 |
|
Case Reports Indian Pediatrics 2000;37: 898-900 |
||
|
Focal Segmental Myelitis |
||
From the Department of Pediatrics, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi 110 002, India. Reprint requests: Dr. A.P. Dubey, Professor, Department of Pediatrics, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi 110 002, India.
Manuscript Received: October 26, 1999;
Acute Transverse Myelopathy (ATM) is an uncommon disorder characterized by evidence of lesions in motor and sensory tracts on both sides of the spinal cord. The etiology of most cases of ATM remains unknown; however, there are well known associations with a variety of infectious diseases, including herpes zoster, human immunodeficiency virus, Ebstein-Barr virus, mumps, Mycoplasma pneumoniae, brucellosis, psittacosis and after vaccination against cholera, typhoid and poliomyelitis. The disease in most cases is described as an idiopathic or transverse myelopathy if no distinct causative factor is identifiable. Most textbooks on pediatric neurology and western literature describe ATM as a homogeneous entity with little variation from the classical description of acute onset paraplegia with bladder involvement and a symmetrical sensory level(1,2). A great emphasis has been laid on the symmetry of presentation for both motor and sensory involvement. Scott et al. even(3) suggested a symmetry score on the pattern of involvement in a patient presenting with motor and sensory symptoms; greater the score the more the likelihood of the case being of ATM. Rarely asymmetrical involvement has also been reported in some cases(4). We are reporting a case of asymmetrical myelitis with unusual features.
An 11-year-old male child presented to our hospital with sudden onset weakness of the right lower limb and inability to void the bladder, noticed after a trivial fall. The weakness was non-progressive in nature, not involving the opposite leg or either of the upper limbs. There was no history of alteration of sensorium, seizures or pain or tenderness in the back. There was decreased appreciation of fine touch on the left side of the body, the sensory level being perceived at the nipple level. On examination, the child had weakness of the right lower limb, the power being 0/5 at hip, knee and ankle joints with hypotonia and deep tendon jerks not elicitible. The plantar reflex was normal on the left side but not elicitible on the right side. The cremasteric, abdominal, paraspinal and other superficial reflexes were bilaterally absent. The sensory system examina-tion revealed decreased perception of fine touch and pain on the left side with a sharp sensory level at T2-T3 level, with the posterior column sensations being preserved. The child had a full bladder palpable upto the umbilicus with no feeling of bladder fullness and inability to void the bladder voluntarily. Two days after admission the child started to complain of paresthesia on moving the left upper limb. The X-rays of the cervical and dorso-lumbar spine were normal. The fundus did not show any changes. The CSF study was also normal, as was the nerve conduction velocity. Stool for poliovirus culture was negative. MRI of the spine was done which showed mild enlarge-ment of the cervical cord with multiple hyperintense signals in the lower cervical and upper thoracic region, the changes being maximum in the lower cervical area diagnostic of myelitis (Fig. 1). In the absence of any specific guidelines with respect to the treatment of ATM it was decided to give methylprednisolone therapy which has shown some promise in hastening recovery in this condition. Methylprednisolone was started 7 days after the onset of symptoms in dose of 1.73 g/m2 for a period of 5 days. The child did seem to benefit from the therapy with improvement in power and disappearance of paresthesiae within 7 days of administration of the drug. Two months after discharge from the hospital, the child regained motor power completely with no sensory or bladder involvement.
Acute transverse myelopathy has for long been considered a homogeneous entity with rarely cases being reported with patchy involvement of the spinal cord. In fact the presence of asymmetrical involvement of the cord has been considered to be against the diagnosis of ATM. However a number of cases are now being reported with patchy cord involvement. It has also been suggested that the condition be renamed as para-infectious myelitis (PM)(4) in view of the hitherto unrecognized variability of presentation of this condition. Furthermore, PM has been further subdivided into 3 categories based on the clinical and radiological features:
According to this proposed classification this patient would be classified as a case of focal segmental myelitis with respect to both clinical and radiological criteria. Strangely enough, there are no definite guidelines with respect to treatment of PM, therapy being mainly supportive. Only recently a number of drugs have been tried which include intravenous immunoglobulin(5) and steroids(6,7). Of these the most notable has been high dose methylprednisolone (1g/1.73 m2 BSA for 5 consecutive days) with considerable success as in the present case. However, a randomized controlled trial to establish the definite therapeutic efficacy of this drug in PM is still awaited. Contributors: TG worked up the case, reviewed the literatue and drafted the manuscript. APD co-drafted the manuscript. SS was involved in day to day care of the patient and review of the final manuscript.
Funding:
None.
| ||
|
Key Messages |
||
|
||
|
|
