulse polio immunization. A correct diagnosis was extremely vital in this case, because of its serious consequences to the pulse polio proramme.

Case Report

A one-year-girl who was apparently healthy previously was admitted with a history of insidious onset of weakness of both lower limbs, one week after a pulse polio immunization. She had low grade intermittent fever for 3 days. There was no history of convulsions. She was conscious and oriented. The muscle power was Grade I in both lower limbs which became Grade a within 3 days of admission. The deep tendon reflexes were absent in the lower limbs and plantars were equivocal. Abdominal reflexes were normal. Sensory system and bladder functions could not be tested. The upper limbs and neck muscles were normal. She. did not have significant lymphadenopathy or hepatosplenomegaly. She was unimmunized, except for the single pulse polio vaccine.

CSF showed features of Froin syndrome, the protein being 6g/dl. X-ray spine and USG abdomen were normal. MRI scan showed a large intradural lesion with extradural extension in L₁ to L₅ region, of intermediate signal density in both T₁ and T₂ weighted image (Figs. 1&2).
L₃-L₄ right sided laminectomy and biopsy decompression were done under GA. Histopathology was suggestive of neuroblastoma. Twenty four hour urinary VMA level was normal. The neurologic recovery was poor inspite of post-operative radiation.

Fig. 1. T3 weighted MR coronal images of the spine showing a large intradural tumor (arrow) with extradural extension (L1-L5) with intermediate signal intensity.

 

Fig. 2. T2 weighted MR axial images of the spine showing the tumor (arrow).

Discussion

Spinal cord tumors are .rarer than intracranial tumors, in both adults and children. The ratio of intracranial/intraspinal neoplasms for children varies from 20:1 to 5:1(2). These are most commonly found In the thoracic spinal cord followed by cervical, thoraco-lumbar and lumbo sacral areas(3).

Signs and symptoms of spinal cord tumors are usually insidious and misleading. Because of the vague history and paucity of clinical signs initially, a definitive diagnosis may be delayed. The most common com- plaints are abnormalities of gait or pain in the extremities or back. In the very young, who are not bowel or bladder trained, sphincter abnormalities may be over- looked(4).

Traditionally, tumors of the spinal cord are divided into intramedullary and extramedullary. Extramedullary tumors usually represent benign neurofibroma or malignant metastases from tumors like neuroblastoma. Neuroblastoma usually produces spinal cord compression, due to dumb bell extension of the tumor arising from the para-spinal sympathetic ganglia(5).

The point of interest in this case was the development of acute flaccid paralysis in an unimmunized child, following a dose of pulse polio immunization. This created a lot of panic in the public and medical personnel and it would have been a severe set back to the entire Pulse Polio Immunization Programme, if the correct diagnosis had not been established in time.
 

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