Indian Journal of Medical Sciences Medknow Publications on behalf of Indian Journal of Medical Sciences Trust ISSN: 0019-5359 EISSN: 1998-3654 Vol. 57, Num. 12, 2003, pp. 556-558

Indian Journal of Medical Sciences, Volume 57, Number 12, December 2003, pp. 556-558

Case report

Necrotizing myelitis in an immunocompetent child: A case report with review of literature

R C Parmar, S B Bavdekar,* P Sira,** J R Kamat*

MD, DNB, DCH, FCPS; *MD, DCH; **DCH; Department of Pediatrics, Seth G. S. Medical College and K.E.M. Hospital, Parel, Mumbai - 400012, India.
Correspondence: Ramesh C. Parmar, 2/11, Ambika Nivas, Prabhat Colony, Santacruz (E), Mumbai - 400055, India.

Accepted Date: 21-11-2002

Code Number: ms03047

ABSTRACT

A few cases of necrotizing myelitis have been reported in adults since its first description in 1973. No case has been described in the pediatric age group. A 12-year-old boy, who presented with acute flaccid paraplegia, loss of sphincter control and sensory loss showed features suggestive of necrotizing myelitis on magnetic resonance imaging. Investigations carried out could not reveal a specific etiological or pre-disposing factor. No clinical improvement occurred despite the therapy.

KEY WORDS: Necrotising myelitis, Paraplegia, Spinal disorders, Magnetic resonance imaging.

INTRODUCTION

Necrotizing myelitis is a rare neurological disorder with fewer than 50 cases being reported in the world literature (MEDLINE search 1965-2003). We report a case of necrotizing myelitis in a 12-year-old boy, an entity not previously reported in childhood.

CASE REPORT

A 12-year-old completely immunized boy presented with a sudden onset weakness involving both the lower limbs of 12 days duration. Incontinence of bowel, retention of urine and loss of sensations in the lower limbs accompanied it. There was no history of trauma, fever, tuberculosis or tuberculous contact. The patient had not received any sera, vaccinations or intra-muscular injections in the recent past. His examination revealed a symmetrical lower motor neuron type of weakness affecting both lower limbs with total inability to perform any movement at hips, knees, ankles and smaller joints of the feet. Both the lower limbs were hypotonic and knee and ankle deep tendon reflexes were absent. All the sensations were lost below the T 10 dermatomal level. The gluteal, abdominal and paraspinal reflexes were absent. There were no abnormalities detected in examination of higher functions, cranial nerves or upper limbs. Signs of cerebellar dysfunction and meningeal irritation were absent. Examination of the spine did not reveal any abnormality. The rest of the clinical examination was non-contributory.

Magnetic resonance imaging (MRI) revealed markedly abnormal hyperintense signals suggesting hemorrhagic changes involving the cord parenchyma involving the entire dorsal cord on T1 weighted images. It was associated with a marked diffuse cord swelling with effacement of the subarachnoid space. The T2 weighted images revealed abnormal bright signals in the entire cord. No abnormal enhancements were seen on post-Gadolinium T1-weighted images. These features were suggestive of necrotizing myelitis.

The patient's serum was negative for antibodies against HIV-1, HIV-2 and Herpes simplex viruses and IgM antibodies against Mycoplasma. Because of financial constraints, tests for detection of antibodies against cytomegalovirus could not be carried out. Despite treatment with high dose methylprednisolone therapy in conjunction with oral acyclovir and tetracycline, patient's clinical condition further deteriorated. He developed respiratory paralysis from which he could not be revived in spite of respiratory support. At autopsy, on gross examination the spinal cord was edematous and distorted in shape. Histopathological examination of spinal cord showed confluent necrosis of both neurons and supportive stroma with gliosis and marked swelling of axons with edematous spongy white matter. Few areas in addition revealed fibrinoid necrosis of larger blood vessels with perivascular lymphocytic aggregation. All the above changes were most marked in the cervical spinal cord.

DISCUSSION

Necrotizing myelitis is a rare inflammatory disorder of the spinal cord.¹ Only a few cases have been reported after Nagaswami et al first described the entity in 1973.²

The reported cases have presented with acute flaccid paraplegia with sphincteric involvement and loss of sensations.^2-4 This has also been the presentation in our case. Atypical presentations have included progressive gait disturbances, lumbago⁵ and fatality due to respiratory involvement. Neuroimaging provides clue to the diagnosis of this entity. MRI remains investigation of choice.⁶ MRI scans showing hyperintense signals on T1 weighted and T2 weighted images indicate the presence of hemorrhagic necrosis and are diagnostic of the entity if these signals are not enhanced after gadolinium administration. The latter is also important in ruling out thrombosed arteriovenous malformation (Foix-Alajounnaie disease). The histopathological examination of the cord in patients who have died of the disease shows classical changes of cord edema, hemorrhagic necrosis and lymphocytic infiltration as in our case.⁷ Electron microscopy, when done may prove helpful in identification of confounding organism.

A number of infectious agents have been incriminated. These include HIV, Herpes simplex virus type 1 and type 2, and Diplococcus pneumoniae, Mycoplasma pneumoniae and schistosomiasis.^1-5 In the setting of acquired immunodeficiency syndrome, varicella zoster virus and cytomegalovirus have also been associated with the development of necrotizing myelitis.^8,9 Malignancies constitute the second largest group of etiological agents. Malignant disorders that have been described to be associated with necrotizing myelitis include clear cell carcinoma, malignant melanoma, multiple myeloma, T cell leukemia, and non-Hodgkin disease.^5,10-12 Other associated disorders have included Sjogren's syndrome and primary biliary cirrhosis.¹³

The management strategies have included supportive treatment and therapy directed at the causative agent. The results of therapy have not always been rewarding^4,13 as in our case. An intense search for etiological factor should be carried out, as therapy directed at such an identified factor may prove beneficial in many instances. The pediatrician should also be on the look out for hitherto unreported factor, since the causative factor in pediatric age group could be different from those described in adult cases reported, so far.

REFERENCES

1. Radhakrishnan VV, Saraswati A, Mohan PR, Narayan SK. Necrotising myelopathy - a report of two cases with review of literature. Ind J Pathol Microbiol 1994;37:439-45.

2. Nagaswami S, Keeps J, Foster B, Twemlow SW. Necrotising myelitis: A clinicopathological report of two cases associated with diplococcus pneumoniae and mycoplasma infections. Trans Am Neurol Assoc 1973;98:290-2.

3. Folpe A, Lapham LW, Smith HC. Herpes simplex myelitis as a cause of acute necrotising myelitis syndrome. Neurology 1994;44:1955-7.

4. Lechtenberg R, Vaida GA. Schistosomiasis of the spinal cord. Neurology 1977;27:55-9.

5. Ito Y, Yamaha M, Koide T, Sakai S, Seko A, Watnabe K. A case of bilateral adrenal non-Hodgkin lymphoma. Acta Urologica Japonica 1988;34:137-40.

6. Mirich DR, Kucharczyk W, Keller MA, Deck J. Subacute necrotising myelopathy: MR imaging in four pathologically proven cases. Am J Neuroradio 1991;12:1077-83.

7. Iwamasa T, Yashitake H, Sakuda H, Kamada Y, Miyazato M, Utsumi Y, et al. Acute ascending necrotising myelitis in Okinawa caused by herpes simplex virus type 2. Virchows Archiv-A, Pathol Anat & Histopathol 1991;418:71-5.

8. Gray F, Belec L, Lescs MC, Chretien F, Ciardi A, Hassine D, et al. Varicella zoster virus infection of the central nervous system in the acquired immune deficiency syndrome. Brain 1994;117:981-99.

9. Budka H. Neuropathology of myelitis, myelopathy and spinal infections in AIDS. Neuroimag Clin North Am 1997;7:639-50.

10. Martin Escudeno JC, Aparicio Blanco M, Barrego Pintado H, de Viller Negro A, Carreterosastre JL, Velasco FC. Necrotising myelopathy associated with neoplasia. A clinicopathological study of 2 cases and a review of literature. Annales de Medicina Interna 1991;8:497-500

11. Storey E, McKelview PA. Necrotising myelopathy associated with multiple myeloma. Acta Neurologica Scandinavica 1991;84:98-101.

12. Nakagawa M, Naka Meera A, Kubota R, Kakazu T, Kuba M, Nakasore M, et al. Necrotising myelopathy associated with malignancy caused by herpes simplex virus type 2. Clinical report of two cases and literature review. Jap J Med 1991;30:182-8.

13. Ruttan G, Martinez AJ, Fieshko JT, Van Thiel DH. Primary biliary cirrhosis, Sjogren's syndrome and transverse myelitis. Gastroenterol 1986;90: 206-10.

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